Abbreviation: R_XOLTRIOLtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: xoltriol[c] <==> xoltriol[r] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
lipid, flip-flop intracellular transport
Abbreviation: R_G6Pter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: g6p[c] <==> g6p[r] Confidence Level: 4 NCD genes: LOCUS:_2542#TRANSCRIPT:1#ABBREVIATION:SLC37A4# proteins: NAME:solute carrier family 37 (glycerol-6-phosphate transporter), member 4#ABBREVIATION:Slc37a4.1# citations: AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# AUTHORS:van Schaftingen E, Gerin I#TITLE:The glucose-6-phopsphatase system#JOURNAL:Biochem J#YEAR:2002#PUBMED:11879177# AUTHORS:Gerin I, Veiga-da-Cunha M, Achouri Y, Collet JF, Van Schaftingen E#TITLE:Sequence of a putative glucose 6-phosphate translocase, mutated in glycogen storage disease type Ib#JOURNAL:FEBS Lett#YEAR:1997#PUBMED:9428641# AUTHORS:Ihara K, Nomura A, Hikino S, Takada H, Hara T#TITLE:Quantitative analysis of glucose-6-phosphate translocase gene expression in various human tissues and haematopoietic progenitor cells#JOURNAL:J Inherit Metab Dis#YEAR:2000#PUBMED:11032333# AUTHORS:Lin B, Pan CJ, Chou JY#TITLE:Human variant glucose-6-phosphate transporter is active in microsomal transport#JOURNAL:Hum Genet#YEAR:2000#PUBMED:11140953# GENE ASSOCIATION: (2542.1)
glucose 6-phosphate endoplasmic reticular transport via diffusion
Abbreviation: R_EX_nrvnc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : nrvnc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_10fthf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 10fthf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NIFEDIPINEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: nifedipine[e] <==> nifedipine[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ASPTAm Synonyms: _0 EC Number: 2.6.1.1 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [m] : akg + asp-L <==> glu-L + oaa Confidence Level: 4 NCD genes: LOCUS:_2806#TRANSCRIPT:1#ABBREVIATION:GOT2#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase, mitochondrial#ABBREVIATION:Got2.1-m# citations: AUTHORS:Ford GC, Eichele G, Jansonius JN.#TITLE:Three-dimensional structure of a pyridoxal-phosphate-dependent enzyme, mitochondrial aspartate aminotransferase.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1980#PUBMED:6930651# GENE ASSOCIATION: (2806.1)
Abbreviation: R_ACACt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: acac[c] + h[c] <==> acac[m] + h[m] Confidence Level: 4 IT, TDV changed name from "tm2" to "t2m" - NCD genes: LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1 #ABBREVIATION:Slc16a1.1-m# citations: AUTHORS:Pande SV, Parvin R.#TITLE:Pyruvate and acetoacetate transport in mitochondria. A reappraisal.#JOURNAL:J Biol Chem#YEAR:1975#PUBMED:627555# AUTHORS:Halestrap AP.#TITLE:Pyruvate and ketone-body transport across the mitochondrial membrane. Exchange properties, pH-dependence and mechanism of the carrier.#JOURNAL:Biochem J#YEAR:1978#PUBMED:28726# GENE ASSOCIATION: (6566.1)
Acetoacetate mitochondrial transport via H+ symport
Abbreviation: R_HPCLx Synonyms: _1 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : (4) h + phyt2ohcoa --> formcoa + pristanal Confidence Level: 4 pristanal is the fatty aldehyde analog of pristanic acid Catalyzes a carbon-carbon cleavage reaction; cleaves a 2-hydroxy-3-methylacyl-CoA into formyl-CoA and a 2-methyl-branched fatty aldehyde. NJ genes: LOCUS:_26062#TRANSCRIPT:1#ABBREVIATION:HYALP1# proteins: NAME:_2-hydroxyphytanoyl-CoA lyase#ABBREVIATION:Hpcl2.1# citations: AUTHORS:Foulon V, Antonenkov VD, Croes K, Waelkens E, Mannaerts GP, Van Veldhoven PP, Casteels M.#TITLE:Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1999#PUBMED:10468558# GENE ASSOCIATION: (26062.1)
Abbreviation: R_DNDPt41m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[m] + dutp[c] --> dcdp[c] + dutp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
alpha 1,4-N-acetylglucosaminyltransferase, Golgi apparatus
Abbreviation: R_A4GNT2g Synonyms: _5 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : core2 + uacgam --> gncore2 + h + udp Confidence Level: 0 EC 2.4.1.- NCD genes: LOCUS:_51146#TRANSCRIPT:1#ABBREVIATION:A4GNT# proteins: NAME:alpha-1,4-N-acetylglucosaminyltransferase, Golgi apparatus (stomach, pancreas)#ABBREVIATION:A4gnt.1-g# citations: AUTHORS:Nakayama J, Yeh JC, Misra AK, Ito S, Katsuyama T, Fukuda M.#TITLE:Expression cloning of a human alpha1, 4-N-acetylglucosaminyltransferase that forms GlcNAcalpha1-->4Galbeta-->R, a glycan specifically expressed in the gastric gland mucous cell-type mucin#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1999#PUBMED:10430883# GENE ASSOCIATION: (51146.1)
Abbreviation: R_FUM Synonyms: _0 EC Number: 4.2.1.2 SUBSYSTEM: Citric Acid Cycle Equation: [c] : fum + h2o <==> mal-L Confidence Level: 0 genes: LOCUS:_2271#TRANSCRIPT:1#ABBREVIATION:FH#ECNUMBERS:4.2.1.2# proteins: NAME:fumarate hydratase#ABBREVIATION:Fh.1# citations: none GENE ASSOCIATION: (2271.1)
R total 2 coenzyme A_CO2FULLR2C21H31N7O15P3S
Adenosine 3',5'-bisphosphate_C10H11N5O10P2
Sulfate derivative of norepinephrine_C8H11NO6S
_3'-Phosphoadenylyl sulfate_C10H11N5O13P2S
Abbreviation: R_NRPPHRSULT Synonyms: _0 EC Number: 2.8.2.1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : nrpphr + paps --> h + nrpphrsf + pap Confidence Level: 4 RS/TV (6/14/05) genes: LOCUS:_6818#TRANSCRIPT:1#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# LOCUS:_6818#TRANSCRIPT:2#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# LOCUS:_6818#TRANSCRIPT:3#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# proteins: NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.2# NAME:sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.3# citations: AUTHORS:Chapman E, Best MD, Hanson SR, Wong CH.#TITLE:Sulfotransferases: structure, mechanism, biological activity, inhibition, and synthetic utility.#JOURNAL:Angew Chem Int Ed Engl#YEAR:2004#PUBMED:15293241# AUTHORS:Glatt H, Boeing H, Engelke CE, Ma L, Kuhlow A, Pabel U, Pomplun D, Teubner W, Meinl W.#TITLE:Human cytosolic sulphotransferases: genetics, characteristics, toxicological aspects.#JOURNAL:Mutat Res#YEAR:2001#PUBMED:11535246# GENE ASSOCIATION: (6818.1) or (6818.3) or (6818.2)
Abbreviation: R_DURIK1 Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [c] : atp + duri --> adp + dump + h Confidence Level: 2 genes: LOCUS:_7083#TRANSCRIPT:1#ABBREVIATION:TK1#ECNUMBERS:2.7.1.21# LOCUS:_7084#TRANSCRIPT:1#ABBREVIATION:TK2#ECNUMBERS:2.7.1.21# proteins: NAME:thymidine kinase 1, soluble #ABBREVIATION:Tk1.1# NAME:thymidine kinase 2, mitochondrial #ABBREVIATION:Tk2.1# citations: AUTHORS:Johansson M, Brismar S, Karlsson A.#TITLE:Human deoxycytidine kinase is located in the cell nucleus.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9342341# AUTHORS:Krawiec K, Kierdaszuk B, Shugar D.#TITLE:Inorganic tripolyphosphate (PPP(i)) as a phosphate donor for human deoxyribonucleoside kinases. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12535661# GENE ASSOCIATION: (7083.1) or (7084.1)
heparan sulfate, degradation product 17_C43H65N2O48S4
(Gal)3 (Glc)1 (GlcNAc)1 (LFuc)1 (Cer)1_C56H99N2O31FULLRCO
Abbreviation: R_GALFUC12GAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: galfuc12gal14acglcgalgluside_hs[g] <==> galfuc12gal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
tetracosanoyl-CoA (n-C24:0CoA)_C45H78N7O17P3S
(alpha-D-mannosyl)5-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine (protein)_C52H87N2O40X
(alpha-D-mannosyl)6-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine (protein)_C58H97N2O45X
Abbreviation: R_MM6B1ag Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m6masnB1 --> m5masnB1 + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
mannosyl-oligosaccharide 1,2-alpha-mannosidase, Golgi apparatus
keratan sulfate I biosynthesis, precursor 34_C241H385N17O202S10X
Octadecynoyl-ACP (n-C18:2ACP)_C29H51N2O8PRS
Abbreviation: R_5HLTDL Synonyms: _0 EC Number: 4.1.1.28 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 5htrp + h --> co2 + srtn Confidence Level: 4 SAB genes: LOCUS:_1644#TRANSCRIPT:1#ABBREVIATION:DDC#ECNUMBERS:4.1.1.28# proteins: NAME:aromatic-L-amino-acid decarboxylase#ABBREVIATION:Ddc.1# citations: AUTHORS:Sumi-Ichinose C, Ichinose H, Takahashi E, Hori T, Nagatsu T#TITLE:Molecular cloning of genomic DNA and chromosomal assignment of the gene for human aromatic L-amino acid decarboxylase, the enzyme for catecholamine and serotonin biosynthesis#JOURNAL:Biochemistry#YEAR:1992#PUBMED:1540578# GENE ASSOCIATION: (1644.1)
_5-Phospho-alpha-D-ribose 1-diphosphate_C5H8O14P3
Abbreviation: R_GUAPRT Synonyms: _0 EC Number: 2.4.2.8 SUBSYSTEM: Salvage Pathway Equation: [c] : gua + prpp --> gmp + ppi Confidence Level: 4 Guanine and 6-mercaptopurine can replace hypoxanthine EC added (2.4.2.7 makes the same reaction) IT genes: LOCUS:_3251#TRANSCRIPT:1#ABBREVIATION:HPRT1#ECNUMBERS:2.4.2.8# proteins: NAME:hypoxanthine phosphoribosyltransferase 1 (Lesch-Nyhan syndrome) #ABBREVIATION:HprT1.1# citations: AUTHORS:Wilson JM, Kobayashi R, Fox IH, Kelley WN.#TITLE: Human hypoxanthine-guanine phosphoribosyltransferase. #JOURNAL:J Biol Chem.#YEAR:1983#PUBMED:6853490# AUTHORS:Kim SH, Moores JC, David D, Respess JG, Jolly DJ, Friedmann T.#TITLE:The organization of the human HPRT gene.#JOURNAL:Nucleic Acids Res#YEAR:1986#PUBMED:3008106# GENE ASSOCIATION: (3251.1)
Abbreviation: R_RDH1a Synonyms: _0 EC Number: 1.1.1.105 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : nadp + retinol <==> h + nadph + retinal Confidence Level: 4 IT genes: LOCUS:_145226#TRANSCRIPT:1#ABBREVIATION:RDH12# LOCUS:_157506#TRANSCRIPT:1#ABBREVIATION:RDH10# LOCUS:_50700#TRANSCRIPT:1#ABBREVIATION:RDH8# LOCUS:_57665#TRANSCRIPT:1#ABBREVIATION:RDH14# LOCUS:_112724#TRANSCRIPT:1#ABBREVIATION:RDH13# LOCUS:_51109#TRANSCRIPT:1#ABBREVIATION:RDH11#ECNUMBERS:1.1.1.-# LOCUS:_195814#TRANSCRIPT:1#ABBREVIATION:RDHE2# proteins: NAME:retinol dehydrogenase 12 (all-trans and 9-cis) #ABBREVIATION:Rdh12.1# NAME:retinol dehydrogenase 14 (all-trans and 9-cis) #ABBREVIATION:Rdh14.1# NAME:retinol dehydrogenase 11 (all-trans and 9-cis) #ABBREVIATION:Rdh11.1# NAME:retinol dehydrogenase 13 (all-trans and 9-cis) #ABBREVIATION:Rdh13.1# NAME:retinol dehydrogenase 8 (all-trans) #ABBREVIATION:Rdh8.1# NAME:retinol dehydrogenase 10 (all-trans) #ABBREVIATION:Rdh10.1# NAME:epidermal retinal dehydrogenase 2 #ABBREVIATION:Rdhe2.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Haeseleer F, Jang GF, Imanishi Y, Driessen CA, Matsumura M, Nelson PS, Palczewski K.#TITLE:Dual-substrate specificity short chain retinol dehydrogenases from the vertebrate retina.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12226107# AUTHORS:Janecke AR, Thompson DA, Utermann G, Becker C, Hubner CA, Schmid E, McHenry CL, Nair AR, Ruschendorf F, Heckenlively J, Wissinger B, Nurnberg P, Gal #TITLE:Mutations in RDH12 encoding a photoreceptor cell retinol dehydrogenase cause childhood-onset severe retinal dystrophy. #JOURNAL:Nat Genet. #YEAR:2004#PUBMED:15258582# AUTHORS:Perrault I, Hanein S, Gerber S, Barbet F, Ducroq D, Dollfus H, Hamel C, Dufier JL, Munnich A, Kaplan J, Rozet JM. #TITLE:Retinal dehydrogenase 12 (RDH12) mutations in leber congenital amaurosis. #JOURNAL:Am J Hum Genet#YEAR:2004#PUBMED:15322982# AUTHORS:Matsuzaka Y, Okamoto K, Tsuji H, Mabuchi T, Ozawa A, Tamiya G, Inoko H.#TITLE:Identification of the hRDH-E2 gene, a novel member of the SDR family, and its increased expression in psoriatic lesion. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2002#PUBMED:12372410# AUTHORS:Wu BX, Chen Y, Chen Y, Fan J, Rohrer B, Crouch RK, Ma JX.#TITLE:Cloning and characterization of a novel all-trans retinol short-chain dehydrogenase/reductase from the RPE. #JOURNAL:Invest Ophthalmol Vis Sci#YEAR:2002#PUBMED:12407145# GENE ASSOCIATION: (157506.1) or (112724.1) or (145226.1) or (51109.1) or (195814.1) or (57665.1) or (50700.1)
Nicotinamide adenine dinucleotide phosphate - reduced_C21H26N7O17P3
Nicotinamide adenine dinucleotide phosphate_C21H25N7O17P3
Abbreviation: R_AGLPR Synonyms: _0 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : akdhap_hs + h + nadph --> akgp_hs + nadp Confidence Level: 0 takes place on outer peroxisomal membrane NJ genes: none proteins: none citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Hajra AK#TITLE:Glycerolipid biosynthesis in peroxisomes#JOURNAL:Prog Lipid Res#YEAR:1995# AUTHORS:Brites P, Waterham HR, Wander RJA#TITLE:Functions and biosynthesis of plasmalogens in health and disease#JOURNAL:Biochimica et Biophysica Acta#YEAR:2004# GENE ASSOCIATION:
_1-alkylglycerol 3-phosphate (homo sapiens)_C4H10O4PFULLRCO2
_1-alkyldihydroxyacetone phosphate_C3H4O6PFULLRC2H4
Abbreviation: R_OCDCAtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ocdca[e] <==> ocdca[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
chondroitin sulfate A (GalNAc4S-GlcA), degradation product 3_C31H48NO32S2
chondroitin sulfate A (GalNAc4S-GlcA), degradation product 2_C37H55NO38S2
Abbreviation: R_GLCAASE4ly Synonyms: _3 EC Number: 3.2.1.31 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_a_deg2 + h2o --> cs_a_deg3 + glcur Confidence Level: 4 NCD genes: LOCUS:_2990#TRANSCRIPT:1#ABBREVIATION:GUSB#ECNUMBERS:3.2.1.31# proteins: NAME:glucuronidase, beta, lysosomal#ABBREVIATION:Gusb.1-l# citations: AUTHORS:Guise KS, Korneluk RG, Waye J, Lamhonwah AM, Quan F, Palmer R, Ganschow RE, Sly WS, Gravel RA#TITLE:Isolation and expression in Escherichia coli of a cDNA clone encoding human beta-glucuronidase#JOURNAL:Gene#YEAR:1985#PUBMED:3924735# AUTHORS:Ho YC, Ho LH, Ho KJ#TITLE:Human hepatic beta-glucuronidase: an enzyme kinetic study#JOURNAL:Enzyme#YEAR:1985#PUBMED:3987656# AUTHORS:Oshima A, Kyle JW, Miller RD, Hoffmann JW, Powell PP, Grubb JH, Sly WS, Tropak M, Guise KS, Gravel RA#TITLE:Cloning, sequencing, and expression of cDNA for human beta-glucuronidase#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3468507# GENE ASSOCIATION: (2990.1)
Abbreviation: R_ECOAH1m Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: Tryptophan metabolism Equation: [m] : 3hbcoa <==> b2coa + h2o Confidence Level: 2 SAB for mito genes: LOCUS:_1892#TRANSCRIPT:1#ABBREVIATION:ECHS1# LOCUS:_3030#TRANSCRIPT:1#ABBREVIATION:HADHA#ECNUMBERS:4.2.1.17,1.1.1.35# LOCUS:_3032#TRANSCRIPT:1#ABBREVIATION:HADHB# LOCUS:_549#TRANSCRIPT:1#ABBREVIATION:AUH# proteins: NAME:AU RNA binding protein/enoyl-Coenzyme A hydratase (mito)#ABBREVIATION:Auh.1-m# NAME:enoyl Coenzyme A hydratase, short chain, 1, mitochondrial#ABBREVIATION:Echs1.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit #ABBREVIATION:Hadha.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit #ABBREVIATION:Hadhb.1-m# citations: AUTHORS:FitzPatrick DR, Germain-Lee E, Valle D.#TITLE:Isolation and characterization of rat and human cDNAs encoding a novel putative peroxisomal enoyl-CoA hydratase. #YEAR:1995#PUBMED:7558027# GENE ASSOCIATION: (549.1) or (3030.1) and (3032.1) or (1892.1)
_3-hydroxyacyl-CoA dehydratase (3-hydroxybutanoyl-CoA) (mitochondria)
_9-O-Acetylated GT3 (homo sapiens)_C65H106N4O37FULLRCO
globoside alpha-1,3-N-acetylgalactosaminyltransferase 1
Abbreviation: R_GBGT1 Synonyms: _7 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gbside_hs + udpacgal --> acgagbside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_26301#TRANSCRIPT:1#ABBREVIATION:FS#ECNUMBERS:2.4.1.88# proteins: NAME:globoside alpha-1,3-N-acetylgalactosaminyltransferase 1/Forssman glycolipid synthetase#ABBREVIATION:GBGT1.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Xu H, Storch T, Yu M, Elliott SP, Haslam DB.#TITLE:haracterization of the human Forssman synthetase gene. An evolving association between glycolipid synthesis and host-microbial interactions. #JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10506200# GENE ASSOCIATION: (26301.1)
alpha GalNAc globoside (homo sapiens)_C52H92N3O27FULLRCO
globoside (homo sapiens)_C44H79N2O22FULLRCO
Abbreviation: R_HYPOE Synonyms: _0 SUBSYSTEM: Vitamin B6 Metabolism Equation: [c] : h2o + pyam5p --> pi + pydam Confidence Level: 4 NCD genes: LOCUS:_57026#TRANSCRIPT:1#ABBREVIATION:PDXP#ECNUMBERS:3.1.3.-# proteins: NAME:Pyridoxal Phosphatase #ABBREVIATION:PdxP.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# AUTHORS:Jang YM, Kim DW, Kang TC, Won MH, Baek NI, Moon BJ, Choi SY, Kwon OS.#TITLE:Human pyridoxal phosphatase. Molecular cloning, functional expression, and tissue distribution.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:14522954# GENE ASSOCIATION: (57026.1)
Nicotinamide adenine dinucleotide - reduced_C21H27N7O14P2
Abbreviation: R_ALATHRNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + na1[e] + thr-L[c] --> ala-L[c] + na1[c] + thr-L[e] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
L-alanine/L-threonine Na-dependent exchange (Ala-L in)
Abbreviation: R_DIGALSIDEtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: digalside_hs[c] <==> digalside_hs[l] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
diacylglycerol (homo sapiens)_C3H6OFULLRCO2FULLR2CO2
Abbreviation: R_DAG_HSter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: dag_hs[r] --> dag_hs[c] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Beta-galactoside alpha-2,3-sialyltransferase
Abbreviation: R_ST8SIA52g Synonyms: _13 EC Number: 2.4.99.8 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gt1b_hs --> cmp + gq1b_hs + h Confidence Level: 4 NJ genes: LOCUS:_29906#TRANSCRIPT:1#ABBREVIATION:SIAT8E# proteins: NAME:ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 5 #ABBREVIATION:St8sia5.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Harduin-Lepers A, Vallejo-Ruiz V, Krzewinski-Recchi MA, Smyn-Petit B, Julien S, Delannoy P#TITLE:The human sialyltransferase family#JOURNAL:Biochimie#YEAR:2001# AUTHORS:Kim YJ, Kim KS, Do S, Kim CH, Kim SK, Lee YC.#TITLE:Molecular cloning and expression of human alpha2,8-sialyltransferase (hST8Sia V).#JOURNAL:Biochem Biophys Res Commun.#YEAR:1997#PUBMED:9199191# GENE ASSOCIATION: (29906.1)
Abbreviation: R_GLUt7l Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: glu-L[l] --> glu-L[c] Confidence Level: 1 IT genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate A (GalNAc4S-GlcA) and B (IdoA2S-GalNAc4S), precursor 3_C45H67N2O42S2X
chondroitin sulfate A (GalNAc4S-GlcA) and B (IdoA2S-GalNAc4S), precursor 2_C37H54NO37S2X
uridine diphosphoacetylgalactosamine-chondroitin acetylgalactosaminyltransferase II, Golgi
Abbreviation: R_GALNACT2g Synonyms: _6 EC Number: 2.4.1.175 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_a,b_pre2 + uacgam --> cs_a,b_pre3 + h + udp Confidence Level: 4 NCD genes: LOCUS:_22856#TRANSCRIPT:1#ABBREVIATION:CHSY1# LOCUS:_55454#TRANSCRIPT:1#ABBREVIATION:GALNACT-2# LOCUS:_55790#TRANSCRIPT:1#ABBREVIATION:ChGn# LOCUS:_79586#TRANSCRIPT:1#ABBREVIATION:CHPF# LOCUS:_337876#TRANSCRIPT:1#ABBREVIATION:CSS3# proteins: NAME:chondroitin sulfate GalNAcT-2, Golgi apparatus#ABBREVIATION:Galnact-2.1-g# NAME:chondroitin beta1,4 N-acetylgalactosaminyltransferase, Golgi apparatus#ABBREVIATION:Chgn.1-g# NAME:carbohydrate (chondroitin) synthase 1, Golgi apparatus#ABBREVIATION:Chsy1.1-g# NAME:chondroitin polymerizing factor, Golgi apparatus#ABBREVIATION:Chpf.1-g# NAME:chondroitin sulfate synthase 3, Golgi apparatus#ABBREVIATION:Css3.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Sato T, Gotoh M, Kiyohara K, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Togayachi A, Kudo T, Asada M, Watanabe H, Imamura T, Kimata K, Narimatsu H#TITLE:Differential roles of two N-acetylgalactosaminyltransferases, CSGalNAcT-1, and a novel enzyme, CSGalNAcT-2. Initiation and elongation in synthesis of chondroitin sulfate#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12446672# AUTHORS:Uyama T, Kitagawa H, Tamura Ji J, Sugahara K#TITLE:Molecular cloning and expression of human chondroitin N-acetylgalactosaminyltransferase: the key enzyme for chain initiation and elongation of chondroitin/dermatan sulfate on the protein linkage region tetrasaccharide shared by heparin/heparan sulfate#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11788602# AUTHORS:Gotoh M, Sato T, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Zhang Y, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Nishihara S, Watanabe H, Kimata K, Narimatsu H#TITLE:Enzymatic synthesis of chondroitin with a novel chondroitin sulfate N-acetylgalactosaminyltransferase that transfers N-acetylgalactosamine to glucuronic acid in initiation and elongation of chondroitin sulfate synthesis#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12163485# AUTHORS:Kitagawa H, Uyama T, Sugahara K#TITLE:Molecular cloning and expression of a human chondroitin synthase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11514575# AUTHORS:Kitagawa H, Izumikawa T, Uyama T, Sugahara K#TITLE:Molecular cloning of a chondroitin polymerizing factor that cooperates with chondroitin synthase for chondroitin polymerization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12716890# AUTHORS:Yada T, Sato T, Kaseyama H, Gotoh M, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-3. Molecular cloning and characterization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12907687# AUTHORS:Yada T, Gotoh M, Sato T, Shionyu M, Go M, Kaseyama H, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12761225# GENE ASSOCIATION: (55790.1) or (79586.1) and (22856.1) or (55454.1) or (337876.1)
de-Fuc, reducing GlcNAc removed, de-Sia, de-Gal form of PA6 (w/o peptide linkage)_C42H71N3O31
heparan sulfate, degradation product 2_C79H116N5O96S10
Abbreviation: R_CITMCOAHm Synonyms: _0 EC Number: 4.2.1.56 SUBSYSTEM: C5-Branched dibasic acid metabolism Equation: [m] : h2o + itaccoa <==> citmcoa-L Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:WANG SF, ADLER J, LARDY HA#TITLE:The pathway of itaconate metabolism by liver mitochondria#JOURNAL:J Biol Chem#YEAR:1961#PUBMED:13783048# AUTHORS:ADLER J, WANG SF, LARDY HA#TITLE:The metabolism of itaconic acid by liver mitochondria.#JOURNAL:J Biol Chem#YEAR:1957#PUBMED:13502348# GENE ASSOCIATION:
L-Citramalyl-CoA hydro-lyase, mitochondrial
Abbreviation: R_NACHEXA13ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg18 --> acgam + h + ksi_deg20 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
keratan sulfate I, degradation product 18_C146H235N10O127S7
keratan sulfate I, degradation product 20_C138H223N9O119S6
beta-N-acetylhexosaminidase A, lysosomal
Stearoyl-CoA (n-C18:0CoA)_C39H66N7O17P3S
hyaluronan biosynthesis, precursor 1_C14H20NO11
Iodide:hydrogen-peroxide oxidoreductase 3
Abbreviation: R_IDHPOXOX3 Synonyms: _0 EC Number: 1.11.1.8 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 35diotyr + 3ityr-L + h2o2 --> 2amac + (2) h2o + triodthy Confidence Level: 4 SAB genes: LOCUS:_7173#TRANSCRIPT:1#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:2#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:5#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:4#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:3#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# proteins: NAME:thyroid peroxidase#ABBREVIATION:Tpo.1# NAME:thyroid peroxidase#ABBREVIATION:Tpo.2# NAME:thyroid peroxidase#ABBREVIATION:Tpo.3# NAME:thyroid peroxidase#ABBREVIATION:Tpo.4# NAME:thyroid peroxidase#ABBREVIATION:Tpo.5# citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# AUTHORS:Nakamura M, Yamazaki I, Kotani T, Ohtaki S#TITLE:Thyroid peroxidase selects the mechanism of either 1- or 2-electron oxidation of phenols, depending on their substituents#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2997169# AUTHORS:Kimura S, Kotani T, McBride OW, Umeki K, Hirai K, Nakayama T, Ohtaki S#TITLE:Human thyroid peroxidase: complete cDNA and protein sequence, chromosome mapping, and identification of two alternately spliced mRNAs#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1987#PUBMED:3475693# AUTHORS:Magnusson RP, Chazenbalk GD, Gestautas J, Seto P, Filetti S, DeGroot LJ, Rapoport B. #TITLE:Molecular cloning of the complementary deoxyribonucleic acid for human thyroid peroxidase.#JOURNAL:Mol Endocrinol#YEAR:1987#PUBMED:3153466# GENE ASSOCIATION: (7173.4) or (7173.1) or (7173.3) or (7173.2) or (7173.5)
Abbreviation: R_EX_biocyt_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : biocyt <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MGACONm Synonyms: _0 SUBSYSTEM: C5-Branched dibasic acid metabolism Equation: [m] : citmcoa-L <==> h2o + mescoa Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:WANG SF, ADLER J, LARDY HA#TITLE:The pathway of itaconate metabolism by liver mitochondria#JOURNAL:J Biol Chem#YEAR:1961#PUBMED:13783048# GENE ASSOCIATION:
Abbreviation: R_GLYtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: gly[c] <==> gly[m] Confidence Level: 4 TDV Biochim Biophys Acta. 1980 Jun 6;598(3):588-94 genes: none proteins: none citations: AUTHORS:Benavides J, Garcia ML, Lopez-Lahoya J, Ugarte M, Valdivieso F.#TITLE: Glycine transport in rat brain and liver mitochondria. #JOURNAL:Biochim Biophys Acta#YEAR:1980#PUBMED:7388024# GENE ASSOCIATION:
glycine passive transport to mitochondria
Abbreviation: R_DURItn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: duri[c] <==> duri[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
(N-acetyl-D-glucosaminyl-(alpha-D-mannosyl)2-beta-D-mannosyl-diacetylchitobiosyl)-L-asparagine (protein)_C42H70N3O30X
(N-acetyl-D-glucosaminyl-(alpha-D-mannosyl)4-beta-D-mannosyl-diacetylchitobiosyl)-L-asparagine (protein)_C54H90N3O40X
Abbreviation: R_M1316Mg Synonyms: _7 EC Number: 3.2.1.114 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : (2) h2o + nm4masn --> (2) man + nm2masn Confidence Level: 0 NCD genes: LOCUS:_4122#TRANSCRIPT:1#ABBREVIATION:MAN2A2# LOCUS:_4124#TRANSCRIPT:1#ABBREVIATION:MAN2A1#ECNUMBERS:3.2.1.114# proteins: NAME:mannosidase, alpha, class 2A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man2a2.1-g# NAME:mannosidase, alpha, class 2A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man2a1.1-g# citations: AUTHORS:Misago M, Liao YF, Kudo S, Eto S, Mattei MG, Moremen KW, Fukuda MN.#TITLE:Molecular cloning and expression of cDNAs encoding human alpha-mannosidase II and a previously unrecognized alpha-mannosidase IIx isozyme.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1995#PUBMED:8524845# GENE ASSOCIATION: (4124.1) or (4122.1)
mannosyl-oligosaccharide 1,3-1,6-alpha-mannosidase
Abbreviation: R_UGT1A10r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Steroid Metabolism Equation: [r] : bilirub + (2) h + udpglcur <==> bilglcur + udp Confidence Level: 1 bil is taken up by cell - made reversible (actual measurements of deltaG/reversibility not described in literature currently) NJ genes: LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# citations: none GENE ASSOCIATION: (54576.1)
UDP-glucuronosyltransferase 1-10 precursor, microsomal
(Gal)2 (GalNAc)1 (Glc)1 (GlcNAc)1 (LFuc)2 (Cer)1_C64H112N3O35FULLRCO
_1-alkyl 2-acylglycerol_C5H10O2FULLRFULLR2CO2
alkyl acylglycerol phosphoethanolamine transferase
Abbreviation: R_AGLPET Synonyms: _1 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : ak2g_hs + cdpea + h --> ak2gpe_hs + cmp + h2o Confidence Level: 2 see Harper's Biochemistry 'Metabolism of Acylglycerols and Sphingolipids' chapter currently charge imbalanced (+2) - charging of individual molecules consistent w/ known info at pH 7.4 NJ genes: none proteins: none citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Brites P, Waterham HR, Wander RJA#TITLE:Functions and biosynthesis of plasmalogens in health and disease#JOURNAL:Biochimica et Biophysica Acta#YEAR:2004# AUTHORS:Nagan N, Zoeller RA#TITLE:Plasmalogens: biosynthesis and functions#JOURNAL:Progress in Lipid Research#YEAR:2001# GENE ASSOCIATION:
_1-alkyl 2-acylglycerol 3-phosphoethanolamine_C7H16O5NPFULLRFULLR2CO
Abbreviation: R_PIt7ir Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) na1[e] + pi[e] --> (3) na1[c] + pi[c] Confidence Level: 2 SMP SAB ir genes: LOCUS:_6568#TRANSCRIPT:1#ABBREVIATION:SLC17A1# LOCUS:_10050#TRANSCRIPT:1#ABBREVIATION:SLC17A4# LOCUS:_10246#TRANSCRIPT:1#ABBREVIATION:SLC17A2# LOCUS:_10786#TRANSCRIPT:1#ABBREVIATION:SLC17A3# proteins: NAME:solute carrier family 17 (sodium phosphate), member 1#ABBREVIATION:Slc17a1.1# NAME:solute carrier family 17 (sodium phosphate), member 2#ABBREVIATION:Slc17a2.1# NAME:solute carrier family 17 (sodium phosphate), member 3#ABBREVIATION:Slc17a3.1# NAME:solute carrier family 17 (sodium phosphate), member 4#ABBREVIATION:Slc17a4.1# citations: AUTHORS:Murer H, Biber J#TITLE:Molecular mechanisms of renal apical Na/phosphate cotransport#JOURNAL:Annu Rev Physiol#YEAR:1996#PUBMED:8815811# AUTHORS:Reimer RJ, Edwards RH#TITLE:Organic anion transport is the primary function of the SLC17/type I phosphate transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12811560# GENE ASSOCIATION: (10050.1) or (10786.1) or (10246.1) or (6568.1)
phosphate transport in/out via three Na+ symporter (irreversible)
Abbreviation: R_CREATt4_LPAREN_2_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: creat[e] + (2) na1[e] <==> creat[c] + (2) na1[c] Confidence Level: 4 SAB genes: LOCUS:_6537#TRANSCRIPT:1#ABBREVIATION:SLC6A10# LOCUS:_6535#TRANSCRIPT:1#ABBREVIATION:SLC6A8# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, creatine), member 8#ABBREVIATION:Slc6a8.1# NAME:solute carrier family 6 (neurotransmitter transporter, creatine), member 10#ABBREVIATION:Slc6a10.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (6535.1) or (6537.1)
Creatine transport (sodium symport) (2:1)
Abbreviation: R_ACt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: ac[c] + h[c] <==> ac[m] + h[m] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Casal M, Paiva S, Andrade RP, Gancedo C, Leao C.#TITLE:The lactate-proton symport of Saccharomyces cerevisiae is encoded by JEN1.#JOURNAL:J Bacteriol#YEAR:1999#PUBMED:10198029# AUTHORS:Wolfe AJ.#TITLE:The acetate switch#JOURNAL:Microbiol Mol Biol Rev#YEAR:2005#PUBMED:15755952# GENE ASSOCIATION:
acetate mitochondrial transport via proton symport
Abbreviation: R_FOLR2 Synonyms: _0 EC Number: 1.5.1.3 SUBSYSTEM: Folate Metabolism Equation: [c] : fol + nadph --> dhf + nadp Confidence Level: 2 Reaction can be catalyzed by dihydrofolate dehydrogenase (EC 1.5.1.3) - to H2F genes: LOCUS:_1719#TRANSCRIPT:1#ABBREVIATION:DHFR#ECNUMBERS:1.5.1.3# proteins: NAME:dihydrofolate reductase #ABBREVIATION:Dhfr.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Funanage VL, Myoda TT, Moses PA, Cowell HR.#TITLE:Assignment of the human dihydrofolate reductase gene to the q11----q22 region of chromosome 5.#JOURNAL:Mol Cell Biol. #YEAR:1984#PUBMED:6504041# GENE ASSOCIATION: (1719.1)
sphingomyelin (homo sapiens)_C23H48N2O5PFULLRCO
Abbreviation: R_SPHMYLNtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: sphmyln_hs[c] <==> sphmyln_hs[g] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# GENE ASSOCIATION:
Abbreviation: R_GMAND Synonyms: _0 EC Number: 4.2.1.47 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : gdpmann --> gdpddman + h2o Confidence Level: 0 JLR genes: LOCUS:_2762#TRANSCRIPT:1#ABBREVIATION:GMDS#ECNUMBERS:4.2.1.47# proteins: NAME:GDP-mannose 4,6-dehydratase#ABBREVIATION:Gmds.1# citations: none GENE ASSOCIATION: (2762.1)
Abbreviation: R_FUT97g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fuc132galacglcgal14acglcgalgluside_hs + gdpfuc --> fucfuc132galacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
(Gal)3 (Glc)1 (GlcNAc)1 (LFuc)2 (Cer)1_C62H109N2O35FULLRCO
Abbreviation: R_EX_fucgalfucgalacglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucgalfucgalacglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(Gal)3 (Glc)1 (GlcNAc)1 (LFuc)2 (Cer)1 exchange
Abbreviation: R_LDH_L Synonyms: _0 EC Number: 1.1.1.27 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : lac-L + nad <==> h + nadh + pyr Confidence Level: 0 Also see LDL_D genes: LOCUS:_3945#TRANSCRIPT:1#ABBREVIATION:LDHB#ECNUMBERS:1.1.1.27# LOCUS:_3948#TRANSCRIPT:1#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_3939#TRANSCRIPT:1#ABBREVIATION:LDHA#ECNUMBERS:1.1.1.27# LOCUS:_160287#TRANSCRIPT:1#ABBREVIATION:LDHAL6A# LOCUS:_92483#TRANSCRIPT:1#ABBREVIATION:LDHAL6B# LOCUS:_3948#TRANSCRIPT:2#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_55293#TRANSCRIPT:1#ABBREVIATION:UEV3# proteins: NAME:lactate dehydrogenase A (liver, sk muscle)#ABBREVIATION:Ldha.1# NAME:lactate dehydrogenase B (heart, RBC)#ABBREVIATION:Ldhb.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.2# NAME:lactate dehydrogenase A-like 6B (testis)#ABBREVIATION:Ldhal6b.1# NAME:lactate dehydrogenase A-like 6A#ABBREVIATION:Ldhal6a.1# NAME:ubiquitin-conjugating enzyme E2-like#ABBREVIATION:Uev3.1# NAME:lactate dehydrogenase (heart, RBC, brain, kidney, liver, sk muscle)#ABBREVIATION:LdhAB# citations: AUTHORS:Yu Y, Deck JA, Hunsaker LA, Deck LM, Royer RE, Goldberg E, Vander Jagt DL#TITLE:Selective active site inhibitors of human lactate dehydrogenases A4, B4, and C4.#JOURNAL:Biochem Pharmacol#YEAR:2001#PUBMED:11377399# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Li X, Qin C, Burghardt R, Safe S. #TITLE:Hormonal regulation of lactate dehydrogenase-A through activation of protein kinase C pathways in MCF-7 breast cancer cells.#JOURNAL:Biochem Biophys Res Commun.#YEAR:2004#PUBMED:15240094# AUTHORS:Millan JL, Driscoll CE, LeVan KM, Goldberg E.#TITLE:Epitopes of human testis-specific lactate dehydrogenase deduced from a cDNA sequence.#JOURNAL:Proc Natl Acad Sci U S A. #YEAR:1985#PUBMED:2440048# GENE ASSOCIATION: (3945.1 and 3939.1) or (160287.1) or (3948.2) or (3939.1) or (3948.1) or (55293.1) or (3945.1) or (92483.1)
Nicotinamide adenine dinucleotide_C21H26N7O14P2
Abbreviation: R_DNDPt40m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[m] + dutp[c] --> dadp[c] + dutp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_RDH3 Synonyms: _0 EC Number: 1.1.1.105 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : nad + retinol-cis-11 <==> h + nadh + retinal-11-cis Confidence Level: 4 IT genes: LOCUS:_5959#TRANSCRIPT:1#ABBREVIATION:RDH5#ECNUMBERS:1.1.1.105# proteins: NAME:retinol dehydrogenase 5 (11-cis and 9-cis) #ABBREVIATION:Rdh5.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Haeseleer F, Jang GF, Imanishi Y, Driessen CA, Matsumura M, Nelson PS, Palczewski K.#TITLE:Dual-substrate specificity short chain retinol dehydrogenases from the vertebrate retina.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12226107# AUTHORS:Simon A, Hellman U, Wernstedt C, Eriksson U. #TITLE:The retinal pigment epithelial-specific 11-cis retinol dehydrogenase belongs to the family of short chain alcohol dehydrogenases. #JOURNAL:J Biol Chem.#YEAR:1995#PUBMED:7836368# GENE ASSOCIATION: (5959.1)
protein-linked asparagine residue (N-glycosylation site)_XH
sphingosylphosphorylcholine (homo sapiens)_C23H50N2O5P
Abbreviation: R_SPC_HSt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: spc_hs[c] <==> spc_hs[e] Confidence Level: 2 known to get out of cell, mechanism/transporters not yet found, see for example PMID: 12069827 NJ genes: none proteins: none citations: AUTHORS:Meyer zu Heringdorf D, Himmel HM, Jakobs KH.#TITLE:Sphingosylphosphorylcholine-biological functions and mechanisms of action.#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12069827# GENE ASSOCIATION:
sphingosylphosphorylcholine transport (diffusion)
Abbreviation: R_EX_tetpent6_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tetpent6 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate D (GlcNAc6S-GlcA2S), degradation product 4_C31H47NO35S3
chondroitin sulfate D (GlcNAc6S-GlcA2S), degradation product 3_C37H54NO41S3
Abbreviation: R_GLCAASE6ly Synonyms: _3 EC Number: 3.2.1.31 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_d_deg3 + h2o --> cs_d_deg4 + glcur Confidence Level: 4 NCD genes: LOCUS:_2990#TRANSCRIPT:1#ABBREVIATION:GUSB#ECNUMBERS:3.2.1.31# proteins: NAME:glucuronidase, beta, lysosomal#ABBREVIATION:Gusb.1-l# citations: AUTHORS:Guise KS, Korneluk RG, Waye J, Lamhonwah AM, Quan F, Palmer R, Ganschow RE, Sly WS, Gravel RA#TITLE:Isolation and expression in Escherichia coli of a cDNA clone encoding human beta-glucuronidase#JOURNAL:Gene#YEAR:1985#PUBMED:3924735# AUTHORS:Ho YC, Ho LH, Ho KJ#TITLE:Human hepatic beta-glucuronidase: an enzyme kinetic study#JOURNAL:Enzyme#YEAR:1985#PUBMED:3987656# AUTHORS:Oshima A, Kyle JW, Miller RD, Hoffmann JW, Powell PP, Grubb JH, Sly WS, Tropak M, Guise KS, Gravel RA#TITLE:Cloning, sequencing, and expression of cDNA for human beta-glucuronidase#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3468507# GENE ASSOCIATION: (2990.1)
Abbreviation: R_APOCFm Synonyms: _0 SUBSYSTEM: Biotin Metabolism Equation: [m] : apoC + lys-L --> apoC-Lys + h + h2o Confidence Level: 2 IT faked reaction to balance reaction in human biotin metabolism genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Dolichol, human uterine homolog_C1025H1680O10
Abbreviation: R_DOLICHOL_Uter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: (0.1) dolichol_U[r] <==> (0.1) dolichol_U[c] Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FUCFUC132GALACGLCGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucfuc132galacglcgal14acglcgalgluside_hs[e] <==> fucfuc132galacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FACOAL200 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : arach + atp + coa <==> amp + arachcoa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
_1D-myo-Inositol 1,3,4,5-tetrakisphosphate_C6H8O18P4
Lacto-N-fucopentaosyl III ceramide_C50H89N2O26FULLRCO
Abbreviation: R_EX_fucgal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucgal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Lacto-N-fucopentaosyl III ceramide exchange
Abbreviation: R_MHISOR Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Histidine Metabolism Equation: [c] : h2o + mhista + o2 --> 3mldz + h2o2 + nh4 Confidence Level: 4 SAB genes: LOCUS:_314#TRANSCRIPT:1#ABBREVIATION:AOC2#ECNUMBERS:1.4.3.6# LOCUS:_8639#TRANSCRIPT:1#ABBREVIATION:AOC3#ECNUMBERS:1.4.3.6# LOCUS:_26#TRANSCRIPT:1#ABBREVIATION:ABP1#ECNUMBERS:1.4.3.6# LOCUS:_314#TRANSCRIPT:2#ABBREVIATION:AOC2# proteins: NAME:amine oxidase, copper containing 3#ABBREVIATION:Aoc3.1# NAME:amiloride binding protein 1 (amine oxidase (copper-containing))#ABBREVIATION:Abp1.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.2# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# AUTHORS:Elmore BO, Bollinger JA, Dooley DM#TITLE:Human kidney diamine oxidase: heterologous expression, purification, and characterization#JOURNAL:J Biol Inorg Chem#YEAR:2002#PUBMED:12072962# GENE ASSOCIATION: (26.1) or (314.1) or (8639.1) or (314.2)
N-Methylhistamine:oxygen oxidoreductase (deaminating)
(alpha-D-Mannosyl)4-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human liver homolog_C1540H2528N20O420P20
Abbreviation: R_RAI2 Synonyms: _0 EC Number: 5.2.1.3 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : retinal <==> retinal-cis-9 Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_FDH Synonyms: _0 EC Number: 1.2.1.2 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : for + nad --> co2 + nadh Confidence Level: 2 genes: LOCUS:_10840#TRANSCRIPT:1#ABBREVIATION:FTHFD# proteins: NAME:formyltetrahydrofolate dehydrogenase #ABBREVIATION:Fthfdh.1# citations: none GENE ASSOCIATION: (10840.1)
Abbreviation: R_Htx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: h[c] <==> h[x] Confidence Level: 0 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NDPK10 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + didp <==> adp + ditp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.2) or (10201.1) or (4831.1 and 4830.1) or (4832.1) or (4831.1 and 4830.2) or (29922.1)
nucleoside-diphosphate kinase (ATP:dIDP)
Abbreviation: R_FACOAL1812 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + vacc <==> amp + ppi + vacccoa Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_DOGULNO1 Synonyms: _0 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : 23doguln + h2o2 --> (2) h + oxa + thrnt Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Banhegyi G, Braun L, Csala M, Puskas F, Mandl J#TITLE:Ascorbate metabolism and its regulation in animals#JOURNAL:Free Radic Biol Med#YEAR:1997#PUBMED:9296457# AUTHORS:Simpson GL, Ortwerth BJ#TITLE:The non-oxidative degradation of ascorbic acid at physiological conditions#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10727845# GENE ASSOCIATION:
_2,3-dioxo-L-gulonate:hydrogen peroxide oxireductase
(alpha-D-Mannosyl)5-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human liver homolog_C1600H2628N20O470P20
Dolichol phosphate, human liver homolog_C1080H1758O40P10
(alpha-D-Mannosyl)6-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human liver homolog_C1660H2728N20O520P20
Dolichyl phosphate D-mannose, human liver homolog_C1140H1868O90P10
dolichyl-phosphate-mannose-glycolipid alpha-mannosyltransferase (liver)
Abbreviation: R_DOLPMT2_Ler Synonyms: _2 EC Number: 2.4.1.130 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolmanp_L + (0.1) m5mpdol_L --> (0.1) dolp_L + h + (0.1) m6mpdol_L Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
keratan sulfate I, degradation product 14_C166H267N11O145S8
linoelaidic acid (all trans C18:2)_C18H31O2
Abbreviation: R_EX_lneldc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lneldc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAOXC2051843m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : coa + h2o + nad + tmndnccoa --> accoa + h + nadh + strdnccoa Confidence Level: 4 _1 round beta ox, 1 degree loss of sat -> no fad oxidation peroxisomal (very long chain FA) c20:5,n-3 -> c18:4, n-3 - final '3' in abbreviation indicates omega3 FA 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_ACETONEt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: acetone[c] + h[c] <==> acetone[m] + h[m] Confidence Level: 0 NCD genes: LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1 #ABBREVIATION:Slc16a1.1-m# citations: none GENE ASSOCIATION: (6566.1)
acetone mitochondrial transport via proton symport
_2-Amino-4-hydroxy-6-(erythro-1,2,3-trihydroxypropyl)dihydropteridine triphosphate_C9H12N5O13P3
(Gal)3 (GalNAc)1 (Glc)1 (GlcNAc)1 (LFuc)1 (Cer)1_C64H112N3O36FULLRCO
Abbreviation: R_S2L2FN2M2MASNtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: s2l2fn2m2masn[e] --> s2l2fn2m2masn[l] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
s2l2fn2m2masn transport, extracellular to lysosome
Abbreviation: R_EX_adrnl_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : adrnl <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UMPK2 Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : ctp + ump <==> cdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
_4-Trimethylammoniobutanoate,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating)
Abbreviation: R_BBHOX Synonyms: _4 SUBSYSTEM: Lysine Metabolism Equation: [c] : 4tmeabutn + akg + o2 --> co2 + crn + succ Confidence Level: 4 MM genes: LOCUS:_8424#TRANSCRIPT:1#ABBREVIATION:BBOX1#ECNUMBERS:1.14.11.1# proteins: NAME:butyrobetaine (gamma), 2-oxoglutarate dioxygenase (gamma-butyrobetaine hydroxylase) 1 #ABBREVIATION:Bbox1.1# citations: AUTHORS:Vaz,F.M. , van Gool,S. , Ofman,R. , Ijlst,L. , Wanders,R.J. #TITLE:Carnitine biosynthesis: identification of the cDNA encoding human gamma-butyrobetaine hydroxylase.#YEAR:1998#PUBMED:9753662# GENE ASSOCIATION: (8424.1)
Abbreviation: R_EX_ala_DASH_B_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ala-B <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I, degradation product 15_C160H257N11O140S8
Abbreviation: R_HEXCCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: hexccrn[c] --> hexccrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
transport into the mitochondria (carnitine)
Abbreviation: R_OCTAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: octa[e] <==> octa[c] Confidence Level: 2 JLR genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GGH_DASH_5DHFe Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [e] : 5dhf + (4) h2o --> dhf + (4) glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) - last step from 5-glutamyl to monoglutamyl is in one step IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-e# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (5DHF), extracellular
_1-Phosphatidyl-1D-myo-inositol 3-phosphate (Homo sapiens)_C9H15O12P2FULLRCO2FULLR2CO2
phosphatidylinositol-3,4-bisphosphate (Homo sapiens)_C9H14O15P3FULLRCO2FULLR2CO2
Abbreviation: R_PI34P4Pn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail34p_hs --> pail3p_hs + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
phosphatidylinositol-3,4-bisphosphate 4-phosphatase, nuclear
Abbreviation: R_GLXtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: glx[c] <==> glx[m] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP.#TITLE:Glycolate and glyoxylate metabolism in HepG2 cells.#YEAR:2004#PUBMED:15240345# GENE ASSOCIATION:
phosphatidylinositol 4,5-bisphosphate (Homo sapiens)_C9H14O15P3FULLRCO2FULLR2CO2
phosphatidylinositol-3,4,5-trisphosphate (Homo sapiens)_C9H13O18P4FULLRCO2FULLR2CO2
Abbreviation: R_PI345P3Pn Synonyms: _0 EC Number: 3.1.3.67 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail345p_hs --> pail45p_hs + pi Confidence Level: 4 NCD genes: LOCUS:_5728#TRANSCRIPT:1#ABBREVIATION:PTEN# proteins: NAME:phosphatase and tensin homolog (mutated in multiple advanced cancers 1), nuclear#ABBREVIATION:Pten.1-n# citations: AUTHORS:Ono H, Katagiri H, Funaki M, Anai M, Inukai K, Fukushima Y, Sakoda H, Ogihara T, Onishi Y, Fujishiro M, Kikuchi M, Oka Y, Asano T#TITLE:Regulation of phosphoinositide metabolism, Akt phosphorylation, and glucose transport by PTEN (phosphatase and tensin homolog deleted on chromosome 10) in 3T3-L1 adipocytes#JOURNAL:Mol Endocrinol#YEAR:2001#PUBMED:11463863# AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION: (5728.1)
phosphatidylinositol-3,4,5-trisphosphate 3-phosphatase, nuclear
Abbreviation: R_3HLYTCL Synonyms: _0 EC Number: 4.1.1.28 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 34dhphe + h --> co2 + dopa Confidence Level: 4 SAB genes: LOCUS:_1644#TRANSCRIPT:1#ABBREVIATION:DDC#ECNUMBERS:4.1.1.28# proteins: NAME:aromatic-L-amino-acid decarboxylase#ABBREVIATION:Ddc.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (1644.1)
ceramide (homo sapiens)_C18H36NO2FULLRCO
glucocerebroside (homo sapiens)_C24H46NO7FULLRCO
Abbreviation: R_GBAl Synonyms: _5 EC Number: 3.2.1.45 SUBSYSTEM: Sphingolipid Metabolism Equation: [l] : gluside_hs + h2o --> crm_hs + glc-D Confidence Level: 4 NJ genes: LOCUS:_2629#TRANSCRIPT:1#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# LOCUS:_2629#TRANSCRIPT:5#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# LOCUS:_2629#TRANSCRIPT:3#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# LOCUS:_2629#TRANSCRIPT:4#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# LOCUS:_2629#TRANSCRIPT:2#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# proteins: NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.1# NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.2# NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.3# NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.4# NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.5# citations: AUTHORS:Orvisky E, Park JK, Parker A, Walker JM, Martin BM, Stubblefield BK, Uyama E, Tayebi N, Sidransky E.#TITLE:The identification of eight novel glucocerebrosidase (GBA) mutations in patients with Gaucher disease.#JOURNAL:Hum Mutat#YEAR:2002#PUBMED:11933202# AUTHORS:Tettamanti G, Bassi R, Viani P, Riboni L#TITLE:Salvage pathways in glycosphingolipid metabolism#JOURNAL:Biochimie#YEAR:2003# AUTHORS:Berrin JG, Czjzek M, Kroon PA, McLauchlan WR, Puigserver A, Williamson G, Juge N.#TITLE:Substrate (aglycone) specificity of human cytosolic beta-glucosidase.#JOURNAL:Biochem J#YEAR:2003#PUBMED:12667141# GENE ASSOCIATION: (2629.4) or (2629.1) or (2629.3) or (2629.2) or (2629.5)
Abbreviation: R_NTD9e Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [e] : gmp + h2o --> gsn + pi Confidence Level: 2 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_4907#TRANSCRIPT:1#ABBREVIATION:NT5E#ECNUMBERS:3.1.3.5# proteins: NAME:_5'-nucleotidase, ecto (CD73) #ABBREVIATION:Nt5E.1-e# citations: none GENE ASSOCIATION: (4907.1)
Abbreviation: R_PIACGT Synonyms: _2 EC Number: 2.4.1.198 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [c] : pail_hs + uacgam --> acgpail_hs + h + udp Confidence Level: 4 NCD genes: LOCUS:_5277#TRANSCRIPT:1#ABBREVIATION:PIGA# LOCUS:_8818#TRANSCRIPT:1#ABBREVIATION:DPM2# LOCUS:_5283#TRANSCRIPT:1#ABBREVIATION:PIGH# LOCUS:_51227#TRANSCRIPT:1#ABBREVIATION:DSCR5# LOCUS:_5277#TRANSCRIPT:2#ABBREVIATION:PIGA# LOCUS:_51227#TRANSCRIPT:2#ABBREVIATION:DSCR5# LOCUS:_8818#TRANSCRIPT:2#ABBREVIATION:DPM2# LOCUS:_51227#TRANSCRIPT:3#ABBREVIATION:DSCR5# LOCUS:_5277#TRANSCRIPT:3#ABBREVIATION:PIGA# LOCUS:_5279#TRANSCRIPT:1#ABBREVIATION:PIGC# LOCUS:_5279#TRANSCRIPT:2#ABBREVIATION:PIGC# LOCUS:_9091#TRANSCRIPT:1#ABBREVIATION:PIGQ# LOCUS:_9091#TRANSCRIPT:2#ABBREVIATION:PIGQ# proteins: NAME:Down syndrome critical region gene 5#ABBREVIATION:Dscr5.1# NAME:Down syndrome critical region gene 5#ABBREVIATION:Dscr5.2# NAME:Down syndrome critical region gene 5#ABBREVIATION:Dscr5.3# NAME:phosphatidylinositol glycan, class A (paroxysmal nocturnal hemoglobinuria)#ABBREVIATION:Piga.1# NAME:phosphatidylinositol glycan, class A (paroxysmal nocturnal hemoglobinuria)#ABBREVIATION:Piga.2# NAME:phosphatidylinositol glycan, class A (paroxysmal nocturnal hemoglobinuria)#ABBREVIATION:Piga.3# NAME:phosphatidylinositol glycan, class C#ABBREVIATION:Pigc.1# NAME:phosphatidylinositol glycan, class C#ABBREVIATION:Pigc.2# NAME:phosphatidylinositol glycan, class Q#ABBREVIATION:Pigq.1# NAME:phosphatidylinositol glycan, class Q#ABBREVIATION:Pigq.2# NAME:phosphatidylinositol glycan, class H#ABBREVIATION:Pigh.1# NAME:dolichyl-phosphate mannosyltransferase polypeptide 2, regulatory subunit #ABBREVIATION:Dpm2.1# NAME:dolichyl-phosphate mannosyltransferase polypeptide 2, regulatory subunit #ABBREVIATION:Dpm2.2# citations: AUTHORS:Shibuya K, Kudoh J, Minoshima S, Kawasaki K, Asakawa S, Shimizu N#TITLE:Isolation of two novel genes, DSCR5 and DSCR6, from Down syndrome critical region on human chromosome 21q22.2#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10814524# AUTHORS:Togashi T, Choi DK, Taylor TD, Suzuki Y, Sugano S, Hattori M, Sakaki Y#TITLE:A novel gene, DSCR5, from the distal Down syndrome critical region on chromosome 21q22.2#JOURNAL:DNA Res#YEAR:2000#PUBMED:10907851# AUTHORS:Choi DK, Suzuki Y, Yoshimura S, Togashi T, Hida M, Taylor TD, Wang Y, Sugano S, Hattori M, Sakaki Y#TITLE:Molecular cloning and characterization of a gene expressed in mouse developing tongue, mDscr5 gene, a homolog of human DSCR5 (Down syndrome Critical Region gene 5)#JOURNAL:Mamm Genome#YEAR:2001#PUBMED:11331941# AUTHORS:Miyata T, Takeda J, Iida Y, Yamada N, Inoue N, Takahashi M, Maeda K, Kitani T, Kinoshita T#TITLE:The cloning of PIG-A, a component in the early step of GPI-anchor biosynthesis#JOURNAL:Science#YEAR:1993#PUBMED:7680492# AUTHORS:Iida Y, Takeda J, Miyata T, Inoue N, Nishimura J, Kitani T, Maeda K, Kinoshita T#TITLE:Characterization of genomic PIG-A gene: a gene for glycosylphosphatidylinositol-anchor biosynthesis and paroxysmal nocturnal hemoglobinuria#JOURNAL:Blood#YEAR:1994#PUBMED:8193350# AUTHORS:Eisenhaber B, Maurer-Stroh S, Novatchkova M, Schneider G, Eisenhaber F#TITLE:Enzymes and auxiliary factors for GPI lipid anchor biosynthesis and post-translational transfer to proteins#JOURNAL:Bioesssays#YEAR:2003#PUBMED:12655644# AUTHORS:Inoue N, Watanabe R, Takeda J, Kinoshita T#TITLE:PIG-C, one of the three human genes involved in the first step of glycosylphosphatidylinositol biosynthesis is a homologue of Saccharomyces cerevisiae GPI2#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8806613# AUTHORS:Hong Y, Ohishi K, Inoue N, Endo Y, Fujita T, Takeda J, Kinoshita T#TITLE:Structures and chromosomal localizations of the glycosylphosphatidylinositol synthesis gene PIGC and its pseudogene PIGCP1#JOURNAL:Genomics#YEAR:1997#PUBMED:9325057# AUTHORS:Kamitani T, Chang HM, Rollins C, Waneck GL, Yeh ET. #TITLE:Correction of the class H defect in glycosylphosphatidylinositol anchor biosynthesis in Ltk- cells by a human cDNA clone#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8407896# AUTHORS:Watanabe R, Inoue N, Westfall B, Taron CH, Orlean P, Takeda J, Kinoshita T#TITLE:The first step of glycosylphosphatidylinositol biosynthesis is mediated by a complex of PIG-A, PIG-H, PIG-C and GPI1#JOURNAL:EMBO J#YEAR:1998#PUBMED:9463366# AUTHORS:Tiede A, Daniels RJ, Higgs DR, Mehrein Y, Schmidt RE, Schubert J#TITLE:The human GPI1 gene is required for efficient glycosylphosphatidylinositol biosynthesis#JOURNAL:Gene#YEAR:2001#PUBMED:11418246# GENE ASSOCIATION: (5279.1) and (51227.3) and (5283.1) and (5277.2) and (9091.1) and (8818.1) or (5279.1) and (9091.2) and (51227.2) and (5277.2) and (5283.1) and (8818.2) or (51227.1) and (9091.2) and (5277.3) and (5283.1) and (5279.2) and (8818.1) or (51227.3) and (5277.1) and (5283.1) and (9091.1) and (8818.1) and (5279.2) or (51227.3) and (9091.2) and (5277.3) and (5283.1) and (5279.2) and (8818.1) or (5279.1) and (51227.2) and (9091.2) and (5277.1) and (5283.1) and (8818.1) or (5279.1) and (51227.1) and (5283.1) and (5277.2) and (9091.1) and (8818.2) or (51227.2) and (5277.1) and (5283.1) and (9091.1) and (8818.2) and (5279.2) or (51227.3) and (9091.2) and (5277.3) and (5283.1) and (5279.2) and (8818.2) or (51227.1) and (9091.2) and (5277.1) and (5283.1) and (8818.2) and (5279.2) or (9091.2) and (51227.2) and (5283.1) and (5277.2) and (5279.2) and (8818.2) or (5279.1) and (9091.2) and (51227.2) and (5277.3) and (5283.1) and (8818.1) or (5279.1) and (51227.1) and (9091.2) and (5277.2) and (5283.1) and (8818.1) or (5279.1) and (51227.3) and (5277.3) and (5283.1) and (9091.1) and (8818.1) or (51227.1) and (5277.3) and (5283.1) and (9091.1) and (8818.1) and (5279.2) or (9091.2) and (51227.2) and (5277.1) and (5283.1) and (8818.2) and (5279.2) or (5279.1) and (51227.3) and (5277.1) and (5283.1) and (9091.1) and (8818.2) or (5279.1) and (9091.2) and (51227.2) and (5277.2) and (5283.1) and (8818.1) or (5279.1) and (51227.3) and (9091.2) and (5283.1) and (5277.2) and (8818.1) or (5279.1) and (51227.3) and (9091.2) and (5277.1) and (5283.1) and (8818.1) or (51227.3) and (9091.2) and (5283.1) and (5277.2) and (8818.2) and (5279.2) or (51227.2) and (5277.3) and (5283.1) and (9091.1) and (8818.1) and (5279.2) or (5279.1) and (51227.2) and (5283.1) and (9091.1) and (5277.2) and (8818.1) or (5279.1) and (51227.1) and (5277.3) and (5283.1) and (9091.1) and (8818.2) or (51227.1) and (9091.2) and (5283.1) and (5277.2) and (8818.1) and (5279.2) or (51227.1) and (9091.2) and (5277.3) and (5283.1) and (8818.2) and (5279.2) or (5279.1) and (51227.3) and (5277.3) and (9091.1) and (5283.1) and (8818.2) or (51227.2) and (5283.1) and (9091.1) and (5277.2) and (8818.1) and (5279.2) or (5279.1) and (51227.3) and (9091.2) and (5277.1) and (5283.1) and (8818.2) or (51227.1) and (9091.2) and (5277.1) and (5283.1) and (8818.1) and (5279.2) or (5279.1) and (51227.1) and (9091.2) and (5277.3) and (5283.1) and (8818.2) or (5279.1) and (51227.3) and (9091.2) and (5277.3) and (5283.1) and (8818.2) or (5279.1) and (9091.2) and (51227.2) and (5277.3) and (5283.1) and (8818.2) or (51227.2) and (5277.3) and (9091.1) and (5283.1) and (8818.2) and (5279.2) or (51227.3) and (5283.1) and (5277.2) and (9091.1) and (8818.1) and (5279.2) or (9091.2) and (51227.2) and (5277.1) and (5283.1) and (5279.2) and (8818.1) or (51227.1) and (5277.3) and (5283.1) and (9091.1) and (5279.2) and (8818.2) or (5279.1) and (51227.2) and (5277.3) and (5283.1) and (9091.1) and (8818.2) or (51227.2) and (9091.2) and (5277.3) and (5283.1) and (8818.1) and (5279.2) or (51227.3) and (5277.3) and (9091.1) and (5283.1) and (5279.2) and (8818.2) or (9091.2) and (51227.2) and (5277.3) and (5283.1) and (5279.2) and (8818.2) or (5279.1) and (51227.1) and (5277.1) and (9091.1) and (5283.1) and (8818.2) or (51227.2) and (5277.1) and (5283.1) and (9091.1) and (8818.1) and (5279.2) or (5279.1) and (51227.1) and (5277.1) and (5283.1) and (9091.1) and (8818.1) or (5279.1) and (51227.2) and (5283.1) and (5277.2) and (9091.1) and (8818.1) or (5279.1) and (51227.1) and (9091.1) and (5277.2) and (5283.1) and (8818.1) or (5279.1) and (51227.1) and (9091.2) and (5283.1) and (5277.2) and (8818.2) or (51227.1) and (9091.2) and (5283.1) and (5277.2) and (8818.2) and (5279.2) or (51227.1) and (5277.1) and (5283.1) and (9091.1) and (8818.2) and (5279.2) or (51227.3) and (5279.1) and (9091.2) and (5277.3) and (5283.1) and (8818.1) or (51227.3) and (5277.3) and (5283.1) and (9091.1) and (8818.1) and (5279.2) or (5279.1) and (51227.1) and (5277.3) and (5283.1) and (9091.1) and (8818.1) or (51227.3) and (9091.2) and (5277.1) and (5283.1) and (8818.2) and (5279.2) or (5279.1) and (51227.2) and (5277.3) and (9091.1) and (5283.1) and (8818.1) or (51227.3) and (5277.1) and (9091.1) and (5283.1) and (5279.2) and (8818.2) or (51227.2) and (9091.2) and (5277.2) and (5283.1) and (8818.1) and (5279.2) or (51227.3) and (9091.1) and (5283.1) and (5277.2) and (5279.2) and (8818.2) or (51227.3) and (9091.2) and (5277.1) and (5283.1) and (5279.2) and (8818.1) or (51227.1) and (5277.1) and (5283.1) and (9091.1) and (5279.2) and (8818.1) or (5279.1) and (51227.2) and (5277.1) and (9091.1) and (5283.1) and (8818.2) or (5279.1) and (51227.1) and (9091.2) and (5277.1) and (5283.1) and (8818.1) or (5279.1) and (51227.3) and (9091.2) and (5283.1) and (5277.2) and (8818.2) or (5279.1) and (51227.3) and (5277.1) and (5283.1) and (9091.1) and (8818.1) or (5279.1) and (51227.1) and (9091.2) and (5277.3) and (5283.1) and (8818.1) or (51227.3) and (9091.2) and (5283.1) and (5277.2) and (5279.2) and (8818.1) or (5279.1) and (9091.2) and (51227.2) and (5277.1) and (5283.1) and (8818.2) or (51227.1) and (5283.1) and (5277.2) and (9091.1) and (5279.2) and (8818.2) or (5279.1) and (51227.1) and (9091.2) and (5277.1) and (5283.1) and (8818.2) or (5279.1) and (51227.3) and (9091.1) and (5277.2) and (5283.1) and (8818.2) or (51227.1) and (5283.1) and (9091.1) and (5277.2) and (5279.2) and (8818.1) or (51227.2) and (5283.1) and (5277.2) and (9091.1) and (8818.2) and (5279.2) or (5279.1) and (51227.2) and (5277.1) and (9091.1) and (5283.1) and (8818.1)
phosphatidylinositol N-acetylglucosaminyltransferase
phosphatidylinositol (homo sapiens)_C9H16O9PFULLRCO2FULLR2CO2
Abbreviation: R_COQ7m Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 2dp6mobq_me + h + nadph + o2 --> 2dpmhobq + h2o + nadp Confidence Level: 4 _1.14.13.- IT genes: LOCUS:_10229#TRANSCRIPT:1#ABBREVIATION:COQ7# proteins: NAME:coenzyme Q7 homolog, ubiquinone (yeast) #ABBREVIATION:Coq7.1-m# citations: AUTHORS:Kang DC, Takeshige K, Minakami S.#TITLE:An intermediate of ubiquinone biosynthesis exists in the microsomal fraction of HepG2 cells. #JOURNAL:J Biochem (Tokyo).#YEAR:1990#PUBMED:1965190# AUTHORS:Nambudiri AM, Ranganathan S, Rudney H#TITLE:The role of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in the regulation of ubiquinone synthesis in human fibroblasts. #JOURNAL:J Biol Chem.#YEAR:1980#PUBMED:7380842# AUTHORS:Szkopinska A.#TITLE:Ubiquinone. Biosynthesis of quinone ring and its isoprenoid side chain. Intracellular localization.#JOURNAL:Acta Biochim Pol. #YEAR:2000#PUBMED:11051212# GENE ASSOCIATION: (10229.1)
Abbreviation: R_5HTRPVESSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) 5htrp[c] + (2) atp[c] + (2) h2o[c] --> (3) 5htrp[e] + (2) adp[c] + (2) h[c] + (2) pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of 5htrp from the cell. It leaves out the vesicle because that is not a compartment. ATP:H ratio is assumed as 1:3 for vacuolar ATPase and substrate:h ratio is 1:2. This gives a 3:2 ratio for substrate:ATP genes: LOCUS:_6571#TRANSCRIPT:1#ABBREVIATION:SLC18A2# LOCUS:_6570#TRANSCRIPT:1#ABBREVIATION:SLC18A1# proteins: NAME:solute carrier family 18 (vesicular monoamine), member 1#ABBREVIATION:Slc18a1.1# NAME:solute carrier family 18 (vesicular monoamine), member 2#ABBREVIATION:Slc18a2.1# citations: AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# AUTHORS:Eiden LE, Schafer MK, Weihe E, Schutz B#TITLE:The vesicular amine transporter family (SLC18): amine/proton antiporters required for vesicular accumulation and regulated exocytotic secretion of monoamines and acetylcholine#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12827358# GENE ASSOCIATION: (6570.1) or (6571.1)
_5-hydroxy-L-tryptophan secretion via secretory vesicle (ATP driven)
Abbreviation: R_GLNS Synonyms: _0 EC Number: 6.3.1.2 SUBSYSTEM: Glutamate metabolism Equation: [c] : atp + glu-L + nh4 --> adp + gln-L + h + pi Confidence Level: 2 genes: LOCUS:_2752#TRANSCRIPT:1#ABBREVIATION:GLUL#ECNUMBERS:6.3.1.2# LOCUS:_51557#TRANSCRIPT:1#ABBREVIATION:GLULD1# proteins: NAME:glutamate-ammonia ligase (glutamine synthase)#ABBREVIATION:Glul.1# NAME:glutamate-ammonia ligase (glutamine synthase)#ABBREVIATION:Gluld1.1# citations: AUTHORS:Gibbs CS, Campbell KE, Wilson RH.#TITLE:Sequence of a human glutamine synthetase cDNA.#YEAR:1987#PUBMED:2888076# GENE ASSOCIATION: (2752.1) or (51557.1)
keratan sulfate I, degradation product 22_C132H214N9O111S5
Abbreviation: R_FUCFUCGALACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucfucgalacglcgalgluside_hs[e] <==> fucfucgalacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_OCBTm Synonyms: _0 EC Number: 2.1.3.3 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : cbp + orn --> citr-L + h + pi Confidence Level: 4 TDV genes: LOCUS:_5009#TRANSCRIPT:1#ABBREVIATION:OTC#ECNUMBERS:2.1.3.3# proteins: NAME:ornithine carbamoyltransferase#ABBREVIATION:Otc.1-m# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Horwich AL, Fenton WA, Williams KR, Kalousek F, Kraus JP, Doolittle RF, Konigsberg W, Rosenberg LE#TITLE:Structure and expression of a complementary DNA for the nuclear coded precursor of human mitochondrial ornithine transcarbamylase#JOURNAL:Science#YEAR:1984#PUBMED:6372096# AUTHORS:Dekaney CM, Wu G, Jaeger LA.#TITLE:Gene expression and activity of enzymes in the arginine biosynthetic pathway in porcine fetal small intestine.#JOURNAL:Pediatr Res#YEAR:2003#PUBMED:12538786# GENE ASSOCIATION: (5009.1)
ornithine carbamoyltransferase, irreversible
N-Acetyl-D-glucosamine 6-phosphate_C8H14NO9P
Abbreviation: R_AGDC Synonyms: _0 EC Number: 3.5.1.25 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acgam6p + h2o --> ac + gam6p Confidence Level: 2 acetylglucosamine phosphate deacetylase; acetylaminodeoxyglucosephosphate acetylhydrolase; 2-acetamido-2-deoxy-D-glucose-6-phosphate amidohydrolase genes: LOCUS:_51005#TRANSCRIPT:1#ABBREVIATION:CGI-14# proteins: NAME:CGI-14 protein#ABBREVIATION:Cgi-14.1# citations: none GENE ASSOCIATION: (51005.1)
N-acetylglucosamine-6-phosphate deacetylase
Gal-Gal-Gal-Gal-Gal-Glc-Cer (homo sapiens)_C54H96NO32FULLRCO
heparan sulfate, precursor 5_C59H89N3O47X
Abbreviation: R_S4TASE3ly Synonyms: _5 EC Number: 3.1.6.12 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_b + h2o <==> cs_b_deg1 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_411#TRANSCRIPT:1#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# LOCUS:_411#TRANSCRIPT:2#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# proteins: NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.1-l# NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.2-l# citations: AUTHORS:Schuchman EH, Jackson CE, Desnick RJ#TITLE:Human arylsulfatase B: MOPAC cloning, nucleotide sequence of a full-length cDNA, and regions of amino acid identity with arylsulfatases A and C#JOURNAL:Genomics#YEAR:1990#PUBMED:1968043# AUTHORS:Peters C, Schmidt B, Rommerskirch W, Rupp K, Zuhlsdorf M, Vingron M, Meyer HE, Pohlmann R, von Figura K#TITLE:Phylogenetic conservation of arylsulfatases. cDNA cloning and expression of human arylsulfatase B.#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2303452# GENE ASSOCIATION: (411.1) or (411.2)
N-acetylgalactosamine-4-sulfatase, lysosomal
chondroitin sulfate B / dermatan sulfate (IdoA2S-GalNAc4S), degradation product 1_C45H67N2O46S3
chondroitin sulfate B / dermatan sulfate (IdoA2S-GalNAc4S), free chain_C45H66N2O49S4
Abbreviation: R_IMACTD_m Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Histidine Metabolism Equation: [m] : h2o + im4act + nad --> (2) h + im4ac + nadh Confidence Level: 2 SAB 2 H's on RHS are there to balance--not certain genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (219.1) or (8659.1) or (217.1) or (8659.2)
Imidazole acetaldeyde dehydrogenase (mito)
Abbreviation: R_PACCOAL Synonyms: _0 EC Number: 6.2.1.30 SUBSYSTEM: Phenylalanine metabolism Equation: [c] : atp + coa + pac --> amp + phaccoa + ppi Confidence Level: 2 JLR genes: none proteins: none citations: AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# GENE ASSOCIATION:
Abbreviation: R_25HVITD2tin_DASH_m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _25hvitd2[c] --> 25hvitd2[m] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_25-hydroxyvitamin D2 transport in mitochondria
lysophosphatidylcholine (homo sapiens)_C8H19NO5PFULLRCO2
Abbreviation: R_PLA2_2 Synonyms: _0 EC Number: 3.1.1.4 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : h2o + pchol_hs --> Rtotal2 + h + lpchol_hs Confidence Level: 4 phosphatidyl choline degradation NJ genes: LOCUS:_5320#TRANSCRIPT:1#ABBREVIATION:PLA2G2A# LOCUS:_8398#TRANSCRIPT:1#ABBREVIATION:PLA2G6# LOCUS:_8398#TRANSCRIPT:2#ABBREVIATION:PLA2G6# proteins: NAME:phospholipase A2, group VI (cytosolic, Ca independent)#ABBREVIATION:Pla2g6.1# NAME:phospholipase A2, group VI (cytosolic, Ca dependent)#ABBREVIATION:Pla2g6.2# NAME:phospholipase A2, groupIIA (platelets, synovial fluid)#ABBREVIATION:Pla2g2a.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Larsson PK, Claesson HE, Kennedy BP.#TITLE:Multiple splice variants of the human calcium-independent phospholipase A2 and their effect on enzyme activity.#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9417066# AUTHORS:Nimmrich I, Friedl W, Kruse R, Pietsch S, Hentsch S, Deuter R, Winde G, Muller O.#TITLE:Loss of the PLA2G2A gene in a sporadic colorectal tumor of a patient with a PLA2G2A germline mutation and absence of PLA2G2A germline alterations in patients with FAP.#JOURNAL:Hum Genet#YEAR:1997#PUBMED:9272153# GENE ASSOCIATION: (8398.1) or (5320.1) or (8398.2)
Phosphatidylcholine (homo sapiens)_C8H18NO4PFULLRCO2FULLR2CO2
(Gal)3 (Glc)1 (GlcNAc)3 (Cer)1_C66H115N4O37FULLRCO
Abbreviation: R_NTD2l Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleic acid degradation Equation: [l] : h2o + ump --> pi + uri Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:R.L. Pisoni#TITLE:Lysosomal Nucleic Acid and Phosphate Metabolism and Related Metabolic Reactions, Chapter 9#YEAR:1996# GENE ASSOCIATION: (53.1)
Abbreviation: R_PEAMNO Synonyms: _0 EC Number: 1.4.3.6 SUBSYSTEM: Phenylalanine metabolism Equation: [c] : h2o + o2 + peamn --> h2o2 + nh4 + pacald Confidence Level: 2 JLR- Kegg reaction R02613 genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# LOCUS:_314#TRANSCRIPT:1#ABBREVIATION:AOC2#ECNUMBERS:1.4.3.6# LOCUS:_26#TRANSCRIPT:1#ABBREVIATION:ABP1#ECNUMBERS:1.4.3.6# LOCUS:_314#TRANSCRIPT:2#ABBREVIATION:AOC2# proteins: NAME:amiloride binding protein 1 (amine oxidase (copper-containing))#ABBREVIATION:Abp1.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.2# NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# AUTHORS: Shih JC, Chen K, Ridd MJ#TITLE:Role of MAO A and B in neurotransmitter metabolism and behavior#JOURNAL:Pol J Pharmacol#YEAR:1999#PUBMED:10389141# GENE ASSOCIATION: (4129.1) or (26.1) or (4128.1) or (314.1) or (314.2)
Abbreviation: R_HTAXOLte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: htaxol[e] <==> htaxol[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_P4502A6 Synonyms: _2 SUBSYSTEM: CYP Metabolism Equation: [c] : coumarin + h + nadph + o2 --> h2o + hcoumarin + nadp Confidence Level: 4 coumarin: 7 hydroxylation assumed to take place on outer membrane - xenobiotic metabolism NJ genes: LOCUS:_1553#TRANSCRIPT:1#ABBREVIATION:CYP2A13#ECNUMBERS:1.14.14.1# LOCUS:_1548#TRANSCRIPT:1#ABBREVIATION:CYP2A6#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 2, subfamily A, polypeptide 13#ABBREVIATION:Cyp2a13.1# NAME:cytochrome P450, family 2, subfamily A, polypeptide 6#ABBREVIATION:Cyp2a6.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Oscarson M, Gullsten H, Rautio A, Bernal ML, Sinues B, Dahl ML, Stengard JH, Pelkonen O, Raunio H, Ingelman-Sundberg M.#TITLE:Genotyping of human cytochrome P450 2A6 (CYP2A6), a nicotine C-oxidase.#JOURNAL:FEBS Let#YEAR:1998#PUBMED:9827545# GENE ASSOCIATION: (1553.1) or (1548.1)
Abbreviation: R_LIMNENte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: limnen[e] <==> limnen[c] Confidence Level: 2 unknown mechanism, xenobiotic transport (original and metabolized) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UMPK2n Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : ctp + ump <==> cdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_CYSLYSL Synonyms: _0 EC Number: 4.4.1.1 SUBSYSTEM: Cysteine Metabolism Equation: [c] : Lcystin + h2o --> nh4 + pyr + thcys Confidence Level: 4 according to reaction in KEGG MM genes: none proteins: none citations: AUTHORS:Stipanuk MH#TITLE:Sulfur amino acid metabolism: pathways for production and removal of homocysteine and cysteine#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189131# GENE ASSOCIATION:
Abbreviation: R_EX_4abut_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 4abut <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Dolichol phosphate, human uterine homolog_C1025H1670O40P10
Abbreviation: R_DOLPMT3_Uer Synonyms: _2 EC Number: 2.4.1.130 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolmanp_U + (0.1) m6mpdol_U --> (0.1) dolp_U + h + (0.1) m7mpdol_U Confidence Level: 0 NCD genes: LOCUS:_79087#TRANSCRIPT:1#ABBREVIATION:ALG12# proteins: NAME:alpha-1,6-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg12.1-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (79087.1)
(alpha-D-Mannosyl)7-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1665H2740N20O570P20
Dolichyl phosphate D-mannose, human uterine homolog_C1085H1780O90P10
(alpha-D-Mannosyl)6-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1605H2640N20O520P20
dolichyl-phosphate-mannose-glycolipid alpha-mannosyltransferase (uterus)
Abbreviation: R_3HCO3_NAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) hco3[e] + na1[e] <==> (3) hco3[c] + na1[c] Confidence Level: 4 MM genes: LOCUS:_8671#TRANSCRIPT:1#ABBREVIATION:SLC4A4# LOCUS:_57835#TRANSCRIPT:1#ABBREVIATION:SLC4A5# LOCUS:_57835#TRANSCRIPT:2#ABBREVIATION:SLC4A5# LOCUS:_57835#TRANSCRIPT:3#ABBREVIATION:SLC4A5# LOCUS:_57835#TRANSCRIPT:4#ABBREVIATION:SLC4A5# proteins: NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 4, extracellular#ABBREVIATION:Slc4a4.1-e# NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 5, extracellular#ABBREVIATION:Slc4a5.1-e# NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 5, extracellular#ABBREVIATION:Slc4a5.2-e# NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 5, extracellular#ABBREVIATION:Slc4a5.3-e# NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 5, extracellular#ABBREVIATION:Slc4a5.4-e# citations: AUTHORS:Romero MF, Fulton CM, Boron WF.#TITLE:The SLC4 family of HCO 3 - transporters.#YEAR:2004#PUBMED:14722772# GENE ASSOCIATION: (57835.3) or (8671.1) or (57835.2) or (57835.1) or (57835.4)
bicarbonate transport (Na/HCO3 1:3 cotransport)
Abbreviation: R_THYMDt1 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: thymd[e] --> thymd[c] Confidence Level: 4 TDV genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# AUTHORS:Brown NS, Bicknell R.#TITLE:Thymidine phosphorylase, 2-deoxy-D-ribose and angiogenesis#JOURNAL:Biochem J#YEAR:1998#PUBMED:9693094# GENE ASSOCIATION: (3177.1) or (2030.1)
P1,P4-Bis(5'-adenosyl) tetraphosphate_C20H24N10O19P4
Abbreviation: R_FUCGALFUCGALACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucgalfucgalacglcgalgluside_hs[e] <==> fucgalfucgalacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_aflatoxin_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : aflatoxin <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate II (core 2-linked)_C81H129N6O67S3X
Abbreviation: R_STS3r Synonyms: _0 EC Number: 3.1.6.2 SUBSYSTEM: Steroid Metabolism Equation: [r] : chsterols + h2o --> chsterol + h + so4 Confidence Level: 4 NJ genes: LOCUS:_412#TRANSCRIPT:1#ABBREVIATION:STS#ECNUMBERS:3.1.6.2# proteins: NAME:steroid sulfatase (microsomal), arylsulfatase C#ABBREVIATION:Sts.1# citations: AUTHORS:Stein C, Hille A, Seidel J, Rijnbout S, Waheed A, Schmidt B, Geuze H, von Figura K.#TITLE:Cloning and expression of human steroid-sulfatase. Membrane topology, glycosylation, and subcellular distribution in BHK-21 cells.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2668275# AUTHORS:Migeon BR, Shapiro LJ, Norum RA, Mohandas T, Axelman J, Dabora RL.#TITLE:Abstract Differential expression of steroid sulphatase locus on active and inactive human X chromosome.#JOURNAL:Nature#YEAR:1982#PUBMED:6957717# GENE ASSOCIATION: (412.1)
Abbreviation: R_GD1Ctg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gd1c_hs[c] <==> gd1c_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_EX_yvite_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : yvite <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(Gal)6 (Glc)1 (GlcNAc)3 (LFuc)2 (Cer)1_C96H165N4O60FULLRCO
Abbreviation: R_EX_hestratriol_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hestratriol <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
alpha-D-Glucosyl-(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1785H2940N20O670P20
(mannosyl),(phosphoethanolaminyl)-mannosyl-glucosylaminyl-acylphosphatidylinositol (B)_C45H84N2O27P2FULLRCO2FULLR2CO2
keratan sulfate II biosynthesis, precursor 1_C33H53N3O23X
Etiocholan-3alpha-ol-17-one 3-glucuronide_C25H38O8
Abbreviation: R_AHANDROSTANGLCte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ahandrostanglc[c] + atp[c] + h2o[c] --> adp[c] + ahandrostanglc[e] + h[c] + pi[c] Confidence Level: 2 for MDR transport NJ genes: LOCUS:_4363#TRANSCRIPT:1#ABBREVIATION:ABCC1.1# proteins: NAME:ATP-binding cassette, subfamily C (CFTR/MRP), member 1#ABBREVIATION:ABCC1.1# citations: none GENE ASSOCIATION: (4363.1)
_1-Phosphatidyl-1D-myo-inositol 4-phosphate (Homo sapiens)_C9H15O12P2FULLRCO2FULLR2CO2
_4-Hydroxy-2-quinolinecarboxylic acid_C10H6NO3
Abbreviation: R_TETTET6t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tettet6[e] <==> tettet6[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
glutamate dehydrogenase (NAD) (mitochondrial)
Abbreviation: R_GLUDxm Synonyms: _0 EC Number: 1.4.1.2 SUBSYSTEM: Glutamate metabolism Equation: [m] : glu-L + h2o + nad <==> akg + h + nadh + nh4 Confidence Level: 4 genes: LOCUS:_2746#TRANSCRIPT:1#ABBREVIATION:GLUD1#ECNUMBERS:1.4.1.3# LOCUS:_2747#TRANSCRIPT:1#ABBREVIATION:GLUD2# proteins: NAME:glutamate dehydrogenase 1, mitochondrial#ABBREVIATION:Glud1.1-m# NAME:glutamate dehydrogenase 2, mitochondrial#ABBREVIATION:Glud2.1-m# citations: AUTHORS:Jie FANG , Betty Y. L. HSU , Courtney M. MACMULLEN , Mortimer PONCZ , Thomas J. SMITH and Charles A. STANLEY#TITLE:Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations #YEAR:2002# AUTHORS:Zaganas I, Spanaki C, Karpusas M, Plaitakis A.#TITLE:Substitution of Ser for Arg-443 in the regulatory domain of human housekeeping (GLUD1) glutamate dehydrogenase virtually abolishes basal activity and markedly alters the activation of the enzyme by ADP and L-leucine.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12324473# AUTHORS:Mavrothalassitis G, Tzimagiorgis G, Mitsialis A, Zannis V, Plaitakis A, Papamatheakis J, Moschonas N.#TITLE:Isolation and characterization of cDNA clones encoding human liver glutamate dehydrogenase: evidence for a small gene family. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1988#PUBMED:3368458# GENE ASSOCIATION: (2747.1) or (2746.1)
Abbreviation: R_SGPL12r Synonyms: _3 EC Number: 4.1.2.27 SUBSYSTEM: Sphingolipid Metabolism Equation: [r] : h2o + sphs1p --> ethamp + h + hdca Confidence Level: 2 SGPL1 gene as per GeneCards states that hexadecanal is produced - the only way to do this and to maintain charge balance would be to have H2 gas as substrate - remarkably implausible. Due to lack of information to indicate otherwise, added water to breakdown components into ethamp and palmitate NJ genes: LOCUS:_8879#TRANSCRIPT:1#ABBREVIATION:SGPL1# proteins: NAME:sphingosine-1-phosphate lyase 1 #ABBREVIATION:Sgpl.1# citations: AUTHORS:Van Veldhoven PP, Gijsbers S, Mannaerts GP, Vermeesch JR, Brys V.#TITLE:Human sphingosine-1-phosphate lyase: cDNA cloning, functional expression studies and mapping to chromosome 10q22(1).#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:11018465# AUTHORS:Reiss U, Oskouian B, Zhou J, Gupta V, Sooriyakumaran P, Kelly S, Wang E, Merrill AH Jr, Saba JD.#TITLE:Sphingosine-phosphate lyase enhances stress-induced ceramide generation and apoptosis.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14570870# GENE ASSOCIATION: (8879.1)
Abbreviation: R_EX_man_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : man <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_H2O2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h2o2[e] <==> h2o2[c] Confidence Level: 2 AMF genes: none proteins: none citations: AUTHORS:Ohno Y, Gallin JI. #TITLE:Diffusion of extracellular hydrogen peroxide into intracellular compartments of human neutrophils. Studies utilizing the inactivation of myeloperoxidase by hydrogen peroxide and azide.#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2989289# GENE ASSOCIATION:
hydrogen peroxide transport via diffusion
Abbreviation: R_P4503A43r Synonyms: _1 SUBSYSTEM: Steroid Metabolism Equation: [r] : h + nadph + o2 + tststerone --> h2o + nadp + whtststerone Confidence Level: 2 omega hydroxylation of testosterone NJ genes: LOCUS:_64816#TRANSCRIPT:1#ABBREVIATION:CYP3A43#ECNUMBERS:1.14.14.1# LOCUS:_64816#TRANSCRIPT:2#ABBREVIATION:CYP3A43#ECNUMBERS:1.14.14.1# LOCUS:_64816#TRANSCRIPT:3#ABBREVIATION:CYP3A43#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 3, subfamily A, polypeptide#ABBREVIATION:Cyp3a43.1# NAME:cytochrome P450, family 3, subfamily A, polypeptide#ABBREVIATION:Cyp3a43.2# NAME:cytochrome P450, family 3, subfamily A, polypeptide#ABBREVIATION:Cyp3a43.3# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# GENE ASSOCIATION: (64816.1) or (64816.3) or (64816.2)
Abbreviation: R_EX_35cgmp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 35cgmp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
trans-Hexadec-2-enoyl carnitine_C23H43NO4
Abbreviation: R_ACALDtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: acald[x] <==> acald[c] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_TSTSTERONEtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: tststerone[r] <==> tststerone[c] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACGPID Synonyms: _5 EC Number: 3.5.1.89 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [c] : acgpail_hs + h2o --> ac + gpail_hs Confidence Level: 4 NCD genes: LOCUS:_9487#TRANSCRIPT:1#ABBREVIATION:PIGL# proteins: NAME:phosphatidylinositol glycan, class L#ABBREVIATION:Pigl.1# citations: AUTHORS:Pottekat A, Menon AK#TITLE:Subcellular localization and targeting of N-acetylglucosaminyl phosphatidylinositol de-N-acetylase, the second enzyme in the glycosylphosphatidylinositol biosynthetic pathway#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14742432# AUTHORS:Watanabe R, Ohishi K, Maeda Y, Nakamura N, Kinoshita T#TITLE:Mammalian PIG-L and its yeast homologue Gpi12p are N-acetylglucosaminylphosphatidylinositol de-N-acetylases essential in glycosylphosphatidylinositol biosynthesis#JOURNAL:Biochem J#YEAR:1999#PUBMED:10085243# GENE ASSOCIATION: (9487.1)
N-acetylglucosaminylphosphatidylinositol deacetylase
Abbreviation: R_TETTET6CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: tettet6crn[c] --> tettet6crn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_OCCOAtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: occoa[c] --> occoa[m] Confidence Level: 0 TDV genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_34HPLFM Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 34hpp + h + nadh --> 34hpl + nad Confidence Level: 2 IT reaction involved in formation of 4-hydroxybenzoate from tyrosine. genes: none proteins: none citations: AUTHORS:Meganathan R.#TITLE:Ubiquinone biosynthesis in microorganisms.#JOURNAL:FEMS Microbiol Lett. #YEAR:2001#PUBMED:11583838# AUTHORS:Loscher R, Heide L.#TITLE:Biosynthesis of p-Hydroxybenzoate from p-Coumarate and p-Coumaroyl-Coenzyme A in Cell-Free Extracts of Lithospermum erythrorhizon Cell Cultures. #JOURNAL:Plant Physiol.#YEAR:1994#PUBMED:12232327# AUTHORS:Booth, A. N., Masri, M. S., Robbins, D. J., Emerson, O. H., Jones, F. T. & DeEds, F.#TITLE:Urinary Phenolic Acid Metabolites of Tyrosine#JOURNAL:J. Biol. Chem#YEAR:1960# GENE ASSOCIATION:
Abbreviation: R_XOL27OHtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: xol27oh[r] <==> xol27oh[m] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_B3GALT42g Synonyms: _7 EC Number: 2.4.1.62 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gm2_hs + udpgal --> gm1_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_8705#TRANSCRIPT:1#ABBREVIATION:B3GALT4# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 4#ABBREVIATION:B3galt4.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Amado M, Almeida R, Carneiro F, Levery ST, Holmes EH, Nomoto M, Hollingsworth MA, Hassan H, Schwientek T, Nielsen PA, Bennett EP, Clausen H#TITLE:A family of human beta3-galactosyltransferases#JOURNAL:The Journal of Biological Chemistry#YEAR:1998# GENE ASSOCIATION: (8705.1)
ganglioside GM1 (homo sapiens)_C55H95N3O30FULLRCO
ganglioside GM2 (homo sapiens)_C49H85N3O25FULLRCO
keratan sulfate I biosynthesis, precursor 31_C227H363N16O189S9X
Octadecenoyl-CoA (n-C18:1CoA)_C39H64N7O17P3S
Abbreviation: R_C181CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + odecrn --> crn + odecoa Confidence Level: 4 TDV genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# GENE ASSOCIATION: (1376.1)
_15-Hydroperoxyeicosatetraenoic acid_C20H31O4
Abbreviation: R_ALOX15 Synonyms: _2 EC Number: 1.13.11.33 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : arachd + o2 --> 15HPET Confidence Level: 4 Converts arachidonic acid to 15S-hydroperoxyeicosatetraenoic acid. Also acts on C-12 of arachidonate as well as on linoleic acid. NJ genes: LOCUS:_246#TRANSCRIPT:1#ABBREVIATION:ALOX15#ECNUMBERS:1.13.11.33# LOCUS:_247#TRANSCRIPT:1#ABBREVIATION:ALOX15B# proteins: NAME:arachidonate 15-lipoxygenase, second type#ABBREVIATION:Alox15b.1# NAME:arachidonate 15-lipoxygenase#ABBREVIATION:Alox15.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Tang S, Bhatia B, Maldonado CJ, Yang P, Newman RA, Liu J, Chandra D, Traag J, Klein RD, Fischer SM, Chopra D, Shen J, Zhau HE, Chung LW, Tang DG.#TITLE:Evidence that arachidonate 15-lipoxygenase 2 is a negative cell cycle regulator in normal prostate epithelial cells.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11839751# GENE ASSOCIATION: (247.1) or (246.1)
(Gal)3 (Glc)1 (GlcNAc)2 (LFuc)2 (Cer)1_C70H122N3O40FULLRCO
Abbreviation: R_COAtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: coa[c] <==> coa[r] Confidence Level: 1 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_fuc14galacglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fuc14galacglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
saccharopine dehydrogenase (NAD, L-glutamate forming), mitochondrial
Abbreviation: R_SACCD4m Synonyms: _0 EC Number: 1.5.1.10 SUBSYSTEM: Lysine Metabolism Equation: [m] : h2o + nadp + saccrp-L --> L2aadp6sa + glu-L + h + nadph Confidence Level: 4 MM genes: LOCUS:_10157#TRANSCRIPT:1#ABBREVIATION:AASS# proteins: NAME:aminoadipate-semialdehyde synthase, mitochondrial #ABBREVIATION:Aass.1-m# citations: AUTHORS:Sacksteder,K.A., Biery,B.J., Morrell,J.C., Goodman,B.K., Geisbrecht,B.V., Cox,R.P., Gould,S.J., Geraghty,M.T., #TITLE:Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia.#YEAR:2000#PUBMED:10775527# AUTHORS:Cox, RP#TITLE:Errors of lysine metabolism#JOURNAL:The Metabolic and Molecular Bases of Inherited Disease, 8th ed#YEAR:2001# GENE ASSOCIATION: (10157.1)
L-2-Aminoadipate 6-semialdehyde_C6H11NO3
Abbreviation: R_CAT2p Synonyms: _0 EC Number: 1.11.1.6 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [x] : etoh + h2o2 --> acald + (2) h2o Confidence Level: 2 NCD genes: LOCUS:_847#TRANSCRIPT:1#ABBREVIATION:CAT#ECNUMBERS:1.11.1.6# proteins: NAME:catalase#ABBREVIATION:Cat.1-x# citations: AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# GENE ASSOCIATION: (847.1)
keratan sulfate I biosynthesis, precursor 21_C177H284N12O145S6X
keratan sulfate I biosynthesis, precursor 22_C185H297N13O150S6X
Abbreviation: R_AG13T11g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre21 + uacgam --> h + ksi_pre22 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase, Golgi apparatus
Abbreviation: R_PPM Synonyms: _0 EC Number: 5.4.2.7 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : r1p <==> r5p Confidence Level: 0 genes: LOCUS:_5236#TRANSCRIPT:1#ABBREVIATION:PGM1#ECNUMBERS:5.4.2.2# proteins: NAME:phosphoglucomutase 1#ABBREVIATION:Pgm1.1# citations: none GENE ASSOCIATION: (5236.1)
Abbreviation: R_PMEVKx Synonyms: _0 EC Number: 2.7.4.2 SUBSYSTEM: Cholesterol Metabolism Equation: [x] : 5pmev + atp --> 5dpmev + adp Confidence Level: 4 peroxisomal version - NJ genes: LOCUS:_10654#TRANSCRIPT:1#ABBREVIATION:PMVK#ECNUMBERS:2.7.4.2# proteins: NAME:phosphomevalonate kinase#ABBREVIATION:Pmvk.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (10654.1)
_9-O-Acetylated GD3 (homo sapiens)_C54H90N3O29FULLRCO
Abbreviation: R_ORNt4m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: citr-L[c] + h[c] + orn[m] <==> citr-L[m] + h[m] + orn[c] Confidence Level: 4 TDV (ornithine carrier) genes: LOCUS:_83884#TRANSCRIPT:1#ABBREVIATION:SLC25A2# LOCUS:_10166#TRANSCRIPT:1#ABBREVIATION:SLC25A15# proteins: NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 2#ABBREVIATION:Slc25a2.1-m# NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15#ABBREVIATION:Slc25a15.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (10166.1) or (83884.1)
ornithine mitochondrial transport exchange with citruline
Abbreviation: R_METtec Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: met-L[e] <==> met-L[c] Confidence Level: 4 SAB genes: LOCUS:_124935#TRANSCRIPT:1#ABBREVIATION:SLC43A2# LOCUS:_8501#TRANSCRIPT:1#ABBREVIATION:SLC43A1# proteins: NAME:solute carrier family 43, member 2#ABBREVIATION:Slc43a2.1# NAME:solute carrier family 43, member 1#ABBREVIATION:Slc43a1.1# citations: AUTHORS:Bodoy S, Martin L, Zorzano A, Palacin M, Estevez R, Bertran J#TITLE:Identification of LAT4, a novel amino acid transporter with system L activity#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15659399# AUTHORS:Babu E, Kanai Y, Chairoungdua A, Kim do K, Iribe Y, Tangtrongsup S, Jutabha P, Li Y, Ahmed N, Sakamoto S, Anzai N, Nagamori S, Endou H#TITLE:Identification of a novel system L amino acid transporter structurally distinct from heterodimeric amino acid transporters#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12930836# AUTHORS:Hediger MA, Romero MF, Peng JB, Rolfs A, Takanaga H, Bruford EA#TITLE:The ABCs of solute carriers: physiological, pathological and therapeutic implications of human membrane transport proteinsIntroduction#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14624363# GENE ASSOCIATION: (8501.1) or (124935.1)
L-methionine transport via diffusion (extracellular to cytosol)
Abbreviation: R_GD1Cte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gd1c_hs[c] <==> gd1c_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_PRISTCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: pristcoa[x] <==> pristcoa[c] Confidence Level: 0 Mechanism not known, see PMID 11591435 for justification. NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S23T3g Synonyms: _0 EC Number: 2.4.99.6 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : cmpacna + l2fn2m2masn --> cmp + h + ksi_pre1 Confidence Level: 4 NCD genes: LOCUS:_6487#TRANSCRIPT:1#ABBREVIATION:ST3GAL3#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:10#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:2#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:11#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:9#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:4#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:3#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:6#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:5#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:8#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:7#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# proteins: NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.1-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.10-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.11-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.2-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.3-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.4-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.5-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.6-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.7-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.8-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.9-g# citations: AUTHORS:Kitagawa H, Paulson JC#TITLE:Cloning and expression of human Gal beta 1,3(4)GlcNAc alpha 2,3-sialyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1993#PUBMED:8333853# GENE ASSOCIATION: (6487.4) or (6487.9) or (6487.11) or (6487.6) or (6487.7) or (6487.10) or (6487.5) or (6487.1) or (6487.2) or (6487.8) or (6487.3)
keratan sulfate I biosynthesis, precursor 1_C79H129N5O57X
beta-galactoside alpha-2,3-sialyltransferase (complex N-glycan)
Abbreviation: R_NDPK6n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + dudp <==> adp + dutp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:dUDP), nuclear
Abbreviation: R_AG13T14g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre30 + uacgam --> h + ksi_pre31 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
keratan sulfate I biosynthesis, precursor 30_C219H350N15O184S9X
Abbreviation: R_LNLCCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + lnlccoa --> coa + lnlccrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
chondroitin sulfate A (GalNAc4S-GlcA) proteoglycan_C45H66N2O45S3X
ganglioside GM3 (homo sapiens)_C41H72N2O20FULLRCO
Abbreviation: R_DOPAt4_LPAREN_2_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dopa[e] + (2) na1[e] <==> dopa[c] + (2) na1[c] Confidence Level: 4 SAB genes: LOCUS:_6531#TRANSCRIPT:1#ABBREVIATION:SLC6A3# LOCUS:_6530#TRANSCRIPT:1#ABBREVIATION:SLC6A2# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, noradrenalin), member 2#ABBREVIATION:Slc6a2.1# NAME:solute carrier family 6 (neurotransmitter transporter, dopamine), member 3#ABBREVIATION:Slc6a3.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (6530.1) or (6531.1)
Dopamine reversible transport in via sodium symport (1:2)
Abbreviation: R_ESTRONEtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: estrone[r] <==> estrone[c] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_BIOCYTtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: biocyt[c] <==> biocyt[n] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Pispa J.#TITLE:Animal biotinidase.#JOURNAL:Ann Med Exp Biol Fenn. #YEAR:1965#PUBMED:5867120# AUTHORS:Zempleni J., Mock D.M.#TITLE:Biotin homeostasis during the cell cycle #JOURNAL:Nutrition Research Reviews#YEAR:2001# AUTHORS:Hymes J, Fleischhauer K, Wolf B.#TITLE:Biotinylation of biotinidase following incubation with biocytin.#JOURNAL:Clin Chim Acta#YEAR:1995#PUBMED:7758201# AUTHORS:Hymes J, Fleischhauer K, Wolf B.#TITLE:Biotinylation of histones by human serum biotinidase: assessment of biotinyl-transferase activity in sera from normal individuals and children with biotinidase deficiency.#JOURNAL:Biochem Mol Med.#YEAR:1995#PUBMED:8593541# AUTHORS:Zempleni J.#TITLE:Uptake, localization, and noncarboxylase roles of biotin.#JOURNAL:Annu Rev Nutr#YEAR:2005#PUBMED:16011464# GENE ASSOCIATION:
Abbreviation: R_NACASPAH Synonyms: _0 EC Number: 3.5.1.15 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [c] : Nacasp + h2o --> ac + asp-L Confidence Level: 4 SAB genes: LOCUS:_443#TRANSCRIPT:1#ABBREVIATION:ASPA#ECNUMBERS:3.5.1.15# LOCUS:_91703#TRANSCRIPT:1#ABBREVIATION:ACY3# proteins: NAME:aspartoacylase (aminoacylase 2)#ABBREVIATION:Aspa.1# NAME:aspartoacylase (aminocyclase) 3#ABBREVIATION:Acy3.1# citations: AUTHORS:Kaul R, Gao GP, Balamurugan K, Matalon R#TITLE:Cloning of the human aspartoacylase cDNA and a common missense mutation in Canavan disease#JOURNAL:Nat Genet#YEAR:1993#PUBMED:8252036# GENE ASSOCIATION: (443.1) or (91703.1)
(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine (protein)_C70H117N2O55X
Abbreviation: R_EX_vacc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : vacc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_2,6 dimethylheptanoyl carnitine_C16H35NO4
Abbreviation: R_DNDPt1m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[m] + datp[c] --> adp[c] + datp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_GLYCLTDy Synonyms: _1 EC Number: 1.1.1.26 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : glx + h + nadph --> glyclt + nadp Confidence Level: 4 JLR genes: LOCUS:_9380#TRANSCRIPT:1#ABBREVIATION:GRHPR#ECNUMBERS:1.1.1.79# proteins: NAME:glyoxylate reductase/hydroxypyruvate reductase#ABBREVIATION:Grhpr.1# citations: AUTHORS:Van Schaftingen E, Draye JP, Van Hoof F#TITLE:Coenzyme specificity of mammalian liver D-glycerate dehydrogenase#JOURNAL:Eur J Biochem#YEAR:1989#PUBMED:2689175# AUTHORS:Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP.#TITLE:Glycolate and glyoxylate metabolism in HepG2 cells.#YEAR:2004#PUBMED:15240345# AUTHORS:Taylor SW, Fahy E, Zhang B, Glenn GM, Warnock DE, Wiley S, Murphy AN, Gaucher SP, Capaldi RA, Gibson BW, Ghosh SS#TITLE:Characterization of the human heart mitochondrial proteome#JOURNAL:Nat Biotechnol#YEAR:2003#PUBMED:1252411# GENE ASSOCIATION: (9380.1)
Abbreviation: R_HKt Synonyms: _0 EC Number: 3.6.3.10 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + k[e] --> adp[c] + h[e] + k[c] + pi[c] Confidence Level: 4 TV, RS (5/16/2005) This reaction are catalyzed by two classes of ATPase pumps (P-and ABC types) genes: LOCUS:_496#TRANSCRIPT:1#ABBREVIATION:ATP4B#ECNUMBERS:3.6.1.36# LOCUS:_495#TRANSCRIPT:1#ABBREVIATION:ATP4A#ECNUMBERS:3.6.1.36# proteins: NAME:ATPase, H+/K+ exchanging (gastric)#ABBREVIATION:Atp4ab# citations: AUTHORS:Maeda M, Oshiman K, Tamura S, Futai M.#TITLE:Human gastric (H+ + K+)-ATPase gene. Similarity to (Na+ + K+)-ATPase genes in exon/intron organization but difference in control region. #JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2160952# AUTHORS:Callaghan JM, Tan SS, Khan MA, Curran KA, Campbell WG, Smolka AJ, Toh BH, Gleeson PA, Wingo CS, Cain BD, et al.#TITLE:Renal expression of the gene encoding the gastric H(+)-K(+)-ATPase beta-subunit.#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7900835# GENE ASSOCIATION: (495.1 and 496.1)
H+/K+ gastric/non-gastric P-ATPase and ABC ATPase
Abbreviation: R_CGMPt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _35cgmp[c] + atp[c] + h2o[c] --> 35cgmp[e] + adp[c] + h[c] + pi[c] Confidence Level: 4 IT genes: LOCUS:_10257#TRANSCRIPT:1#ABBREVIATION:ABCC4# LOCUS:_10057#TRANSCRIPT:1#ABBREVIATION:ABCC5# LOCUS:_10057#TRANSCRIPT:2#ABBREVIATION:ABCC5# proteins: NAME:ATP-binding cassette, sub-family C (CFTR/MRP), member 4 #ABBREVIATION:Abcc4.1-e# NAME:ATP-binding cassette, sub-family C (CFTR/MRP), member 5 #ABBREVIATION:Abcc5.1-e# NAME:ATP-binding cassette, sub-family C (CFTR/MRP), member 5 #ABBREVIATION:Abcc5.2-e# citations: AUTHORS:Chen ZS, Lee K, Kruh GD.#TITLE:Transport of cyclic nucleotides and estradiol 17-beta-D-glucuronide by multidrug resistance protein 4. Resistance to 6-mercaptopurine and 6-thioguanine.#JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11447229# AUTHORS:Reid G, Wielinga P, Zelcer N, van der Heijden I, Kuil A, de Haas M, Wijnholds J, Borst P. #TITLE:The human multidrug resistance protein MRP4 functions as a prostaglandin efflux transporter and is inhibited by nonsteroidal antiinflammatory drugs.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2003#PUBMED:12835412# AUTHORS:Dazert P, Meissner K, Vogelgesang S, Heydrich B, Eckel L, Bohm M, Warzok R, Kerb R, Brinkmann U, Schaeffeler E, Schwab M, Cascorbi I, Jedlitschky G, Kroemer HK. #TITLE:Expression and localization of the multidrug resistance protein 5 (MRP5/ABCC5), a cellular export pump for cyclic nucleotides, in human heart.#JOURNAL:Am J Pathol. #YEAR:2003#PUBMED:14507663# AUTHORS:Meyer Zu Schwabedissen HE, Grube M, Heydrich B, Linnemann K, Fusch C, Kroemer HK, Jedlitschky G. #TITLE:Expression, localization, and function of MRP5 (ABCC5), a transporter for cyclic nucleotides, in human placenta and cultured human trophoblasts: effects of gestational age and cellular differentiation. #JOURNAL:Am J Pathol.#YEAR:2005#PUBMED:15631998# AUTHORS:Wielinga PR, van der Heijden I, Reid G, Beijnen JH, Wijnholds J, Borst P. #TITLE:Characterization of the MRP4- and MRP5-mediated transport of cyclic nucleotides from intact cells. #JOURNAL: J Biol Chem. #YEAR:2003#PUBMED:12637526# GENE ASSOCIATION: (10257.1) or (10057.2) or (10057.1)
Abbreviation: R_CYTK4n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : dcmp + gtp <==> dcdp + gdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
((N-acetyl-D-glucosaminyl)5-(alpha-D-mannosyl)2-beta-D-mannosyl-diacetylchitobiosyl)-L-asparagine (protein)_C74H122N7O50X
reducing GlcNAc removed form of n2m2nmasn (w/o peptide)_C50H84N4O36
n2m2nmasn (w/o peptide linkage)_C58H97N5O41
Abbreviation: R_ENGASE2ly Synonyms: _15 EC Number: 3.2.1.96 SUBSYSTEM: N-Glycan Degradation Equation: [l] : h2o + n2m2nm --> acgam + n2m2nmn Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
endo-beta-N-acetylglucosaminidase, lysosomal
sialyl (1,3) sialyl (2,6) galactosylgloboside (homo sapiens)_C72H121N4O43FULLRCO
heparan sulfate proteoglycan_C79H113N5O101S12X
Abbreviation: R_5HOXINDACTO2OX Synonyms: _0 EC Number: 1.2.3.1 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 5hoxindact + h2o + o2 --> 5hoxindoa + h + h2o2 Confidence Level: 2 SAB questionable--balanced, but h2o2 production is based on an obscure comment in entrez genes: LOCUS:_316#TRANSCRIPT:1#ABBREVIATION:AOX1# proteins: NAME:aldehyde oxidase 1#ABBREVIATION:Aox1.1# citations: AUTHORS:Keung WM#TITLE:Biogenic aldehyde(s) derived from the action of monoamine oxidase may mediate the antidipsotropic effect of daidzin#JOURNAL:Chem Biol Interact#YEAR:2001#PUBMED:11306106# GENE ASSOCIATION: (316.1)
_5-Hydroxyindoleacetaldehyde:oxygen oxidoreductase
tetracosahexaenoyl coenzyme A_C45H66N7O17P3S
Abbreviation: R_TAURtcx Synonyms: _0 SUBSYSTEM: Taurine and hypotaurine metabolism Equation: na1[c] + taur[c] <==> na1[x] + taur[x] Confidence Level: 1 SAB genes: LOCUS:_6533#TRANSCRIPT:1#ABBREVIATION:SLC6A6# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, taurine), member 6#ABBREVIATION:Slc6a6.1# citations: AUTHORS:Ramamoorthy S, Leibach FH, Mahesh VB, Han H, Yang-Feng T, Blakely RD, Ganapathy V#TITLE:Functional characterization and chromosomal localization of a cloned taurine transporter from human placenta#JOURNAL:Biochem J#YEAR:1994#PUBMED:8010975# GENE ASSOCIATION: (6533.1)
taurine transport (sodium symport) (cytosol to peroxisome)
dihomo-gamma-linolenic acid (n-6)_C20H33O2
Abbreviation: R_EX_dlnlcg_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dlnlcg <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
dihomo-gamma-linolenic acid (n-6) exchange
Type IIIA glycolipid_C78H135N4O45FULLRCO
methylmalonate-semialdehyde dehydrogenase
Abbreviation: R_MMSAD1m Synonyms: _0 EC Number: 1.2.1.27 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 2mop + coa + nad --> co2 + nadh + ppcoa Confidence Level: 4 TV Also converts propanal to propanoyl-CoA genes: LOCUS:_4329#TRANSCRIPT:1#ABBREVIATION:ALDH6A1#ECNUMBERS:1.2.1.27# proteins: NAME:Aldehyde dehydrogenase 6 family, member A1, mitochondrial#ABBREVIATION:Aldh6a1.1-m# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Kedishvili NY, Popov KM, Rougraff PM, Zhao Y, Crabb DW, Harris RA.#TITLE:CoA-dependent methylmalonate-semialdehyde dehydrogenase, a unique member of the aldehyde dehydrogenase superfamily. cDNA cloning, evolutionary relationships, and tissue distribution. #YEAR:1992#PUBMED:1527093# AUTHORS:Chambliss,K.L. , Gray,R.G. , Rylance,G. , Pollitt,R.J. , Gibson,K.M. #TITLE:Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency.#YEAR:2000#PUBMED:10947204# GENE ASSOCIATION: (4329.1)
Abbreviation: R_EX_fucacgalfucgalacglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucacgalfucgalacglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(Gal)2 (GalNAc)1 (Glc)1 (GlcNAc)1 (LFuc)2 (Cer)1 exchange
triacylglycerol (homo sapiens)_C3H5FULLRCO2FULLR2CO2FULLR3CO2
Abbreviation: R_EX_dad_DASH_2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dad-2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Bilirubin beta-diglucuronide_C45H50N4O18
Abbreviation: R_NOS2 Synonyms: _0 EC Number: 1.14.13.39 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : nadph + (2) nwharg + (2) o2 --> (2) citr-L + h + (2) h2o + nadp + (2) no Confidence Level: 4 SAB genes: LOCUS:_4842#TRANSCRIPT:1#ABBREVIATION:NOS1#ECNUMBERS:1.14.13.39# LOCUS:_4843#TRANSCRIPT:1#ABBREVIATION:NOS2A#ECNUMBERS:1.14.13.39# LOCUS:_4846#TRANSCRIPT:1#ABBREVIATION:NOS3#ECNUMBERS:1.14.13.39# LOCUS:_4845#TRANSCRIPT:1#ABBREVIATION:NOS2C# LOCUS:_4844#TRANSCRIPT:1#ABBREVIATION:NOS2B# LOCUS:_4843#TRANSCRIPT:2#ABBREVIATION:NOS2A#ECNUMBERS:1.14.13.39# proteins: NAME:nitric oxide synthase 1 (neuronal)#ABBREVIATION:Nos1.1# NAME:nitric oxide synthase 2A (inducible, hepatocytes)#ABBREVIATION:Nos2a.1# NAME:nitric oxide synthase 2A (inducible, hepatocytes)#ABBREVIATION:Nos2a.2# NAME:nitric oxide synthase 2B#ABBREVIATION:Nos2b.1# NAME:nitric oxide synthase 2C#ABBREVIATION:Nos2c.1# NAME:nitric oxide synthase 3 (endothelial cell)#ABBREVIATION:Nos3.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Brenman JE, Chao DS, Xia H, Aldape K, Bredt DS#TITLE:Nitric oxide synthase complexed with dystrophin and absent from skeletal muscle sarcolemma in Duchenne muscular dystrophy#JOURNAL:Cell#YEAR:1995#PUBMED:7545544# AUTHORS:Geoffrey M. Cooper#TITLE:The Cell A Molecular Approach#YEAR:2000# AUTHORS:Bloch KD, Wolfram JR, Brown DM, Roberts JD Jr, Zapol DG, Lepore JJ, Filippov G, Thomas JE, Jacob HJ, Bloch DB. #TITLE:Three members of the nitric oxide synthase II gene family (NOS2A, NOS2B, and NOS2C) colocalize to human chromosome 17. #JOURNAL:Genomics#YEAR:1995#PUBMED:7558036# AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# GENE ASSOCIATION: (4846.1) or (4844.1) or (4842.1) or (4843.2) or (4843.1) or (4845.1)
disialyl galactosylgloboside (homo sapiens)_C72H121N4O43FULLRCO
Abbreviation: R_ST8SIA11 Synonyms: _13 EC Number: 2.4.99.8 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : acngalgbside_hs + cmpacna --> acnacngalgbside_hs + cmp + h Confidence Level: 4 NJ genes: LOCUS:_6489#TRANSCRIPT:1#ABBREVIATION:SIAT8A#ECNUMBERS:2.4.99.8# proteins: NAME:ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 1#ABBREVIATION:St8sia1.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Takashima S, Tsuji S, Tsujimoto#TITLE:Characterization of second type of human beta-galactoside alpha2,6-sialyltransferase which sialates gal-beta1,4GlcNAc structures on oligosaccharides preferentially#JOURNAL:Journal of Biological Chemistry#YEAR:2002# AUTHORS:Kim YJ, Kim KS, Do S, Kim CH, Kim SK, Lee YC.#TITLE:Molecular cloning and expression of human alpha2,8-sialyltransferase (hST8Sia V).#JOURNAL:Biochem Biophys Res Commun.#YEAR:1997#PUBMED:9199191# GENE ASSOCIATION: (6489.1)
sialyl galactosylgloboside (homo sapiens)_C61H105N3O35FULLRCO
Flavin adenine dinucleotide oxidized_C27H31N9O15P2
R group 4 Coenzyme A homo sapiens_XCO2C21H31N7O15P3S
Hexadecenoyl-CoA (n-C16:1CoA)_C37H60N7O17P3S
Flavin adenine dinucleotide reduced_C27H33N9O15P2
Abbreviation: R_ARTFR41 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + hdcoa[c] --> R4coa_hs[c] + fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ADK3 Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [c] : amp + gtp <==> adp + gdp Confidence Level: 4 JLR genes: LOCUS:_26289#TRANSCRIPT:1#ABBREVIATION:AK5# LOCUS:_26289#TRANSCRIPT:2#ABBREVIATION:AK5# proteins: NAME:adenylate kinase 5 #ABBREVIATION:Ak5.1# NAME:adenylate kinase 5 #ABBREVIATION:Ak5.2# citations: AUTHORS:Noma T, Song S, Yoon YS, Tanaka S, Nakazawa A. #TITLE:cDNA cloning and tissue-specific expression of the gene encoding human adenylate kinase isozyme 2. #JOURNAL:Biochim Biophys Acta. #YEAR:1998#PUBMED:9434148# GENE ASSOCIATION: (26289.1) or (26289.2)
linoleic acid (all cis C18:2) n-6_C18H31O2
Abbreviation: R_EX_Rtotal3_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : Rtotal3 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
nucleoside-diphosphatase (UDP), endoplasmic reticulum
Abbreviation: R_NDP7er Synonyms: _0 EC Number: 3.6.1.6 SUBSYSTEM: Nucleotides Equation: [r] : h2o + udp --> h + pi + ump Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Trombetta ES, Helenius A.#TITLE:Glycoprotein reglucosylation and nucleotide sugar utilization in the secretory pathway: identification of a nucleoside diphosphatase in the endoplasmic reticulum.#JOURNAL:EMBO J#YEAR:1999#PUBMED:10369669# GENE ASSOCIATION:
Abbreviation: R_DNDPt49m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[m] + dttp[c] --> dadp[c] + dttp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_NTD9l Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleic acid degradation Equation: [l] : gmp + h2o --> gsn + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:R.L. Pisoni#TITLE:Lysosomal Nucleic Acid and Phosphate Metabolism and Related Metabolic Reactions, Chapter 9#YEAR:1996# GENE ASSOCIATION: (53.1)
Abbreviation: R_FE3R2e Synonyms: _0 SUBSYSTEM: Heme Biosynthesis Equation: [e] : ascb-L + (2) fe3 --> dhdascb + (2) fe2 + (2) h Confidence Level: 4 stoichiometry unknown; inferred from EC1.16.1.7 NCD genes: LOCUS:_79901#TRANSCRIPT:1#ABBREVIATION:CYBRD1# proteins: NAME:cytochrome b reductase 1#ABBREVIATION:Cybrd1.1-e# citations: AUTHORS:Chung J, Wessling-Resnick M#TITLE:Molecular mechanisms and regulation of iron transport.#JOURNAL:Crit Rev Clin Lab Sci#YEAR:2003#PUBMED:12755454# AUTHORS:McKie AT, Barrow D, Latunde-Dada GO, Rolfs A, Sager G, Mudaly E, Mudaly M, Richardson C, Barlow D, Bomford A, Peters TJ, Raja KB, Shirali S, Hediger MA, Farzaneh F, Simpson RJ.#TITLE:An iron-regulated ferric reductase associated with the absorption of dietary iron#JOURNAL:Science#YEAR:2001#PUBMED:11230685# GENE ASSOCIATION: (79901.1)
Abbreviation: R_UGLCNACtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: uacgam[c] + ump[g] <==> uacgam[g] + ump[c] Confidence Level: 4 NCD genes: LOCUS:_11046#TRANSCRIPT:1#ABBREVIATION:SLC35D2# proteins: NAME:solute carrier family 35, member D2#ABBREVIATION:Slc35d2.1-g# citations: AUTHORS:Suda T, Kamiyama S, Suzuki M, Kikuchi N, Nakayama K, Narimatsu H, Jigami Y, Aoki T, Nishihara S#TITLE:Molecular cloning and characterization of a human multisubstrate specific nucleotide-sugar transporter homologous to Drosophila fringe connection#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15082721# AUTHORS:Ishida N, Kuba T, Aoki K, Miyatake S, Kawakita M, Sanai Y#TITLE:Identification and characterization of human Golgi nucleotide sugar transporter SLC35D2, a novel member of the SLC35 nucleotide sugar transporter family#JOURNAL:Genomics#YEAR:2005#PUBMED:15607426# GENE ASSOCIATION: (11046.1)
UDP-GlcNAc Golgi transport via CMP antiport
Abbreviation: R_AMANK Synonyms: _0 EC Number: 2.7.1.60 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acmana + atp --> acmanap + adp + h Confidence Level: 4 JLR - Kegg R02705 genes: LOCUS:_55577#TRANSCRIPT:1#ABBREVIATION:NAGK#ECNUMBERS:2.7.1.59# LOCUS:_10020#TRANSCRIPT:1#ABBREVIATION:GNE#ECNUMBERS:5.1.3.14,2.7.1.60# proteins: NAME:N-acetylglucosamine kinase#ABBREVIATION:Nagk.1# NAME:glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase#ABBREVIATION:Gne.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Seppala R, Lehto VP, Gahl WA#TITLE:Mutations in the human UDP-N-acetylglucosamine 2-epimerase gene define the disease sialuria and the allosteric site of the enzyme#JOURNAL:Am J Hum Genet#YEAR:1999#PUBMED:10330343# AUTHORS:Lucka L, Krause M, Danker K, Reutter W, Horstkorte R#TITLE:Primary structure and expression analysis of human UDP-N-acetyl-glucosamine-2-epimerase/N-acetylmannosamine kinase, the bifunctional enzyme in neuraminic acid biosynthesis#JOURNAL:FEBS Lett#YEAR:1999#PUBMED:10431835# AUTHORS:Hinderlich S, Berger M, Keppler OT, Pawlita M, Reutter W#TITLE:Biosynthesis of N-acetylneuraminic acid in cells lacking UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase#JOURNAL:Biol Chem#YEAR:2001#PUBMED:11308027# GENE ASSOCIATION: (10020.1) or (55577.1)
N-Acetyl-D-mannosamine 6-phosphate_C8H14NO9P
Abbreviation: R_STRDNCCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: strdnccrn[c] --> strdnccrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
_1-alkyl 2-acteylglycerol 3-phosphocholine (homo sapiens)_C11H23O4NPFULLRCO2
Abbreviation: R_EX_adn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : adn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAOXC183806m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (5) coa + (5) h2o + lnlncgcoa + (5) nad + (2) o2 --> (5) accoa + (5) h + (2) h2o2 + (5) nadh + occoa Confidence Level: 4 _5 rounds of beta ox, 3 degrees loss of saturation -> only 2 fad oxidized to fadh2 - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) peroxisomal (very long chain FA) c18:3,n-6 -> c8:0, 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_EX_gluala_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gluala <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Holocarboxylase (biotin covalent bound to Lys residue of apoC)_C16H27N4O3SX
Abbreviation: R_BTNPLm Synonyms: _0 SUBSYSTEM: Biotin Metabolism Equation: [m] : apoC-Lys + btamp --> amp + apoC-Lys_btn + h Confidence Level: 4 IT for human biotin metabolism genes: LOCUS:_3141#TRANSCRIPT:1#ABBREVIATION:HLCS#ECNUMBERS:6.3.4.10# proteins: NAME:holocarboxylase synthetase (biotin-[proprionyl-Coenzyme A-carboxylase (ATP-hydrolysing)] ligase) #ABBREVIATION:Hcls.1-m# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (3141.1)
holocarboxylase synthestase (biotin protein ligase), mitochondrial
Abbreviation: R_EX_cit_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cit <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_fe2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fe2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(Gal)3 (Glc)1 (GlcNAc)1 (Cer)1_C55H95N3O30FULLRCO
ST3 beta-galactoside alpha-2,3-sialyltransferase 3
Abbreviation: R_ST3GAL31g Synonyms: _9 EC Number: 2.4.99.6 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : cmpacna + galacglcgalgluside_hs --> acngalacglcgalgluside_hs + cmp + h Confidence Level: 4 NJ genes: LOCUS:_6487#TRANSCRIPT:1#ABBREVIATION:ST3GAL3#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:10#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:2#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:11#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:9#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:4#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:3#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:6#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:5#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:8#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# LOCUS:_6487#TRANSCRIPT:7#ABBREVIATION:SIAT6#ECNUMBERS:2.4.99.6# proteins: NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.1-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.10-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.11-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.2-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.3-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.4-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.5-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.6-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.7-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.8-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 3, Golgi apparatus#ABBREVIATION:St3gal3.9-g# citations: AUTHORS:Kitagawa H, Paulson JC#TITLE:Cloning and expression of human Gal beta 1,3(4)GlcNAc alpha 2,3-sialyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1993#PUBMED:8333853# GENE ASSOCIATION: (6487.6) or (6487.11) or (6487.5) or (6487.7) or (6487.9) or (6487.1) or (6487.8) or (6487.4) or (6487.3) or (6487.10) or (6487.2)
Abbreviation: R_EX_dcyt_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dcyt <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ADPRDP Synonyms: _0 EC Number: 3.6.1.13 SUBSYSTEM: Nucleotides Equation: [c] : adprib + h2o --> amp + (2) h + r5p Confidence Level: 4 genes: LOCUS:_11164#TRANSCRIPT:1#ABBREVIATION:NUDT5# proteins: NAME:nudix (nucleoside diphosphate linked moiety X)-type motif 5 #ABBREVIATION:NudT5.1# citations: AUTHORS:Yang H, Slupska MM, Wei YF, Tai JH, Luther WM, Xia YR, Shih DM, Chiang JH, Baikalov C, Fitz-Gibbon S, Phan IT, Conrad A, Miller JH.#TITLE:Cloning and characterization of a new member of the Nudix hydrolases from human and mouse.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10722730# AUTHORS:Ishibashi T, Hayakawa H, Sekiguchi M.#TITLE:A novel mechanism for preventing mutations caused by oxidation of guanine nucleotides.#JOURNAL:EMBO Rep.#YEAR:2003#PUBMED:12717453# AUTHORS:Gasmi L, Cartwright JL, McLennan AG. #TITLE:Cloning, expression and characterization of YSA1H, a human adenosine 5'-diphosphosugar pyrophosphatase possessing a MutT motif.#JOURNAL:Biochem J.#YEAR:1999#PUBMED:10567213# GENE ASSOCIATION: (11164.1)
Abbreviation: R_ALDD20xm Synonyms: _0 SUBSYSTEM: Tryptophan metabolism Equation: [m] : h2o + id3acald + nad --> (2) h + ind3ac + nadh Confidence Level: 1 NCD genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: none GENE ASSOCIATION: (219.1) or (8659.1) or (217.1) or (8659.2)
aldehyde dehydrogenase (indole-3-acetaldehyde, NAD), mitochondrial
Abbreviation: R_B3GNT39g Synonyms: _9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gluside_hs + udpgal --> galgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10331.1)
UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus
galactosyl glucosyl ceramide_C30H56NO12FULLRCO
Abbreviation: R_ELAIDt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: elaid[e] <==> elaid[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_DNDPt36m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[m] + dgdp[c] --> dcdp[c] + dgdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_BILIRUBt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: bilirub[e] + hco3[c] <==> bilirub[c] + hco3[e] Confidence Level: 4 NJ genes: LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# citations: AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# AUTHORS:Abe T, Kakyo M, Tokui T, Nakagomi R, Nishio T, Nakai D, Nomura H, Unno M, Suzuki M, Naitoh T, Matsuno S, Yawo H.#TITLE:Identification of a novel gene family encoding human liver-specific organic anion transporter LST-1.#JOURNAL:J Biol Chem.#YEAR:1999#PUBMED:10358072# GENE ASSOCIATION: (10599.1)
bilirubin transport via bicarbonate countertransport
Abbreviation: R_HPDCACRNCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + hpdcacrn --> crn + hpdcacoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# AUTHORS:Fraser F, Padovese R, Zammit VA.#TITLE:Distinct kinetics of carnitine palmitoyltransferase i in contact sites and outer membranes of rat liver mitochondria.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11274214# GENE ASSOCIATION: (1376.1)
heptadecanoate transport into the mitochondria
Abbreviation: R_CYTK5n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : cmp + gtp <==> cdp + gdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# GENE ASSOCIATION: (51727.1)
Abbreviation: R_SELCYSLY Synonyms: _2 EC Number: 4.4.1.16 SUBSYSTEM: Selenoamino acid metabolism Equation: [c] : dtt + selcys --> ala-L + dttOX + h + seln Confidence Level: 1 SAB genes: LOCUS:_51540#TRANSCRIPT:1#ABBREVIATION:SCLY# proteins: NAME:selenocysteine lyase#ABBREVIATION:Scly.1# citations: AUTHORS:Mihara H, Kurihara T, Watanabe T, Yoshimura T, Esaki N. #TITLE:cDNA cloning, purification, and characterization of mouse liver selenocysteine lyase. Candidate for selenium delivery protein in selenoprotein synthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10692412# AUTHORS:Katsuhiko Nakamuro, Tomofumi Okuno, and Tatsuya Hasegawa#TITLE:Metabolism of Selenoamino Acids and Contribution of Selenium Methylation to Their Toxicity#JOURNAL:Journal of Health Science#YEAR:2000#PUBMED:?# GENE ASSOCIATION: (51540.1)
Abbreviation: R_SELCYSTS Synonyms: _0 EC Number: 4.2.1.22 SUBSYSTEM: Selenoamino acid metabolism Equation: [c] : selhcys + ser-L --> h2o + selcyst Confidence Level: 4 NCD SAB genes: LOCUS:_875#TRANSCRIPT:1#ABBREVIATION:CBS#ECNUMBERS:4.2.1.22# proteins: NAME:cystathionine-beta-synthase #ABBREVIATION:Cbs.1# citations: AUTHORS:Esaki N, Nakamura T, Tanaka H, Soda K#TITLE:Selenocysteine lyase, a novel enzyme that specifically acts on selenocysteine. Mammalian distribution and purification and properties of pig liver enzyme#JOURNAL:J Biol Chem#YEAR:1982#PUBMED:6461656# GENE ASSOCIATION: (875.1)
sulfatide galactocerebroside (homo sapiens)_C24H45NO10SFULLRCO
Abbreviation: R_CITRtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: citr-L[m] <==> citr-L[c] Confidence Level: 4 TDV genes: LOCUS:_83884#TRANSCRIPT:1#ABBREVIATION:SLC25A2# LOCUS:_10166#TRANSCRIPT:1#ABBREVIATION:SLC25A15# proteins: NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 2#ABBREVIATION:Slc25a2.1-m# NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15#ABBREVIATION:Slc25a15.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (83884.1) or (10166.1)
citrulline mitochondrial transport via proton antiport
Abbreviation: R_CSNATr Synonyms: _0 EC Number: 2.3.1.7 SUBSYSTEM: Carnitine shuttle Equation: [c] : accoa + crn <==> acrn + coa Confidence Level: 4 IF genes: none proteins: none citations: AUTHORS:Corti O, Finocchiaro G, Rossi E, Zuffardi O, DiDonato S.#TITLE:Molecular cloning of cDNAs encoding human carnitine acetyltransferase and mapping of the corresponding gene to chromosome 9q34.1.#JOURNAL:Genomics#YEAR:1994#PUBMED:7829107# AUTHORS:Ramsay, Gandour, van der Leij#TITLE:Molecular enzymology of carnitine transfer and transport#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11257506# GENE ASSOCIATION:
Abbreviation: R_3HXKYNDCL Synonyms: _0 EC Number: 4.1.1 SUBSYSTEM: Tryptophan metabolism Equation: [c] : h + hLkynr --> 3hxkynam + co2 Confidence Level: 1 SAB made up name genes: LOCUS:_1644#TRANSCRIPT:1#ABBREVIATION:DDC#ECNUMBERS:4.1.1.28# proteins: NAME:aromatic-L-amino-acid decarboxylase#ABBREVIATION:Ddc.1# citations: none GENE ASSOCIATION: (1644.1)
Abbreviation: R_EX_ura_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ura <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PI45P3Kn Synonyms: _0 EC Number: 2.7.1.153 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + pail45p_hs --> adp + h + pail345p_hs Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
phosphatidylinositol 4,5-bisphosphate 3-kinase, nuclear
_1-Phosphatidyl-1D-myo-inositol 5-phosphate (Homo sapiens)_C9H15O12P2FULLRCO2FULLR2CO2
Abbreviation: R_PI45P4P Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail45p_hs --> pail5p_hs + pi Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Tolias KF, Cantley LC#TITLE:Pathways for phosphoinositide synthesis#JOURNAL:Chem Phys Lipids#YEAR:1999#PUBMED:10358929# GENE ASSOCIATION:
phosphatidylinositol-4,5-bisphosphate 4-phosphatase
Abbreviation: R_ARACHDCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: arachdcoa[c] <==> arachdcoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_ptdca_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ptdca <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CYSAMO Synonyms: _0 SUBSYSTEM: Taurine and hypotaurine metabolism Equation: [c] : cysam + o2 --> (2) h + hyptaur Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# GENE ASSOCIATION:
Abbreviation: R_HYXNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hxan[e] <==> hxan[c] Confidence Level: 4 tv genes: LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Yao SY, Ng AM, Vickers MF, Sundaram M, Cass CE, Baldwin SA, Young JD.#TITLE:Functional and molecular characterization of nucleobase transport by recombinant human and rat equilibrative nucleoside transporters 1 and 2.#JOURNAL:J Biol Chem. #YEAR:2002#PUBMED:12006583# GENE ASSOCIATION: (3177.1)
_3-sulfino-alanine transaminase (irreversible), mitochondrial
Abbreviation: R_3SALATAim Synonyms: _0 SUBSYSTEM: Cysteine Metabolism Equation: [m] : 3sala + akg + h --> 3snpyr + glu-L Confidence Level: 2 MM genes: LOCUS:_2806#TRANSCRIPT:1#ABBREVIATION:GOT2#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase, mitochondrial#ABBREVIATION:Got2.1-m# citations: none GENE ASSOCIATION: (2806.1)
Abbreviation: R_DPCOAK Synonyms: _0 EC Number: 2.7.1.24 SUBSYSTEM: CoA Biosynthesis Equation: [c] : atp + dpcoa --> adp + coa + h Confidence Level: 4 genes: LOCUS:_80347#TRANSCRIPT:1#ABBREVIATION:KDTB/COAE#ECNUMBERS:2.7.7.3/2.7.1.24# proteins: NAME:dephospho-CoA kinase#ABBREVIATION:CoaE.1# citations: AUTHORS:Daugherty M, Polanuyer B, Farrell M, Scholle M, Lykidis A, de Crecy-Lagard V, Osterman A. #TITLE:Complete reconstitution of the human coenzyme A biosynthetic pathway via comparative genomics.#JOURNAL:J Biol Chem.#YEAR:2002#PUBMED:11923312# AUTHORS:Aghajanian S, Worrall DM.#TITLE:Identification and characterization of the gene encoding the human phosphopantetheine adenylyltransferase and dephospho-CoA kinase bifunctional enzyme (CoA synthase).#JOURNAL:Biochem J.#YEAR:2002#PUBMED:11994049# GENE ASSOCIATION: (80347.1)
Abbreviation: R_G12MT1_U Synonyms: _0 EC Number: 2.4.1.131 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : gdpmann + (0.1) m2mpdol_U --> gdp + h + (0.1) m3mpdol_U Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Glycolipid 1,2-alpha-D-mannosyltransferase (uterus)
(alpha-D-mannosyl)3-beta-D-mannosyl-diacetylchitodiphosphodolichol, human uterine homolog_C1425H2340N20O370P20
(alpha-D-mannosyl)2-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1365H2240N20O320P20
Abbreviation: R_FACOAL170 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + hpdca <==> amp + hpdcacoa + ppi Confidence Level: 4 AR genes: LOCUS:_2182#TRANSCRIPT:1#ABBREVIATION:ACSL4# LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# LOCUS:_2181#TRANSCRIPT:1#ABBREVIATION:ACSL3# LOCUS:_2181#TRANSCRIPT:2#ABBREVIATION:ACSL3# LOCUS:_22305#TRANSCRIPT:1#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# LOCUS:_22305#TRANSCRIPT:2#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.1# NAME:acyl-CoA synthetase long-chain family member 4#ABBREVIATION:Acsl4.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.2# NAME:acyl-CoA synthetase long-chain family member 6#ABBREVIATION:Acsl6.1# NAME:acyl-CoA synthetase long-chain family member 6 #ABBREVIATION:Acsl6.2# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (22305.2) or (2182.1) or (2180.1) or (2181.2) or (2181.1) or (22305.1)
Abbreviation: R_GTHDH Synonyms: _0 EC Number: 1.8.5.1 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : dhdascb + (2) gthrd --> ascb-L + gthox Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# GENE ASSOCIATION:
Glutathione dehydrogenase (dehydroascorbate reductase)
Abbreviation: R_24NPHte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _24nph[e] <==> 24nph[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate E (GalNAc4,6diS-GlcA) proteoglycan_C45H63N2O54S6X
Abbreviation: R_FACOAL241 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + nrvnc <==> amp + nrvnccoa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_ORETNtn2 Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: _13-cis-oretn[c] <==> 13-cis-oretn[n] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
_13-cis-4-oxo-retinoic acid transport, Nuclear
protein-linked serine residue (glycosaminoglycan attachment site)_XH
Abbreviation: R_CYTK14 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : dcmp + utp <==> dcdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
heparan sulfate, degradation product 1_C79H115N5O99S11
Abbreviation: R_TKT1 Synonyms: _0 EC Number: 2.2.1.1 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : r5p + xu5p-D <==> g3p + s7p Confidence Level: 0 SMP Two reactions for transketolase: TKT1 and TKT2 genes: LOCUS:_7086#TRANSCRIPT:1#ABBREVIATION:TKT#ECNUMBERS:2.2.1.1# LOCUS:_8277#TRANSCRIPT:1#ABBREVIATION:TKTL1# LOCUS:_84076#TRANSCRIPT:1#ABBREVIATION:DKFZP434L1717# proteins: NAME:transketolase#ABBREVIATION:Tkt.1# NAME:transketolase#ABBREVIATION:Tktl1.1# NAME:similar to transketolase#ABBREVIATION:Dkfzp434l1717.1# citations: none GENE ASSOCIATION: (7086.1) or (84076.1) or (8277.1)
Abbreviation: R_H2O2tly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: h2o2[c] <==> h2o2[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Ohno Y, Gallin JI. #TITLE:Diffusion of extracellular hydrogen peroxide into intracellular compartments of human neutrophils. Studies utilizing the inactivation of myeloperoxidase by hydrogen peroxide and azide.#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2989289# GENE ASSOCIATION:
hydrogen peroxide lysosomal transport via diffusion
fatty acyl-CoA desaturase (n-C18:2CoA -> n-C18:3CoA)
Abbreviation: R_DESAT18_10 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + lnlncacoa + nadh + o2 --> (2) h2o + nad + strdnccoa Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_9415#TRANSCRIPT:1#ABBREVIATION:FADS2#ECNUMBERS:1.14.99.25# proteins: NAME:fatty acid desaturase 2#ABBREVIATION:Fads2.1# citations: AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Cho HP, Nakamura MT, Clarke SD#TITLE:Cloning, expression, and nutritional regulation of the mammalian delta-6 desaturase#JOURNAL:The Journal of Biological Chemistry#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION: (9415.1)
Abbreviation: R_ACP1_LPAREN_FMN_RPAREN_ Synonyms: _0 EC Number: 3.1.3.2 SUBSYSTEM: Riboflavin Metabolism Equation: [c] : fmn + h2o --> pi + ribflv Confidence Level: 2 genes: LOCUS:_52#TRANSCRIPT:1#ABBREVIATION:ACP1#ECNUMBERS:3.1.3.2# LOCUS:_52#TRANSCRIPT:2#ABBREVIATION:ACP1#ECNUMBERS:3.1.3.2# LOCUS:_52#TRANSCRIPT:3#ABBREVIATION:ACP1#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 1, soluble #ABBREVIATION:Acp1.1# NAME:acid phosphatase 1, soluble #ABBREVIATION:Acp1.2# NAME:acid phosphatase 1, soluble #ABBREVIATION:Acp1.3# citations: none GENE ASSOCIATION: (52.1) or (52.3) or (52.2)
Abbreviation: R_EX_n2m2nmasn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : n2m2nmasn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
N-Acetyl-beta-D-glucosaminyl-1,2-alpha-D-mannosyl-1,3-(N-acetyl-beta-D-glucosaminyl-1,2-alpha-D-mannosyl-1,6)-(N-acetyl-beta-D-glucosaminyl-1,4)-beta-D-mannosyl-1,4-N-acetyl-beta-D-glucosaminyl-R exchange
Abbreviation: R_EX_5dhf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 5dhf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PDX5PO Synonyms: _0 EC Number: 1.4.3.5 SUBSYSTEM: Vitamin B6 Metabolism Equation: [c] : o2 + pdx5p <==> h2o2 + pydx5p Confidence Level: 4 NCD genes: LOCUS:_55163#TRANSCRIPT:1#ABBREVIATION:PNPO#ECNUMBERS:1.4.3.5# proteins: NAME:Pyridoxine-5'-phosphate oxidase #ABBREVIATION:PnpO.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# AUTHORS:Kang JH, Hong ML, Kim DW, Park J, Kang TC, Won MH, Baek NI, Moon BJ, Choi SY, Kwon OS.#TITLE:Genomic organization, tissue distribution and deletion mutation of human pyridoxine 5'-phosphate oxidase.#JOURNAL:Eur J Biochem.#YEAR:2004#PUBMED:15182361# AUTHORS:Mehansho H, Henderson LM.#TITLE:Transport and accumulation of pyridoxine and pyridoxal by erythrocytes.#JOURNAL:J Biol Chem. #YEAR:1980#PUBMED:J Biol Chem.# GENE ASSOCIATION: (55163.1)
Abbreviation: R_HPYRRy Synonyms: _0 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : h + hpyr + nadph --> glyc-R + nadp Confidence Level: 4 JLR genes: LOCUS:_9380#TRANSCRIPT:1#ABBREVIATION:GRHPR#ECNUMBERS:1.1.1.79# proteins: NAME:glyoxylate reductase/hydroxypyruvate reductase#ABBREVIATION:Grhpr.1# citations: AUTHORS:Van Schaftingen E, Draye JP, Van Hoof F#TITLE:Coenzyme specificity of mammalian liver D-glycerate dehydrogenase#JOURNAL:Eur J Biochem#YEAR:1989#PUBMED:2689175# AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# GENE ASSOCIATION: (9380.1)
Abbreviation: R_NAGAly Synonyms: _3 EC Number: 3.2.1.49 SUBSYSTEM: Sphingolipid Metabolism Equation: [l] : acgagbside_hs + h + udp --> gbside_hs + udpacgal Confidence Level: 4 Alpha-galactosidase B NJ genes: LOCUS:_4668#TRANSCRIPT:1#ABBREVIATION:NAGA#ECNUMBERS:3.2.1.49# proteins: NAME:N-acetylgalactosaminidase, alpha-, lysosomal#ABBREVIATION:Naga.1-l# citations: AUTHORS:Wang AM, Bishop DF, Desnick RJ.#TITLE:Human alpha-N-acetylgalactosaminidase-molecular cloning, nucleotide sequence, and expression of a full-length cDNA. Homology with human alpha-galactosidase A suggests evolution from a common ancestral gene. #JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2174888# AUTHORS:Tsuji S, Yamauchi T, Hiraiwa M, Isobe T, Okuyama T, Sakimura K, Takahashi Y, Nishizawa M, Uda Y, Miyatake T.#TITLE:Molecular cloning of a full-length cDNA for human alpha-N-acetylgalactosaminidase (alpha-galactosidase B)#JOURNAL:Biochem Biophys Res Commun#YEAR:1989#PUBMED:2551294# GENE ASSOCIATION: (4668.1)
_4,8 dimethylnonanoyl-CoA_C32H54N7O17P3S
Abbreviation: R_FAOXC11 Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : coa + dmnoncoa + h2o + nad --> accoa + dmhptcoa + h + nadh Confidence Level: 2 TV Other enzymes: 1.3.3.6, 4.2.1.74, 4.2.1.17, 1.1.1.35, 1.1.1.211 1 round of beta ox of branched chain FA from peroxisome NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Verhoeven NM, Wanders RJ, Poll-The BT, Saudubray JM, Jakobs C.#TITLE:The metabolism of phytanic acid and pristanic acid in man: a review.#JOURNAL:J Inherit Metab Dis#YEAR:1998#PUBMED:9819701# GENE ASSOCIATION: (34.1)
_2,6 dimethylheptanoyl-CoA_C30H52N7O17P3S
_2-keto-3-deoxy-D-glycero-D-galactononic acid_C9H15O9
Abbreviation: R_3DSPHR Synonyms: _0 EC Number: 1.1.1.102 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : 3dsphgn + h + nadph --> nadp + sphgn Confidence Level: 4 NCD genes: LOCUS:_2531#TRANSCRIPT:1#ABBREVIATION:FVT1# proteins: NAME:folliclular lymphoma variant translocation 1#ABBREVIATION:Fvt1.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION: (2531.1)
Abbreviation: R_FUCFUC12GAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucfuc12gal14acglcgalgluside_hs[g] <==> fucfuc12gal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
R group 3 Coenzyme A homo sapiens_XCO2C21H31N7O15P3S
Abbreviation: R_ARTFR31 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : stcoa --> (1.125) R3coa_hs Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ARTFR211 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: clpndcoa[c] + (3) fadh2[m] + (2) h[m] + (2) nadph[m] --> (1.375) R2coa_hs[c] + (3) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
R group 2 Coenzyme A homo sapiens_XCO2C21H31N7O15P3S
keratan sulfate I, degradation product 6_C210H336N15O184S11
keratan sulfate I, degradation product 5_C222H356N15O194S11
Abbreviation: R_GALASE3ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg5 --> (2) gal + ksi_deg6 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
glucosyl-(alpha-D-mannosyl)7-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine, isoform B (protein)_C70H117N2O55X
Abbreviation: R_HKYNH Synonyms: _0 EC Number: 3.7.1.3 SUBSYSTEM: Tryptophan metabolism Equation: [c] : h2o + hLkynr --> 3hanthrn + ala-L Confidence Level: 4 NCD genes: LOCUS:_8942#TRANSCRIPT:1#ABBREVIATION:KYNU#ECNUMBERS:3.7.1.3# proteins: NAME:kynureninase (L-kynurenine hydrolase)#ABBREVIATION:Kynu.1# citations: AUTHORS:Toma S, Nakamura M, Tone S, Okuno E, Kido R, Breton J, Avanzi N, Cozzi L, Speciale C, Mostardini M, Gatti S, Benatti L#TITLE:Cloning and recombinant expression of rat and human kynureninase#JOURNAL:FEBS Lett#YEAR:1997#PUBMED:9180257# GENE ASSOCIATION: (8942.1)
trimannosyl-glucosaminyl-acylphosphatidylinositol (H4)_C49H88NO29PFULLRCO2FULLR2CO2
dimannosyl-glucosaminyl-acylphosphatidylinositol (H3)_C43H78NO24PFULLRCO2FULLR2CO2
Abbreviation: R_H3MTer_L Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_L + m2gacpail_hs --> (0.1) dolp_L + h + m3gacpail_hs Confidence Level: 4 NCD genes: LOCUS:_9488#TRANSCRIPT:1#ABBREVIATION:PIGB# proteins: NAME:phosphatidylinositol glycan, class B, endoplasmic reticulum#ABBREVIATION:Pigb.1-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Takahashi M, Inoue N, Ohishi K, Maeda Y, Nakamura N, Endo Y, Fujita T, Takeda J, Kinoshita T#TITLE:PIG-B, a membrane protein of the endoplasmic reticulum with a large lumenal domain, is involved in transferring the third mannose of the GPI anchor#JOURNAL:EMBO J#YEAR:1996#PUBMED:8861954# GENE ASSOCIATION: (9488.1)
H3 mannosyltransferase, endoplasmic reticulum
_1D-myo-Inositol 1,3,4,5,6-pentakisphosphate_C6H7O21P5
Abbreviation: R_EX_crn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : crn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
delta1-piperideine-2-carboxylate reductase, perixosomal
Abbreviation: R_1PPDCRp Synonyms: _3 SUBSYSTEM: Lysine Metabolism Equation: [x] : 1pipdn2c + h + nadh --> Lpipecol + nad Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:IJlst L, de Kromme I, Oostheim W, Wanders RJ.#TITLE:Molecular cloning and expression of human L-pipecolate oxidase.#YEAR:2000#PUBMED:10772957# AUTHORS:Murthy SN, Janardanasarma MK.#TITLE:Identification of L-amino acid/L-lysine alpha-amino oxidase in mouse brain.#YEAR:1999#PUBMED:10485319# GENE ASSOCIATION:
Myristoyl-ACP (n-C14:0ACP)_C25H47N2O8PRS
Abbreviation: R_EX_cl_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cl <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_fucfucgalacglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucfucgalacglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I, degradation product 36_C62H103N4O49S1
keratan sulfate I, degradation product 35_C68H113N4O54S1
Abbreviation: R_GALASE13ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg35 --> gal + ksi_deg36 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
ABO blood group (transferase A, alpha 1-3-N-acetylgalactosaminyltransferase; transferase B, alpha 1-3-galactosyltransferase)
Abbreviation: R_ABO3g Synonyms: _11 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fuc12gal14acglcgalgluside_hs + udpgal --> galfuc12gal14acglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_28#TRANSCRIPT:1#ABBREVIATION:ABO# proteins: NAME:ABO blood group#ABBREVIATION:Abo.1# citations: AUTHORS:Lloyd KO.#TITLE:The chemistry and immunochemistry of blood group A, B, H, and Lewis antigens: past, present and future.#JOURNAL:Glycoconj J#YEAR:2000#PUBMED:11421346# AUTHORS:Yamamoto F.-I., Hakomori S.-I.;#TITLE:Sugar-nucleotide donor specificity of histo-blood group A and B transferases is based on amino acid substitutions#JOURNAL:J. Biol. Chem#YEAR:1990#PUBMED:2121736# AUTHORS:Patenaude S.I., Seto N.O.L., Borisova S.N., Szpacenko A., Marcus S.L., Palcic M.M., Evans S.V.;#TITLE:The structural basis for specificity in human ABO(H) blood group biosynthesis#JOURNAL:Nat. Struct. Biol#YEAR:2002#PUBMED:12198488# GENE ASSOCIATION: (28.1)
beta-D-Mannosyldiacetylchitobiosyldiphosphodolichol, human liver homolog_C1300H2128N20O220P20
alpha-D-mannosyl-beta-D-mannosyl-diacylchitobiosyldiphosphodolichol, human liver homolog_C1360H2228N20O270P20
Glycolipid 1,3-alpha-D-mannosyltransferase (liver)
Abbreviation: R_G13MT_L Synonyms: _0 EC Number: 2.4.1.132 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : gdpmann + (0.1) mpdol_L --> gdp + h + (0.1) m1mpdol_L Confidence Level: 0 NCD genes: LOCUS:_85365#TRANSCRIPT:1#ABBREVIATION:ALG2# LOCUS:_85365#TRANSCRIPT:2#ABBREVIATION:ALG2# proteins: NAME:alpha-1,3-mannosyltransferase#ABBREVIATION:Alg2.1# NAME:asparagine-linked glycosylation 2 homolog (yeast, alpha-1,3-mannosyltransferase) #ABBREVIATION:Alg2.2# citations: none GENE ASSOCIATION: (85365.1) or (85365.2)
formate-tetrahydrofolate ligase, mitochondrial
Abbreviation: R_FTHFLm Synonyms: _0 EC Number: 6.3.4.3 SUBSYSTEM: Folate Metabolism Equation: [m] : atp + for + thf <==> 10fthf + adp + pi Confidence Level: 4 NCD genes: LOCUS:_25902#TRANSCRIPT:1#ABBREVIATION:MTHFD1L# proteins: NAME:methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like, mitochondrial#ABBREVIATION:Mthfd1l-m# citations: AUTHORS:Prasannan P, Pike S, Peng K, Shane B, Appling DR#TITLE:Human mitochondrial C1-tetrahydrofolate synthase: gene structure, tissue distribution of the mRNA, and immunolocalization in Chinese hamster ovary calls#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12937168# GENE ASSOCIATION: (25902.1)
Abbreviation: R_ALASm Synonyms: _0 EC Number: 2.3.1.37 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : gly + h + succoa --> 5aop + co2 + coa Confidence Level: 2 NCD genes: LOCUS:_212#TRANSCRIPT:1#ABBREVIATION:ALAS2#ECNUMBERS:2.3.1.37# LOCUS:_211#TRANSCRIPT:1#ABBREVIATION:ALAS1#ECNUMBERS:2.3.1.37# LOCUS:_23464#TRANSCRIPT:1#ABBREVIATION:GCAT#ECNUMBERS:2.3.1.29# LOCUS:_211#TRANSCRIPT:2#ABBREVIATION:ALAS1#ECNUMBERS:2.3.1.37# proteins: NAME:glycine C-acetyltransferase (2-amino-3-ketobutyrate coenzyme A ligase), mitochondrial #ABBREVIATION:Gcat.1-m# NAME:aminolevulinate, delta-, synthase 1, mitochondrial #ABBREVIATION:Alas1.1-m# NAME:minolevulinate, delta-, synthase 1, mitochondrial#ABBREVIATION:Alas1.2-m# NAME:_5-aminolevulinate synthase, erythroid-specific, mitochondrial precursor #ABBREVIATION:Alas2.1-m# citations: AUTHORS:Bishop DF.#TITLE:Two different genes encode delta-aminolevulinate synthase in humans: nucleotide sequences of cDNAs for the housekeeping and erythroid genes. #YEAR:1990#PUBMED:2263504# GENE ASSOCIATION: (212.1) or (23464.1) or (211.2) or (211.1)
hyaluronan degradation product 1_C22H33N2O16
Abbreviation: R_25VITD3Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : 25hvitd3 + h + nadph + o2 --> 1a25dhvitd3 + h2o + nadp Confidence Level: 4 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.196 IT genes: LOCUS:_1594#TRANSCRIPT:1#ABBREVIATION:CYP27B1#ECNUMBERS:1.14.-.-# proteins: NAME:cytochrome P450, family 27, subfamily B, polypeptide 1 #ABBREVIATION:Cyp27b1.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Yokomura K, Suda T, Sasaki S, Inui N, Chida K, Nakamura H. #TITLE:Increased expression of the 25-hydroxyvitamin D(3)-1alpha-hydroxylase gene in alveolar macrophages of patients with lung cancer.#JOURNAL:J Clin Endocrinol Metab. #YEAR:2003#PUBMED:14671156# AUTHORS:St-Arnaud R, Messerlian S, Moir JM, Omdahl JL, Glorieux FH.#TITLE:The 25-hydroxyvitamin D 1-alpha-hydroxylase gene maps to the pseudovitamin D-deficiency rickets (PDDR) disease locus.#JOURNAL: J Bone Miner Res. #YEAR:1997#PUBMED:9333115# GENE ASSOCIATION: (1594.1)
_1-alpha,25-Dihydroxyvitamin D3_C27H44O3
Abbreviation: R_FAOXC161802m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid activation Equation: [m] : (4) coa + (3) fad + (4) h2o + hdd2coa + (4) nad --> (4) accoa + (3) fadh2 + (4) h + (4) nadh + occoa Confidence Level: 4 _4 rounds of beta ox, 1 loss of degree of unsat -> only 3 fad oxidized c16:1 -> c8:0 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Dolichol, human liver homolog_C1080H1768O10
Abbreviation: R_MECOAS1m Synonyms: _0 EC Number: 6.2.1.4 SUBSYSTEM: C5-Branched dibasic acid metabolism Equation: [m] : coa + gtp + mescon <==> gdp + mescoa + pi Confidence Level: 2 IF genes: LOCUS:_8802#TRANSCRIPT:1#ABBREVIATION:SUCLG1#ECNUMBERS:6.2.1.4# LOCUS:_8801#TRANSCRIPT:1#ABBREVIATION:SUCLG2#ECNUMBERS:6.2.1.4# proteins: NAME:Succinate-CoA ligase (GDP-forming), mitochondiral#ABBREVIATION:Suclg-m# citations: AUTHORS:WANG SF, ADLER J, LARDY HA#TITLE:The pathway of itaconate metabolism by liver mitochondria#JOURNAL:J Biol Chem#YEAR:1961#PUBMED:13783048# GENE ASSOCIATION: (8802.1 and 8801.1)
Abbreviation: R_ALACYSNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + cys-L[c] + na1[e] --> ala-L[c] + cys-L[e] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
L-alanine/L-cysteine Na-dependent exchange (Ala-L in)
L-serine/L-asparagine Na-dependent exchange (Ser-L in)
Abbreviation: R_SERASNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[c] + na1[e] + ser-L[e] --> asn-L[e] + na1[c] + ser-L[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
Itaconate--CoA ligase (ADP-forming), mitochondrial
Abbreviation: R_ITCOALm Synonyms: _1 EC Number: 6.2.1.5 SUBSYSTEM: C5-Branched dibasic acid metabolism Equation: [m] : atp + coa + itacon <==> adp + itaccoa + pi Confidence Level: 1 NCD genes: LOCUS:_8802#TRANSCRIPT:1#ABBREVIATION:SUCLG1#ECNUMBERS:6.2.1.4# LOCUS:_8803#TRANSCRIPT:1#ABBREVIATION:SUCLA2#ECNUMBERS:6.2.1.5# proteins: NAME:succinate--CoA ligase, ADP forming, mitochondrial#ABBREVIATION:Sucla-m# citations: AUTHORS:WANG SF, ADLER J, LARDY HA#TITLE:The pathway of itaconate metabolism by liver mitochondria#JOURNAL:J Biol Chem#YEAR:1961#PUBMED:13783048# GENE ASSOCIATION: (8802.1 and 8803.1)
Abbreviation: R_NORANMT Synonyms: _6 EC Number: 2.1.1.28 SUBSYSTEM: Tyrosine metabolism Equation: [c] : amet + nrpphr --> adrnl + ahcys + h Confidence Level: 4 SAB genes: LOCUS:_5409#TRANSCRIPT:1#ABBREVIATION:PNMT#ECNUMBERS:2.1.1.28# proteins: NAME:phenylethanolamine N-methyltransferase#ABBREVIATION:Pnmt.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (5409.1)
Abbreviation: R_UAGALDP Synonyms: _0 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acgal1p + h + utp --> ppi + udpacgal Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
UDP-N-acetylgalactosamine diphosphorylase
N-Acetyl-D-galactosamine 1-phosphate_C8H14NO9P
chondroitin sulfate B / dermatan sulfate (IdoA2S-GalNAc4S), degradation product 2_C37H54NO41S3
Abbreviation: R_NACHEX3ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_b_deg1 + h2o --> acgal + cs_b_deg2 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1) or (3074.1)
Abbreviation: R_METLEUex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: leu-L[c] + met-L[e] --> leu-L[e] + met-L[c] Confidence Level: 4 SAB see PMID 14770310 genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_8140#TRANSCRIPT:1#ABBREVIATION:SLC7A5# proteins: NAME:solute carrier family 7 member 5/family 3 member 2 heterodimer#ABBREVIATION:Slc3a2/7a5# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (6520.1 and 8140.1)
Abbreviation: R_DPCOAPP Synonyms: _0 SUBSYSTEM: CoA Catabolism Equation: [c] : dpcoa + h2o --> amp + (2) h + pan4p Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# GENE ASSOCIATION:
Abbreviation: R_NDPK3n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + cdp <==> adp + ctp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:CDP),nuclear
_1D-myo-Inositol 1,4-bisphosphate_C6H10O12P2
Abbreviation: R_HS1ly Synonyms: _4 EC Number: 3.10.1.1 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg1 --> h + hs_deg2 + so4 Confidence Level: 4 NCD genes: LOCUS:_6448#TRANSCRIPT:1#ABBREVIATION:SGSH# proteins: NAME:N-sulfoglucosamine sulfohydrolase (sulfamidase), lysosomal#ABBREVIATION:Sgsh.1-l# citations: AUTHORS:Scott HS, Blanch L, Guo XH, Freeman C, Orsborn A, Baker E, Sutherland GR, Morris CP, Hopwood JJ#TITLE:Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7493035# AUTHORS:Bielicki J, Hopwood JJ, Melville EL, Anson DS#TITLE:Recombinant human sulphamidase: expression, amplification, purification and characterization#JOURNAL:Biochem J#YEAR:1998#PUBMED:9405287# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION: (6448.1)
Abbreviation: R_GLUt6 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glu-L[e] + h[e] + k[c] + (3) na1[e] --> glu-L[c] + h[c] + k[e] + (3) na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2003 genes: LOCUS:_6511#TRANSCRIPT:1#ABBREVIATION:SLC1A6# LOCUS:_6507#TRANSCRIPT:1#ABBREVIATION:SLC1A3# LOCUS:_6506#TRANSCRIPT:1#ABBREVIATION:SLC1A2# LOCUS:_6505#TRANSCRIPT:1#ABBREVIATION:SLC1A1# LOCUS:_6512#TRANSCRIPT:1#ABBREVIATION:SLC1A7# proteins: NAME:solute carrier family 1 (neuronal/epithelial high affinity glutamate transporter, system Xag), member 1#ABBREVIATION:Slc1a1.1# NAME:solute carrier family 1 (glial high affinity glutamate transporter), member 2#ABBREVIATION:Slc1a2.1# NAME:solute carrier family 1 (glial high affinity glutamate transporter), member 3#ABBREVIATION:Slc1a3.1# NAME:solute carrier family 1 (high affinity aspartate/glutamate transporter), member 6#ABBREVIATION:Slc1a6.1# NAME:solute carrier family 1 (glutamate transporter), member 7#ABBREVIATION:Slc1a7.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6511.1) or (6505.1) or (6507.1) or (6506.1) or (6512.1)
Glutamate transport via Na, H symport and K antiport
Abbreviation: R_DCTPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: dctp[c] <==> dctp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_gtp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gtp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FUCASEe Synonyms: _1 EC Number: 3.2.1.51 SUBSYSTEM: N-Glycan Degradation Equation: [e] : h2o + s2l2fn2m2masn --> fuc-L + s2l2n2m2masn Confidence Level: 0 NCD genes: LOCUS:_2519#TRANSCRIPT:1#ABBREVIATION:FUCA2#ECNUMBERS:3.2.1.51# proteins: NAME:alpha-fucosidase 2, extracellular (plasma)#ABBREVIATION:Fuca2.1-e# citations: none GENE ASSOCIATION: (2519.1)
Abbreviation: R_P4503A4 Synonyms: _5 SUBSYSTEM: CYP Metabolism Equation: [c] : h + nadph + nifedipine + o2 --> h2o + hnifedipine + nadp Confidence Level: 4 N-oxidation of nifedipine assumed to take place on outer membrane NJ genes: LOCUS:_1576#TRANSCRIPT:1#ABBREVIATION:CYP3A4#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 3, subfamily A, polypeptide 4#ABBREVIATION:Cyp3a4.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Molowa DT, Schuetz EG, Wrighton SA, Watkins PB, Kremers P, Mendez-Picon G, Parker GA, Guzelian PS.#TITLE:Complete cDNA sequence of a cytochrome P-450 inducible by glucocorticoids in human liver.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1986#PUBMED:3460094# AUTHORS:Finta C, Zaphiropoulos PG.#TITLE:Intergenic mRNA molecules resulting from trans-splicing.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11726664# GENE ASSOCIATION: (1576.1)
glycophosphatidylinositol (GPI) deacylase, endoplasmic reticulum
glycophosphatidylinositol (GPI)-anchored protein_C55H106N4O38P4FULLRCO2FULLR2CO2X
Abbreviation: R_GPIDAer Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : gpi_prot_hs + h2o --> dgpi_prot_hs + h + hdca Confidence Level: 4 NCD genes: LOCUS:_80055#TRANSCRIPT:1#ABBREVIATION:PGAP1# proteins: NAME:GPI deacylase, endoplasmic reticulum#ABBREVIATION:Pgap1.1-r# citations: AUTHORS:Tanaka S, Maeda Y, Tashima Y, Kinoshita T#TITLE:Inositol deacylation of glycosylphosphatidylinositol-anchored proteins is mediated by mammalian PGAP1 and yeast Bst1p#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14734546# GENE ASSOCIATION: (80055.1)
deacylated-glycophosphatidylinositol (GPI)-anchored protein_C39H76N4O37P4FULLRCO2FULLR2CO2X
_2-Amino-3-carboxymuconate semialdehyde_C7H6NO5
Palmitoyl-CoA (n-C16:0CoA)_C37H62N7O17P3S
Abbreviation: R_C160CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + pmtcrn --> crn + pmtcoa Confidence Level: 4 TV genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# GENE ASSOCIATION: (1376.1)
Abbreviation: R_LCTStl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: lcts[c] --> lcts[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Gordon PB, Seglen PO.#TITLE:Prelysosomal convergence of autophagic and endocytic pathways#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3126737# GENE ASSOCIATION:
lactose transport from cytosol to lysosome (via autophagocytosis)
Abbreviation: R_CRTNsyn Synonyms: _0 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : pcreat --> crtn + h + pi Confidence Level: 2 SAB genes: none proteins: none citations: AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_SLDt Synonyms: _0 SUBSYSTEM: Cysteine Metabolism Equation: sl-L[c] --> sl-L[e] Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Rein U, Gueta R, Denger K, Ruff J, Hollemeyer K, Cook AM.#TITLE:Dissimilation of cysteate via 3-sulfolactate sulfo-lyase and a sulfate exporter in Paracoccus pantotrophus NKNCYSA.#YEAR:2005#PUBMED:15758220# GENE ASSOCIATION:
L-sulfolactate transport (cytosol to extracellular)
Abbreviation: R_GCHOLAte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gchola[e] <==> gchola[c] Confidence Level: 2 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Hydrogen peroxide synthesis (NADPH dependent)
Abbreviation: R_H2O2syn Synonyms: _0 SUBSYSTEM: Tyrosine metabolism Equation: [c] : h + nadph + o2 --> h2o2 + nadp Confidence Level: 2 SAB lumped reaction--first O2 goes to superoxide radical, then dismutes to H2O2 genes: LOCUS:_53905#TRANSCRIPT:1#ABBREVIATION:DUOX1# LOCUS:_53905#TRANSCRIPT:2#ABBREVIATION:DUOX1# LOCUS:_50506#TRANSCRIPT:1#ABBREVIATION:DUOX2# proteins: NAME:dual oxidase 1#ABBREVIATION:Duox1.1# NAME:dual oxidase 1#ABBREVIATION:Doux1.2# NAME:dual oxidase 2#ABBREVIATION:Duox2.1# citations: AUTHORS:Ameziane-El-Hassani R, Morand S, Boucher JL, Frapart YM, Apostolou D, Agnandji D, Gnidehou S, Ohayon R, Noel-Hudson MS, Francon J, Lalaoui K, Virion A, Dupuy C#TITLE:Dual Oxidase-2 Has an Intrinsic Ca2+-dependent H2O2-generating Activity#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15972824# AUTHORS:Forteza R, Salathe M, Miot F, Forteza R, Conner GE#TITLE:Regulated hydrogen peroxide production by Duox in human airway epithelial cells#JOURNAL:Am J Respir Cell Mol Biol#YEAR:2005#PUBMED:15677770# GENE ASSOCIATION: (53905.1) or (50506.1) or (53905.2)
Abbreviation: R_EX_oxa_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : oxa <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DPCOAtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: dpcoa[l] <==> dpcoa[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Seetharam B, Alpers DH.#TITLE:Absorption and transport of cobalamin (vitamin B12).#JOURNAL:Annu Rev Nutr.#YEAR:1982#PUBMED:6313022# GENE ASSOCIATION:
dephospho-CoA transport from lysosome via diffusion
monoacylglycerol 2 (homo sapiens)_C3H7O2FULLR2CO2
R total Coenzyme A_CO2FULLRC21H31N7O15P3S
Abbreviation: R_MOGAT Synonyms: _0 SUBSYSTEM: Triacylglycerol Synthesis Equation: [c] : Rtotalcoa + mag_hs --> coa + dag_hs Confidence Level: 2 FULLR corresponds to acyl/R groups in texts. NJ genes: LOCUS:_80168#TRANSCRIPT:1#ABBREVIATION:MOGAT2# LOCUS:_116255#TRANSCRIPT:1#ABBREVIATION:MOGAT1# proteins: NAME:monoacylglycerol O-acyltransferse 1#ABBREVIATION:Mogat1.1# NAME:monoacylglycerol O-acyltransferase 2#ABBREVIATION:Mogat2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Coleman RA, Lee DP#TITLE:Enzymes of triacylglycerol synthesis and their regulation#JOURNAL:Prog in Lipid Research#YEAR:2004# GENE ASSOCIATION: (116255.1) or (80168.1)
cytochrome P450 lanosterol 14-alpha-demethylase
Abbreviation: R_LNS14DMr Synonyms: _2 EC Number: 1.14.14.1 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : (2) h + lanost + (3) nadph + (3) o2 --> 44mctr + for + (4) h2o + (3) nadp Confidence Level: 4 NCD ER version NJ genes: LOCUS:_1595#TRANSCRIPT:1#ABBREVIATION:CYP51A1# proteins: NAME:cytochrom P450, family 51, subfamily A, polypeptide 1#ABBREVIATION:Cyp51a1.1# citations: AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# AUTHORS:Lepesheva GI, Waterman MR#TITLE:CYP51 - the omnipotent P450#JOURNAL:Molecular and Cell Endocrinology#YEAR:2004# GENE ASSOCIATION: (1595.1)
Abbreviation: R_SARCOXp Synonyms: _0 EC Number: 1.5.3.1 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [x] : h2o + o2 + sarcs --> fald + gly + h2o2 Confidence Level: 2 genes: LOCUS:_51268#TRANSCRIPT:1#ABBREVIATION:PIPOX#ECNUMBERS:1.5.3.1,,1.5.3.7# proteins: NAME:pipecolic acid/sarcosine oxidase, peroxisomal#ABBREVIATION:Pipox.1-x# citations: AUTHORS:Dodt G, Kim DG, Reimann SA, Reuber BE, McCabe K, Gould SJ, Mihalik SJ. #TITLE:L-Pipecolic acid oxidase, a human enzyme essential for the degradation of L-pipecolic acid, is most similar to the monomeric sarcosine oxidases.#YEAR:2000#PUBMED:10642506# GENE ASSOCIATION: (51268.1)
Abbreviation: R_7DHFtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _7dhf[m] --> 7dhf[c] Confidence Level: 2 unknown mechanism. IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_7-glutamyl-DHF transport,m mitochondrial
Abbreviation: R_NRPPHRt4_LPAREN_2_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) na1[e] + nrpphr[e] <==> (2) na1[c] + nrpphr[c] Confidence Level: 4 SAB genes: LOCUS:_6531#TRANSCRIPT:1#ABBREVIATION:SLC6A3# LOCUS:_6530#TRANSCRIPT:1#ABBREVIATION:SLC6A2# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, noradrenalin), member 2#ABBREVIATION:Slc6a2.1# NAME:solute carrier family 6 (neurotransmitter transporter, dopamine), member 3#ABBREVIATION:Slc6a3.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (6530.1) or (6531.1)
Norepinephrine reversible transport in via sodium symport (1:2)
Histamine secretion via secretory vesicle (ATP driven)
Abbreviation: R_HISTAVESSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) atp[c] + (2) h2o[c] + (3) hista[c] --> (2) adp[c] + (2) h[c] + (3) hista[e] + (2) pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of hista from the cell. It leaves out the vesicle because that is not a compartment. ATP:H ratio is assumed as 1:3 for vacuolar ATPase and substrate:h ratio is 1:2. This gives a 3:2 ratio for substrate:ATP genes: LOCUS:_6571#TRANSCRIPT:1#ABBREVIATION:SLC18A2# LOCUS:_6570#TRANSCRIPT:1#ABBREVIATION:SLC18A1# proteins: NAME:solute carrier family 18 (vesicular monoamine), member 1#ABBREVIATION:Slc18a1.1# NAME:solute carrier family 18 (vesicular monoamine), member 2#ABBREVIATION:Slc18a2.1# citations: AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# AUTHORS:Eiden LE, Schafer MK, Weihe E, Schutz B#TITLE:The vesicular amine transporter family (SLC18): amine/proton antiporters required for vesicular accumulation and regulated exocytotic secretion of monoamines and acetylcholine#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12827358# GENE ASSOCIATION: (6570.1) or (6571.1)
Abbreviation: R_4NPHSULT Synonyms: _3 EC Number: 2.8.2.1 SUBSYSTEM: CYP Metabolism Equation: [c] : 4nph + paps --> 4nphsf + h + pap Confidence Level: 4 RS/TV (6/14/05) genes: LOCUS:_6818#TRANSCRIPT:1#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# LOCUS:_6799#TRANSCRIPT:1#ABBREVIATION:SULT1A2#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:1#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:2#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:3#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:4#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:5#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6799#TRANSCRIPT:2#ABBREVIATION:SULT1A2#ECNUMBERS:2.8.2.1# LOCUS:_6818#TRANSCRIPT:2#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# LOCUS:_6818#TRANSCRIPT:3#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# proteins: NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.2# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.3# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.4# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.5# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 2 #ABBREVIATION:Sult1a2.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 2#ABBREVIATION:Sult1a2.2# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.2# NAME:sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.3# citations: AUTHORS:Chapman E, Best MD, Hanson SR, Wong CH.#TITLE:Sulfotransferases: structure, mechanism, biological activity, inhibition, and synthetic utility.#JOURNAL:Angew Chem Int Ed Engl#YEAR:2004#PUBMED:15293241# AUTHORS:Glatt H, Boeing H, Engelke CE, Ma L, Kuhlow A, Pabel U, Pomplun D, Teubner W, Meinl W.#TITLE:Human cytosolic sulphotransferases: genetics, characteristics, toxicological aspects.#JOURNAL:Mutat Res#YEAR:2001#PUBMED:11535246# GENE ASSOCIATION: (6799.1) or (6817.3) or (6799.2) or (6818.2) or (6817.2) or (6818.1) or (6817.1) or (6817.4) or (6818.3) or (6817.5)
Abbreviation: R_LCADi_D Synonyms: _0 EC Number: 1.2.1.21 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : h2o + lald-D + nad --> (2) h + lac-D + nadh Confidence Level: 2 JLR- added irreversible form of LCAD genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Ewaschuk JB, Naylor JM, Zello GA#TITLE:D-lactate in human and ruminant metabolism#JOURNAL:J Nutr#YEAR:2005#PUBMED:15987839# AUTHORS:Christopher MM, Eckfeldt JH, Eaton JW.#TITLE:Propylene glycol ingestion causes D-lactic acidosis#JOURNAL:Lab Invest.#YEAR:1990#PUBMED:2296157# AUTHORS:Ray S, Ray M.#TITLE:Oxidation of lactaldehyde by cytosolic aldehyde dehydrogenase and inhibition of cytosolic and mitochondrial aldehyde dehydrogenase by metabolites.#JOURNAL:Biochim Biophys Acta.#YEAR:1984#PUBMED:6487654# GENE ASSOCIATION: (218.1) or (223.1) or (8854.2) or (224.1) or (222.1) or (8854.1) or (221.1) or (216.1) or (8854.3) or (501.1) or (220.1)
(alpha-D-Mannosyl)7-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human liver homolog_C1720H2828N20O570P20
Abbreviation: R_DNDPt20m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + dtdp[c] --> atp[c] + dtdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_URIt5 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) na1[e] + uri[e] --> (2) na1[c] + uri[c] Confidence Level: 4 MM genes: LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (64078.1)
uridine transport in via sodium symport (1:2)
b-ketoacyl synthetase (palmitate, n-C16:0)
Abbreviation: R_KAS8 Synonyms: _1 EC Number: 2.3.1.41 SUBSYSTEM: Fatty acid elongation Equation: [c] : accoa + (20) h + (7) malcoa + (14) nadph --> (7) co2 + (8) coa + (6) h2o + hdca + (14) nadp Confidence Level: 4 genes: LOCUS:_2194#TRANSCRIPT:1#ABBREVIATION:FASN#ECNUMBERS:2.3.1.85# proteins: NAME:fatty acid synthase#ABBREVIATION:Fasn.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Chakravarty B, Gu Z, Chirala SS, Wakil SJ, Quiocho FA.#TITLE:Human fatty acid synthase: structure and substrate selectivity of the thioesterase domain.#YEAR:2004#PUBMED:15507492# AUTHORS:Brink J, Ludtke SJ, Yang CY, Gu ZW, Wakil SJ, Chiu W.#TITLE:Quaternary structure of human fatty acid synthase by electron cryomicroscopy.#JOURNAL:Proc Natl Acad Sci#YEAR:2002#PUBMED:11756679# AUTHORS:Wakil SJ.#TITLE:Fatty acid synthase, a proficient multifunctional enzyme.#JOURNAL:Biochemistry#YEAR:1989#PUBMED:2669958# GENE ASSOCIATION: (2194.1)
Abbreviation: R_PRODt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[e] + pro-D[e] <==> h[c] + pro-D[c] Confidence Level: 2 NCD SAB D form genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
D-proline reversible transport via proton symport
de-Fuc, reducing GlcNAc removed, de-Sia form of PA6 (w/o peptide linkage)_C54H91N3O41
beta GalNAc globoside (homo sapiens)_C52H92N3O27FULLRCO
Abbreviation: R_ACGBGBSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acgbgbside_hs[c] <==> acgbgbside_hs[g] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# GENE ASSOCIATION:
Abbreviation: R_GLYKm Synonyms: _0 EC Number: 2.7.1.30 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [m] : atp + glyc --> adp + glyc3p + h Confidence Level: 2 TV genes: LOCUS:_2710#TRANSCRIPT:1#ABBREVIATION:GK#ECNUMBERS:2.7.1.30# LOCUS:_2712#TRANSCRIPT:1#ABBREVIATION:GK2# LOCUS:_2713#TRANSCRIPT:1#ABBREVIATION:GKP3# LOCUS:_2710#TRANSCRIPT:2#ABBREVIATION:GK#ECNUMBERS:2.7.1.30# proteins: NAME:glycerol kinase#ABBREVIATION:Gk.1# NAME:glycerol kinase#ABBREVIATION:Gk.2# NAME:glycerol kinase 2#ABBREVIATION:Gk2.1# NAME:glycerol kinase 3 (pseudogene)#ABBREVIATION:Gkp3.1# citations: AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# AUTHORS:Sargent CA, Young C, Marsh S, Ferguson-Smith MA, Affara NA.#TITLE:The glycerol kinase gene family: structure of the Xp gene, and related intronless retroposons.#JOURNAL:Hum Mol Genet#YEAR:1994#PUBMED:7987308# GENE ASSOCIATION: (2710.2) or (2712.1) or (2710.1) or (2713.1)
GQ1balpha (homo sapiens)_C88H143N6O54FULLRCO
Abbreviation: R_GQ1BALPHAte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gq1balpha_hs[c] <==> gq1balpha_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_PTHPS Synonyms: _0 EC Number: 4.2.3.12 SUBSYSTEM: Tetrahydrobiopterin Equation: [c] : ahdt --> 6pthp + pppi Confidence Level: 4 Formery EC 4.6.1.10; New EC 4.2.3.12 catalyses triphosphate elimination and an intramolecular redox reaction in the presence of Mg2+. It has been identified in human liver. The product is 6-pyruvoyltetrahydrobiopterin genes: LOCUS:_5805#TRANSCRIPT:1#ABBREVIATION:PTS#ECNUMBERS:4.6.1.10# proteins: NAME:_6-pyruvoyltetrahydropterin synthase #ABBREVIATION:Pts1.1# citations: AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (5805.1)
cis-tetradec-7-enoyl-[acyl-carrier protein] (n-C14:1)_C25H45N2O8PRS
Abbreviation: R_GLXO1 Synonyms: _0 EC Number: 1.2.3.5 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : glx + h2o + nad --> (2) h + nadh + oxa Confidence Level: 4 NCD genes: LOCUS:_3945#TRANSCRIPT:1#ABBREVIATION:LDHB#ECNUMBERS:1.1.1.27# LOCUS:_3948#TRANSCRIPT:1#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_3939#TRANSCRIPT:1#ABBREVIATION:LDHA#ECNUMBERS:1.1.1.27# LOCUS:_160287#TRANSCRIPT:1#ABBREVIATION:LDHAL6A# LOCUS:_92483#TRANSCRIPT:1#ABBREVIATION:LDHAL6B# LOCUS:_197257#TRANSCRIPT:1#ABBREVIATION:LDHD# LOCUS:_3948#TRANSCRIPT:2#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_197257#TRANSCRIPT:2#ABBREVIATION:LDHD# LOCUS:_55293#TRANSCRIPT:1#ABBREVIATION:UEV3# proteins: NAME:lactate dehydrogenase A (liver, sk muscle)#ABBREVIATION:Ldha.1# NAME:lactate dehydrogenase B (heart, RBC)#ABBREVIATION:Ldhb.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.2# NAME:lactate dehydrogenase A-like 6B (testis)#ABBREVIATION:Ldhal6b.1# NAME:lactate dehydrogenase A-like 6A#ABBREVIATION:Ldhal6a.1# NAME:ubiquitin-conjugating enzyme E2-like#ABBREVIATION:Uev3.1# NAME:lactate dehydrogenase D#ABBREVIATION:Ldhd.1# NAME:lactate dehydrogenase D#ABBREVIATION:Ldhd.2# NAME:lactate dehydrogenase (heart, RBC, brain, kidney, liver, sk muscle)#ABBREVIATION:LdhAB# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Holmes RP, Assimos DG#TITLE:Glyoxylate synthesis, and its modulation and influence on oxalate synthesis#JOURNAL:J Urol#YEAR:1998#PUBMED:9783918# AUTHORS:Holmes RP#TITLE:Pharmacological approaches in the treatment of primary hyperoxaluria#JOURNAL:J Nephrol#YEAR:1998#PUBMED:9604807# AUTHORS:Pirulli D, Marangella M, Amoroso A#TITLE:Primary hyperoxaluria: genotype-phenotype correlation#JOURNAL:J Nephrol.#YEAR:2003#PUBMED:12768081# AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# GENE ASSOCIATION: (3939.1) or (55293.1) or (3945.1 and 3939.1) or (197257.1) or (92483.1) or (3945.1) or (160287.1) or (3948.1) or (197257.2) or (3948.2)
Abbreviation: R_SPODM Synonyms: _1 EC Number: 1.15.1.1 SUBSYSTEM: ROS Detoxification Equation: [c] : (2) h + (2) o2s --> h2o2 + o2 Confidence Level: 4 JLR genes: LOCUS:_6647#TRANSCRIPT:1#ABBREVIATION:SOD1#ECNUMBERS:1.15.1.1# proteins: NAME:superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))#ABBREVIATION:Sod1.1# citations: AUTHORS:Crapo JD, Oury T, Rabouille C, Slot JW, Chang LY#TITLE:Copper,zinc superoxide dismutase is primarily a cytosolic protein in human cells#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1992#PUBMED:1332049# AUTHORS:Hallewell RA, Masiarz FR, Najarian RC, Puma JP, Quiroga MR, Randolph A, Sanchez-Pescador R, Scandella CJ, Smith B, Steimer KS, et al#TITLE:Human Cu/Zn superoxide dismutase cDNA: isolation of clones synthesising high levels of active or inactive enzyme from an expression library#JOURNAL:Nucleic Acids Res#YEAR:1985#PUBMED:3889846# GENE ASSOCIATION: (6647.1)
Abbreviation: R_CSNAT3x Synonyms: _0 EC Number: 2.3.1.7 SUBSYSTEM: Carnitine shuttle Equation: [x] : crn + ppcoa <==> coa + pcrn Confidence Level: 4 NJ genes: LOCUS:_1384#TRANSCRIPT:2#ABBREVIATION:CRAT#ECNUMBERS:2.3.1.7# proteins: NAME:carnitine acetyltransferase, perixosomal#ABBREVIATION:Crat.2-x# citations: AUTHORS:Corti O, Finocchiaro G, Rossi E, Zuffardi O, DiDonato S.#TITLE:Molecular cloning of cDNAs encoding human carnitine acetyltransferase and mapping of the corresponding gene to chromosome 9q34.1.#JOURNAL:Genomics#YEAR:1994#PUBMED:7829107# AUTHORS:Jakobs BS, Wanders RJ.#TITLE:Fatty acid beta-oxidation in peroxisomes and mitochondria: the first, unequivocal evidence for the involvement of carnitine in shuttling propionyl-CoA from peroxisomes to mitochondria.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1995#PUBMED:7654220# GENE ASSOCIATION: (1384.2)
carnitine O-aceyltransferase, peroxisomal
Abbreviation: R_EX_acac_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acac <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NADK Synonyms: _0 EC Number: 2.7.1.23 SUBSYSTEM: NAD Metabolism Equation: [c] : atp + nad --> adp + h + nadp Confidence Level: 4 genes: LOCUS:_65220#TRANSCRIPT:1#ABBREVIATION:FLJ13052#ECNUMBERS:2.7.1.23# proteins: NAME:NAD kinase #ABBREVIATION:Nadk.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Lerner F, Niere M, Ludwig A, Ziegler M.#TITLE:Structural and functional characterization of human NAD kinase.#JOURNAL:Biochem Biophys Res Commun.#YEAR:2001#PUBMED:11594753# AUTHORS:Williams MB, Jones HP.#TITLE:Calmodulin-dependent NAD kinase of human neutrophils.#JOURNAL:Arch Biochem Biophys. #YEAR:1985#PUBMED:2982330# GENE ASSOCIATION: (65220.1)
Abbreviation: R_GDCHOLAte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gdchola[e] <==> gdchola[c] Confidence Level: 2 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I biosynthesis, precursor 18_C163H262N11O132S5X
keratan sulfate I biosynthesis, precursor 19_C171H275N12O137S5X
Abbreviation: R_AG13T10g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre18 + uacgam --> h + ksi_pre19 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
Abbreviation: R_MM6bg Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m6masnC --> m5masnC + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
Core 3 beta-GlcNAc-transferase, Golgi apparatus
Abbreviation: R_CORE3GTg Synonyms: _7 EC Number: 2.4.1.147 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : Tn_antigen + uacgam --> core3 + h + udp Confidence Level: 0 NCD genes: LOCUS:_192134#TRANSCRIPT:1#ABBREVIATION:IMAGE:4907098# proteins: NAME:beta-1,3-N-acetylglucosaminyltransferase protein (somtach, colon, sm intestine, sk muscle, testis)#ABBREVIATION:Image:4907098-g# citations: AUTHORS:Iwai T, Inaba N, Naundorf A, Zhang Y, Gotoh M, Iwasaki H, Kudo T, Togayachi A, Ishizuka Y, Nakanishi H, Narimatsu H#TITLE:Molecular cloning and characterization of a novel UDP-GlcNAc:GalNAc-peptide beta1,3-N-acetylglucosaminyltransferase (beta 3Gn-T6), an enzyme synthesizing the core 3 structure of O-glycans#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11821425# GENE ASSOCIATION: (192134.1)
Abbreviation: R_CDSm Synonyms: _7 EC Number: 2.7.7.41 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [m] : ctp + h + pa_hs --> cdpdag_hs + ppi Confidence Level: 4 inner mitochondrial membrane NJ genes: LOCUS:_8760#TRANSCRIPT:1#ABBREVIATION:CDS2#ECNUMBERS:2.7.7.41# proteins: NAME:CDP-diacylglycerol synthase#ABBREVIATION:Cds2.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Kent C#TITLE:Eukaryotic phospholipid biosynthesis#JOURNAL:Annu Rev Biochem#YEAR:1995# AUTHORS:Halford S, Dulai KS, Daw SC, Fitzgibbon J, Hunt DM#TITLE:Isolation and chromosomal localization of two human cdp-diacylglycerol synthase genes#JOURNAL:Genomics#YEAR:1998# AUTHORS:Weeks R, Dowhan W, Shen H, Balantac N, Meengs B, Nudelman E, Leung DW#TITLE:Isolation and expression of an isoform of human CDP-diacylglycerol synthease cDNA#JOURNAL:DNA Cell Biol#YEAR:1997# GENE ASSOCIATION: (8760.1)
CDP diacylglycerol (homo sapiens)_C12H17N3O11P2FULLRCO2FULLR2CO2
phosphatidic acid (homo sapiens)_C3H5O4PFULLRCO2FULLR2CO2
Abbreviation: R_TXA2te Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: txa2[e] <==> txa2[c] Confidence Level: 2 unknown mechanism for transport - known to be secreted from cells - unknown mechanism or pathway, TXA2tc -> TXA2te to get to EC space NJ genes: none proteins: none citations: TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_DM_dsT_antigen_LPAREN_g_RPAREN_ Synonyms: _0 Equation: [g] : dsT_antigen --> Confidence Level: NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_lcts_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lcts <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_asp_DASH_D_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : asp-D <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
eicosatetranoyl coenzyme A_C41H62N7O17P3S
Abbreviation: R_FAOXC204184m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : coa + eicostetcoa + fad + h2o + nad --> accoa + fadh2 + h + nadh + strdnccoa Confidence Level: 4 _1 rounds of beta ox c20:4 -> c18:4 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_EX_uri_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : uri <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_1-alpha,24R,25-Trihydroxyvitamin D3_C27H44O4
_1-alpha,24R,25-Vitamin D-hydroxylase (D3)
Abbreviation: R_1a_COMMA_25VITD3Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : 1a25dhvitd3 + h + nadph + o2 --> 1a2425thvitd3 + h2o + nadp Confidence Level: 4 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.198 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_UDPtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: udp[c] <==> udp[l] Confidence Level: 1 unknown mechanism for transport - not known if it is energy dependent or not, recycling and IC transport is important to get it to other areas of the cell (e.g. plasma membrane) - translocators have been found for ER and Golgi - none found for lysosome yet so non-mechanistic transporter added - in future this will need to be revised _when_ a translocator is found NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_XOLTRI27te Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: xoltri27[c] <==> xoltri27[e] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_O2ter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: o2[c] <==> o2[r] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Dehydroepiandrosterone sulfate_C19H27O5S
Abbreviation: R_UREAt5 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h2o[e] + urea[e] <==> h2o[c] + urea[c] Confidence Level: 4 NCD genes: LOCUS:_6523#TRANSCRIPT:1#ABBREVIATION:SLC5A1# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 1, extracellular#ABBREVIATION:Slc5a1.1-e# citations: AUTHORS:Hirayama BA, Loo DD, Wright EM.#TITLE:Protons drive sugar transport through the Na+/glucose cotransporter (SGLT1)#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8063771# AUTHORS:Hediger MA, Turk E, Wright EM#TITLE:Homology of the human intestinal Na+/glucose and Escherichia coli Na+/proline cotransporters#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1989#PUBMED:2490366# AUTHORS:Quick M, Loo DD, Wright EM#TITLE:Neutralization of a conserved amino acid residue in the human Na+/glucose transporter (hSGLT1) generates a glucose-gated H+ channel#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11024018# AUTHORS:Wright EM, Hirayama BA, Loo DDF, Turk E, Hager K#TITLE:Intestinal sugar transport#YEAR:1994# AUTHORS:Leung DW, Loo DD, Hirayama BA, Zeuthen T, Wright EM#TITLE:Urea transport by cotransporters#JOURNAL:J Physiol#YEAR:2000#PUBMED:11034615# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# GENE ASSOCIATION: (6523.1)
heparan sulfate, precursor 3_C45H69N2O36X
Abbreviation: R_NKCCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) cl[e] + k[e] + na1[e] <==> (2) cl[c] + k[c] + na1[c] Confidence Level: 4 NCD genes: LOCUS:_6558#TRANSCRIPT:1#ABBREVIATION:SLC12A2# LOCUS:_6557#TRANSCRIPT:1#ABBREVIATION:SLC12A1# proteins: NAME:solute carrier family 12 (sodium/potassium/chloride transporters), member 1#ABBREVIATION:Slc12a1.1-e# NAME:solute carrier family 12 (sodium/potassium/chloride transporters), member 2#ABBREVIATION:Slc12a2.1-e# citations: AUTHORS:Simon DB, Karet FE, Hamdan JM, DiPietro A, Sanjad SA, Lifton RP#TITLE:Bartter's syndrome, hypokalaemic alkalosis with hypercalciuria, is caused by mutations in the Na-K-2Cl cotransporter NKCC2#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8640224# AUTHORS:Hebert SC, Mount DB, Gamba G#TITLE:Molecular physiology of cation-coupled Cl- cotransport: the SLC12 family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739168# AUTHORS:Payne JA, Xu JC, Haas M, Lytle CY, Ward D, Forbush B 3rd#TITLE:Primary structure, functional expression, and chromosomal localization of the bumetanide-sensitive Na-K-Cl cotransporter in human colon#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7629105# AUTHORS:Russell JM#TITLE:Sodium-potassium-chloride cotransport#JOURNAL:Physiol Rev#YEAR:2000#PUBMED:10617769# AUTHORS:Bergeron MJ, Gagnon E, Wallendorff B, Lapointe JY, Isenring P.#TITLE:Ammonium transport and pH regulation by K(+)-Cl(-) cotransporters#JOURNAL:Am J Physiol Renal Physiol#YEAR:2003#PUBMED:12657561# GENE ASSOCIATION: (6557.1) or (6558.1)
heparan sulfate, degradation product 10_C55H80N3O70S8
heparan sulfate, degradation product 9_C61H87N3O76S8
Abbreviation: R_GLCAASE1ly Synonyms: _3 EC Number: 3.2.1.31 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg9 --> glcur + hs_deg10 Confidence Level: 4 NCD genes: LOCUS:_2990#TRANSCRIPT:1#ABBREVIATION:GUSB#ECNUMBERS:3.2.1.31# proteins: NAME:glucuronidase, beta, lysosomal#ABBREVIATION:Gusb.1-l# citations: AUTHORS:Guise KS, Korneluk RG, Waye J, Lamhonwah AM, Quan F, Palmer R, Ganschow RE, Sly WS, Gravel RA#TITLE:Isolation and expression in Escherichia coli of a cDNA clone encoding human beta-glucuronidase#JOURNAL:Gene#YEAR:1985#PUBMED:3924735# AUTHORS:Ho YC, Ho LH, Ho KJ#TITLE:Human hepatic beta-glucuronidase: an enzyme kinetic study#JOURNAL:Enzyme#YEAR:1985#PUBMED:3987656# AUTHORS:Oshima A, Kyle JW, Miller RD, Hoffmann JW, Powell PP, Grubb JH, Sly WS, Tropak M, Guise KS, Gravel RA#TITLE:Cloning, sequencing, and expression of cDNA for human beta-glucuronidase#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3468507# GENE ASSOCIATION: (2990.1)
Abbreviation: R_SERDGLNexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gln-L[c] + ser-D[e] <==> gln-L[e] + ser-D[c] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_CBL2OR Synonyms: _0 SUBSYSTEM: Vitamin B12 Metabolism Equation: [c] : (2) aqcobal + h + nadh --> (2) cbl2 + (2) h2o + nad Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Carmel R, Green R, Rosenblatt DS, Watkins D.#TITLE:Update on cobalamin, folate, and homocysteine.#JOURNAL:Hematology (Am Soc Hematol Educ Program).#YEAR:2003#PUBMED:14633777# GENE ASSOCIATION:
Abbreviation: R_UDPACGALtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: udpacgal[c] <==> udpacgal[l] Confidence Level: 1 unknown mechanism for transport - there are UDPACGAL-translocators for ER and Golgi, however no info on lysosomal - poor support for this NJ genes: none proteins: none citations: none GENE ASSOCIATION:
(alpha-D-Glucosyl)2-(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human liver homolog_C1900H3128N20O720P20
Abbreviation: R_DOLPGT2_Ler Synonyms: _2 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolglcp_L + (0.1) g1m8mpdol_L --> (0.1) dolp_L + (0.1) g2m8mpdol_L + h Confidence Level: 0 EC 2.4.1.- NCD genes: LOCUS:_79053#TRANSCRIPT:1#ABBREVIATION:ALG8# LOCUS:_79053#TRANSCRIPT:2#ABBREVIATION:ALG8# LOCUS:_79053#TRANSCRIPT:3#ABBREVIATION:ALG8# proteins: NAME:alpha-1,3-glucosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg8.1-er# NAME:alpha-1,3-glucosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg8.2-er# NAME:alpha-1,3-glucosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg8.3-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (79053.1) or (79053.3) or (79053.2)
Dolichyl beta-D-glucosyl phosphate, human liver homolog_C1140H1868O90P10
dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase (liver)
alpha-D-Glucosyl-(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human liver homolog_C1840H3028N20O670P20
Abbreviation: R_UDPDOLPT_L Synonyms: _0 EC Number: 2.4.1.117 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dolp_L + udpg --> (0.1) dolglcp_L + udp Confidence Level: 0 NCD genes: LOCUS:_29880#TRANSCRIPT:1#ABBREVIATION:ALG5#ECNUMBERS:2.4.1.117# proteins: NAME:dolichyl-phosphate beta-glucosyltransferase (ubiquitous)#ABBREVIATION:Alg5.1# citations: AUTHORS:Bossuyt X, Blanckaert N.#TITLE:Topology of nucleotide-sugar:dolichyl phosphate glycosyltransferases involved in the dolichol pathway for protein glycosylation in native rat liver microsomes#JOURNAL:Biochem J#YEAR:1993#PUBMED:8280060# GENE ASSOCIATION: (29880.1)
UDPglucose:dolichyl-phosphate beta-D-glucosyltransferase (liver)
Abbreviation: R_MDH Synonyms: _0 EC Number: 1.1.1.37 SUBSYSTEM: Citric Acid Cycle Equation: [c] : mal-L + nad <==> h + nadh + oaa Confidence Level: 0 genes: LOCUS:_4190#TRANSCRIPT:1#ABBREVIATION:MDH1#ECNUMBERS:1.1.1.37# LOCUS:_130752#TRANSCRIPT:1#ABBREVIATION:MDH1B# proteins: NAME:malate dehydrogenase 1 (NAD), soluble#ABBREVIATION:Mdh1.1# NAME:malate dehydrogenase 1b (NAD), soluble#ABBREVIATION:Mdh1b.1# citations: none GENE ASSOCIATION: (130752.1) or (4190.1)
Abbreviation: R_N4Tg Synonyms: _2 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : core6 + udpgal --> f1a + h + udp Confidence Level: 0 EC 2.4.1.- NCD genes: none proteins: none citations: none GENE ASSOCIATION:
N-acetylgalactosamine 4-beta-galactosyltransferase, Golgi apparatus
Abbreviation: R_LRAT Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : pchol_hs + retinol --> lpchol_hs + retfa Confidence Level: 4 IT genes: LOCUS:_9227#TRANSCRIPT:1#ABBREVIATION:LRAT#ECNUMBERS:2.3.1.135# proteins: NAME:lecithin retinol acyltransferase (phosphatidylcholine--retinol O-acyltransferase) #ABBREVIATION:Lrat.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Jahng WJ, Xue L, Rando RR.#TITLE:Lecithin retinol acyltransferase is a founder member of a novel family of enzymes.#JOURNAL:Biochemistry.#YEAR:2003#PUBMED:14596594# AUTHORS:Zolfaghari R, Ross AC. #TITLE:Cloning, gene organization and identification of an alternative splicing process in lecithin:retinol acyltransferase cDNA from human liver.#JOURNAL:Gene#YEAR:2004#PUBMED:15474300# GENE ASSOCIATION: (9227.1)
Abbreviation: R_URIK1 Synonyms: _0 EC Number: 2.7.1.48 SUBSYSTEM: Pyrimidine Biosynthesis Equation: [c] : atp + uri --> adp + h + ump Confidence Level: 4 genes: LOCUS:_7371#TRANSCRIPT:1#ABBREVIATION:UCK2#ECNUMBERS:2.7.4.*# LOCUS:_83549#TRANSCRIPT:1#ABBREVIATION:UCK1#ECNUMBERS:2.7.1.48# proteins: NAME:uridine-cytidine kinase 2 #ABBREVIATION:Uck2.1# NAME:uridine-cytidine kinase 1 #ABBREVIATION:Uck1.1# citations: AUTHORS:Kashuba E, Kashuba V, Sandalova T, Klein G, Szekely L#TITLE:Epstein-Barr virus encoded nuclear protein EBNA-3 binds a novel human uridine kinase/uracil phosphoribosyltransferase. #JOURNAL:BMC Cell Biol.#YEAR:2002#PUBMED:12199906# AUTHORS:Ozaki K, Kuroki T, Hayashi S, Nakamura Y.#TITLE:Isolation of three testis-specific genes (TSA303, TSA806, TSA903) by a differential mRNA display method. #JOURNAL:Genomics#YEAR:1996#PUBMED:8812458# AUTHORS:Van Rompay AR, Norda A, Linden K, Johansson M, Karlsson A.#TITLE:Phosphorylation of uridine and cytidine nucleoside analogs by two human uridine-cytidine kinases.#JOURNAL:Mol Pharmacol. #YEAR:2001#PUBMED:11306702# GENE ASSOCIATION: (7371.1) or (83549.1)
Abbreviation: R_ACNACNGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acnacngal14acglcgalgluside_hs[g] <==> acnacngal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
galactose/N-acetylglucosamine 6-O-sulfotransferase, Golgi apparatus
Abbreviation: R_S6T5g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre7 + paps --> h + ksi_pre8 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
keratan sulfate I biosynthesis, precursor 7_C115H187N8O85SX
keratan sulfate I biosynthesis, precursor 8_C115H186N8O88S2X
Malonyl-[acyl-carrier protein]_C14H22N2O10PRS
Abbreviation: R_MCOATAm Synonyms: _0 EC Number: 2.3.1.39 SUBSYSTEM: Fatty Acid Metabolism Equation: [m] : ACP + malcoa <==> coa + malACP Confidence Level: 4 NCD genes: LOCUS:_27349#TRANSCRIPT:1#ABBREVIATION:MT#ECNUMBERS:2.3.1.39# LOCUS:_27349#TRANSCRIPT:2#ABBREVIATION:MT#ECNUMBERS:2.3.1.39# proteins: NAME:malonyl-CoA:acyl carrier protein transacylase#ABBREVIATION:Mt.1# NAME:malonyl-CoA:acyl carrier protein transacylase, mitochondrial#ABBREVIATION:Mt.2# citations: AUTHORS:Zhang L, Joshi AK, Smith S.#TITLE:Cloning, expression, characterization, and interaction of two components of a human mitochondrial fatty acid synthase. Malonyltransferase and acyl carrier protein.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12882974# GENE ASSOCIATION: (27349.1) or (27349.2)
Malonyl-CoA-ACP transacylase, mitochondrial
glucosyl-(alpha-D-mannosyl)6-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine (protein)_C64H107N2O50X
Abbreviation: R_MAN2_6B1er Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : g1m7masnB + h2o --> g1m6masnB1 + man Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# AUTHORS:Roth J, Zuber C, Guhl B, Fan JY, Ziak M.#TITLE:The importance of trimming reactions on asparagine-linked oligosaccharides for protein quality control.#JOURNAL:Histochem Cell Biol#YEAR:2002#PUBMED:11935292# GENE ASSOCIATION:
mannosidase II, endoplasmic reticulum (g1m6masnB1-producing)
tetracosapentaenoyl coenzyme A, n-3_C45H68N7O17P3S
Abbreviation: R_FACOAL245_2 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + tetpent3 <==> amp + ppi + tetpent3coa Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_GALT Synonyms: _0 EC Number: 2.7.7.10 SUBSYSTEM: Galactose metabolism Equation: [c] : gal1p + h + utp <==> ppi + udpgal Confidence Level: 0 genes: LOCUS:_2592#TRANSCRIPT:1#ABBREVIATION:GALT#ECNUMBERS:2.7.7.12# LOCUS:_2592#TRANSCRIPT:3#ABBREVIATION:GALT#ECNUMBERS:2.7.7.12# LOCUS:_2592#TRANSCRIPT:2#ABBREVIATION:GALT#ECNUMBERS:2.7.7.12# proteins: NAME:galactose-1-phosphate uridylyltransferase#ABBREVIATION:Galt.1# NAME:galactose-1-phosphate uridylyltransferase#ABBREVIATION:Galt.2# NAME:galactose-1-phosphate uridylyltransferase#ABBREVIATION:Galt.3# citations: AUTHORS:Leslie ND, Immerman EB, Flach JE, Florez M, Fridovich-Keil JL, Elsas LJ#TITLE:The human galactose-1-phosphate uridyltransferase gene#JOURNAL:Genomics#YEAR:1992#PUBMED:1427861# GENE ASSOCIATION: (2592.1) or (2592.3) or (2592.2)
galactose-1-phosphate uridylyltransferase
Abbreviation: R_TKT2 Synonyms: _0 EC Number: 2.2.1.1 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : e4p + xu5p-D <==> f6p + g3p Confidence Level: 0 SMP Two reactions for transketolase: TKT1 and TKT2 genes: LOCUS:_7086#TRANSCRIPT:1#ABBREVIATION:TKT#ECNUMBERS:2.2.1.1# LOCUS:_8277#TRANSCRIPT:1#ABBREVIATION:TKTL1# LOCUS:_84076#TRANSCRIPT:1#ABBREVIATION:DKFZP434L1717# proteins: NAME:transketolase#ABBREVIATION:Tkt.1# NAME:transketolase#ABBREVIATION:Tktl1.1# NAME:similar to transketolase#ABBREVIATION:Dkfzp434l1717.1# citations: none GENE ASSOCIATION: (7086.1) or (84076.1) or (8277.1)
Decanoyl-CoA (n-C10:0CoA)_C31H50N7O17P3S
Abbreviation: R_ACACT5p Synonyms: _0 EC Number: 2.3.1.16 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : 3oddcoa + coa --> accoa + dcacoa Confidence Level: 4 NCD genes: LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# proteins: NAME:acetyl-Coenzyme A acyltransferase 1#ABBREVIATION:Acaa1.1# citations: AUTHORS:Bout A, Franse MM, Collins J, Blonden L, Tager JM, Benne R.#TITLE:Characterization of the gene encoding human peroxisomal 3-oxoacyl-CoA thiolase (ACAA). No large DNA rearrangement in a thiolase-deficient patient.#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1679347# GENE ASSOCIATION: (30.1)
acetyl-CoA C-acyltransferase (decanoyl-CoA), peroxisomal
N-Acetyl-L-glutamyl 5-phosphate_C7H9NO8P
fatty acid 11-cis-retinol_C20H29OFULLR2CO
Abbreviation: R_EX_13_DASH_cis_DASH_retnglc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 13-cis-retnglc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_glc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : glc-D <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACITL Synonyms: _0 SUBSYSTEM: Citric Acid Cycle Equation: [c] : atp + cit + coa --> accoa + adp + oaa + pi Confidence Level: 0 ATP dependent citrate lyase. Reaction is reversible in KEGG (new EC 2.3.3.8) NCD genes: LOCUS:_47#TRANSCRIPT:1#ABBREVIATION:ACLY#ECNUMBERS:4.1.3.8# LOCUS:_47#TRANSCRIPT:2#ABBREVIATION:ACLY#ECNUMBERS:4.1.3.8# proteins: NAME:ATP citrate lyase#ABBREVIATION:Acly.1# NAME:ATP citrate lyase#ABBREVIATION:Acly.2# citations: AUTHORS:Elshourbagy NA, Near JC, Kmetz PJ, Sathe GM, Southan C, Strickler JE, Gross M, Young JF, Wells TN, Groot PH.#TITLE:Rat ATP citrate-lyase. Molecular cloning and sequence analysis of a full-length cDNA and mRNA abundance as a function of diet, organ, and age#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2295639# GENE ASSOCIATION: (47.1) or (47.2)
Abbreviation: R_AMETtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: amet[c] <==> amet[n] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
S-adenosyl-L-methionine nuclear transport
Abbreviation: R_ILEtec Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ile-L[e] <==> ile-L[c] Confidence Level: 4 SAB genes: LOCUS:_124935#TRANSCRIPT:1#ABBREVIATION:SLC43A2# LOCUS:_8501#TRANSCRIPT:1#ABBREVIATION:SLC43A1# proteins: NAME:solute carrier family 43, member 2#ABBREVIATION:Slc43a2.1# NAME:solute carrier family 43, member 1#ABBREVIATION:Slc43a1.1# citations: AUTHORS:Bodoy S, Martin L, Zorzano A, Palacin M, Estevez R, Bertran J#TITLE:Identification of LAT4, a novel amino acid transporter with system L activity#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15659399# AUTHORS:Babu E, Kanai Y, Chairoungdua A, Kim do K, Iribe Y, Tangtrongsup S, Jutabha P, Li Y, Ahmed N, Sakamoto S, Anzai N, Nagamori S, Endou H#TITLE:Identification of a novel system L amino acid transporter structurally distinct from heterodimeric amino acid transporters#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12930836# AUTHORS:Hediger MA, Romero MF, Peng JB, Rolfs A, Takanaga H, Bruford EA#TITLE:The ABCs of solute carriers: physiological, pathological and therapeutic implications of human membrane transport proteinsIntroduction#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14624363# GENE ASSOCIATION: (8501.1) or (124935.1)
L-isoleucine transport via diffusion (extracellular to cytosol)
Abbreviation: R_TETPENT6CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: tetpent6crn[c] --> tetpent6crn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_EX_pheacgln_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pheacgln <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
alpha-N-Phenylacetyl-L-glutamine exchange
Abbreviation: R_ACONT Synonyms: _0 EC Number: 4.2.1.3 SUBSYSTEM: Citric Acid Cycle Equation: [c] : cit <==> icit Confidence Level: 0 SMP genes: LOCUS:_48#TRANSCRIPT:1#ABBREVIATION:ACO1#ECNUMBERS:4.2.1.3# LOCUS:_3658#TRANSCRIPT:1#ABBREVIATION:IREB2# proteins: NAME:aconitase 1, soluble#ABBREVIATION:Aco1.1# NAME:iron-responsive element binding protein#ABBREVIATION:Ireb2.1# citations: none GENE ASSOCIATION: (3658.1) or (48.1)
Abbreviation: R_ACACT1r Synonyms: _0 EC Number: 2.3.1.9 SUBSYSTEM: Tryptophan metabolism Equation: [c] : (2) accoa <==> aacoa + coa Confidence Level: 0 JLR- reversible form of ACACT1 genes: LOCUS:_39#TRANSCRIPT:1#ABBREVIATION:ACAT2#ECNUMBERS:2.3.1.9# proteins: NAME:acetyl-Coenzyme A acetyltransferase 2 (acetoacetyl Coenzyme A thiolase)#ABBREVIATION:Acat2.1# citations: none GENE ASSOCIATION: (39.1)
Abbreviation: R_ME1m Synonyms: _0 EC Number: 1.1.1.38 SUBSYSTEM: Pyruvate Metabolism Equation: [m] : mal-L + nad --> co2 + nadh + pyr Confidence Level: 0 NCD genes: LOCUS:_4200#TRANSCRIPT:1#ABBREVIATION:ME2#ECNUMBERS:1.1.1.40# proteins: NAME:malic enzyme 2, NAD(+)-dependent, mitochondrial#ABBREVIATION:Me2.1-m# citations: none GENE ASSOCIATION: (4200.1)
S-Adenosyl-L-methionine:amine N-methyltransferase (srtn)
Abbreviation: R_SRTNMTX Synonyms: _0 EC Number: 2.1.1.49 SUBSYSTEM: Tryptophan metabolism Equation: [c] : amet + srtn --> ahcys + h + nmthsrtn Confidence Level: 1 SAB seratonin methyltransferase genes: LOCUS:_11185#TRANSCRIPT:1#ABBREVIATION:INMT# proteins: NAME:indolethylamine N-methyltransferase#ABBREVIATION:Inmt.1# citations: none GENE ASSOCIATION: (11185.1)
Abbreviation: R_PIt8 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (1.5) na1[e] + pi[e] <==> (1.5) na1[c] + pi[c] Confidence Level: 4 MM genes: LOCUS:_6575#TRANSCRIPT:1#ABBREVIATION:SLC20A2# LOCUS:_6574#TRANSCRIPT:1#ABBREVIATION:SLC20A1# proteins: NAME:solute carrier family 20 (phosphate transporter), member 1, extracellular #ABBREVIATION:Slc20a1.1-e# NAME:solute carrier family 20 (phosphate transporter), member 1, extracellular #ABBREVIATION:Slc20a2.1-e# citations: AUTHORS:Collins JF, Bai L, Ghishan FK.#TITLE:The SLC20 family of proteins: dual functions as sodium-phosphate cotransporters and viral receptors.#YEAR:2003#PUBMED:12759754# GENE ASSOCIATION: (6574.1) or (6575.1)
phosphate transport in/out via Na+ symporter
glycine-cleavage system (lipoamide) irreversible, mitochondrial
Abbreviation: R_GCC2bim Synonyms: _0 EC Number: 2.1.2.10 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : alpam + thf --> dhlam + mlthf + nh4 Confidence Level: 4 NCD genes: LOCUS:_2731#TRANSCRIPT:1#ABBREVIATION:GLDC#ECNUMBERS:1.4.4.2# LOCUS:_275#TRANSCRIPT:1#ABBREVIATION:AMT#ECNUMBERS:2.1.2.10# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_2653#TRANSCRIPT:1#ABBREVIATION:GCSH# proteins: NAME:glycine dehydrogenase (decarboxylating; glycine decarboxylase, glycine cleavage system protein P), mitochondrial #ABBREVIATION:Gldc.1-m# NAME:aminomethyltransferase (glycine cleavage system protein T), mitochondrial #ABBREVIATION:Amt.1-m# NAME:glycine cleavage system protein H (aminomethyl carrier) #ABBREVIATION:Gcsh.1-m# NAME:dihydrolipoamide dehydrogenase, mitochondrial#ABBREVIATION:Dld.1-m# citations: AUTHORS:Lee HH, Kim do J, Ahn HJ, Ha JY, Suh SW.#TITLE:Crystal structure of T-protein of the glycine cleavage system. Cofactor binding, insights into H-protein recognition, and molecular basis for understanding nonketotic hyperglycinemia.#YEAR:2004#PUBMED:15355973# GENE ASSOCIATION: (275.1) and (1738.1) and (2731.1) and (2653.1)
Abbreviation: R_GLUPRT Synonyms: _0 EC Number: 2.4.2.14 SUBSYSTEM: IMP Biosynthesis Equation: [c] : gln-L + h2o + prpp --> glu-L + ppi + pram Confidence Level: 4 genes: LOCUS:_5471#TRANSCRIPT:1#ABBREVIATION:PPAT#ECNUMBERS:2.4.2.14# proteins: NAME:phosphoribosyl pyrophosphate amidotransferase#ABBREVIATION:Ppat.1# citations: AUTHORS:Chen S, Nagy PL, Zalkin H#TITLE:Role of NRF-1 in bidirectional transcription of the human GPAT-AIRC purine biosynthesis locus.#JOURNAL: Nucleic Acids Res. #YEAR:1997#PUBMED:9108165# AUTHORS:Iwahana H, Oka J, Mizusawa N, Kudo E, Ii S, Yoshimoto K, Holmes EW, Itakura M. #TITLE:Molecular cloning of human amidophosphoribosyltransferase. #JOURNAL:Biochem Biophys Res Commun.#YEAR:1993#PUBMED:8380692# AUTHORS:Brayton KA, Chen Z, Zhou G, Nagy PL, Gavalas A, Trent JM, Deaven LL, Dixon JE, Zalkin H. #TITLE:Two genes for de novo purine nucleotide synthesis on human chromosome 4 are closely linked and divergently transcribed.#JOURNAL:J Biol Chem. #YEAR:1994#PUBMED:8106516# GENE ASSOCIATION: (5471.1)
glutamine phosphoribosyldiphosphate amidotransferase
Abbreviation: R_EX_hdcea_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hdcea <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CARVEOLte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: carveol[e] <==> carveol[c] Confidence Level: 1 unknown mechanism, xenobiotic transport (original and metabolized) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
N-Acetyl-D-glucosamine 1-phosphate_C8H14NO9P
Abbreviation: R_UAGDP Synonyms: _0 EC Number: 2.7.7.23 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acgam1p + h + utp --> ppi + uacgam Confidence Level: 4 genes: LOCUS:_6675#TRANSCRIPT:1#ABBREVIATION:UAP1#ECNUMBERS:2.7.7.23# LOCUS:_91373#TRANSCRIPT:1#ABBREVIATION:UAP1L1# proteins: NAME:UDP-N-acteylglucosamine pyrophosphorylase 1#ABBREVIATION:Uap1.1# NAME:UDP-N-acteylglucosamine pyrophosphorylase 1-like 1#ABBREVIATION:Uap1l1.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Mio T, Yabe T, Arisawa M, Yamada-Okabe H#TITLE:The eukaryotic UDP-N-acetylglucosamine pyrophosphorylases. Gene cloning, protein expression, and catalytic mechanism#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9603950# GENE ASSOCIATION: (6675.1) or (91373.1)
Abbreviation: R_H2Otly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: h2o[c] <==> h2o[l] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I biosynthesis, precursor 16_C157H253N11O124S4X
keratan sulfate I biosynthesis, precursor 17_C157H252N11O127S5X
Abbreviation: R_S6T8g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre16 + paps --> h + ksi_pre17 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_EX_nifedipine_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : nifedipine <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_dopasf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dopasf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
heparin-glucosamine 3-O-sulfotransferase
heparan sulfate, precursor 15_C79H114N5O98S11X
heparan sulfate, precursor 14_C79H115N5O95S10X
Abbreviation: R_S3T2g Synonyms: _5 EC Number: 2.8.2.23 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre14 + paps --> h + hs_pre15 + pap Confidence Level: 4 NCD genes: LOCUS:_9956#TRANSCRIPT:1#ABBREVIATION:HS3ST2#ECNUMBERS:2.8.2.23# LOCUS:_222537#TRANSCRIPT:1#ABBREVIATION:HS3ST5# LOCUS:_9951#TRANSCRIPT:1#ABBREVIATION:HS3ST4#ECNUMBERS:2.8.2.23# proteins: NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 2, Golgi apparatus#ABBREVIATION:Hs3st2.1-g# NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 4, Golgi apparatus#ABBREVIATION:Hs3st4.1-g# NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 5, Golgi apparatus#ABBREVIATION:Hs3st5.1-g# citations: AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Shworak NW, Liu J, Petros LM, Zhang L, Kobayashi M, Copeland NG, Jenkins NA, Rosenberg RD#TITLE:Multiple isoforms of heparan sulfate D-glucosaminyl 3-O-sulfotransferase. Isolation, characterization, and expression of human cdnas and identification of distinct genomic loci#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9988767# AUTHORS:Xia G, Chen J, Tiwari V, Ju W, Li JP, Malmstrom A, Shukla D, Liu J#TITLE:Heparan sulfate 3-O-sulfotransferase isoform 5 generates both an antithrombin-binding site and an entry receptor for herpes simplex virus, type 1#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12138164# AUTHORS:Mochizuki H, Yoshida K, Gotoh M, Sugioka S, Kikuchi N, Kwon YD, Tawada A, Maeyama K, Inaba N, Hiruma T, Kimata K, Narimatsu H#TITLE:Characterization of a heparan sulfate 3-O-sulfotransferase-5, an enzyme synthesizing a tetrasulfated disaccharide#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12740361# AUTHORS:Liu J, Shworak NW, Sinay P, Schwartz JJ, Zhang L, Fritze LM, Rosenberg RD#TITLE:Expression of heparan sulfate D-glucosaminyl 3-O-sulfotransferase isoforms reveals novel substrate specificities#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9988768# GENE ASSOCIATION: (9956.1) or (9951.1) or (222537.1)
Abbreviation: R_C4STMO2r Synonyms: _2 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : 4mzym_int2 + nad + o2 --> co2 + h + nadh + zym_int2 Confidence Level: 4 double check rxn specifics - see lit ref: Marijanovic, Molecular Endocrinology 2003 17:1715-1725 ER version NJ genes: LOCUS:_6307#TRANSCRIPT:1#ABBREVIATION:SC4MOL# LOCUS:_50814#TRANSCRIPT:1#ABBREVIATION:NSDHL# proteins: NAME:sterol-C4-methyl oxidase#ABBREVIATION:Sc4mol.1# NAME:NAD(P) dependent steroid dehydrogenase#ABBREVIATION:Nsdhl.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (6307.1) or (50814.1)
_4-Methylzymosterol intermediate 2_C28H44O
Abbreviation: R_B3GNT315g Synonyms: _9 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acglc13galacglcgal14acglcgalgluside_hs + udpgal --> galacglc13galacglcgal14acglcgalgluside_hs + h + udp Confidence Level: 2 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3#ABBREVIATION:B3gnt3.1# citations: AUTHORS:Yokoyama-Kobayashi M, Yamaguchi T, Sekine S, Kato S.#TITLE:Selection of cDNAs encoding putative type II membrane proteins on the cell surface from a human full-length cDNA bank.#JOURNAL:Gene#YEAR:1999#PUBMED:10072769# GENE ASSOCIATION: (10331.1)
Procollagen 5-hydroxy-L-lysine_C6H14N2O3
R group 5 Coenzyme A homo sapiens_XCO2C21H31N7O15P3S
heparan sulfate, degradation product 12_C55H82N3O64S6
Abbreviation: R_EX_s2l2fn2m2masn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : s2l2fn2m2masn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate D (GlcNAc6S-GlcA2S) proteoglycan_C45H64N2O51S5X
Abbreviation: R_ADEt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ade[e] <==> ade[c] Confidence Level: 4 NCD genes: LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Yao SY, Ng AM, Vickers MF, Sundaram M, Cass CE, Baldwin SA, Young JD.#TITLE:Functional and molecular characterization of nucleobase transport by recombinant human and rat equilibrative nucleoside transporters 1 and 2.#JOURNAL:J Biol Chem. #YEAR:2002#PUBMED:12006583# GENE ASSOCIATION: (3177.1)
keratan sulfate I biosynthesis, precursor 2_C87H142N6O62X
docosa-4,7,10,13,16-pentaenoyl coenzyme A_C43H64N7O17P3S
docosa-4,7,10,13,16-pentaenoyl carnitine_C29H47NO4
Abbreviation: R_DCSPTN1CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + dcsptn1crn --> crn + dcsptn1coa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Octadecanoyl-ACP (n-C18:0ACP)_C29H55N2O8PRS
Abbreviation: R_3MOX4HOXPGALDOX_LPAREN_NADP_RPAREN_ Synonyms: _0 EC Number: 1.2.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 3m4hpga + h2o + nadp <==> 3mox4hoxm + (2) h + nadph Confidence Level: 2 SAB genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (222.1) or (220.1) or (221.1) or (218.1)
_3-Methoxy-4-hydroxyphenylglycolaldehyde:NADP+ oxidoreductase
Abbreviation: R_TCHOLAt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) na1[e] + tchola[e] --> (2) na1[c] + tchola[c] Confidence Level: 4 electrogenic uptake NJ genes: LOCUS:_6555#TRANSCRIPT:1#ABBREVIATION:SLC10A2# LOCUS:_6554#TRANSCRIPT:1#ABBREVIATION:SLC10A1# proteins: NAME:solute carrier family 10 (sodium/bile acid cotransporter family), member 1#ABBREVIATION:Slc10a1.1# NAME:solute carrier family 10 (sodium/bile acid cotransporter family), member 2#ABBREVIATION:Slc10a2.1# citations: AUTHORS:Hagenbuch B, Dawson P.#TITLE:The sodium bile salt cotransport family SLC10.#JOURNAL:Pflugers Arch.#YEAR:2004#PUBMED:12851823# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:Molecular cloning, chromosomal localization, and functional characterization of a human liver Na+/bile acid cotransporter.#JOURNAL:J Clin Invest.#YEAR:1994#PUBMED:8132774# AUTHORS:Oelkers P, Kirby LC, Heubi JE, Dawson PA.#TITLE:Primary bile acid malabsorption caused by mutations in the ileal sodium-dependent bile acid transporter gene (SLC10A2).#JOURNAL:J Clin Invest.#YEAR:1997#PUBMED:9109432# AUTHORS:Wong MH, Oelkers P, Dawson PA.#TITLE:Identification of a mutation in the ileal sodium-dependent bile acid transporter gene that abolishes transport activity.#JOURNAL:J Biol Chem.#YEAR:1995#PUBMED:7592981# GENE ASSOCIATION: (6554.1) or (6555.1)
taurocholate transport via sodium cotransport
Abbreviation: R_SUCCt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: pi[m] + succ[c] <==> pi[c] + succ[m] Confidence Level: 4 TDV genes: LOCUS:_1468#TRANSCRIPT:1#ABBREVIATION:SLC25A10# proteins: NAME:Solute carrier family 25 (dicarboxylate transporter) member 10, mitochondrial #ABBREVIATION:Slc25a10.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (1468.1)
Abbreviation: R_METS Synonyms: _0 EC Number: 2.1.1.13 SUBSYSTEM: Methionine Metabolism Equation: [c] : 5mthf + hcys-L --> h + met-L + thf Confidence Level: 4 genes: LOCUS:_4548#TRANSCRIPT:1#ABBREVIATION:MTR#ECNUMBERS:2.1.1.13# proteins: NAME:_5-methyltetrahydrofolate-homocysteine methyltransferase #ABBREVIATION:Metr.1# citations: none GENE ASSOCIATION: (4548.1)
Abbreviation: R_PHACCOAGLNAC Synonyms: _0 EC Number: 2.3.1.14 SUBSYSTEM: Phenylalanine metabolism Equation: [c] : gln-L + phaccoa --> coa + pheacgln Confidence Level: 2 SAB genes: none proteins: none citations: AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# GENE ASSOCIATION:
Phenylacetyl-CoA:L-glutamine alpha-N-phenylacetyltransferase
Abbreviation: R_TRDRm Synonyms: _0 EC Number: 1.8.1.9 SUBSYSTEM: Nucleotides Equation: [m] : h + nadph + trdox --> nadp + trdrd Confidence Level: 4 NCD genes: LOCUS:_10587#TRANSCRIPT:1#ABBREVIATION:TXNRD2# LOCUS:_10587#TRANSCRIPT:2#ABBREVIATION:TXNRD2#ECNUMBERS:1.8.1.9# LOCUS:_10587#TRANSCRIPT:3#ABBREVIATION:TXNRD2#ECNUMBERS:1.8.1.9# LOCUS:_114112#TRANSCRIPT:1#ABBREVIATION:TXNRD3# proteins: NAME:thioredoxin reductase 2 #ABBREVIATION:Txnrd2.1-m# NAME:thioredoxin reductase 2 #ABBREVIATION:Txnrd2.2-m# NAME:thioredoxin reductase 2#ABBREVIATION:Txnrd2.3-m# NAME:thioredoxin reductase 3 #ABBREVIATION:Txnrd3.1-m# citations: AUTHORS:Kim MR, Chang HS, Kim BH, Kim S, Baek SH, Kim JH, Lee SR, Kim JR.#TITLE:Involvements of mitochondrial thioredoxin reductase (TrxR2) in cell proliferation.#JOURNAL:Biochem Biophys Res Commun#YEAR:2003#PUBMED:12705894# AUTHORS:Miranda-Vizuete A, Damdimopoulos AE, Pedrajas JR, Gustafsson JA, Spyrou G.#TITLE:Human mitochondrial thioredoxin reductase cDNA cloning, expression and genomic organization. #JOURNAL:Eur J Biochem.#YEAR:1999#PUBMED:10215850# AUTHORS:Sun QA, Kirnarsky L, Sherman S, Gladyshev VN.#TITLE:Selenoprotein oxidoreductase with specificity for thioredoxin and glutathione systems.#JOURNAL:Proc Natl Acad Sci U S A. #YEAR:2001#PUBMED:11259642# GENE ASSOCIATION: (114112.1) or (10587.1) or (10587.3) or (10587.2)
Abbreviation: R_EX_acn13acngalgbside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acn13acngalgbside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
sialyl (1,3) sialyl (2,6) galactosylgloboside (homo sapiens) exchange
_5alpha-Dihydrotestosterone glucuronide_C25H38O8
Abbreviation: R_UGT1A9r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Steroid Metabolism Equation: [r] : 5adtststerone + udpglcur --> 5adtststeroneglc + udp Confidence Level: 4 NJ genes: LOCUS:_54490#TRANSCRIPT:1#ABBREVIATION:UGT2B28# proteins: NAME:UDP glycosyltransferase 2 family, polypeptide B28#ABBREVIATION:Ugt2b28.1# citations: AUTHORS:Levesque E, Turgeon D, Carrier JS, Montminy V, Beaulieu M, Belanger A.#TITLE:Isolation and characterization of the UGT2B28 cDNA encoding a novel human steroid conjugating UDP-glucuronosyltransferase.#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11300766# GENE ASSOCIATION: (54490.1)
Abbreviation: R_ESTRADIOLGLCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: estradiolglc[e] + hco3[c] <==> estradiolglc[c] + hco3[e] Confidence Level: 4 NJ genes: LOCUS:_53919#TRANSCRIPT:1#ABBREVIATION:SLCO1C1# LOCUS:_28234#TRANSCRIPT:1#ABBREVIATION:SLCO1B3# LOCUS:_6579#TRANSCRIPT:1#ABBREVIATION:SLCO1A2# LOCUS:_28231#TRANSCRIPT:1#ABBREVIATION:SLCO4A1# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1A2#ABBREVIATION:Slco1a2.1# NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# NAME:solute carrier organic anion transporter family, member 1B3#ABBREVIATION:Slco1b3.1# NAME:solute carrier organic anion transporter family, member 4A1#ABBREVIATION:Slco4a1.1# NAME:solute carrier organic anion transporter family, member 1C1#ABBREVIATION:Slco1c1.1# citations: AUTHORS:Kullak-Ublick GA, Beuers U, Meier PJ, Domdey H, Paumgartner G.#TITLE:Assignment of the human organic anion transporting polypeptide (OATP) gene to chromosome 12p12 by fluorescence in situ hybridization.#JOURNAL:J Hepatol.#YEAR:1996#PUBMED:9007731# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (28234.1) or (28231.1) or (10599.1) or (6579.1) or (53919.1)
estradiol glucuronide transport via bicarbonate countertransport
beta-ureidopropionase (D-3-amino-isobutanoate forming)
Abbreviation: R_BUP2 Synonyms: _0 EC Number: 3.5.1.6 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : 3uib + (2) h + h2o --> 3aib-D + co2 + nh4 Confidence Level: 4 MM genes: LOCUS:_51733#TRANSCRIPT:1#ABBREVIATION:UPB1# proteins: NAME:ureidopropionase, beta#ABBREVIATION:Upb1.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Sakamoto T, Sakata SF, Matsuda K, Horikawa Y, Tamaki N.#TITLE:Expression and properties of human liver beta-ureidopropionase.#JOURNAL:J Nutr Sci Vitaminol (Tokyo). #YEAR:2001#PUBMED:11508704# AUTHORS:Kakimoto Y, Taniguchi K, Sano I.#TITLE:D-beta-aminoisobutyrate:pyruvate aminotransferase in mammalian liver and excretion of beta-aminoisobutyrate by man.#YEAR:1969#PUBMED:5773299# GENE ASSOCIATION: (51733.1)
GD1beta (homo sapiens)_C66H111N4O38FULLRCO
Tyr-194 of apo-glycogenin protein (primer for glycogen synthesis)_XOH
primed glycogenin (glycogenin-8[1,4-Glc])_C48H81O41X
Abbreviation: R_GGNG Synonyms: _0 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : Tyr-ggn + (8) udpg --> ggn + (8) h + (8) udp Confidence Level: 4 NCD genes: LOCUS:_2998#TRANSCRIPT:1#ABBREVIATION:GYS2#ECNUMBERS:2.4.1.11# LOCUS:_2997#TRANSCRIPT:1#ABBREVIATION:GYS1#ECNUMBERS:2.4.1.11# LOCUS:_2992#TRANSCRIPT:1#ABBREVIATION:GYG# LOCUS:_8908#TRANSCRIPT:1#ABBREVIATION:GYG2# proteins: NAME:glycogenin#ABBREVIATION:Gyg.1# NAME:glycogenin 2#ABBREVIATION:Gyg2.1# NAME:glycogen synthase 1 (muscle)#ABBREVIATION:Gys1.1# NAME:glycogen synthase 2 (liver)#ABBREVIATION:Gys2.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Barbetti F, Rocchi M, Bossolasco M, Cordera R, Sbraccia P, Finelli P, Consalez GG.#TITLE:The human skeletal muscle glycogenin gene: cDNA, tissue expression and chromosomal localization#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8602861# AUTHORS:Mu J, Skurat AV, Roach PJ#TITLE:Glycogenin-2, a novel self-glucosylating protein involved in liver glycogen biosynthesis#JOURNAL:J Biol Chem#YEAR:1997# AUTHORS:Mu J, Roach PJ#TITLE:Characterization of human glycogenin-2, a self-glucosylating initiator of liver glycogen metabolism#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9857012# AUTHORS:Lomako J, Lomako WM, Whelan WJ#TITLE:Glycogenin: the primer for mammalian and yeast glycogen synthesis#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15238248# GENE ASSOCIATION: (8908.1) and (2998.1) or (2998.1) and (2992.1) or (2997.1) and (2992.1) or (8908.1) and (2997.1)
Abbreviation: R_BTNtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: btn[c] <==> btn[n] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Pispa J.#TITLE:Animal biotinidase.#JOURNAL:Ann Med Exp Biol Fenn. #YEAR:1965#PUBMED:5867120# AUTHORS:Zempleni J., Mock D.M.#TITLE:Biotin homeostasis during the cell cycle #JOURNAL:Nutrition Research Reviews#YEAR:2001# AUTHORS:Hymes J, Fleischhauer K, Wolf B.#TITLE:Biotinylation of biotinidase following incubation with biocytin.#JOURNAL:Clin Chim Acta#YEAR:1995#PUBMED:7758201# AUTHORS:Hymes J, Fleischhauer K, Wolf B.#TITLE:Biotinylation of histones by human serum biotinidase: assessment of biotinyl-transferase activity in sera from normal individuals and children with biotinidase deficiency.#JOURNAL:Biochem Mol Med.#YEAR:1995#PUBMED:8593541# AUTHORS:Zempleni J.#TITLE:Uptake, localization, and noncarboxylase roles of biotin.#JOURNAL:Annu Rev Nutr#YEAR:2005#PUBMED:16011464# GENE ASSOCIATION:
Abbreviation: R_AMETr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: amet[c] <==> amet[r] Confidence Level: 1 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
S-Adenosyl-L-methionine intracellular diffusion
Abbreviation: R_G5SDym Synonyms: _0 EC Number: 1.2.1.41 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : glu5p + h + nadph --> glu5sa + nadp + pi Confidence Level: 2 SAB m TDV: name change from G5SDm genes: LOCUS:_5832#TRANSCRIPT:1#ABBREVIATION:ALDH18A1# proteins: NAME:aldehyde dehydrogenase 18 family, member A1#ABBREVIATION:Aldh18a1.1-m# citations: AUTHORS:Hu CA, Lin WW, Obie C, Valle D#TITLE:Molecular enzymology of mammalian Delta1-pyrroline-5-carboxylate synthase. Alternative splice donor utilization generates isoforms with different sensitivity to ornithine inhibition#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10037775# GENE ASSOCIATION: (5832.1)
glutamate-5-semialdehyde dehydrogenase (m)
Abbreviation: R_FRDPtr Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: frdp[x] <==> frdp[r] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). See also Krisans (PMID: 14713247, PMID: 12121718) for synthesis of cholesterol pathway (why FRDP needs to be transported between ER and peroxisome) NJ genes: none proteins: none citations: AUTHORS:Kovacs WJ, Olivier LM, Krisans SK.#TITLE:Central role of peroxisomes in isoprenoid biosynthesis.#JOURNAL:Prog Lipid Res.#YEAR:2002#PUBMED:12121718# AUTHORS:Kovacs WJ, Krisans S.#TITLE:Cholesterol biosynthesis and regulation: role of peroxisomes.#JOURNAL:Adv Exp Med Biol.#YEAR:2003#PUBMED:14713247# AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
phosphatidylserine (homo sapiens)_C6H11NO6PFULLRCO2FULLR2CO2
Abbreviation: R_PSSA1_hs Synonyms: _0 EC Number: 2.7.8.8 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : pchol_hs + ser-L <==> chol + ps_hs Confidence Level: 4 phosphatidylcholine as substrate NJ genes: LOCUS:_9791#TRANSCRIPT:1#ABBREVIATION:PTDSS1# proteins: NAME:phosphatidylserine synthase 1#ABBREVIATION:PTDSS1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION: (9791.1)
Phosphatidylserine synthase homo sapiens
Abbreviation: R_FACOAL240 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + lgnc <==> amp + lgnccoa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_CYTK4 Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [c] : dcmp + gtp <==> dcdp + gdp Confidence Level: 4 genes: LOCUS:_26289#TRANSCRIPT:1#ABBREVIATION:AK5# LOCUS:_26289#TRANSCRIPT:2#ABBREVIATION:AK5# proteins: NAME:adenylate kinase 5 #ABBREVIATION:Ak5.1# NAME:adenylate kinase 5 #ABBREVIATION:Ak5.2# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Identification of a novel human adenylate kinase. cDNA cloning, expression analysis, chromosome localization and characterization of the recombinant protein. #JOURNAL:Eur J Biochem. #YEAR:1999#PUBMED:10215863# GENE ASSOCIATION: (26289.1) or (26289.2)
Abbreviation: R_ARTFR57 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (3) fadh2[m] + (3) h[m] + (3) nadph[m] + tethex3coa[c] --> (1.5) R5coa_hs[c] + (3) fad[m] + (3) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
GM1alpha (homo sapiens)_C55H95N3O30FULLRCO
Abbreviation: R_PA_HStg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: pa_hs[c] <==> pa_hs[g] Confidence Level: 2 for assumed reversible, non-energy dep scramblase mechanism (PMID: 10856717). NJ genes: none proteins: none citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# GENE ASSOCIATION:
Abbreviation: R_KYN3OX Synonyms: _3 EC Number: 1.14.13.9 SUBSYSTEM: Tryptophan metabolism Equation: [c] : Lkynr + h + nadph + o2 --> h2o + hLkynr + nadp Confidence Level: 4 NCD genes: LOCUS:_8564#TRANSCRIPT:1#ABBREVIATION:KMO#ECNUMBERS:1.14.13.9# proteins: NAME:kynurenine 3-monooxygenase#ABBREVIATION:Kmo.1# citations: AUTHORS:Alberati-Giani D, Cesura AM, Broger C, Warren WD, Rover S, Malherbe P#TITLE:Cloning and functional expression of human kynurenine 3-monooxygenase#JOURNAL:FEBS Lett#YEAR:1997#PUBMED:9237672# GENE ASSOCIATION: (8564.1)
Abbreviation: R_EX_omeprazole_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : omeprazole <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_lys_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lys-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_3-mercaptolactate-cysteine disulfide_C6H10O5S2N
Abbreviation: R_ST8SIA55g Synonyms: _13 EC Number: 2.4.99.8 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gq1c_hs --> cmp + gp1calpha_hs + h Confidence Level: 4 NJ genes: LOCUS:_29906#TRANSCRIPT:1#ABBREVIATION:SIAT8E# proteins: NAME:ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 5 #ABBREVIATION:St8sia5.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Sandhoff K, Kolter T#TITLE:Biosynthesis and degradation of mammalian glycosphingolipids#JOURNAL:Phil Trans R Soc Lond B#YEAR:2003# AUTHORS:Kim YJ, Kim KS, Do S, Kim CH, Kim SK, Lee YC.#TITLE:Molecular cloning and expression of human alpha2,8-sialyltransferase (hST8Sia V).#JOURNAL:Biochem Biophys Res Commun.#YEAR:1997#PUBMED:9199191# GENE ASSOCIATION: (29906.1)
GP1c alpha (homo sapiens)_C99H159N7O62FULLRCO
Abbreviation: R_EICOSTETCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: eicostetcrn[c] --> eicostetcrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_DMHPTCRNCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + dmhptcrn <==> crn + dmhptcoa Confidence Level: 2 made reversible since Keq ~ 0.6. dmhptcoa is a special case because its biochemical fate has not be elucidated. NJ genes: none proteins: none citations: AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# AUTHORS:Verhoeven NM, Wanders RJ, Poll-The BT, Saudubray JM, Jakobs C.#TITLE:The metabolism of phytanic acid and pristanic acid in man: a review.#JOURNAL:J Inherit Metab Dis#YEAR:1998#PUBMED:9819701# GENE ASSOCIATION:
_2,6 dimethylheptanoly CoA carnitine transferase
L-4-Hydroxyglutamate semialdehyde_C5H9NO4
L-4-hydroxyglutamate semialdehyde dehydrogenase, mitochondrial
Abbreviation: R_4HGLSDm Synonyms: _0 EC Number: 1.5.1.12 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : 4hglusa + h2o + nad <==> e4hglu + (2) h + nadh Confidence Level: 1 NCD genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: AUTHORS:Knight J, Holmes RP#TITLE:Mitochondrial hydroxyproline metabolism: implications for primary hyperoxaluria#JOURNAL:Am J Nephrol#YEAR:2005#PUBMED:15849464# GENE ASSOCIATION: (8659.1) or (8659.2)
phosphatidylglycerol (homo sapiens)_C6H12O6PFULLRCO2FULLR2CO2
Abbreviation: R_EX_dmhptcrn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dmhptcrn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_2,6 dimethylheptanoyl carnitine exchange
Abbreviation: R_EX_nrpphrsf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : nrpphrsf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Sulfate derivative of norepinephrine exchange
Itaconate--CoA ligase (GDP-forming), mitochondrial
Abbreviation: R_ITCOAL1m Synonyms: _1 EC Number: 6.2.1.4 SUBSYSTEM: C5-Branched dibasic acid metabolism Equation: [m] : coa + gtp + itacon <==> gdp + itaccoa + pi Confidence Level: 2 NCD genes: LOCUS:_8802#TRANSCRIPT:1#ABBREVIATION:SUCLG1#ECNUMBERS:6.2.1.4# LOCUS:_8801#TRANSCRIPT:1#ABBREVIATION:SUCLG2#ECNUMBERS:6.2.1.4# proteins: NAME:Succinate-CoA ligase (GDP-forming), mitochondiral#ABBREVIATION:Suclg-m# citations: AUTHORS:WANG SF, ADLER J, LARDY HA#TITLE:The pathway of itaconate metabolism by liver mitochondria#JOURNAL:J Biol Chem#YEAR:1961#PUBMED:13783048# GENE ASSOCIATION: (8802.1 and 8801.1)
Dolichyl beta-D-glucosyl phosphate, human uterine homolog_C1085H1780O90P10
Abbreviation: R_CRNCARtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: acrn[x] + crn[c] --> acrn[c] + crn[x] Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
carnitine-acetylcarnitine carrier, peroxisomal
Abbreviation: R_LDH_Lm Synonyms: _0 EC Number: 1.1.1.27 SUBSYSTEM: Pyruvate Metabolism Equation: [m] : lac-L + nad <==> h + nadh + pyr Confidence Level: 4 This follows the convention of L-LACtm Also see LDL_D. genes: LOCUS:_3939#TRANSCRIPT:1#ABBREVIATION:LDHA#ECNUMBERS:1.1.1.27# proteins: NAME:lactate dehydrogenase A (liver, sk muscle)#ABBREVIATION:Ldha.1# citations: AUTHORS:Brooks GA, Dubouchaud H, Brown M, Sicurello JP, Butz CE.#TITLE:Role of mitochondrial lactate dehydrogenase and lactate oxidation in the intracellular lactate shuttle.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1999#PUBMED:9927705# AUTHORS:Ewaschuk JB, Naylor JM, Zello GA#TITLE:D-lactate in human and ruminant metabolism#JOURNAL:J Nutr#YEAR:2005#PUBMED:15987839# GENE ASSOCIATION: (3939.1)
phosphatidyl glycerol phosphate (homo sapiens)_C6H11O9P2FULLRCO2FULLR2CO2
Abbreviation: R_FUCACGALFUCGALACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucacgalfucgalacglcgalgluside_hs[g] <==> fucacgalfucgalacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PPAn Synonyms: _3 EC Number: 3.6.1.1 SUBSYSTEM: Miscellaneous Equation: [n] : h2o + ppi --> h + (2) pi Confidence Level: 1 genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TETPENT3CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + tetpent3coa --> coa + tetpent3crn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_2OXOADPTm Synonyms: _0 SUBSYSTEM: Tryptophan metabolism Equation: _2oxoadp[c] + akg[m] <==> 2oxoadp[m] + akg[c] Confidence Level: 4 SAB PMID 11083877 genes: LOCUS:_89874#TRANSCRIPT:1#ABBREVIATION:SLC25A21# proteins: NAME:solute carrier family 25 (mitochondrial oxodicarboxylate carrier), member 21#ABBREVIATION:Slc25a21.1-m# citations: AUTHORS:Fiermonte G, Dolce V, Palmieri L, Ventura M, Runswick MJ, Palmieri F, Walker JE#TITLE:Identification of the human mitochondrial oxodicarboxylate carrier. Bacterial expression, reconstitution, functional characterization, tissue distribution, and chromosomal location#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11083877# GENE ASSOCIATION: (89874.1)
_2-oxoadipate shuttle (cytosol/mitochondria)
Abbreviation: R_VITD3t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: vitd3[c] --> vitd3[e] Confidence Level: 2 I am not sure how vitamin D3 is released from Skin cells based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_ADK1m Synonyms: _0 EC Number: 2.7.4.3 SUBSYSTEM: Nucleotides Equation: [m] : amp + atp <==> (2) adp Confidence Level: 4 NCD genes: LOCUS:_205#TRANSCRIPT:1#ABBREVIATION:AK3#ECNUMBERS:2.7.4.10# LOCUS:_204#TRANSCRIPT:1#ABBREVIATION:AK2#ECNUMBERS:2.7.4.3# LOCUS:_204#TRANSCRIPT:3#ABBREVIATION:AK2#ECNUMBERS:2.7.4.3# LOCUS:_204#TRANSCRIPT:2#ABBREVIATION:AK2#ECNUMBERS:2.7.4.3# LOCUS:_205#TRANSCRIPT:3#ABBREVIATION:AK3#ECNUMBERS:2.7.4.10# LOCUS:_205#TRANSCRIPT:2#ABBREVIATION:AK3#ECNUMBERS:2.7.4.10# proteins: NAME:adenylate kinase 2 #ABBREVIATION:Ak2.1-m# NAME:adenylate kinase 2 #ABBREVIATION:Ak2.2-m# NAME:adenylate kinase 2 #ABBREVIATION:Ak2.3-m# NAME:adenylate kinase 3 #ABBREVIATION:Ak3.1-m# NAME:adenylate kinase 3 #ABBREVIATION:Ak3.2-m# NAME:adenylate kinase 3 #ABBREVIATION:Ak3.3-m# citations: AUTHORS:Noma T, Song S, Yoon YS, Tanaka S, Nakazawa A. #TITLE:cDNA cloning and tissue-specific expression of the gene encoding human adenylate kinase isozyme 2. #JOURNAL:Biochim Biophys Acta. #YEAR:1998#PUBMED:9434148# AUTHORS:Lee Y, Kim JW, Lee IA, Kang HB, Choe YK, Lee HG, Lim JS, Kim HJ, Park C, Choe IS.#TITLE:Cloning and characterization of cDNA for human adenylate kinase 2A.#JOURNAL:Biochem Mol Biol Int. #YEAR:1996#PUBMED:8843353# AUTHORS:Bruns GA, Regina VM.#TITLE:Adenylate kinase 2, a mitochondrial enzyme.#JOURNAL:Biochem Genet.#YEAR:1977#PUBMED:195572# AUTHORS:Noma T, Fujisawa K, Yamashiro Y, Shinohara M, Nakazawa A, Gondo T, Ishihara T, Yoshinobu K.#TITLE:Structure and expression of human mitochondrial adenylate kinase targeted to the mitochondrial matrix. #JOURNAL:Biochem J.#YEAR:2001#PUBMED:11485571# GENE ASSOCIATION: (204.3) or (204.1) or (205.1) or (205.3) or (205.2) or (204.2)
Abbreviation: R_P4507B11r Synonyms: _3 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : h + nadph + o2 + xol25oh --> h2o + nadp + xoltri25 Confidence Level: 4 NJ - added, substrates by analogy to other xol hydroxylase reactions (NADH in general can be used in place of NADPH) genes: LOCUS:_9420#TRANSCRIPT:1#ABBREVIATION:CYP7B1# proteins: NAME:cytochrom P450, family 7, subfamily B, polypeptide 1#ABBREVIATION:Cyp7b1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (9420.1)
Abbreviation: R_GLCNACT1g Synonyms: _3 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs,hs_linkage + uacgam --> h + hs_pre1 + udp Confidence Level: 4 EC 2.4.1.223 NCD genes: LOCUS:_2135#TRANSCRIPT:1#ABBREVIATION:EXTL2# LOCUS:_2137#TRANSCRIPT:1#ABBREVIATION:EXTL3#ECNUMBERS:2.4.1.-# proteins: NAME:exostoses (multiple)-like 2, Golgi apparatus#ABBREVIATION:Extl2.1-g# NAME:exostoses (multiple)-like 3, Golgi apparatus#ABBREVIATION:Extl3.1-g# citations: AUTHORS:Wuyts W, Van Hul W, Hendrickx J, Speleman F, Wauters J, De Boulle K, Van Roy N, Van Agtmael T, Bossuyt P, Willems PJ#TITLE:Identification and characterization of a novel member of the EXT gene family, EXTL2#JOURNAL:Eur J Hum Genet#YEAR:1997#PUBMED:9450183# AUTHORS:Saito T, Seki N, Yamauchi M, Tsuji S, Hayashi A, Kozuma S, Hori T#TITLE:Structure, chromosomal location, and expression profile of EXTR1 and EXTR2, new members of the multiple exostoses gene family#JOURNAL:Biochem Biophys Res Commun#YEAR:1998#PUBMED:9473480# AUTHORS:Kitagawa H, Shimakawa H, Sugahara K.#TITLE:The tumor suppressor EXT-like gene EXTL2 encodes an alpha1, 4-N-acetylhexosaminyltransferase that transfers N-acetylgalactosamine and N-acetylglucosamine to the common glycosaminoglycan-protein linkage region.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10318803# AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Kim BT, Kitagawa H, Tamura J, Saito T, Kusche-Gullberg M, Lindahl U, Sugahara K#TITLE:Human tumor suppressor EXT gene family members EXTL1 and EXTL3 encode alpha 1,4- N-acetylglucosaminyltransferases that likely are involved in heparan sulfate/ heparin biosynthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390981# AUTHORS:Van Hul W, Wuyts W, Hendrickx J, Speleman F, Wauters J, De Boulle K, Van Roy N, Bossuyt P, Willems PJ#TITLE:Identification of a third EXT-like gene (EXTL3) belonging to the EXT gene family#JOURNAL:Genomics#YEAR:1998#PUBMED:9479495# GENE ASSOCIATION: (2137.1) or (2135.1)
chondroitin sulfate/heparan sulfate linkage region (GlcA-(Gal)2-Xyl-L-Ser (protein))_C23H36O20X
heparan sulfate, precursor 1_C31H49NO25X
alpha-N-acetylglucosaminyltransferase I, Golgi apparatus
Dehydrodolichol diphosphate, human liver homolog_C1080H1738O70P20
trans,trans,cis-Geranylgeranyl diphosphate_C20H33O7P2
Abbreviation: R_GGT_L Synonyms: _1 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (17.6) ipdp + ttc-ggdp --> (0.1) dedoldp_L + (17.6) ppi Confidence Level: 0 EC 2.5.1.- NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CYTK2 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : atp + dcmp <==> adp + dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_ASPt6 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asp-L[e] + h[e] + k[c] + (3) na1[e] --> asp-L[c] + h[c] + k[e] + (3) na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2003 genes: LOCUS:_6511#TRANSCRIPT:1#ABBREVIATION:SLC1A6# LOCUS:_6507#TRANSCRIPT:1#ABBREVIATION:SLC1A3# LOCUS:_6506#TRANSCRIPT:1#ABBREVIATION:SLC1A2# LOCUS:_6505#TRANSCRIPT:1#ABBREVIATION:SLC1A1# LOCUS:_6512#TRANSCRIPT:1#ABBREVIATION:SLC1A7# proteins: NAME:solute carrier family 1 (neuronal/epithelial high affinity glutamate transporter, system Xag), member 1#ABBREVIATION:Slc1a1.1# NAME:solute carrier family 1 (glial high affinity glutamate transporter), member 2#ABBREVIATION:Slc1a2.1# NAME:solute carrier family 1 (glial high affinity glutamate transporter), member 3#ABBREVIATION:Slc1a3.1# NAME:solute carrier family 1 (high affinity aspartate/glutamate transporter), member 6#ABBREVIATION:Slc1a6.1# NAME:solute carrier family 1 (glutamate transporter), member 7#ABBREVIATION:Slc1a7.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6511.1) or (6505.1) or (6507.1) or (6506.1) or (6512.1)
L-aspartate transport via Na, H symport and K antiport
Abbreviation: R_PI4PP Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail4p_hs --> pail_hs + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Holub BJ#TITLE:Metabolism and function of myo-inositol and inositol phospholipids#JOURNAL:Annu Rev Nutr#YEAR:1986#PUBMED:2425833# GENE ASSOCIATION:
phosphatidylinositol-4-phosphate 4-phosphatase
Abbreviation: R_OMPDC Synonyms: _0 EC Number: 4.1.1.23 SUBSYSTEM: Pyrimidine Biosynthesis Equation: [c] : h + orot5p --> co2 + ump Confidence Level: 4 genes: LOCUS:_7372#TRANSCRIPT:1#ABBREVIATION:UMPS#ECNUMBERS:2.4.2.10,,4.1.1.23# proteins: NAME:uridine monophosphate synthetase (orotate phosphoribosyl transferase and orotidine-5'-decarboxylase) #ABBREVIATION:Umps.1# citations: AUTHORS:Patterson D, Jones C, Morse H, Rumsby P, Miller Y, Davis R. #TITLE:Structural gene coding for multifunctional protein carrying orotate phosphoribosyltransferase and OMP decarboxylase activity is located on long arm of human chromosome 3.#JOURNAL:Somatic Cell Genet#YEAR:1983#PUBMED:6574608# AUTHORS:McClard RW, Black MJ, Livingstone LR, Jones ME.#TITLE:Isolation and initial characterization of the single polypeptide that synthesizes uridine 5'-monophosphate from orotate in Ehrlich ascites carcinoma. Purification by tandem affinity chromatography of uridine-5'-monophosphate synthase.#JOURNAL:Biochemistry#YEAR:1980#PUBMED:6893554# AUTHORS:Suttle DP, Bugg BY, Winkler JK, Kanalas JJ.#TITLE:Molecular cloning and nucleotide sequence for the complete coding region of human UMP synthase. #JOURNAL:Proc Natl Acad Sci U S A#YEAR:1988#PUBMED:3279416# AUTHORS:Suchi M, Mizuno H, Kawai Y, Tsuboi T, Sumi S, Okajima K, Hodgson ME, Ogawa H, Wada Y. #TITLE:Molecular cloning of the human UMP synthase gene and characterization of point mutations in two hereditary orotic aciduria families.#JOURNAL:Am J Hum Genet.#YEAR:1997#PUBMED:9042911# GENE ASSOCIATION: (7372.1)
Abbreviation: R_ADAe Synonyms: _0 EC Number: 3.5.4.4 SUBSYSTEM: Nucleotides Equation: [e] : adn + h + h2o --> ins + nh4 Confidence Level: 4 adenosine deaminase has been found on cell surface in human (Gonzales-Gronow et al, JBC, 2004,279,20993-20998). IT genes: LOCUS:_100#TRANSCRIPT:1#ABBREVIATION:ADA#ECNUMBERS:3.5.4.4# proteins: NAME:adenosine deaminase #ABBREVIATION:Ada.1# citations: AUTHORS:Gonzalez-Gronow M, Hershfield MS, Arredondo-Vega FX, Pizzo SV.#TITLE:Cell surface adenosine deaminase binds and stimulates plasminogen activation on 1-LN human prostate cancer cells.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15016824# AUTHORS:Iwaki-Egawa S, Watanabe Y.#TITLE:Characterization and purification of adenosine deaminase 1 from human and chicken liver.#JOURNAL:Comp Biochem Physiol B Biochem Mol Biol.#YEAR:2002#PUBMED:12381379# GENE ASSOCIATION: (100.1)
Abbreviation: R_DNDPt23m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgdp[m] + dtdp[c] --> dgdp[c] + dtdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_FACOAL180i Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + ocdca --> amp + ppi + stcoa Confidence Level: 4 TV catalyzed by acyl-CoA synthetases genes: LOCUS:_51703#TRANSCRIPT:1#ABBREVIATION:ACSL5# LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# LOCUS:_2181#TRANSCRIPT:1#ABBREVIATION:ACSL3# LOCUS:_2181#TRANSCRIPT:2#ABBREVIATION:ACSL3# LOCUS:_22305#TRANSCRIPT:1#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# LOCUS:_22305#TRANSCRIPT:2#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.1# NAME:acyl-CoA synthetase long-chain family member 5#ABBREVIATION:Acsl5.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.2# NAME:acyl-CoA synthetase long-chain family member 6#ABBREVIATION:Acsl6.1# NAME:acyl-CoA synthetase long-chain family member 6 #ABBREVIATION:Acsl6.2# citations: none GENE ASSOCIATION: (22305.2) or (2181.2) or (51703.1) or (2181.1) or (2180.1) or (22305.1)
Abbreviation: R_DOLGPP_Uer Synonyms: _3 EC Number: 3.1.4.48 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolglcp_U + h2o --> (0.1) dolp_U + glc-D + h Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION:
Dolichyl-beta-D-glucosyl-phosphate dolichylphosphohydrolase (uterus)
_2-keto-3-deoxy-D-glycero-D-galactononic acid 9-phosphate_C9H14O12P
Abbreviation: R_ATPH2e Synonyms: _5 EC Number: 3.6.1.5 SUBSYSTEM: Nucleotides Equation: [e] : adp + h2o --> amp + h + pi Confidence Level: 4 IT genes: LOCUS:_953#TRANSCRIPT:1#ABBREVIATION:ENTPD1# LOCUS:_956#TRANSCRIPT:1#ABBREVIATION:ENTPD3# LOCUS:_955#TRANSCRIPT:1#ABBREVIATION:ENTPD6# LOCUS:_954#TRANSCRIPT:1#ABBREVIATION:ENTPD2# LOCUS:_954#TRANSCRIPT:2#ABBREVIATION:ENTPD2# LOCUS:_957#TRANSCRIPT:1#ABBREVIATION:ENTPD5# proteins: NAME:ectonucleoside triphosphate diphosphohydrolase 1 #ABBREVIATION:Entpd1.1# NAME:ectonucleoside triphosphate diphosphohydrolase 6#ABBREVIATION:Entpd6.1# NAME:ectonucleoside triphosphate diphosphohydrolase 3 #ABBREVIATION:Entpd3.1# NAME:ectonucleoside triphosphate diphosphohydrolase 2 #ABBREVIATION:Entpd2.1# NAME:ectonucleoside triphosphate diphosphohydrolase 2 #ABBREVIATION:Entpd2.2# NAME:ectonucleoside triphosphate diphosphohydrolase 5 #ABBREVIATION:Entpd5.1# citations: AUTHORS:Kaczmarek E, Koziak K, Sevigny J, Siegel JB, Anrather J, Beaudoin AR, Bach FH, Robson SC.#TITLE:Identification and characterization of CD39/vascular ATP diphosphohydrolase.#JOURNAL:J Biol Chem.#YEAR:1996#PUBMED:8955160# AUTHORS:Chadwick BP, Frischauf AM.#TITLE:The CD39-like gene family: identification of three new human members (CD39L2, CD39L3, and CD39L4), their murine homologues, and a member of the gene family from Drosophila melanogaster. #JOURNAL:Genomics#YEAR:1998#PUBMED:9676430# GENE ASSOCIATION: (954.2) or (954.1) or (953.1) or (956.1) or (955.1) or (957.1)
keratan sulfate II (core 2-linked), degradation product 9_C30H50N3O20X
keratan sulfate II (core 2-linked), degradation product 8_C36H60N3O25X
Abbreviation: R_GALASE18ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core2_deg8 --> gal + ksii_core2_deg9 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
(alpha-D-mannosyl)4-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine (protein)_C46H77N2O35X
Abbreviation: R_MM5bg Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m5masnB1 --> m4masn + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
Abbreviation: R_GTPCIn Synonyms: _0 EC Number: 3.5.4.16 SUBSYSTEM: Tetrahydrobiopterin Equation: [n] : gtp + h2o --> ahdt + for + h Confidence Level: 4 The reaction involves hydrolysis of two C-N bonds and isomerization of the pentose unit; the recyclization may be non-enzymic genes: LOCUS:_2643#TRANSCRIPT:1#ABBREVIATION:GCH1#ECNUMBERS:3.5.4.16# proteins: NAME:GTP cyclohydrolase 1 (dopa-responsive dystonia) #ABBREVIATION:Gch1.1-n# citations: AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (2643.1)
Abbreviation: R_ARTFR43 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + vacccoa[c] --> (1.125) R4coa_hs[c] + fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_INSt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ins[e] + na1[e] --> ins[c] + na1[c] Confidence Level: 4 MM genes: LOCUS:_9153#TRANSCRIPT:1#ABBREVIATION:SLC28A2# LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 2, extracellular #ABBREVIATION:Slc28a2.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (9153.1) or (64078.1)
Abbreviation: R_FAOXC2242046m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : adrncoa + coa + fad + h2o + nad --> accoa + arachdcoa + fadh2 + h + nadh Confidence Level: 4 _1 round beta ox peroxisomal (very long chain FA) c22:4,n-6 -> c20:4, n-6 - final '6' in abbreviation indicates omega6 FA 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_LCYSTCBOXL Synonyms: _0 EC Number: 4.1.1.15 SUBSYSTEM: Taurine and hypotaurine metabolism Equation: [c] : Lcyst + h --> co2 + taur Confidence Level: 4 SAB genes: LOCUS:_2572#TRANSCRIPT:1#ABBREVIATION:GAD2#ECNUMBERS:4.1.1.15# LOCUS:_2571#TRANSCRIPT:1#ABBREVIATION:GAD1#ECNUMBERS:4.1.1.15# LOCUS:_51380#TRANSCRIPT:1#ABBREVIATION:CSAD# LOCUS:_2571#TRANSCRIPT:2#ABBREVIATION:GAD1#ECNUMBERS:4.1.1.15# proteins: NAME:glutamate decarboxylase 1 (brain, 67kDa)#ABBREVIATION:Gad1.1# NAME:glutamate decarboxylase 1 (brain, 67kDa)#ABBREVIATION:Gad1.2# NAME:glutamate decarboxylase 2 (pancreatic islets and brain, 65kDa)#ABBREVIATION:Gad2.1# NAME:cysteine sulfinic acid decarboxylase#ABBREVIATION:Csad.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (51380.1) or (2571.1) or (2572.1) or (2571.2)
Abbreviation: R_EX_pro_DASH_D_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pro-D <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EHGLAT Synonyms: _0 EC Number: 2.6.1.1 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : akg + e4hglu --> 4h2oglt + glu-L Confidence Level: 1 NCD genes: LOCUS:_2805#TRANSCRIPT:1#ABBREVIATION:GOT1#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase #ABBREVIATION:Got1.1# citations: none GENE ASSOCIATION: (2805.1)
L-erythro-4-Hydroxyglutamate:2-oxoglutarate aminotransferase
Abbreviation: R_RADH3 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : h2o + nad + retinal-cis-13 <==> 13-cis-retn + (2) h + nadh Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Blaner WS.#TITLE:Cellular metabolism and actions of 13-cis-retinoic acid. #JOURNAL:J Am Acad Dermatol. #YEAR:2001#PUBMED:11606944# GENE ASSOCIATION:
sialyl (2,3) sialyl (2,6) galactosylgloboside (homo sapiens)_C72H121N4O43FULLRCO
Alpha-N-acetylgalactosaminide alpha-2,6-sialyltransferase 2
Abbreviation: R_ST6GALNAC22 Synonyms: _5 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : acngalgbside_hs + cmpacna --> acn23acngalgbside_hs + cmp + h Confidence Level: 2 NJ genes: LOCUS:_10610#TRANSCRIPT:1#ABBREVIATION:SIAT7B#ECNUMBERS:2.4.99.7# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 2#ABBREVIATION:St6galnac2.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10610.1)
Abbreviation: R_EX_paf_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : paf_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_1-alkyl 2-acteylglycerol 3-phosphocholine (homo sapiens) exchange
Abbreviation: R_B3GNT12g Synonyms: _10 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacglcgal14acglcgalgluside_hs + uacgam --> acglc13galacglcgal14acglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# citations: AUTHORS:Isshiki S, Kudo T, Nishihara S, Ikehara Y, Togayachi A, Furuya A, Shitara K, Kubota T, Watanabe M, Kitajima M, Narimatsu H.#TITLE:Lewis type 1 antigen synthase (beta3Gal-T5) is transcriptionally regulated by homeoproteins.#YEAR:2003#PUBMED:12855703# GENE ASSOCIATION: (10678.1) or (10678.2)
UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1
Abbreviation: R_EX_ahandrostanglc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ahandrostanglc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Etiocholan-3alpha-ol-17-one 3-glucuronide exchange
Abbreviation: R_MMCDp Synonyms: _3 EC Number: 4.1.1.41 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [x] : h + mmcoa-S --> co2 + ppcoa Confidence Level: 1 JLR peroxisomal isoform - NCD genes: LOCUS:_23417#TRANSCRIPT:1#ABBREVIATION:MLYCD#ECNUMBERS:4.1.1.9# proteins: NAME:malonyl-CoA decarboxylase, peroxisomal#ABBREVIATION:Mlycd.1-p# citations: AUTHORS:Kerner J, Hoppel CL.#TITLE:Radiochemical malonyl-CoA decarboxylase assay: activity and subcellular distribution in heart and skeletal muscle.#JOURNAL:Anal Biochem#YEAR:2002#PUBMED:12123667# AUTHORS:Wightman PJ, Santer R, Ribes A, Dougherty F, McGill N, Thorburn DR, FitzPatrick DR#TITLE:MLYCD mutation analysis: evidence for protein mistargeting as a cause of MLYCD deficiency#JOURNAL:Hum Mutat#YEAR:2003#PUBMED:12955715# GENE ASSOCIATION: (23417.1)
Methylmalonyl-CoA decarboxylase, peroxisomal
keratan sulfate I, degradation product 30_C90H147N6O75S3
omega hydroxy hexadecanoate (n-C16:0)_C16H31O3
Abbreviation: R_WHHDCAte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: whhdca[e] <==> whhdca[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_H2Oter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: h2o[c] <==> h2o[r] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACGALtlg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acgal[g] <==> acgal[c] Confidence Level: 2 unknown mechanism - may be vesicular NJ genes: none proteins: none citations: none GENE ASSOCIATION:
N-acetyl-galactosamine intracellular transport
Abbreviation: R_GALGLUSIDEtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: galgluside_hs[c] <==> galgluside_hs[l] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
R total 3 Coenzyme A_CO2FULLR3C21H31N7O15P3S
heparan sulfate, degradation product 22_C37H56NO35S
Abbreviation: R_FUMSO4tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: fum[c] + so4[m] <==> fum[m] + so4[c] Confidence Level: 4 MM genes: LOCUS:_1468#TRANSCRIPT:1#ABBREVIATION:SLC25A10# proteins: NAME:Solute carrier family 25 (dicarboxylate transporter) member 10, mitochondrial #ABBREVIATION:Slc25a10.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# AUTHORS:Crompton M, Palmieri F, Capano M, Quagliariello E.#TITLE:The transport of sulphate and sulphite in rat liver mitochondria. #YEAR:1974#PUBMED:4441366# GENE ASSOCIATION: (1468.1)
Fumarate:sulfate antiport, mitochondrial
glycogen, structure 5 (glycogenin-2[1,4-Glc])_C12H21O11X
glycogen, structure 4 (glycogenin-1,6-{2[1,4-Glc], [1,4-Glc]})_C18H31O16X
Abbreviation: R_O16G1e Synonyms: _0 EC Number: 3.2.1.10 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [e] : glygn4 + h2o --> glc-D + glygn5 Confidence Level: 0 NCD genes: LOCUS:_6476#TRANSCRIPT:1#ABBREVIATION:SI#ECNUMBERS:3.2.1.48,3.2.1.10# proteins: NAME:sucrase-isomaltase (alpha-glucosidase), extracellular (sm intestine)#ABBREVIATION:Si.1-e# citations: none GENE ASSOCIATION: (6476.1)
oligo-1,6-glucosidase (glygn4 -> glygn5), extracellular
Abbreviation: R_UGT1A8r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Steroid Metabolism Equation: [r] : estriol + udpglcur --> estriolglc + udp Confidence Level: 4 NJ genes: LOCUS:_7363#TRANSCRIPT:1#ABBREVIATION:UGT2B4#ECNUMBERS:2.4.1.17# LOCUS:_54575#TRANSCRIPT:1#ABBREVIATION:UGT1A10# LOCUS:_54490#TRANSCRIPT:1#ABBREVIATION:UGT2B28# LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A10#ABBREVIATION:Ugt1a10.1# NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# NAME:UDP glycosyltransferase 2 family, polypeptide B28#ABBREVIATION:Ugt2b28.1# NAME:UDP glycosyltransferase 2 family, polypeptide B4#ABBREVIATION:Ugt2b4.1# citations: AUTHORS:Basu NK, Kubota S, Meselhy MR, Ciotti M, Chowdhury B, Hartori M, Owens IS.#TITLE:Free Full Text Gastrointestinally distributed UDP-glucuronosyltransferase 1A10, which metabolizes estrogens and nonsteroidal anti-inflammatory drugs, depends upon phosphorylation.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117964# AUTHORS:Mackenzie PI, Owens IS, Burchell B, Bock KW, Bairoch A, Belanger A, Fournel-Gigleux S, Green M, Hum DW, Iyanagi T, Lancet D, Louisot P, Magdalou J, Chowdhury JR, Ritter JK, Schachter H, Tephly TR, Tipton KF, Nebert DW.#TITLE:The UDP glycosyltransferase gene superfamily: recommended nomenclature update based on evolutionary divergence.#JOURNAL:Pharmacogenetics#YEAR:1997#PUBMED:9295054# AUTHORS:Levesque E, Turgeon D, Carrier JS, Montminy V, Beaulieu M, Belanger A.#TITLE:Isolation and characterization of the UGT2B28 cDNA encoding a novel human steroid conjugating UDP-glucuronosyltransferase.#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11300766# AUTHORS:Jin CJ, Miners JO, Lillywhite KJ, Mackenzie PI.#TITLE:cDNA cloning and expression of two new members of the human liver UDP-glucuronosyltransferase 2B subfamily.#JOURNAL:Biochem Biophys Res Commun#YEAR:1993#PUBMED:8333863# GENE ASSOCIATION: (7363.1) or (54575.1) or (54490.1) or (54576.1)
keratan sulfate I, degradation product 19_C146H236N10O124S6
tetracosapentaenoyl coenzyme A, n-6_C45H68N7O17P3S
Abbreviation: R_ARTFR54 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (3) fadh2[m] + (2) h[m] + (2) nadph[m] + tetpent6coa[c] --> (1.5) R5coa_hs[c] + (3) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_4HBZFm Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 4hbzcoa + h2o --> 4hbz + coa + h Confidence Level: 2 IT reaction involved in formation of 4-hydroxybenzoate from tyrosine. genes: none proteins: none citations: AUTHORS:Meganathan R.#TITLE:Ubiquinone biosynthesis in microorganisms.#JOURNAL:FEMS Microbiol Lett. #YEAR:2001#PUBMED:11583838# AUTHORS:Loscher R, Heide L.#TITLE:Biosynthesis of p-Hydroxybenzoate from p-Coumarate and p-Coumaroyl-Coenzyme A in Cell-Free Extracts of Lithospermum erythrorhizon Cell Cultures. #JOURNAL:Plant Physiol.#YEAR:1994#PUBMED:12232327# AUTHORS:Booth, A. N., Masri, M. S., Robbins, D. J., Emerson, O. H., Jones, F. T. & DeEds, F.#TITLE:Urinary Phenolic Acid Metabolites of Tyrosine#JOURNAL:J. Biol. Chem#YEAR:1960# GENE ASSOCIATION:
Abbreviation: R_ACOAHi Synonyms: _0 EC Number: 3.1.2.1 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : accoa + h2o --> ac + coa + h Confidence Level: 0 NCD genes: LOCUS:_134526#TRANSCRIPT:1#ABBREVIATION:CACH-1# proteins: NAME:cytosolic acetyl-CoA hydrolase#ABBREVIATION:Cach-1.1# citations: none GENE ASSOCIATION: (134526.1)
Abbreviation: R_MEOHtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: meoh[c] <==> meoh[l] Confidence Level: 1 diffusion NCD genes: none proteins: none citations: none GENE ASSOCIATION:
cholesterol ester (from FULLR2)_C27H45FULLR2CO2
Abbreviation: R_PPA2m Synonyms: _3 EC Number: 3.6.1.1 SUBSYSTEM: Others Equation: [m] : h2o + pppi --> h + pi + ppi Confidence Level: 1 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SOAT12r Synonyms: _9 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : R2coa_hs + chsterol --> coa + xolest_hs Confidence Level: 4 SOAT11 and SOAT12 are templates, customized rxns can be formed particular ester moieties. NJ genes: LOCUS:_6646#TRANSCRIPT:1#ABBREVIATION:SOAT1#ECNUMBERS:2.3.1.26# proteins: NAME:sterol O-acetyltransferase (acyl-Coenzyme A: cholesterol acyltransferase) 1#ABBREVIATION:Soat1.1# citations: AUTHORS:Guo ZY, Chang CC, Lu X, Chen J, Li BL, Chang TY.#TITLE:The disulfide linkage and the free sulfhydryl accessibility of acyl-coenzyme A:cholesterol acyltransferase 1 as studied by using mPEG5000-maleimide.#JOURNAL:Biochemistry#YEAR:2005#PUBMED:15850387# AUTHORS:Chang CC, Huh HY, Cadigan KM, Chang TY.#TITLE:Molecular cloning and functional expression of human acyl-coenzyme A:cholesterol acyltransferase cDNA in mutant Chinese hamster ovary cells.#JOURNAL:J Biol Chem.#YEAR:1993#PUBMED:8407899# GENE ASSOCIATION: (6646.1)
sterol O-acyltransferase (acyl-Coenzyme A: cholesterol acyltransferase) 1
phosphoethanolaminyl-trimannosyl-glucosaminyl-acylphosphatidylinositol (H6')_C51H94N2O32P2FULLRCO2FULLR2CO2
phosphatidylethanolamine (homo sapiens)_C5H12NO4PFULLRCO2FULLR2CO2
Abbreviation: R_H4ET3er Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : m3gacpail_hs + pe_hs --> dag_hs + em3gacpail_hs Confidence Level: 4 NCD genes: LOCUS:_5281#TRANSCRIPT:1#ABBREVIATION:PIGF# LOCUS:_84720#TRANSCRIPT:1#ABBREVIATION:PIGO# LOCUS:_84720#TRANSCRIPT:2#ABBREVIATION:PIGO# LOCUS:_5281#TRANSCRIPT:2#ABBREVIATION:PIGF# proteins: NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.1-r# NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.2-r# NAME:phosphatidylinositol glycan, class O, endoplasmic reticulum#ABBREVIATION:Pigo.1-r# NAME:phosphatidylinositol glycan, class O, endoplasmic reticulum#ABBREVIATION:Pigo.2-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Hong Y, Maeda Y, Watanabe R, Inoue N, Ohishi K, Kinoshita T#TITLE:Requirement of PIG-F and PIG-O for transferring phosphoethanolamine to the third mannose in glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781593# AUTHORS:Inoue N, Kinoshita T, Orii T, Takeda J#TITLE:Cloning of a human gene, PIG-F, a component of glycosylphosphatidylinositol anchor biosynthesis, by a novel expression cloning strategy#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8463218# GENE ASSOCIATION: (84720.2) and (5281.1) or (84720.1) and (5281.2) or (5281.1) and (84720.1) or (84720.2) and (5281.2)
H4 phosphoethanolaminyl transferase, endoplasmic reticulum
Abbreviation: R_AGLPED Synonyms: _0 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : ak2gpe_hs + h + nadph + o2 --> dak2gpe_hs + (2) h2o + nadp Confidence Level: 0 NJ genes: none proteins: none citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Brites P, Waterham HR, Wander RJA#TITLE:Functions and biosynthesis of plasmalogens in health and disease#JOURNAL:Biochimica et Biophysica Acta#YEAR:2004# AUTHORS:Nagan N, Zoeller RA#TITLE:Plasmalogens: biosynthesis and functions#JOURNAL:Progress in Lipid Research#YEAR:2001# GENE ASSOCIATION:
_1-alkenyl 2-acylglycerol 3-phosphoethanolamine plasmalogen (homo sapiens)_C7H14O5NPFULLRFULLR2CO
alkyl acyl glycerophosphoethanolamine desaturase
Abbreviation: R_ASNtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: asn-L[c] --> asn-L[m] Confidence Level: 2 TDV genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ST3GAL61g Synonyms: _4 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : cmpacna + gal14acglcgalgluside_hs --> acngal14acglcgalgluside_hs + cmp + h Confidence Level: 4 NJ genes: LOCUS:_10402#TRANSCRIPT:1#ABBREVIATION:SIAT10# proteins: NAME:sialyltransferase 10 (alpha-2,3-sialyltransferase VI)#ABBREVIATION:Siat10.1# citations: AUTHORS:Okajima T, Fukumoto S, Miyazaki H, Ishida H, Kiso M, Furukawa K, Urano T, Furukawa K.#TITLE:Molecular cloning of a novel alpha2,3-sialyltransferase (ST3Gal VI) that sialylates type II lactosamine structures on glycoproteins and glycolipids.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10206952# GENE ASSOCIATION: (10402.1)
Type 2 lactosamine alpha-2,3-sialyltransferase
Abbreviation: R_MEPIVESSte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) atp[c] + (2) h2o[c] + (3) mepi[c] --> (2) adp[c] + (2) h[c] + (3) mepi[e] + (2) pi[c] Confidence Level: 2 metanephrine vesicular, ATP dependent transport - no strict evidence for this mode, added by analogy to adrnl vesicular transport reaction (both catechols that must be secreted out of the cell). NJ genes: none proteins: none citations: none GENE ASSOCIATION:
metanephrine secretion via secretory vesicle (ATP driven)
chondroitin sulfate C (GalNAc6S-GlcA), degradation product 5_C23H36O24S
chondroitin sulfate C (GalNAc6S-GlcA), degradation product 4_C31H49NO29S
Abbreviation: R_NACHEX5ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_c_deg4 + h2o --> acgal + cs_c_deg5 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1) or (3074.1)
Abbreviation: R_P4502C92 Synonyms: _5 SUBSYSTEM: Limonene and pinene degradation Equation: [c] : h + limnen + nadph + o2 --> carveol + h2o + nadp Confidence Level: 4 assumed to take place on outer membrane NJ genes: LOCUS:_1557#TRANSCRIPT:1#ABBREVIATION:CYP2C19#ECNUMBERS:1.14.14.1# LOCUS:_1559#TRANSCRIPT:1#ABBREVIATION:CYP2C9#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 2, subfamily C, polypeptide 19#ABBREVIATION:Cyp2c19.1# NAME:cytochrome P450, family 2, subfamily C, polypeptide 9#ABBREVIATION:Cyp2c9.1# citations: AUTHORS:Rendic S.#TITLE:Summary of information on human CYP enzymes: human P450 metabolism data.#JOURNAL:Drug Metab Rev#YEAR:2002#PUBMED:11996015# AUTHORS:Miyazawa M, Shindo M, Shimada T.#TITLE:Metabolism of (+)- and (-)-limonenes to respective carveols and perillyl alcohols by CYP2C9 and CYP2C19 in human liver microsomes.#JOURNAL:Drug Metab Dispos#YEAR:2002#PUBMED:11950794# GENE ASSOCIATION: (1559.1) or (1557.1)
Abbreviation: R_6HTSTSTERONEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _6htststerone[c] <==> 6htststerone[e] Confidence Level: 2 unknown mechanism, inactivated testosterone needs to be exported NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UGT1A3r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Steroid Metabolism Equation: [r] : andrstrn + udpglcur --> andrstrnglc + udp Confidence Level: 4 NJ genes: LOCUS:_7367#TRANSCRIPT:1#ABBREVIATION:UGT2B17# LOCUS:_7364#TRANSCRIPT:1#ABBREVIATION:UGT2B7#ECNUMBERS:2.4.1.17# LOCUS:_54575#TRANSCRIPT:1#ABBREVIATION:UGT1A10# LOCUS:_54490#TRANSCRIPT:1#ABBREVIATION:UGT2B28# LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A10#ABBREVIATION:Ugt1a10.1# NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# NAME:UDP glycosyltransferase 2 family, polypeptide B28#ABBREVIATION:Ugt2b28.1# NAME:UDP glycosyltransferase 2 family, polypeptide B7#ABBREVIATION:Ugt2b7.1# NAME:UDP glycosyltransferase 2 family, polypeptide B17#ABBREVIATION:Ugt2b17.1# citations: AUTHORS:Basu NK, Kubota S, Meselhy MR, Ciotti M, Chowdhury B, Hartori M, Owens IS.#TITLE:Free Full Text Gastrointestinally distributed UDP-glucuronosyltransferase 1A10, which metabolizes estrogens and nonsteroidal anti-inflammatory drugs, depends upon phosphorylation.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117964# AUTHORS:Mackenzie PI, Owens IS, Burchell B, Bock KW, Bairoch A, Belanger A, Fournel-Gigleux S, Green M, Hum DW, Iyanagi T, Lancet D, Louisot P, Magdalou J, Chowdhury JR, Ritter JK, Schachter H, Tephly TR, Tipton KF, Nebert DW.#TITLE:The UDP glycosyltransferase gene superfamily: recommended nomenclature update based on evolutionary divergence.#JOURNAL:Pharmacogenetics#YEAR:1997#PUBMED:9295054# AUTHORS:Levesque E, Turgeon D, Carrier JS, Montminy V, Beaulieu M, Belanger A.#TITLE:Isolation and characterization of the UGT2B28 cDNA encoding a novel human steroid conjugating UDP-glucuronosyltransferase.#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11300766# AUTHORS:Belanger A, Pelletier G, Labrie F, Barbier O, Chouinard S.#TITLE:Inactivation of androgens by UDP-glucuronosyltransferase enzymes in humans.#JOURNAL:Trends Endocrinol Metab.#YEAR:2003#PUBMED:14643063# GENE ASSOCIATION: (7364.1) or (54575.1) or (54490.1) or (54576.1) or (7367.1)
Abbreviation: R_EX_4nph_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 4nph <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] <==> na1[c] Confidence Level: 4 genes: LOCUS:_6528#TRANSCRIPT:1#ABBREVIATION:SLC5A5# LOCUS:_6523#TRANSCRIPT:1#ABBREVIATION:SLC5A1# LOCUS:_6526#TRANSCRIPT:1#ABBREVIATION:SLC5A3# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 1, extracellular#ABBREVIATION:Slc5a1.1-e# NAME:solute carrier family 5 (inositol transporters), member 3, extracellular#ABBREVIATION:Slc5a3.1-e# NAME:solute carrier family 5 (sodium iodide symporter), member 5, extacellular#ABBREVIATION:Slc5a5.1-e# citations: AUTHORS:Hirayama BA, Loo DD, Wright EM.#TITLE:Protons drive sugar transport through the Na+/glucose cotransporter (SGLT1)#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8063771# AUTHORS:Hediger MA, Turk E, Wright EM#TITLE:Homology of the human intestinal Na+/glucose and Escherichia coli Na+/proline cotransporters#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1989#PUBMED:2490366# AUTHORS:Quick M, Loo DD, Wright EM#TITLE:Neutralization of a conserved amino acid residue in the human Na+/glucose transporter (hSGLT1) generates a glucose-gated H+ channel#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11024018# AUTHORS:Wright EM, Hirayama BA, Loo DDF, Turk E, Hager K#TITLE:Intestinal sugar transport#YEAR:1994# AUTHORS:Leung DW, Loo DD, Hirayama BA, Zeuthen T, Wright EM#TITLE:Urea transport by cotransporters#JOURNAL:J Physiol#YEAR:2000#PUBMED:11034615# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# AUTHORS:Kwon HM, Yamauchi A, Uchida S, Preston AS, Garcia-Perez A, Burg MB, Handler JS#TITLE:Cloning of the cDNa for a Na+/myo-inositol cotransporter, a hypertonicity stress protein#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1372904# AUTHORS:Berry GT, Mallee JJ, Kwon HM, Rim JS, Mulla WR, Muenke M, Spinner NB#TITLE:The human osmoregulatory Na+/myo-inositol cotransporter gene (SLC5A3): molecular cloning and localization to chromosome 21#JOURNAL:Genomics#YEAR:1995#PUBMED:7789985# AUTHORS:Hager K, Hazama A, Kwon HM, Loo DD, Handler JS, Wright EM#TITLE:Kinetics and specificity of the renal Na+/myo-inositol cotransporter expressed in Xenopus oocytes#JOURNAL:J Membr Biol#YEAR:1995#PUBMED:7537337# AUTHORS:Wapnir IL, van de Rijn M, Nowels K, Amenta PS, Walton K, Montgomery K, Greco RS, Dohan O, Carrasco N#TITLE:Immunohistochemical profile of the sodium/iodide symporter in thyroid, breast, and other carcinomas using high density tissue microarrays and conventional sections#JOURNAL:J Clin Endocrinol Metab#YEAR:2003#PUBMED:12679487# AUTHORS:Dai G, Levy O, Carrasco N#TITLE:Cloning and characterization of the thyroid iodide transporter#JOURNAL:Nature#YEAR:1996#PUBMED:8559252# AUTHORS:Smanik PA, Liu Q, Furminger TL, Ryu K, Xing S, Mazzaferri EL, Jhiang SM#TITLE:Cloning of the human sodium lodide symporter#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8806637# AUTHORS:De La Vieja A, Dohan O, Levy O, Carrasco N#TITLE:Molecular analysis of the sodium/iodide symporter: impact on thyroid and extrathyroid pathophysiology#JOURNAL:Physiol Rev#YEAR:2000#PUBMED:10893432# AUTHORS:Eskandari S, Loo DD, Dai G, Levy O, Wright EM, Carrasco N.#TITLE:Thyroid Na+/I- symporter. Mechanism, stoichiometry, and specificity#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9341168# AUTHORS:Wright EM, Loo DD, Hirayama BA, Turk E#TITLE:Surprising versatility of Na+-glucose cotransporters: SLC5#JOURNAL:Physiology (Bethesda)#YEAR:2004#PUBMED:15546855# GENE ASSOCIATION: (6526.1) or (6523.1) or (6528.1)
Abbreviation: R_INSt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ins[e] <==> ins[c] Confidence Level: 4 AR genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (3177.1) or (2030.1)
Abbreviation: R_ACSm Synonyms: _0 EC Number: 6.2.1.1 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [m] : ac + atp + coa --> accoa + amp + ppi Confidence Level: 2 IF genes: LOCUS:_84532#TRANSCRIPT:1#ABBREVIATION:ACAS2L# proteins: NAME:acetyl-CoA synthetase 2 (AMP-forming)-like, mitochondrial#ABBREVIATION:Acas2l.1-m# citations: AUTHORS:Fujino T, Kondo J, Ishikawa M, Morikawa K, Yamamoto TT#TITLE:Acetyl-CoA synthetase 2, a mitochondrial matrix enzyme involved in the oxidation of acetate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11150295# AUTHORS:Scholte HR, Wit-Peeters EM, Bakker JC.#TITLE:The intracellular and intramitochondrial distribution of short-chain acyl-CoA synthetases in guinea-pig heart. #JOURNAL:Biochim Biophys Acta#YEAR:1971#PUBMED:4326157# GENE ASSOCIATION: (84532.1)
heparan sulfate, free chain_C79H114N5O102S12
Abbreviation: R_HSPASEly Synonyms: _0 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hspg --> Ser-Gly/Ala-X-Gly + hs Confidence Level: 2 Note that this reaction occurs in the endosome during endocytosis, but has been compartmentalized as lysosomal. NCD genes: none proteins: none citations: AUTHORS:Burbach BJ, Friedl A, Mundhenke C, Rapraeger AC#TITLE:Syndecan-1 accumulates in lysosomes of poorly differentiated breast carcinoma cells#JOURNAL:Martix Biol#YEAR:2003#PUBMED:12782143# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
heparan sulfate proteoglycan protease, lysosome (endosome)
Abbreviation: R_EX_ksii_core2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ksii_core2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate II (core 2-linked) exchange
Abbreviation: R_GGH_DASH_10FTHF7GLUl Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [l] : 10fthf7glu + h2o --> 10fthf6glu + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-l# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (10FTHF7GLU), lysosomal
Abbreviation: R_SGALSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: sgalside_hs[c] <==> sgalside_hs[g] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NAGLCAly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : core6 + h2o --> Tn_antigen + acgam Confidence Level: 1 EC 3.2.1.- This reaction is my best guess at how the GlcNAc-beta-1,6-GalNAc linkage is cleaved. NCD genes: none proteins: none citations: none GENE ASSOCIATION:
mannosyl-glucosaminyl-acylphosphatidylinositiol (H2)_C37H68NO19PFULLRCO2FULLR2CO2
Abbreviation: R_FACOAL245_1 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + tetpent6 <==> amp + ppi + tetpent6coa Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_AACTtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: aact[m] --> aact[c] Confidence Level: 1 MM genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate A (GalNAc4S-GlcA), free chain_C45H67N2O46S3
Abbreviation: R_CSAPASEly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cspg_a + h2o --> Ser-Gly/Ala-X-Gly + cs_a Confidence Level: 1 Note that this reaction occurs in the endosome during endocytosis, but has been compartmentalized as lysosomal. NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
chondroitin sulfate A proteoglycan protease, lysosome (endosome)
Abbreviation: R_TTDCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: ttdcrn[c] --> ttdcrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_EX_6thf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 6thf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_gp1calpha_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gp1calpha_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ATP2ter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: amp[r] + atp[c] <==> amp[c] + atp[r] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
AMP/ATP transporter, endoplasmic reticulum
Abbreviation: R_EX_ascb_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ascb-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PGM Synonyms: _0 EC Number: 5.4.2.1 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : 2pg <==> 3pg Confidence Level: 0 SMP genes: LOCUS:_669#TRANSCRIPT:1#ABBREVIATION:BPGM#ECNUMBERS:5.4.2.4# LOCUS:_5224#TRANSCRIPT:1#ABBREVIATION:PGAM2#ECNUMBERS:5.4.2.1# LOCUS:_5223#TRANSCRIPT:1#ABBREVIATION:PGAM1#ECNUMBERS:5.4.2.1# LOCUS:_5224#TRANSCRIPT:2#ABBREVIATION:PGAM2#ECNUMBERS:5.4.2.1# LOCUS:_669#TRANSCRIPT:2#ABBREVIATION:BPGM#ECNUMBERS:5.4.2.4# proteins: NAME:brain phosphoglycerate mutase 1#ABBREVIATION:Pgam1.1# NAME:muscle phosphoglycerate mutase 2 (muscle)#ABBREVIATION:Pgam2.1# NAME:muscle phosphoglycerate mutase 2 (muscle)#ABBREVIATION:Pgam2.2# NAME:_2,3-bisphosphoglycerate mutase#ABBREVIATION:Bpgm.1# NAME:_2,3-bisphosphoglycerate mutase#ABBREVIATION:Bpgm.2# citations: none GENE ASSOCIATION: (669.1) or (5223.1) or (5224.2) or (5224.1) or (669.2)
keratan sulfate II biosynthesis, precursor 7_C67H108N5O51SX
Abbreviation: R_AG13T3g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre7 + uacgam --> h + ksii_core2_pre8 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (10331.1) or (11041.1) or (374907.2) or (10678.1) or (79369.1) or (374907.1) or (10678.2) or (93010.1) or (284208.1)
keratan sulfate II biosynthesis, precursor 8_C75H121N6O56SX
Abbreviation: R_EX_sucr_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : sucr <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_tagat_DASH_D_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tagat-D <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UNK3 Synonyms: _0 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : 2kmb + glu-L --> akg + met-L Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Myers RW, Abeles RH#TITLE:Conversion of 5-S-ethyl-5-thio-D-ribose to ethionine in Klebsiella pneumoniae. Basis for the selective toxicity of 5-S-ethyl-5-thio-D-ribose#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2543672# GENE ASSOCIATION:
_2-keto-4-methylthiobutyrate transamination
Abbreviation: R_GUAD Synonyms: _0 EC Number: 3.5.4.3 SUBSYSTEM: Purine Catabolism Equation: [c] : gua + h + h2o --> nh4 + xan Confidence Level: 4 genes: LOCUS:_9615#TRANSCRIPT:1#ABBREVIATION:GDA#ECNUMBERS:3.5.4.3# proteins: NAME:guanine deaminase #ABBREVIATION:Gda.1# citations: AUTHORS:Yuan G, Bin JC, McKay DJ, Snyder FF.#TITLE:Cloning and characterization of human guanine deaminase. Purification and partial amino acid sequence of the mouse protein.#JOURNAL:J Biol Chem.#YEAR:1999#PUBMED:10075721# GENE ASSOCIATION: (9615.1)
Abbreviation: R_CBPS Synonyms: _0 EC Number: 6.3.5.5 SUBSYSTEM: Pyrimidine Biosynthesis Equation: [c] : (2) atp + gln-L + h2o + hco3 --> (2) adp + cbp + glu-L + (2) h + pi Confidence Level: 4 genes: LOCUS:_790#TRANSCRIPT:1#ABBREVIATION:CAD1#ECNUMBERS:6.3.5.5,2.1.3.2,3.5.2.3# proteins: NAME:CAD protein (3.5.2.3, 2.1.3.2, 6.3.5.5)#ABBREVIATION:Cad1.1# citations: AUTHORS:Iwahana H, Fujimura M, Ii S, Kondo M, Moritani M, Takahashi Y, Yamaoka T, Yoshimoto K, Itakura M.#TITLE:Molecular cloning of a human cDNA encoding a trifunctional enzyme of carbamoyl-phosphate synthetase-aspartate transcarbamoylase-dihydroorotase in de Novo pyrimidine synthesis. #JOURNAL:Biochem Biophys Res Commun.#YEAR:1996#PUBMED:8619816# AUTHORS:Chen KC, Vannais DB, Jones C, Patterson D, Davidson JN.#TITLE:Mapping of the gene encoding the multifunctional protein carrying out the first three steps of pyrimidine biosynthesis to human chromosome 2. #JOURNAL: Hum Genet.#YEAR:1989#PUBMED:2565865# GENE ASSOCIATION: (790.1)
carbamoyl-phosphate synthase (glutamine-hydrolysing)
Abbreviation: R_FAOXC1811601m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (5) coa + (4) fad + (5) h2o + (5) nad + od2coa --> (5) accoa + (4) fadh2 + (5) h + (5) nadh + occoa Confidence Level: 4 _5 rounds of beta ox, 1 loss of degree of unsat -> 4 fad oxidized c18:1 -> c8:0 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_LEUtec Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: leu-L[e] <==> leu-L[c] Confidence Level: 4 SAB genes: LOCUS:_124935#TRANSCRIPT:1#ABBREVIATION:SLC43A2# LOCUS:_8501#TRANSCRIPT:1#ABBREVIATION:SLC43A1# proteins: NAME:solute carrier family 43, member 2#ABBREVIATION:Slc43a2.1# NAME:solute carrier family 43, member 1#ABBREVIATION:Slc43a1.1# citations: AUTHORS:Bodoy S, Martin L, Zorzano A, Palacin M, Estevez R, Bertran J#TITLE:Identification of LAT4, a novel amino acid transporter with system L activity#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15659399# AUTHORS:Babu E, Kanai Y, Chairoungdua A, Kim do K, Iribe Y, Tangtrongsup S, Jutabha P, Li Y, Ahmed N, Sakamoto S, Anzai N, Nagamori S, Endou H#TITLE:Identification of a novel system L amino acid transporter structurally distinct from heterodimeric amino acid transporters#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12930836# AUTHORS:Hediger MA, Romero MF, Peng JB, Rolfs A, Takanaga H, Bruford EA#TITLE:The ABCs of solute carriers: physiological, pathological and therapeutic implications of human membrane transport proteinsIntroduction#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14624363# GENE ASSOCIATION: (8501.1) or (124935.1)
L-leucine transport via diffusion (extracellular to cytosol)
Abbreviation: R_EX_retpalm Synonyms: _0 Equation: [e] : retpalm [deleted 10/09/2005 06:18:49 PM] <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DOLPGT3_Ler Synonyms: _2 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolglcp_L + (0.1) g2m8mpdol_L --> (0.1) dolp_L + (0.1) g3m8mpdol_L + h Confidence Level: 0 EC 2.4.1.- NCD genes: LOCUS:_84920#TRANSCRIPT:1#ABBREVIATION:ALG10# proteins: NAME:alpha-1,2-glucosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg10.1-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (84920.1)
(alpha-D-Glucosyl)3-(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human liver homolog_C1960H3228N20O770P20
Abbreviation: R_B3GNT36g Synonyms: _9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : acgal + gbside_hs --> acgbgbside_hs + h2o Confidence Level: 4 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Zhou D, Dinter A, Gutierrez Gallego R, Kamerling JP, Vliegenthart JF, Berger EG, Hennet T. #TITLE:A beta-1,3-N-acetylglucosaminyltransferase with poly-N-acetyllactosamine synthase activity is structurally related to beta-1,3-galactosyltransferases. #JOURNAL:Proc Natl Acad Sci#YEAR:1999# GENE ASSOCIATION: (10331.1)
Abbreviation: R_FAOXC2252053m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : coa + dcsptn1coa + h2o + nad + o2 --> accoa + h + h2o2 + nadh + tmndnccoa Confidence Level: 4 _1 round beta ox - c22:5,n-3 -> c20:5, n-3 - final '3' in abbreviation indicates omega3 FA 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_SADT Synonyms: _0 EC Number: 2.7.7.4 SUBSYSTEM: Nucleotides Equation: [c] : atp + h + so4 --> aps + ppi Confidence Level: 4 genes: LOCUS:_9061#TRANSCRIPT:1#ABBREVIATION:PAPSS1#ECNUMBERS:2.7.1.25,2.7.7.4# LOCUS:_9060#TRANSCRIPT:1#ABBREVIATION:PAPSS2#ECNUMBERS:2.7.7.4,&,2.7.1# proteins: NAME:_3'-phosphoadenosine 5'-phosphosulfate synthase 2 #ABBREVIATION:Papss2.1# NAME:_3'-phosphoadenosine 5'-phosphosulfate synthase 1 #ABBREVIATION:Papss1.1# citations: AUTHORS:Venkatachalam KV.#TITLE:Human 3'-phosphoadenosine 5'-phosphosulfate (PAPS) synthase: biochemistry, molecular biology and genetic deficiency.#JOURNAL:IUBMB Life. #YEAR:2003#PUBMED:12716056# AUTHORS:Fuda H, Shimizu C, Lee YC, Akita H, Strott CA.#TITLE:Characterization and expression of human bifunctional 3'-phosphoadenosine 5'-phosphosulphate synthase isoforms. #JOURNAL:Biochem J.#YEAR:2002#PUBMED:11931637# GENE ASSOCIATION: (9060.1) or (9061.1)
Adenosine 5'-phosphosulfate_C10H12N5O10PS
_1D-myo-Inositol 1,4,5,6-tetrakisphosphate_C6H8O18P4
Abbreviation: R_ARTFR45 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + nrvnccoa[c] --> (1.5) R4coa_hs[c] + fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_for_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : for <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLYK Synonyms: _0 EC Number: 2.7.1.30 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : atp + glyc --> adp + glyc3p + h Confidence Level: 4 genes: LOCUS:_2710#TRANSCRIPT:1#ABBREVIATION:GK#ECNUMBERS:2.7.1.30# LOCUS:_2712#TRANSCRIPT:1#ABBREVIATION:GK2# LOCUS:_2713#TRANSCRIPT:1#ABBREVIATION:GKP3# LOCUS:_2710#TRANSCRIPT:2#ABBREVIATION:GK#ECNUMBERS:2.7.1.30# proteins: NAME:glycerol kinase#ABBREVIATION:Gk.1# NAME:glycerol kinase#ABBREVIATION:Gk.2# NAME:glycerol kinase 2#ABBREVIATION:Gk2.1# NAME:glycerol kinase 3 (pseudogene)#ABBREVIATION:Gkp3.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Guo W, Worley K, Adams V, Mason J, Sylvester-Jackson D, Zhang Y, Towbin JA, Fogt DD, Madu S, Wheeler DA, McCabe ERB#TITLE:Genomic scanning for expressed sequences in Xp21 identifies the glycerol kinase gene#JOURNAL:Nature Genetics#YEAR:1993# AUTHORS:Sargent CA, Young C, Marsh S, Ferguson-Smith MA, Affara NA.#TITLE:The glycerol kinase gene family: structure of the Xp gene, and related intronless retroposons.#JOURNAL:Hum Mol Genet#YEAR:1994#PUBMED:7987308# AUTHORS:Hurley JH, Faber HR, Worthylake D, Meadow ND, Roseman S, Pettigrew DW, Remington SJ#TITLE:Structure of the regulatory complex of Escherichia coli IIIGlc with glycerol kinase#JOURNAL:Science#YEAR:1993#PUBMED:8430315# GENE ASSOCIATION: (2710.2) or (2712.1) or (2710.1) or (2713.1)
Abbreviation: R_DHEASULT Synonyms: _6 EC Number: 2.8.2.2 SUBSYSTEM: Steroid Metabolism Equation: [c] : dhea + paps --> dheas + h + pap Confidence Level: 4 NJ genes: LOCUS:_6783#TRANSCRIPT:1#ABBREVIATION:SULT1E1# LOCUS:_6822#TRANSCRIPT:1#ABBREVIATION:SULT2A1#ECNUMBERS:2.8.2.2# proteins: NAME:sulfotransferase family, cytosolic, 2A, dehydroepiandrosterone#ABBREVIATION:Sult2a1.1# NAME:sulfotransferase family 1E, estrogen#ABBREVIATION:Sult1e1.1# citations: AUTHORS:Otterness DM, Wieben ED, Wood TC, Watson WG, Madden BJ, McCormick DJ, Weinshilboum RM.#TITLE:Human liver dehydroepiandrosterone sulfotransferase: molecular cloning and expression of cDNA.#JOURNAL:Mol Pharmacol#YEAR:1992#PUBMED:1588921# AUTHORS:Glatt H, Boeing H, Engelke CE, Ma L, Kuhlow A, Pabel U, Pomplun D, Teubner W, Meinl W.#TITLE:Human cytosolic sulphotransferases: genetics, characteristics, toxicological aspects.#JOURNAL:Mutat Res#YEAR:2001#PUBMED:11535246# GENE ASSOCIATION: (6783.1) or (6822.1)
Abbreviation: R_NRPPHRSFt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: nrpphrsf[c] --> nrpphrsf[e] Confidence Level: 1 SAB genes: none proteins: none citations: none GENE ASSOCIATION:
norepinephrine sulfate transport (diffusion)
Abbreviation: R_ARTFR202 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (2) fadh2[m] + h[m] + lnlncacoa[c] + nadph[m] --> (1.125) R2coa_hs[c] + (2) fad[m] + nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DOGULND1 Synonyms: _0 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : 23doguln + h2o --> co2 + lyxnt Confidence Level: 2 EC-4.1.1.- NCD genes: none proteins: none citations: AUTHORS:Banhegyi G, Braun L, Csala M, Puskas F, Mandl J#TITLE:Ascorbate metabolism and its regulation in animals#JOURNAL:Free Radic Biol Med#YEAR:1997#PUBMED:9296457# GENE ASSOCIATION:
_2,3-dioxo-L-gulonate decarboxylase (L-lyxonate-forming)
Abbreviation: R_DNDPt19m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + dudp[c] --> atp[c] + dudp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_retn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : retn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAOXC240200x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : (2) coa + (2) h2o + lgnccoa + (2) nad + (2) o2 --> (2) accoa + arachcoa + (2) h + (2) h2o2 + (2) nadh Confidence Level: 4 peroxisomal (very long chain FA) - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) c24:0 -> c20:0 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1) or (51.1 and 30.1 and 1962.1) or (3295.1 and 30.1 and 51.2)
Abbreviation: R_SBTR Synonyms: _0 EC Number: 1.1.1.21 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : glc-D + h + nadph --> nadp + sbt-D Confidence Level: 4 NCD genes: LOCUS:_10327#TRANSCRIPT:1#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# LOCUS:_231#TRANSCRIPT:1#ABBREVIATION:AKR1B1#ECNUMBERS:1.1.1.21# LOCUS:_8574#TRANSCRIPT:1#ABBREVIATION:AKR7A2# LOCUS:_10327#TRANSCRIPT:2#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# proteins: NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.1# NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.2# NAME:aldo-keto reductase family 1, member B1 (aldose reductase)#ABBREVIATION:Akr1b1.1# NAME:aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)#ABBREVIATION:Akr7a2.1# citations: AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# AUTHORS:Kelly VP, Sherratt PJ, Crouch DH, Hayes JD#TITLE:Novel homodimeric and heterodimeric rat gamma-hydroxybutyrate synthases that associate with the Golgi apparatus define a distinct subclass of aldo-keto reductase 7 family proteins.#JOURNAL:Biochem J#YEAR:2002#PUBMED:12071861# AUTHORS:Praml C, Savelyeva L, Perri P, Schwab M.#TITLE:Cloning of the human aflatoxin B1-aldehyde reductase gene at 1p35-1p36.1 in a region frequently altered in human tumor cells.#JOURNAL:Cancer Res#YEAR:1998#PUBMED:9823300# GENE ASSOCIATION: (8574.1) or (231.1) or (10327.2) or (10327.1)
Abbreviation: R_SPHINGStl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: sphings[c] <==> sphings[l] Confidence Level: 1 unknown mechanism, however sphingosine manages to get in different compartments of cells. NOT presumed to be flip-flop. NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MCCCrm Synonyms: _0 EC Number: 6.4.1.4 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 3mb2coa + atp + hco3 <==> 3mgcoa + adp + h + pi Confidence Level: 4 MM genes: LOCUS:_56922#TRANSCRIPT:1#ABBREVIATION:MCCC1#ECNUMBERS:6.4.1.4# LOCUS:_64087#TRANSCRIPT:1#ABBREVIATION:MCCC2#ECNUMBERS:6.4.1.4# proteins: NAME:methylcrotonoyl-Coenzyme A carboxylase, mitochondrial #ABBREVIATION:McccAB-m# citations: AUTHORS:Baumgartner MR, Almashanu S, Suormala T, Obie C, Cole RN, Packman S, Baumgartner ER, Valle D. #TITLE:The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency.#YEAR:2001#PUBMED:11181649# AUTHORS:Holzinger A, Roschinger W, Lagler F, Mayerhofer PU, Lichtner P, Kattenfeld T, Thuy LP, Nyhan WL, Koch HG, Muntau AC, Roscher AA.#TITLE:Cloning of the human MCCA and MCCB genes and mutations therein reveal the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency. #YEAR:2001#PUBMED:11406611# GENE ASSOCIATION: (56922.1 and 64087.1)
methylcrotonoyl-CoA carboxylase, mitochondrial
Abbreviation: R_AP4AH1 Synonyms: _7 EC Number: 3.6.1.17 SUBSYSTEM: Nucleotides Equation: [c] : ap4a + h2o --> amp + atp + (2) h Confidence Level: 4 IT genes: LOCUS:_318#TRANSCRIPT:1#ABBREVIATION:NUDT2#ECNUMBERS:3.6.1.17# LOCUS:_318#TRANSCRIPT:2#ABBREVIATION:NUDT2#ECNUMBERS:3.6.1.17# LOCUS:_318#TRANSCRIPT:3#ABBREVIATION:NUDT2#ECNUMBERS:3.6.1.17# proteins: NAME:nudix (nucleoside diphosphate linked moiety X)-type motif 2 #ABBREVIATION:NudT2.1# NAME:nudix (nucleoside diphosphate linked moiety X)-type motif 2#ABBREVIATION:NudT2.2# NAME:nudix (nucleoside diphosphate linked moiety X)-type motif 2#ABBREVIATION:NudT2.3# citations: AUTHORS:McLennan AG, Flannery AV, Morten JE, Ridanpaa M.#TITLE:Chromosomal localization of the human diadenosine 5',5"'-P1,P4-tetraphosphate pyrophosphohydrolase (Ap4A hydrolase) gene (APAH1) to 9p13.#JOURNAL:Genomics#YEAR:1998#PUBMED:9479504# AUTHORS:Hankin S, Matthew N, Thorne H, McLennan AG.#TITLE:Diadenosine 5',5"'-P1,P4-tetraphosphate hydrolase is present in human erythrocytes, leukocytes and platelets. #JOURNAL:Int J Biochem Cell Biol. #YEAR:1995#PUBMED:7767787# GENE ASSOCIATION: (318.1) or (318.3) or (318.2)
Abbreviation: R_THMP Synonyms: _0 SUBSYSTEM: Thiamine Metabolism Equation: [c] : h2o + thmmp --> pi + thm Confidence Level: 1 EC 3.1.3.- NCD genes: none proteins: none citations: AUTHORS:Bettendorff L, Mastrogiacomo F, Kish SJ, Grisar T.#TITLE:Thiamine, thiamine phosphates, and their metabolizing enzymes in human brain. #JOURNAL:J Neurochem. #YEAR:1996#PUBMED:8522961# AUTHORS:Zhao R, Gao F, Goldman ID. #TITLE:Reduced folate carrier transports thiamine monophosphate: an alternative route for thiamine delivery into mammalian cells.#JOURNAL: Am J Physiol Cell Physiol. #YEAR:2002#PUBMED:11997266# GENE ASSOCIATION:
(Gal)4 (Glc)1 (GlcNAc)2 (LFuc)1 (Cer)1_C70H122N3O41FULLRCO
_3-Phospho-D-glyceroyl phosphate_C3H4O10P2
Abbreviation: R_DPGM Synonyms: _0 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : 13dpg <==> 23dpg + h Confidence Level: 0 genes: LOCUS:_669#TRANSCRIPT:1#ABBREVIATION:BPGM#ECNUMBERS:5.4.2.4# LOCUS:_5224#TRANSCRIPT:1#ABBREVIATION:PGAM2#ECNUMBERS:5.4.2.1# LOCUS:_5223#TRANSCRIPT:1#ABBREVIATION:PGAM1#ECNUMBERS:5.4.2.1# LOCUS:_5224#TRANSCRIPT:2#ABBREVIATION:PGAM2#ECNUMBERS:5.4.2.1# LOCUS:_669#TRANSCRIPT:2#ABBREVIATION:BPGM#ECNUMBERS:5.4.2.4# proteins: NAME:brain phosphoglycerate mutase 1#ABBREVIATION:Pgam1.1# NAME:muscle phosphoglycerate mutase 2 (muscle)#ABBREVIATION:Pgam2.1# NAME:muscle phosphoglycerate mutase 2 (muscle)#ABBREVIATION:Pgam2.2# NAME:_2,3-bisphosphoglycerate mutase#ABBREVIATION:Bpgm.1# NAME:_2,3-bisphosphoglycerate mutase#ABBREVIATION:Bpgm.2# citations: none GENE ASSOCIATION: (669.1) or (5223.1) or (5224.2) or (5224.1) or (669.2)
Abbreviation: R_GUR1PP Synonyms: _0 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : glcur1p + h2o --> glcur + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_MI1345PKn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + mi1345p --> adp + h + mi13456p Confidence Level: 4 NCD genes: LOCUS:_253430#TRANSCRIPT:1#ABBREVIATION:IPMK# proteins: NAME:inositol polyphosphate multikinase, nucleus#ABBREVIATION:Ipmk.1-n# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:Chang SC, Miller AL, Feng Y, Wente SR, Majerus PW. #TITLE:The human homolog of the rat inositol phosphate multikinase is an inositol 1,3,4,6-tetrakisphosphate 5-kinase#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12223481# AUTHORS:Nalaskowski MM, Deschermeier C, Fanick W, Mayr GW#TITLE:The human homologue of yeast ArgRIII protein is an inositol phosphate multikinase with predominantly nuclear localization#JOURNAL:Biochem J#YEAR:2002#PUBMED:12027805# GENE ASSOCIATION: (253430.1)
inositol-1,3,4,5-triphosphate 6-kinase, nucleus
Abbreviation: R_EX_strch2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : strch2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
starch, structure 2 (1,6-{2[1,4-Glc], [1,4-Glc]}) exchange
Abbreviation: R_ACNAMlt Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: acnam[c] + h[c] <==> acnam[l] + h[l] Confidence Level: 4 H symport into lysososome. See PMID: 2768261 and PMID: 10581036 ( for human evidence). Sialic acid storage disorders (due to transporter mutations) require import and export e.g. PMID: 2768266 NJ genes: LOCUS:_26503#TRANSCRIPT:1#ABBREVIATION:SLC17A5# proteins: NAME:solute carrier family 17 (anion/sugar transporter), member 5#ABBREVIATION:Slc17a5.1# citations: AUTHORS:Mancini GM, de Jonge HR, Galjaard H, Verheijen FW.#TITLE:Characterization of a proton-driven carrier for sialic acid in the lysosomal membrane. Evidence for a group-specific transport system for acidic monosaccharides.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2768261# AUTHORS:Verheijen FW, Verbeek E, Aula N, Beerens CE, Havelaar AC, Joosse M, Peltonen L, Aula P, Galjaard H, van der Spek PJ, Mancini GM.#TITLE:A new gene, encoding an anion transporter, is mutated in sialic acid storage diseases.#JOURNAL:Nat Genet#YEAR:1999#PUBMED:10581036# GENE ASSOCIATION: (26503.1)
N-acetylneuraminate transport into lysososme
chondroitin sulfate E (GalNAc4,6diS-GlcA), free chain_C45H64N2O55S6
Abbreviation: R_CSEPASEly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cspg_e + h2o --> Ser-Gly/Ala-X-Gly + cs_e Confidence Level: 1 Note that this reaction occurs in the endosome during endocytosis, but has been compartmentalized as lysosomal. NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
chondroitin sulfate E proteoglycan protease, lysosome (endosome)
Abbreviation: R_PECGONCOATr Synonyms: _0 SUBSYSTEM: Alkaloid biosynthesis II Equation: [r] : atp + coa + h + pecgon --> amp + pecgoncoa + ppi Confidence Level: 1 SAB EC 6.2.1.- name uncertain genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate E (GalNAc4,6diS-GlcA), precursor 5b_C45H64N2O51S5X
keratan sulfate I, degradation product 33_C76H125N5O62S2
Abbreviation: R_NACHEXA18ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg33 --> acgam + h + ksi_deg35 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_SPR Synonyms: _0 EC Number: 1.1.1.153 SUBSYSTEM: Tetrahydrobiopterin Equation: [c] : 6pthp + (2) h + (2) nadph --> (2) nadp + thbpt Confidence Level: 4 IT genes: LOCUS:_6697#TRANSCRIPT:1#ABBREVIATION:SPR#ECNUMBERS:1.1.1.153# proteins: NAME:sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase) #ABBREVIATION:Spr1.1# citations: AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (6697.1)
Abbreviation: R_EX_2425dhvitd2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 2425dhvitd2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TRDR3 Synonyms: _0 EC Number: 1.8.1.9 SUBSYSTEM: Miscellaneous Equation: [c] : h + nadh + q10 --> nad + q10h2 Confidence Level: 4 Xia et al, 2003, JBC, 2141-2146 genes: LOCUS:_7296#TRANSCRIPT:1#ABBREVIATION:TXNRD1# LOCUS:_7296#TRANSCRIPT:4#ABBREVIATION:TXNRD1# LOCUS:_7296#TRANSCRIPT:2#ABBREVIATION:TXNRD1# LOCUS:_7296#TRANSCRIPT:3#ABBREVIATION:TXNRD1# proteins: NAME:thioredoxin reductase 1 #ABBREVIATION:Txnrd1.1# NAME:thioredoxin reductase 1#ABBREVIATION:Txnrd1.2# NAME:thioredoxin reductase 1#ABBREVIATION:Txnrd1.3# NAME:thioredoxin reductase 1#ABBREVIATION:Txnrd1.4# citations: AUTHORS:Tamura T, Stadtman TC.#TITLE:A new selenoprotein from human lung adenocarcinoma cells: purification, properties, and thioredoxin reductase activity. #JOURNAL:Proc Natl Acad Sci U S A. #YEAR:1996#PUBMED:8577704# AUTHORS:Koishi R, Kawashima I, Yoshimura C, Sugawara M, Serizawa N.#TITLE:Cloning and characterization of a novel oxidoreductase KDRF from a human bone marrow-derived stromal cell line KM-102. #JOURNAL:J Biol Chem. #YEAR:1997#PUBMED:8999974# AUTHORS:Xia L, Nordman T, Olsson JM, Damdimopoulos A, Bjorkhem-Bergman L, Nalvarte I, Eriksson LC, Arner ES, Spyrou G, Bjornstedt M.#TITLE:The mammalian cytosolic selenoenzyme thioredoxin reductase reduces ubiquinone. A novel mechanism for defense against oxidative stress.#JOURNAL:J Biol Chem.#YEAR:2003#PUBMED:12435734# GENE ASSOCIATION: (7296.4) or (7296.1) or (7296.3) or (7296.2)
Thioredoxin (ubiquinone 10) reductase (NADH)
Abbreviation: R_AFLATOXINte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: aflatoxin[e] <==> aflatoxin[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DEBRISOQUINEt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: debrisoquine[e] <==> debrisoquine[c] Confidence Level: 4 electrogenic, Na indepdendent & rev NJ genes: LOCUS:_6582#TRANSCRIPT:1#ABBREVIATION:SLC22A2# LOCUS:_6582#TRANSCRIPT:2#ABBREVIATION:SLC22A2# proteins: NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.2# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# AUTHORS:Koepsell H, Schmitt BM, Gorboulev V.#TITLE:Organic cation transporters.#JOURNAL:Rev Physiol Biochem Pharmacol#YEAR:2003#PUBMED:12827517# AUTHORS:Zhang L, Dresser MJ, Gray AT, Yost SC, Terashita S, Giacomini KM.#TITLE:Cloning and functional expression of a human liver organic cation transporter.#JOURNAL:Mol Pharmacol. #YEAR:1997#PUBMED:9187257# GENE ASSOCIATION: (6582.1) or (6582.2)
Abbreviation: R_ILEt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ile-L[e] + na1[e] --> ile-L[c] + na1[c] Confidence Level: 2 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# proteins: NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# AUTHORS:Van Winkle LJ#TITLE:Amino acid transport regulation and early embryo development#JOURNAL:Biol Reprod#YEAR:2001#PUBMED:11133652# GENE ASSOCIATION: (11254.1)
L-isoleucine transport in via sodium symport
Abbreviation: R_SUCCt4_3 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) na1[e] + succ[e] <==> (3) na1[c] + succ[c] Confidence Level: 4 NCD genes: LOCUS:_64849#TRANSCRIPT:1#ABBREVIATION:SLC13A3# LOCUS:_64849#TRANSCRIPT:2#ABBREVIATION:SLC13A3# proteins: NAME:solute carrier family 13 (sodium-dependent dicarboxylate transporter), member 3#ABBREVIATION:Slc13a3.1-e# NAME:solute carrier family 13 (sodium-dependent dicarboxylate transporter), member 3#ABBREVIATION:Slc13a3.2-e# citations: AUTHORS:Wang H, Fei YJ, Kekuda R, Yang-Feng TL, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Structure, function, and genomic organization of human Na(+)-dependent high-affinity dicarboxylate transporter#JOURNAL:Am J Physiol Cell Physiol#YEAR:2000#PUBMED:0794676# AUTHORS:Pajor AM#TITLE:Sodium-coupled transporters for Krebs cycle intermediates#JOURNAL:Annu Rev Physiol#YEAR:1999#PUBMED:10099705# GENE ASSOCIATION: (64849.1) or (64849.2)
Abbreviation: R_ACOAD1fm Synonyms: _0 EC Number: 1.3.99.2 SUBSYSTEM: Butanoate Metabolism Equation: [m] : btcoa + fad --> b2coa + fadh2 Confidence Level: 4 Assume NAD/NADH as acceptors Lumped long-chain acyl-CoA dehydrogenase (EC 1.3.99.13) This reaction is therefore equilivalent to trans-2-enoyl-CoA reductase (NAD) (EC 1.3.1.44) genes: LOCUS:_84129#TRANSCRIPT:1#ABBREVIATION:FLJ12592#ECNUMBERS:1.3.99.3# LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# LOCUS:_36#TRANSCRIPT:1#ABBREVIATION:ACADSB#ECNUMBERS:1.3.99.-# LOCUS:_27034#TRANSCRIPT:1#ABBREVIATION:ACAD8# LOCUS:_80724#TRANSCRIPT:1#ABBREVIATION:ACAD10# LOCUS:_28976#TRANSCRIPT:1#ABBREVIATION:ACAD9# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# NAME:acyl-Coenzyme A dehydrogenase, short/branched chain#ABBREVIATION:Acadsb.1-m# NAME:acyl-Coenzyme A dehydrogenase family, member 8#ABBREVIATION:Acad8.1-m# NAME:acyl-Coenzyme A dehydrogenase family, member 9, mitochondrial#ABBREVIATION:Acad9.1-m# NAME:acyl-Coenzyme A dehydrogenase family, member 10, mitochondrial#ABBREVIATION:Acad10.1-m# NAME:putative acyl-CoA dehydrogenase#ABBREVIATION:Flj12592.1-m# citations: AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# GENE ASSOCIATION: (84129.1) or (28976.1) or (27034.1) or (36.1) or (35.1) or (34.1) or (80724.1)
acyl-CoA dehydrogenase (butanoyl-CoA), mitochondrial
Abbreviation: R_EX_4hdebrisoquine_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 4hdebrisoquine <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HAtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: ha[e] --> ha[l] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
hyaluronan transport, extracellular to lysosome
keratan sulfate I, degradation product 1_C241H383N17O209S12X
Abbreviation: R_FUCASE2e Synonyms: _1 EC Number: 3.2.1.51 SUBSYSTEM: Keratan sulfate degradation Equation: [e] : h2o + ksi --> fuc-L + ksi_deg1 Confidence Level: 4 NCD genes: LOCUS:_2519#TRANSCRIPT:1#ABBREVIATION:FUCA2#ECNUMBERS:3.2.1.51# proteins: NAME:alpha-fucosidase 2, extracellular (plasma)#ABBREVIATION:Fuca2.1-e# citations: AUTHORS:Khunsook S, Bean BS, McGowan SR, Alhadeff JA#TITLE:Purification and characterization of plasma membrane-associated human sperm alpha-L-fucosidase#JOURNAL:Biol Reprod#YEAR:2003#PUBMED:12604617# AUTHORS:Cordero OJ, Merino A, Paez de la Cadena M, Bugia B, Nogueira M, Vinuela JE, Martinez-Zorzano VS, de Carlos A, Rodriguez-Berrocal FJ#TITLE:Cell surface human alpha-L-fucosidase#JOURNAL:Eur J Biochem#YEAR:2001#PUBMED:11389735# AUTHORS:Alhadeff JA, Khunsook S, Choowongkomon K, Baney T, Heredia V, Tweedie A, Bean B#TITLE:Characterization of human semen alpha-L-fucosidases#JOURNAL:Mol Hum Reprod#YEAR:1999#PUBMED:10460218# AUTHORS:Hopfer RL, Johnson SW, Masserini M, Giuliani A, Alhadeff JA#TITLE:Hydrolysis of fucosyl-GM1 ganglioside by purified pellet-associated human brain and human liver alpha-L-fucosidases without activator proteins or detergents#JOURNAL:Biochem J#YEAR:1990#PUBMED:2317201# GENE ASSOCIATION: (2519.1)
(alpha-D-mannosyl)7-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine, isoform B (protein)_C64H107N2O50X
Abbreviation: R_UPP3S Synonyms: _0 EC Number: 4.2.1.75 SUBSYSTEM: Heme Biosynthesis Equation: [c] : hmbil --> h2o + uppg3 Confidence Level: 0 genes: LOCUS:_7390#TRANSCRIPT:1#ABBREVIATION:UROS#ECNUMBERS:4.2.1.75# proteins: NAME:uroporphyrinogen-III synthase#ABBREVIATION:Uros.1# citations: AUTHORS:Tsai SF, Bishop DF, Desnick RJ.#TITLE:Human uroporphyrinogen III synthase: molecular cloning, nucleotide sequence, and expression of a full-length cDNA.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1988#PUBMED:3174619# GENE ASSOCIATION: (7390.1)
Abbreviation: R_DM_sprm_LPAREN_c_RPAREN_ Synonyms: _0 Equation: [c] : sprm --> Confidence Level: SAB potential "biomass" component--may associate with DNA genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_acgalfucgalacgalfuc12gal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acgalfucgalacgalfuc12gal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DASCBR Synonyms: _0 EC Number: 1.6.5.4 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : dhdascb + h + nadph --> ascb-L + nadp Confidence Level: 4 NCD genes: LOCUS:_2745#TRANSCRIPT:1#ABBREVIATION:GLRX# LOCUS:_51022#TRANSCRIPT:1#ABBREVIATION:GLRX2# LOCUS:_51022#TRANSCRIPT:2#ABBREVIATION:GLRX2# proteins: NAME:Glutaredoxin1#ABBREVIATION:Grx1.1# NAME:Glutaredoxin2#ABBREVIATION:Grx2.1# NAME:Glutaredoxin2#ABBREVIATION:Grx2.2# citations: AUTHORS:Lundberg M, Johansson C, Chandra J, Enoksson M, Jacobsson G, Ljung J, Johansson M, Holmgren A.#TITLE:Cloning and expression of a novel human glutaredoxin (Grx2) with mitochondrial and nuclear isoforms#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11297543# GENE ASSOCIATION: (2745.1) or (51022.2) or (51022.1)
Abbreviation: R_TETPENT3COAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: tetpent3coa[c] <==> tetpent3coa[x] Confidence Level: 0 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_BZtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: bz[c] <==> bz[r] Confidence Level: 1 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Benzene transporter, endoplasmic reticulum
Abbreviation: R_MTHFDm Synonyms: _0 EC Number: 1.5.1.5 SUBSYSTEM: Folate Metabolism Equation: [m] : mlthf + nadp <==> methf + nadph Confidence Level: 4 NCD genes: LOCUS:_10797#TRANSCRIPT:1#ABBREVIATION:MTHFD2#ECNUMBERS:1.5.1.15,3.5.4.9# LOCUS:_25902#TRANSCRIPT:1#ABBREVIATION:MTHFD1L# proteins: NAME:methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like, mitochondrial#ABBREVIATION:Mthfd1l-m# NAME:methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2, methenyltetrahydrofolate cyclohydrolase, mitochondrial#ABBREVIATION:Mthfd2.1-m# citations: AUTHORS:Prasannan P, Pike S, Peng K, Shane B, Appling DR#TITLE:Human mitochondrial C1-tetrahydrofolate synthase: gene structure, tissue distribution of the mRNA, and immunolocalization in Chinese hamster ovary calls#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12937168# AUTHORS:Yang XM, MacKenzie RE#TITLE:NAD-dependent methylenetetrahydrofolate dehydrogenase-methenyltetrahydrofolate cyclohydrolase is the mammalian homolog of the mitochondrial enzyme encoded by the yeast MIS1 gene#JOURNAL:Biochemistry#YEAR:1993#PUBMED:8218174# GENE ASSOCIATION: (25902.1) or (10797.1)
methylenetetrahydrofolate dehydrogenase (NADP), mitochondrial
Abbreviation: R_EX_ile_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ile-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ORPT Synonyms: _0 EC Number: 2.4.2.10 SUBSYSTEM: Pyrimidine Biosynthesis Equation: [c] : orot5p + ppi <==> orot + prpp Confidence Level: 4 genes: LOCUS:_7372#TRANSCRIPT:1#ABBREVIATION:UMPS#ECNUMBERS:2.4.2.10,,4.1.1.23# proteins: NAME:uridine monophosphate synthetase (orotate phosphoribosyl transferase and orotidine-5'-decarboxylase) #ABBREVIATION:Umps.1# citations: AUTHORS:Patterson D, Jones C, Morse H, Rumsby P, Miller Y, Davis R. #TITLE:Structural gene coding for multifunctional protein carrying orotate phosphoribosyltransferase and OMP decarboxylase activity is located on long arm of human chromosome 3.#JOURNAL:Somatic Cell Genet#YEAR:1983#PUBMED:6574608# AUTHORS:McClard RW, Black MJ, Livingstone LR, Jones ME.#TITLE:Isolation and initial characterization of the single polypeptide that synthesizes uridine 5'-monophosphate from orotate in Ehrlich ascites carcinoma. Purification by tandem affinity chromatography of uridine-5'-monophosphate synthase.#JOURNAL:Biochemistry#YEAR:1980#PUBMED:6893554# AUTHORS:Suttle DP, Bugg BY, Winkler JK, Kanalas JJ.#TITLE:Molecular cloning and nucleotide sequence for the complete coding region of human UMP synthase. #JOURNAL:Proc Natl Acad Sci U S A#YEAR:1988#PUBMED:3279416# AUTHORS:Suchi M, Mizuno H, Kawai Y, Tsuboi T, Sumi S, Okajima K, Hodgson ME, Ogawa H, Wada Y. #TITLE:Molecular cloning of the human UMP synthase gene and characterization of point mutations in two hereditary orotic aciduria families.#JOURNAL:Am J Hum Genet.#YEAR:1997#PUBMED:9042911# GENE ASSOCIATION: (7372.1)
Abbreviation: R_SO4HCOtex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) hco3[c] + so4[e] --> (2) hco3[e] + so4[c] Confidence Level: 2 SAB genes: LOCUS:_284129#TRANSCRIPT:1#ABBREVIATION:SLC26A11# LOCUS:_10861#TRANSCRIPT:1#ABBREVIATION:SLC26A1# LOCUS:_1836#TRANSCRIPT:1#ABBREVIATION:SLC26A2# LOCUS:_115019#TRANSCRIPT:1#ABBREVIATION:SLC26A9# LOCUS:_10861#TRANSCRIPT:3#ABBREVIATION:SLC26A1# LOCUS:_115019#TRANSCRIPT:2#ABBREVIATION:SLC26A9# LOCUS:_10861#TRANSCRIPT:2#ABBREVIATION:SLC26A1# LOCUS:_1811#TRANSCRIPT:1#ABBREVIATION:SLC26A3# LOCUS:_115111#TRANSCRIPT:1#ABBREVIATION:SLC26A7# LOCUS:_115111#TRANSCRIPT:2#ABBREVIATION:SLC26A7# LOCUS:_116369#TRANSCRIPT:1#ABBREVIATION:SLC26A8# LOCUS:_116369#TRANSCRIPT:2#ABBREVIATION:SLC26A8# proteins: NAME:solute carrier family 26 (sulfate transporter), member 1#ABBREVIATION:Slc26a1.1# NAME:solute carrier family 26 (sulfate transporter), member 1#ABBREVIATION:Slc26a1.2# NAME:solute carrier family 26 (sulfate transporter), member 1#ABBREVIATION:Slc26a1.3# NAME:solute carrier family 26 (sulfate transporter), member 2#ABBREVIATION:Slc26a2.1# NAME:solute carrier family 26, member 3#ABBREVIATION:Slc26a3.1# NAME:solute carrier family 26, member 7#ABBREVIATION:Slc26a7.1# NAME:solute carrier family 26, member 7#ABBREVIATION:Slc26a7.2# NAME:solute carrier family 26, member 8#ABBREVIATION:Slc26a8.1# NAME:solute carrier family 26, member 8#ABBREVIATION:Slc26a8.2# NAME:solute carrier family 26, member 9#ABBREVIATION:Slc26a9.1# NAME:solute carrier family 26, member 9#ABBREVIATION:Slc26a9.2# NAME:solute carrier family 26, member 11#ABBREVIATION:Slc26a11.1# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (115111.1) or (115019.2) or (1836.1) or (115111.2) or (116369.2) or (284129.1) or (10861.2) or (116369.1) or (10861.1) or (10861.3) or (115019.1) or (1811.1)
sulfate transport via bicarbonate countertransport
Abbreviation: R_B3GALT44g Synonyms: _7 EC Number: 2.4.1.62 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gt2_hs + udpgal --> gt1c_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_8705#TRANSCRIPT:1#ABBREVIATION:B3GALT4# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 4#ABBREVIATION:B3galt4.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Amado M, Almeida R, Carneiro F, Levery ST, Holmes EH, Nomoto M, Hollingsworth MA, Hassan H, Schwientek T, Nielsen PA, Bennett EP, Clausen H#TITLE:A family of human beta3-galactosyltransferases#JOURNAL:The Journal of Biological Chemistry#YEAR:1998# GENE ASSOCIATION: (8705.1)
keratan sulfate I, degradation product 28_C104H170N7O85S3
Abbreviation: R_COt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: co[c] <==> co[e] Confidence Level: 2 TDV genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACACtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: acac[c] <==> acac[x] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
acetoacetate intracellular transport unknown mechanism
GlcN-acylPI mannosyltransferase, endoplasmic reticulum
Abbreviation: R_GPIMTer_U Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_U + gacpail_hs --> (0.1) dolp_U + h + mgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_93183#TRANSCRIPT:1#ABBREVIATION:PIGM# LOCUS:_54965#TRANSCRIPT:1#ABBREVIATION:PIGX# proteins: NAME:phosphatidylinositol glycan, class M, endoplasmic reticulum#ABBREVIATION:Pigm.1-r# NAME:phosphatidylinositol glycan, class X, endoplasmic reticulum#ABBREVIATION:Pigx.1-r# citations: AUTHORS:Maeda Y, Watanabe R, Harris CL, Hong Y, Ohishi K, Kinoshita K, Kinoshita T#TITLE:PIG-M transfers the first mannose to glycosylphosphatidylinositol on the lumenal side of the ER#JOURNAL:EMBO J#YEAR:2001#PUBMED:11226175# AUTHORS:Ashida H, Hong Y, Murakami Y, Shishioh N, Sugimoto N, Kim YU, Maeda Y, Kinoshita T#TITLE:Mammalian PIG-X and yeast Pbn1p are the essential components of glycosylphosphatidylinositol-mannosyltransferase I#JOURNAL:Mol Biol Cell#YEAR:2005#PUBMED:15635094# GENE ASSOCIATION: (93183.1) and (54965.1)
Abbreviation: R_GP1CALPHAtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gp1calpha_hs[c] <==> gp1calpha_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
(Gal)2 (GalNAc)1 (Glc)1 (GlcNAc)1 (LFuc)1 (Cer)1_C58H102N3O31FULLRCO
Abbreviation: R_B3GNT311g Synonyms: _9 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acgalfuc12gal14acglcgalgluside_hs + udpgal --> galacgalfuc12gal14acglcgalgluside_hs + h + udp Confidence Level: 2 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3#ABBREVIATION:B3gnt3.1# citations: AUTHORS:Yokoyama-Kobayashi M, Yamaguchi T, Sekine S, Kato S.#TITLE:Selection of cDNAs encoding putative type II membrane proteins on the cell surface from a human full-length cDNA bank.#JOURNAL:Gene#YEAR:1999#PUBMED:10072769# GENE ASSOCIATION: (10331.1)
Abbreviation: R_ASNtN1 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[e] + h[c] + (2) na1[e] <==> asn-L[c] + h[e] + (2) na1[c] Confidence Level: 4 SAB based om PMID 10823827 genes: LOCUS:_92745#TRANSCRIPT:1#ABBREVIATION:SLC38A5# LOCUS:_10991#TRANSCRIPT:1#ABBREVIATION:SLC38A3# proteins: NAME:solute carrier family 38, member 3#ABBREVIATION:Slc38a3.1# NAME:solute carrier family 38, member 5#ABBREVIATION:Slc38a5.1# citations: AUTHORS:Fei YJ, Sugawara M, Nakanishi T, Huang W, Wang H, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, genomic organization, and functional and electrogenic characteristics of human system N 1, a Na+- and H+-coupled glutamine transporter#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10823827# AUTHORS:Mackenzie B, Erickson JD#TITLE:Sodium-coupled neutral amino acid (System N/A) transporters of the SLC38 gene family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12845534# GENE ASSOCIATION: (92745.1) or (10991.1)
Abbreviation: R_H2Otg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: h2o[c] <==> h2o[g] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACGALtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: acgal[l] --> acgal[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Rome LH, Hill DF#TITLE:Lysosomal degradation of glycoproteins and glycosaminoglycans. Efflux and recycling of sulphate and N-acetylhexosamines#JOURNAL:Biochem J#YEAR:1986#PUBMED:3753439# AUTHORS:Jonas AJ, Speller RJ, Conrad PB, Dubinsky WP#TITLE:Transport of N-acetyl-D-glucosamine and N-acetyl-D-galactosamine by rat liver lysosomes#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2784441# GENE ASSOCIATION:
Abbreviation: R_EX_no_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : no <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DMHPTCRNCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + dmhptcoa <==> coa + dmhptcrn Confidence Level: 2 made reversible since Keq ~ 0.6. dmhptcoa is a special case because its biochemical fate has not be elucidated. NJ genes: none proteins: none citations: AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# AUTHORS:Verhoeven NM, Wanders RJ, Poll-The BT, Saudubray JM, Jakobs C.#TITLE:The metabolism of phytanic acid and pristanic acid in man: a review.#JOURNAL:J Inherit Metab Dis#YEAR:1998#PUBMED:9819701# GENE ASSOCIATION:
_4,8 dimethylnonanoyl carnitine_C18H37NO4
Abbreviation: R_FAOXC2252053x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : coa + dcsptn1coa + h2o + nad + o2 --> accoa + h + h2o2 + nadh + tmndnccoa Confidence Level: 4 _1 round beta ox - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) peroxisomal (very long chain FA) c22:5,n-3 -> c20:5, n-3 - final '3' in abbreviation indicates omega3 FA 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1) or (51.1 and 30.1 and 1962.1) or (3295.1 and 30.1 and 51.2)
Abbreviation: R_1a_COMMA_24_COMMA_25VITD3Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : 2425dhvitd3 + h + nadph + o2 --> 1a2425thvitd3 + h2o + nadp Confidence Level: 2 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.196 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_1-alpha-Vitamin D-24,25-hydroxylase (D3)
_1-alpha,24R,25-Trihydroxyvitamin D2_C28H44O4
Abbreviation: R_CYSTAm Synonyms: _0 EC Number: 2.6.1.3 SUBSYSTEM: Cysteine Metabolism Equation: [m] : akg + cys-L <==> glu-L + mercppyr Confidence Level: 2 MM genes: LOCUS:_2806#TRANSCRIPT:1#ABBREVIATION:GOT2#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase, mitochondrial#ABBREVIATION:Got2.1-m# citations: AUTHORS:Stipanuk MH#TITLE:Sulfur amino acid metabolism: pathways for production and removal of homocysteine and cysteine#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189131# AUTHORS:Ubuka T, Umemura S, Yuasa S, Kinuta M, Watanabe K. #TITLE:Purification and characterization of mitochondrial cysteine aminotransferase from rat liver. #YEAR:1978#PUBMED:754189# GENE ASSOCIATION: (2806.1)
Abbreviation: R_ODECOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: odecoa[c] <==> odecoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PAPtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: pap[g] --> pap[c] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
adenosine 3',5'-bisphosphate Golgi transport
Abbreviation: R_ACACT9p Synonyms: _0 EC Number: 2.3.1.16 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : 3ohxccoa + coa --> accoa + ttccoa Confidence Level: 4 NCD genes: LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# proteins: NAME:acetyl-Coenzyme A acyltransferase 1#ABBREVIATION:Acaa1.1# citations: AUTHORS:Bout A, Franse MM, Collins J, Blonden L, Tager JM, Benne R.#TITLE:Characterization of the gene encoding human peroxisomal 3-oxoacyl-CoA thiolase (ACAA). No large DNA rearrangement in a thiolase-deficient patient.#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1679347# GENE ASSOCIATION: (30.1)
acetyl-CoA acyltransferase (tetracosanoyl-CoA), peroxisomal
Abbreviation: R_DOLK_L Synonyms: _0 EC Number: 2.7.1.108 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : ctp + (0.1) dolichol_L --> cdp + (0.1) dolp_L + h Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_P4507B12r Synonyms: _3 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : h + nadph + o2 + xol27oh --> h2o + nadp + xoltri27 Confidence Level: 4 NJ - substrates by similarity/analogy to hydroxylase reactions (literature notes NADH can often be used in place of NADPH) genes: LOCUS:_9420#TRANSCRIPT:1#ABBREVIATION:CYP7B1# proteins: NAME:cytochrom P450, family 7, subfamily B, polypeptide 1#ABBREVIATION:Cyp7b1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (9420.1)
Abbreviation: R_MANtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: man[g] --> man[c] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6T3g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre10 + paps --> h + ksii_core2 + pap Confidence Level: 4 NCD genes: LOCUS:_8534#TRANSCRIPT:1#ABBREVIATION:CHST1# LOCUS:_9469#TRANSCRIPT:1#ABBREVIATION:CHST3# proteins: NAME:carbohydrate (keratan sulfate Gal-6) sulfotransferase 1, Golgi#ABBREVIATION:Chst1.1-g# NAME:carbohydrate (chondroitin 6) sulfotransferase 3, Golgi#ABBREVIATION:Chst3.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Habuchi O, Hirahara Y, Uchimura K, Fukuta M#TITLE:Enzymatic sulfation of galactose residue of keratan sulfate by chondroitin 6-sulfotransferase#JOURNAL:Glycobiology#YEAR:1996#PUBMED:8991509# AUTHORS:Fukuta M, Kobayashi Y, Uchimura K, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of human chondroitin 6-sulfotransferase#JOURNAL:Biochim Biophys Acta#YEAR:1998#PUBMED:9714738# AUTHORS:Fukuta M, Inazawa J, Torii T, Tsuzuki K, Shimada E, Habuchi O#TITLE:Molecular cloning and characterization of human keratan sulfate Gal-6-sulfotransferase#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405439# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (8534.1) or (9469.1)
keratan sulfate II biosynthesis, precursor 10_C81H130N6O64S2X
Abbreviation: R_AG13T7g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre9 + uacgam --> h + ksi_pre10 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
keratan sulfate I biosynthesis, precursor 10_C129H209N9O98S2X
keratan sulfate I biosynthesis, precursor 9_C121H196N8O93S2X
Abbreviation: R_MDHm Synonyms: _0 EC Number: 1.1.1.37 SUBSYSTEM: Citric Acid Cycle Equation: [m] : mal-L + nad <==> h + nadh + oaa Confidence Level: 0 IF genes: LOCUS:_4191#TRANSCRIPT:1#ABBREVIATION:MDH2#ECNUMBERS:1.1.1.37# proteins: NAME:malate dehydrogenase 2 (NAD), mitochondrial#ABBREVIATION:Mdh2.1-m# citations: none GENE ASSOCIATION: (4191.1)
Abbreviation: R_EX_pydx_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pydx <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_B3GNT312g Synonyms: _9 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acglcgal14acglcgalgluside_hs + udpgal --> galacglcgal14acglcgalgluside_hs + h + udp Confidence Level: 2 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3#ABBREVIATION:B3gnt3.1# citations: AUTHORS:Yokoyama-Kobayashi M, Yamaguchi T, Sekine S, Kato S.#TITLE:Selection of cDNAs encoding putative type II membrane proteins on the cell surface from a human full-length cDNA bank.#JOURNAL:Gene#YEAR:1999#PUBMED:10072769# GENE ASSOCIATION: (10331.1)
Abbreviation: R_SMPD3g Synonyms: _4 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : h2o + sphmyln_hs --> cholp + crm_hs + h Confidence Level: 2 NJ genes: LOCUS:_55512#TRANSCRIPT:1#ABBREVIATION:SMPD3# LOCUS:_55512#TRANSCRIPT:2#ABBREVIATION:SMPD3# proteins: NAME:sphingomyelin phosphodiesterase 3, neutral membrane (neutral sphingomyelinase II) #ABBREVIATION:Smpd3.1# NAME:sphingomyelin phosphodiesterase 3, neutral membrane (neutral sphingomyelinase II) #ABBREVIATION:Smpd3.2# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (55512.1) or (55512.2)
sphingomyelin phosphodiesterase 3, neutral membrane (neutral sphingomyelinase II)
Abbreviation: R_ARTPLM2 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : Rtotal2coa <==> pmtcoa Confidence Level: 0 cytosolic conversion of pooled FA into palmitate (so subsequent transport into mit w/ FA oxidation can occur). Since Carbon stoichiometry normalized wrt C16 ratio between Rgroupcoa and pmtcoa is 1:1 HR fatty acid metabolism - R groups = acyl groups, Rtotal, Rtotal2, Rtotal3 correspond to three possible acyl groups added to glycerol R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MI134PK Synonyms: _2 EC Number: 2.7.1.134 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + mi134p --> adp + h + mi1346p Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# GENE ASSOCIATION:
_1D-myo-Inositol 1,3,4-trisphosphate_C6H9O15P3
_1D-myo-Inositol 1,3,4,6-tetrakisphosphate_C6H8O18P4
Abbreviation: R_DM_melanin_LPAREN_c_RPAREN_ Synonyms: _0 Equation: [c] : melanin --> Confidence Level: SAB demand for melanin in cytosol because there is no specific melanosome compartment genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NTD6 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [c] : damp + h2o --> dad-2 + pi Confidence Level: 2 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_22978#TRANSCRIPT:1#ABBREVIATION:NT5C2# proteins: NAME:_5'-nucleotidase, cytosolic II #ABBREVIATION:Nt5C2.1# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# GENE ASSOCIATION: (22978.1)
_1D-myo-Inositol 3,4-bisphosphate_C6H10O12P2
Abbreviation: R_MI34PP Synonyms: _5 EC Number: 3.1.3.66 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi34p --> mi3p-D + pi Confidence Level: 4 NCD genes: LOCUS:_3631#TRANSCRIPT:1#ABBREVIATION:INPP4A#ECNUMBERS:3.1.3.66# LOCUS:_8821#TRANSCRIPT:1#ABBREVIATION:INPP4B# LOCUS:_3631#TRANSCRIPT:2#ABBREVIATION:INPP4A# proteins: NAME:inositol polyphosphate-4-phosphatase, type I, 107kDa#ABBREVIATION:Inpp4a.1# NAME:inositol polyphosphate-4-phosphatase, type I, 107kDa#ABBREVIATION:Inpp4a.2# NAME:inositol polyphosphate-4-phosphatase, type II, 105kDa#ABBREVIATION:Inpp4b.1# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:Norris FA, Auethavekiat V, Majerus PW#TITLE:The isolation and characterization of cDNA encoding human and rat brain inositol polyphosphate 4-phosphatase#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7608176# AUTHORS:Norris FA, Atkins RC, Majerus PW#TITLE:The cDNA cloning and characterization of inositol polyphosphate 4-phosphatase type II. Evidence for conserved alternative splicing in the 4-phosphatase family#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9295334# GENE ASSOCIATION: (3631.2) or (3631.1) or (8821.1)
(Gal)4 (Glc)1 (GlcNAc)3 (LFuc)3 (Cer)1_C90H155N4O54FULLRCO
Abbreviation: R_EX_fucfucfucgalacglc13galacglcgal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucfucfucgalacglc13galacglcgal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(Gal)4 (Glc)1 (GlcNAc)3 (LFuc)3 (Cer)1 exchange
(alpha-D-mannosyl)3-beta-D-mannosyl-diacetylchitodiphosphodolichol, human liver homolog_C1480H2428N20O370P20
Abbreviation: R_ADSELK Synonyms: _0 EC Number: 2.7.1.25 SUBSYSTEM: Selenoamino acid metabolism Equation: [c] : adsel + atp --> 3padsel + adp + h Confidence Level: 1 SAB genes: LOCUS:_9061#TRANSCRIPT:1#ABBREVIATION:PAPSS1#ECNUMBERS:2.7.1.25,2.7.7.4# LOCUS:_9060#TRANSCRIPT:1#ABBREVIATION:PAPSS2#ECNUMBERS:2.7.7.4,&,2.7.1# proteins: NAME:_3'-phosphoadenosine 5'-phosphosulfate synthase 2 #ABBREVIATION:Papss2.1# NAME:_3'-phosphoadenosine 5'-phosphosulfate synthase 1 #ABBREVIATION:Papss1.1# citations: AUTHORS:Xu ZH, Otterness DM, Freimuth RR, Carlini EJ, Wood TC, Mitchell S, Moon E, Kim UJ, Xu JP, Siciliano MJ, Weinshilboum RM#TITLE:Human 3'-phosphoadenosine 5'-phosphosulfate synthetase 1 (PAPSS1) and PAPSS2: gene cloning, characterization and chromosomal localization#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679223# GENE ASSOCIATION: (9061.1) or (9060.1)
Abbreviation: R_FRDPtc Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: frdp[x] <==> frdp[c] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). See also Krisans (PMID: 14713247, PMID: 12121718) for synthesis of cholesterol pathway (why FRDP needs to be transported between ER and peroxisome) NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_PIK3 Synonyms: _4 EC Number: 2.7.1.137 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + pail_hs --> adp + h + pail3p_hs Confidence Level: 4 NCD genes: LOCUS:_5289#TRANSCRIPT:1#ABBREVIATION:PIK3C3# LOCUS:_5291#TRANSCRIPT:1#ABBREVIATION:PIK3CB#ECNUMBERS:2.7.1.137# LOCUS:_5290#TRANSCRIPT:1#ABBREVIATION:PIK3CA#ECNUMBERS:2.7.1.137# LOCUS:_5287#TRANSCRIPT:1#ABBREVIATION:PIK3C2B#ECNUMBERS:2.7.1.137# LOCUS:_5293#TRANSCRIPT:1#ABBREVIATION:PIK3CD#ECNUMBERS:2.7.1.137# LOCUS:_5286#TRANSCRIPT:1#ABBREVIATION:PIK3C2A#ECNUMBERS:2.7.1.137# LOCUS:_5294#TRANSCRIPT:1#ABBREVIATION:PIK3CG#ECNUMBERS:2.7.1.137# LOCUS:_23533#TRANSCRIPT:1#ABBREVIATION:P101-PI3K# LOCUS:_5288#TRANSCRIPT:1#ABBREVIATION:PIK3C2G#ECNUMBERS:2.7.1.137# LOCUS:_5295#TRANSCRIPT:1#ABBREVIATION:PIK3R1# LOCUS:_8503#TRANSCRIPT:1#ABBREVIATION:PIK3R3# LOCUS:_5296#TRANSCRIPT:1#ABBREVIATION:PIK3R2# LOCUS:_5295#TRANSCRIPT:2#ABBREVIATION:PIK3R1# LOCUS:_5295#TRANSCRIPT:3#ABBREVIATION:PIK3R1# LOCUS:_10870#TRANSCRIPT:1#ABBREVIATION:HCST# proteins: NAME:phosphoinositide-3-kinase (p110a, p85a)#ABBREVIATION:Pik3car1.1# NAME:phosphoinositide-3-kinase (p110a, p55a)#ABBREVIATION:Pik3car1.2# NAME:phosphoinositide-3-kinase (p110a, p50a)#ABBREVIATION:Pik3car.3# NAME:phosphoinositide-3-kinase (p110a, p85b)#ABBREVIATION:Pik3car2.1# NAME:phosphoinositide-3-kinase (p110a, p55c)#ABBREVIATION:Pik3car3.1# NAME:phosphoinositide-3-kinase (p110b, p85a)#ABBREVIATION:Pik3cbr1.1# NAME:phosphoinositide-3-kinase (p110b, p55a)#ABBREVIATION:Pik3cbr1.2# NAME:phosphoinositide-3-kinase (p110b, p50a)#ABBREVIATION:Pik3cbr1.3# NAME:phosphoinositide-3-kinase (p110b, p85b)#ABBREVIATION:Pik3cbr2.1# NAME:phosphoinositide-3-kinase (p110b, p55c)#ABBREVIATION:Pik3cbr3.1# NAME:phosphoinositide-3-kinase (p110c, p85a)#ABBREVIATION:Pik3cdr1.1# NAME:phosphoinositide-3-kinase (p110c, p55a)#ABBREVIATION:Pik3cdr1.2# NAME:phosphoinositide-3-kinase (p110c, p50a)#ABBREVIATION:Pik3cdr1.3# NAME:phosphoinositide-3-kinase (p110c, p85b)#ABBREVIATION:Pik3cdr2.1# NAME:phosphoinositide-3-kinase (p110c, p55c)#ABBREVIATION:Pik3cdr3.1# NAME:phosphoinositide-3-kinase (PIK3cg, p101)#ABBREVIATION:Pik3cgr5.1# NAME:phosphoinositide-3-kinase (PIK3CG, p87 adapter protein)#ABBREVIATION:Pik3cgap.1# NAME:phosphoinositide-3-kinase, class 2, alpha polypeptide#ABBREVIATION:Pik3c2a.1# NAME:phosphoinositide-3-kinase, class 2, beta polypeptide#ABBREVIATION:Pik3c2b.1# NAME:phosphoinositide-3-kinase, class 2, gamma polypeptide#ABBREVIATION:Pik3c2g.1# NAME:phosphoinositide-3-kinase, class 3#ABBREVIATION:Pik3c3.1# citations: AUTHORS:Volinia S, Hiles I, Ormondroyd E, Nizetic D, Antonacci R, Rocchi M, Waterfield MD#TITLE:Molecular cloning, cDNA sequence, and chromosomal localization of the human phosphatidylinositol 3-kinase p110 alpha (PIK3CA) gene#JOURNAL:Genomics#YEAR:1994#PUBMED:7713498# AUTHORS:Hu P, Mondino A, Skolnik EY, Schlessinger J#TITLE:Cloning of a novel, ubiquitously expressed human phosphatidylinositol 3-kinase and identification of its binding site on p85#JOURNAL:Mol Cell Biol#YEAR:1993#PUBMED:8246984# AUTHORS:Vanhaesebroeck B, Welham MJ, Kotani K, Stein R, Warne PH, Zvelebil MJ, Higashi K, Volinia S, Downward J, Waterfield MD#TITLE:P110delta, a novel phosphoinositide 3-kinase in leukocytes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9113989# AUTHORS:Stoyanov B, Volinia S, Hanck T, Rubio I, Loubtchenkov M, Malek D, Stoyanova S, Vanhaesebroeck B, Dhand R, Nurnberg B, et al#TITLE:Cloning and characterization of a G protein-activated human phosphoinositide-3 kinase#JOURNAL:Science#YEAR:1995#PUBMED:7624799# AUTHORS:Domin J, Pages F, Volinia S, Rittenhouse SE, Zvelebil MJ, Stein RC, Waterfield MD#TITLE:Cloning of a human phosphoinositide 3-kinase with a C2 domain that displays reduced sensitivity to the inhibitor wortmannin#JOURNAL:Biochem J#YEAR:1997#PUBMED:9337861# AUTHORS:Arcaro A, Volinia S, Zvelebil MJ, Stein R, Watton SJ, Layton MJ, Gout I, Ahmadi K, Downward J, Waterfield MD#TITLE:Human phosphoinositide 3-kinase C2beta, the role of calcium and the C2 domain in enzyme activity#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9830063# AUTHORS:Rozycka M, Lu YJ, Brown RA, Lau MR, Shipley JM, Fry MJ#TITLE:cDNA cloning of a third human C2-domain-containing class II phosphoinositide 3-kinase, PI3K-C2gamma, and chromosomal assignment of this gene (PIK3C2G) to 12p12#JOURNAL:Genomics#YEAR:1998#PUBMED:9878262# AUTHORS:Volinia S, Dhand R, Vanhaesebroeck B, MacDougall LK, Stein R, Zvelebil MJ, Domin J, Panaretou C, Waterfield MD#TITLE:A human phosphatidylinositol 3-kinase complex related to the yeast Vps34p-Vps15p protein sorting system#JOURNAL:EMBO J#YEAR:1995#PUBMED:7628435# GENE ASSOCIATION: (5295.2 and 5291.1) or (23533.1 and 5294.1) or (5290.1 and 5295.1) or (5293.1 and 5295.3) or (5287.1) or (5286.1) or (5293.1 and 5296.1) or (5295.2 and 5293.1) or (5296.1 and 5291.1) or (5290.1 and 5295.3) or (5288.1) or (5290.1 and 8503.1) or (5291.1 and 5295.3) or (10870.1 and 5294.1) or (8503.1 and 5291.1) or (5293.1 and 5295.1) or (5296.1 and 5290.1) or (5289.1) or (5293.1 and 8503.1) or (5291.1 and 5295.1) or (5295.2 and 5290.1)
Galactoside 2-alpha-L-fucosyltransferase 1
Abbreviation: R_FUT16g Synonyms: _4 EC Number: 2.4.1.69 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacglcgal14acglcgalgluside_hs + gdpfuc --> fucgalacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2523#TRANSCRIPT:1#ABBREVIATION:FUT1#ECNUMBERS:2.4.1.69# proteins: NAME:fucosyltransferase 1#ABBREVIATION:Fut1.1# citations: AUTHORS:Larsen RD, Ernst LK, Nair RP, Lowe JB.#TITLE:Molecular cloning, sequence, and expression of a human GDP-L-fucose:beta-D-galactoside 2-alpha-L-fucosyltransferase cDNA that can form the H blood group antigen.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990#PUBMED:2118655# GENE ASSOCIATION: (2523.1)
chondroitin sulfate B / dermatan sulfate (IdoA2S-GalNAc4S) proteoglycan_C45H65N2O48S4X
_5-Methylthio-5-deoxy-D-ribose 1-phosphate_C6H11O7PS
Abbreviation: R_FRTT Synonyms: _1 EC Number: 2.5.1.29 SUBSYSTEM: Cholesterol Metabolism Equation: [c] : frdp + ipdp --> ggdp + ppi Confidence Level: 2 genes: LOCUS:_9453#TRANSCRIPT:1#ABBREVIATION:GGPS1#ECNUMBERS:2.5.1.1,2.5.1.10,,2.5.1.29# proteins: NAME:geranylgeranyl diphosphate synthase 1#ABBREVIATION:Ggps1.1# citations: none GENE ASSOCIATION: (9453.1)
Abbreviation: R_CYTK1m Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [m] : atp + cmp <==> adp + cdp Confidence Level: 1 genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_2HCO3_NAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) hco3[e] + na1[e] <==> (2) hco3[c] + na1[c] Confidence Level: 4 MM genes: LOCUS:_8671#TRANSCRIPT:1#ABBREVIATION:SLC4A4# LOCUS:_57835#TRANSCRIPT:1#ABBREVIATION:SLC4A5# LOCUS:_57835#TRANSCRIPT:2#ABBREVIATION:SLC4A5# LOCUS:_57835#TRANSCRIPT:3#ABBREVIATION:SLC4A5# LOCUS:_57835#TRANSCRIPT:4#ABBREVIATION:SLC4A5# proteins: NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 4, extracellular#ABBREVIATION:Slc4a4.1-e# NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 5, extracellular#ABBREVIATION:Slc4a5.1-e# NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 5, extracellular#ABBREVIATION:Slc4a5.2-e# NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 5, extracellular#ABBREVIATION:Slc4a5.3-e# NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 5, extracellular#ABBREVIATION:Slc4a5.4-e# citations: AUTHORS:Romero MF, Fulton CM, Boron WF.#TITLE:The SLC4 family of HCO 3 - transporters.#YEAR:2004#PUBMED:14722772# GENE ASSOCIATION: (57835.3) or (8671.1) or (57835.2) or (57835.1) or (57835.4)
bicarbonate transport (Na/HCO3 1:2 cotransport)
Abbreviation: R_HMGCOARx Synonyms: _0 EC Number: 1.1.1.34 SUBSYSTEM: Cholesterol Metabolism Equation: [x] : (2) h + hmgcoa + (2) nadph --> coa + mev-R + (2) nadp Confidence Level: 4 NCD Peroxisomal version - NJ genes: LOCUS:_3156#TRANSCRIPT:1#ABBREVIATION:HMGCR#ECNUMBERS:1.1.1.88# proteins: NAME:_3-hydroxy-3-methylglutaryl-Coenzyme A reductase#ABBREVIATION:Hmgcr.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (3156.1)
Hydroxymethylglutaryl CoA reductase (ir)
Abbreviation: R_PIK4n Synonyms: _3 EC Number: 2.7.1.67 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + pail_hs --> adp + h + pail4p_hs Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
Abbreviation: R_RTOTAL2CRNCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : Rtotal2coa + crn --> Rtotal2crn + coa Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (1375.1) or (1375.4) or (1375.3) or (1374.1) or (126129.1) or (1375.2)
Abbreviation: R_PRASCS Synonyms: _0 EC Number: 6.3.2.6 SUBSYSTEM: IMP Biosynthesis Equation: [c] : 5aizc + asp-L + atp <==> 25aics + adp + h + pi Confidence Level: 4 genes: LOCUS:_10606#TRANSCRIPT:1#ABBREVIATION:PAICS#ECNUMBERS:6.3.2.6,4.1.1.21# proteins: NAME:phosphoribosylaminoimidazole carboxylase, phosphoribosylaminoimidazole succinocarboxamide synthetase #ABBREVIATION:PaicS.1# citations: AUTHORS:Brayton KA, Chen Z, Zhou G, Nagy PL, Gavalas A, Trent JM, Deaven LL, Dixon JE, Zalkin H. #TITLE:Two genes for de novo purine nucleotide synthesis on human chromosome 4 are closely linked and divergently transcribed.#JOURNAL:J Biol Chem. #YEAR:1994#PUBMED:8106516# AUTHORS:Minet M, Lacroute F.#TITLE:Cloning and sequencing of a human cDNA coding for a multifunctional polypeptide of the purine pathway by complementation of the ade2-101 mutant in Saccharomyces cerevisiae.#JOURNAL:Curr Genet.#YEAR:1990#PUBMED:2253271# AUTHORS:Barton JW, Hart IM, Patterson D.#TITLE:Mapping of a locus correcting lack of phosphoribosylaminoimidazole carboxylase activity in Chinese hamster ovary cell Ade-D mutants to human chromosome 4. #JOURNAL:Genomics#YEAR:1991#PUBMED:2004782# GENE ASSOCIATION: (10606.1)
phosphoribosylaminoimidazolesuccinocarboxamide synthase
Abbreviation: R_DESAT18_8 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + nadh + o2 + od2coa --> (2) h2o + lneldccoa + nad Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_9415#TRANSCRIPT:1#ABBREVIATION:FADS2#ECNUMBERS:1.14.99.25# proteins: NAME:fatty acid desaturase 2#ABBREVIATION:Fads2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Cho HP, Nakamura MT, Clarke SD#TITLE:Cloning, expression, and nutritional regulation of the mammalian delta-6 desaturase#JOURNAL:The Journal of Biological Chemistry#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION: (9415.1)
fatty acyl-CoA desaturase (n-C18:1CoA -> n-C18:2CoA)
Abbreviation: R_CYSTGLUex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: Lcystin[e] + glu-L[c] --> Lcystin[c] + glu-L[e] Confidence Level: 4 SAB see PMID 14770310 genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_23657#TRANSCRIPT:1#ABBREVIATION:SLC7A11# proteins: NAME:solute carrier family 3 member 2/family 7 member 11#ABBREVIATION:Slc3a2/7a11# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (23657.1 and 6520.1)
Abbreviation: R_URIt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: uri[e] <==> uri[c] Confidence Level: 4 IT genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (3177.1) or (2030.1)
Abbreviation: R_PYAM5POr Synonyms: _0 EC Number: 1.4.3.5 SUBSYSTEM: Vitamin B6 Metabolism Equation: [c] : h2o + o2 + pyam5p <==> h2o2 + nh4 + pydx5p Confidence Level: 4 NCD IT genes: LOCUS:_55163#TRANSCRIPT:1#ABBREVIATION:PNPO#ECNUMBERS:1.4.3.5# proteins: NAME:Pyridoxine-5'-phosphate oxidase #ABBREVIATION:PnpO.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# GENE ASSOCIATION: (55163.1)
Abbreviation: R_PYDXK Synonyms: _0 EC Number: 2.7.1.35 SUBSYSTEM: Vitamin B6 Metabolism Equation: [c] : atp + pydx --> adp + h + pydx5p Confidence Level: 4 NCD genes: LOCUS:_8566#TRANSCRIPT:1#ABBREVIATION:PDXK#ECNUMBERS:2.7.1.35# proteins: NAME:pyridoxal (pyridoxine, vitamin B6) kinase #ABBREVIATION:PdxK.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# AUTHORS:Hanna MC, Turner AJ, Kirkness EF.#TITLE:Human pyridoxal kinase. cDNA cloning, expression, and modulation by ligands of the benzodiazepine receptor. #JOURNAL:J Biol Chem. #YEAR:1997#PUBMED:9099727# GENE ASSOCIATION: (8566.1)
Abbreviation: R_DM_dgtp_LPAREN_m_RPAREN_ Synonyms: _0 Equation: [m] : dgtp --> Confidence Level: IT genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I, degradation product 39_C48H81N3O36
keratan sulfate I, degradation product 38_C54H91N3O41
Abbreviation: R_GALASE14ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg38 --> gal + ksi_deg39 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
_5-Methylthio-5-deoxy-D-ribulose 1-phosphate_C6H11O7PS
Abbreviation: R_MDRPD Synonyms: _0 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : 5mdru1p --> dkmpp + h2o Confidence Level: 2 JLR- Based on reaction given for B. subtilis (Sekowska A and A. Danchin paper(2002) ) genes: none proteins: none citations: AUTHORS:Myers RW, Abeles RH#TITLE:Conversion of 5-S-ethyl-5-thio-D-ribose to ethionine in Klebsiella pneumoniae. Basis for the selective toxicity of 5-S-ethyl-5-thio-D-ribose#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2543672# GENE ASSOCIATION:
_5-Methylthio-5-deoxy-D-ribulose 1-phosphate dehydratase
Abbreviation: R_GCCam Synonyms: _0 EC Number: 1.4.4.2 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : gly + h + lpro <==> alpro + co2 Confidence Level: 4 NCD genes: LOCUS:_2731#TRANSCRIPT:1#ABBREVIATION:GLDC#ECNUMBERS:1.4.4.2# LOCUS:_275#TRANSCRIPT:1#ABBREVIATION:AMT#ECNUMBERS:2.1.2.10# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_2653#TRANSCRIPT:1#ABBREVIATION:GCSH# proteins: NAME:glycine dehydrogenase (decarboxylating; glycine decarboxylase, glycine cleavage system protein P), mitochondrial #ABBREVIATION:Gldc.1-m# NAME:aminomethyltransferase (glycine cleavage system protein T), mitochondrial #ABBREVIATION:Amt.1-m# NAME:glycine cleavage system protein H (aminomethyl carrier) #ABBREVIATION:Gcsh.1-m# NAME:dihydrolipoamide dehydrogenase, mitochondrial#ABBREVIATION:Dld.1-m# citations: AUTHORS:Lee HH, Kim do J, Ahn HJ, Ha JY, Suh SW.#TITLE:Crystal structure of T-protein of the glycine cleavage system. Cofactor binding, insights into H-protein recognition, and molecular basis for understanding nonketotic hyperglycinemia.#YEAR:2004#PUBMED:15355973# GENE ASSOCIATION: (275.1) and (1738.1) and (2731.1) and (2653.1)
glycine-cleavage complex (lipoylprotein), mitochondrial
Abbreviation: R_NAt5 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[c] + nh4[e] <==> na1[e] + nh4[c] Confidence Level: 4 NCD genes: LOCUS:_6550#TRANSCRIPT:1#ABBREVIATION:SLC9A3# LOCUS:_6549#TRANSCRIPT:1#ABBREVIATION:SLC9A2# LOCUS:_6548#TRANSCRIPT:1#ABBREVIATION:SLC9A1# proteins: NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 1 (antiporter, Na+/H+, amiloride sensitive)#ABBREVIATION:Slc9a1.1-e# NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 2#ABBREVIATION:Slc9a2.1-e# NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 3#ABBREVIATION:Slc9a3.1-e# citations: AUTHORS:Aronson PS#TITLE:Kinetic properties of the plasma membrane Na+-H+ exchanger#JOURNAL:Annu Rev Physiol#YEAR:1985#PUBMED:2581505# AUTHORS:Sardet C, Franchi A, Pouyssegur J#TITLE:Molecular cloning, primary structure, and expression of the human growth factor-activatable Na+/H+ antiporter.#JOURNAL:Cell#YEAR:1989#PUBMED:2536298# AUTHORS:Orlowski J, Grinstein S#TITLE:Diversity of the mammalian sodium/proton exchanger SLC9 gene family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12845533# AUTHORS:Malakooti J, Dahdal RY, Schmidt L, Layden TJ, Dudeja PK, Ramaswamy K#TITLE:Molecular cloning, tissue distribution, and functional expression of the human Na(+)/H(+) exchanger NHE2#JOURNAL:Am J Physiol#YEAR:1999#PUBMED:10444453# AUTHORS:Ghishan FK, Knobel SM, Summar M#TITLE:Molecular cloning, sequencing, chromosomal localization, and tissue distribution of the human Na+/H+ exchanger (SLC9A2)#JOURNAL:Genomics#YEAR:1995#PUBMED:8595899# AUTHORS:Brant SR, Yun CH, Donowitz M, Tse CM#TITLE:Cloning, tissue distribution, and functional analysis of the human Na+/N+ exchanger isoform, NHE3#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7631746# AUTHORS:Szpirer C, Szpirer J, Riviere M, Levan G, Orlowski J#TITLE:Chromosomal assignment of four genes encoding Na/H exchanger isoforms in human and rat.#JOURNAL:Mamm Genome#YEAR:1994#PUBMED:8199403# GENE ASSOCIATION: (6549.1) or (6548.1) or (6550.1)
keratan sulfate I, degradation product 17_C152H245N10O132S7
Abbreviation: R_CYStec Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[e] <==> cys-L[c] Confidence Level: 4 SAB genes: LOCUS:_124935#TRANSCRIPT:1#ABBREVIATION:SLC43A2# proteins: NAME:solute carrier family 43, member 2#ABBREVIATION:Slc43a2.1# citations: AUTHORS:Bodoy S, Martin L, Zorzano A, Palacin M, Estevez R, Bertran J#TITLE:Identification of LAT4, a novel amino acid transporter with system L activity#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15659399# AUTHORS:Hediger MA, Romero MF, Peng JB, Rolfs A, Takanaga H, Bruford EA#TITLE:The ABCs of solute carriers: physiological, pathological and therapeutic implications of human membrane transport proteinsIntroduction#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14624363# GENE ASSOCIATION: (124935.1)
L-cysteine transport via diffusion (extracellular to cytosol)
Abbreviation: R_HSD17B4x Synonyms: _3 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : cholcoads + h + nadph + o2 --> cholcoaone + h2o + nadp Confidence Level: 4 see Russell bile acid synthesis review paper in human recon citation db adamanski et al Steroids (1997) 62:159 notes cofactor NADP/NADPH use genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# proteins: NAME:hydroxysteroid (17-beta) dehydrogenase 4#ABBREVIATION:Hsd17b4.1-x# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Leenders F, Dolez V, Begue A, Moller G, Gloeckner JC, deLaunoit Y, Adamski J#TITLE:Structure of the gene for the human 17-beta-hydroxysteroid dehydrogenase type IV#JOURNAL:Mammalian Genome#YEAR:1998# AUTHORS:Adamski J, Leenders F Carstensen JF, Kaumann M, Markus MM, Husen B, Tesdorpf JG, Seedor U, deLaunoit Y, Jakob F#TITLE:Steroids, fatty acyl-CoA, and sterols are substrates of 80-kDa multifunctional protein#JOURNAL:Steroids#YEAR:1997# AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (3295.1)
hydroxysteroid (17-beta) dehydrogenase 4
Abbreviation: R_PRAGSr Synonyms: _1 EC Number: 6.3.4.13 SUBSYSTEM: IMP Biosynthesis Equation: [c] : atp + gly + pram <==> adp + gar + h + pi Confidence Level: 4 JLR- added reversible form of PRAGS genes: LOCUS:_2618#TRANSCRIPT:1#ABBREVIATION:GART#ECNUMBERS:2.1.2.2,,6.3.1.# LOCUS:_2618#TRANSCRIPT:2#ABBREVIATION:GART#ECNUMBERS:2.1.2.2,,6.3.1.# proteins: NAME:phosphoribosylglycinamide formyltransferase, phosphoribosylglycinamide synthetase, phosphoribosylaminoimidazole synthetase #ABBREVIATION:GarT.1# NAME:phosphoribosylglycinamide formyltransferase, phosphoribosylglycinamide synthetase, phosphoribosylaminoimidazole synthetase #ABBREVIATION:GarT.2# citations: AUTHORS:Brodsky G, Barnes T, Bleskan J, Becker L, Cox M, Patterson D.#TITLE:The human GARS-AIRS-GART gene encodes two proteins which are differentially expressed during human brain development and temporally overexpressed in cerebellum of individuals with Down syndrome. #JOURNAL:Hum Mol Genet. #YEAR:1997#PUBMED:9328467# AUTHORS:Zhang Y, Desharnais J, Greasley SE, Beardsley GP, Boger DL, Wilson IA.#TITLE:Crystal structures of human GAR Tfase at low and high pH and with substrate beta-GAR.#JOURNAL:Biochemistry.#YEAR:2002#PUBMED:12450384# AUTHORS:Schild D, Brake AJ, Kiefer MC, Young D, Barr PJ.#TITLE:Cloning of three human multifunctional de novo purine biosynthetic genes by functional complementation of yeast mutations. #JOURNAL:Proc Natl Acad Sci U S A. #YEAR:1990#PUBMED:2183217# AUTHORS:Poch MT, Qin W, Caperelli CA. #TITLE:The human trifunctional enzyme of de novo purine biosynthesis: heterologous expression, purification, and preliminary characterization.#JOURNAL:Protein Expr Purif.#YEAR:1998#PUBMED:9473452# GENE ASSOCIATION: (2618.1) or (2618.2)
Abbreviation: R_DTTPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: dttp[c] <==> dttp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ASNS1 Synonyms: _0 EC Number: 6.3.5.4 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [c] : asp-L + atp + gln-L + h2o --> amp + asn-L + glu-L + h + ppi Confidence Level: 4 genes: LOCUS:_440#TRANSCRIPT:1#ABBREVIATION:ASNS#ECNUMBERS:6.3.5.4# LOCUS:_440#TRANSCRIPT:3#ABBREVIATION:ASNS#ECNUMBERS:6.3.5.4# LOCUS:_440#TRANSCRIPT:2#ABBREVIATION:ASNS#ECNUMBERS:6.3.5.4# proteins: NAME:asparagine synthetase#ABBREVIATION:Asns.1# NAME:asparagine synthetase#ABBREVIATION:Asns.2# NAME:asparagine synthetase#ABBREVIATION:Asns.3# citations: AUTHORS:Van Heeke G, Schuster SM.#TITLE:Expression of human asparagine synthetase in Escherichia coli.#YEAR:1989#PUBMED:2564390# AUTHORS:Andrulis IL, Chen J, Ray PN.#TITLE:Isolation of human cDNAs for asparagine synthetase and expression in Jensen rat sarcoma cells. #YEAR:1987#PUBMED:2886907# GENE ASSOCIATION: (440.1) or (440.3) or (440.2)
asparagine synthase (glutamine-hydrolysing)
Abbreviation: R_ALOX5 Synonyms: _2 EC Number: 1.13.11.34 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : arachd + o2 --> 5HPET Confidence Level: 4 cofactors + usage still not clear see version ALOX5b for ATP concomittant hydrolysis - literature notes in vitro requirement for Ca and ATP for activity in addition to Fe genes: LOCUS:_240#TRANSCRIPT:1#ABBREVIATION:ALOX5#ECNUMBERS:1.13.11.34# proteins: NAME:arachidonate 5-lipoxygenase#ABBREVIATION:Alox5.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Matsumoto T, Funk CD, Radmark O, Hoog JO, Jornvall H, Samuelsson B.#TITLE:Molecular cloning and amino acid sequence of human 5-lipoxygenase.#JOURNAL:Proc Natl Acad Sci#YEAR:1988#PUBMED:2829172# GENE ASSOCIATION: (240.1)
Abbreviation: R_CSNAT2m Synonyms: _0 EC Number: 2.3.1.7 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + pcrn <==> crn + ppcoa Confidence Level: 4 TDV genes: LOCUS:_1384#TRANSCRIPT:1#ABBREVIATION:CRAT#ECNUMBERS:2.3.1.7# proteins: NAME:carnitine acetyltransferase, mitochondrial#ABBREVIATION:Crat.1-m# citations: AUTHORS:Lysiak W, Toth PP, Suelter CH, Bieber LL.#TITLE:Quantitation of the efflux of acylcarnitines from rat heart, brain, and liver mitochondria.#JOURNAL:J Biol Chem#YEAR:1986#PUBMED:3759988# AUTHORS:Ramsay, Gandour, van der Leij#TITLE:Molecular enzymology of carnitine transfer and transport#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11257506# GENE ASSOCIATION: (1384.1)
carnitine O-aceyltransferase, mitochondrial
Abbreviation: R_G14T13g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre23 + udpgal --> h + ksi_pre24 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
keratan sulfate I biosynthesis, precursor 23_C185H296N13O153S7X
keratan sulfate I biosynthesis, precursor 24_C191H306N13O158S7X
beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase, Golgi
Abbreviation: R_GGH_DASH_10FTHF5GLUe Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [e] : 10fthf5glu + (4) h2o --> 10fthf + (4) glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) - last step from 5-glutamyl to monoglutamyl is in one step IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-e# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (10FTHF5GLU), extracellular
Abbreviation: R_PRDX Synonyms: _0 EC Number: 1.11.1.7 SUBSYSTEM: Miscellaneous Equation: [c] : h2o2 + meoh --> fald + (2) h2o Confidence Level: 0 RS/TV (6/3/05) Peroxidases can act on a variety of substrates which are oxidized, not just methanol. genes: LOCUS:_8288#TRANSCRIPT:1#ABBREVIATION:EPX#ECNUMBERS:1.11.1.7# proteins: NAME:Eosinophil peroxidase, cytosolic#ABBREVIATION:Epx.1# citations: AUTHORS:Wu W, Chen Y, Hazen SL.#TITLE:Eosinophil peroxidase nitrates protein tyrosyl residues. Implications for oxidative damage by nitrating intermediates in eosinophilic inflammatory disorders. #JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10464338# GENE ASSOCIATION: (8288.1)
Abbreviation: R_TYRDOPO3 Synonyms: _1 EC Number: 1.14.18.1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : (2) 34dhphe + o2 --> (2) dopaqn + (2) h2o Confidence Level: 4 SAB genes: LOCUS:_7299#TRANSCRIPT:1#ABBREVIATION:TYR#ECNUMBERS:1.14.18.1# proteins: NAME:tyrosinase#ABBREVIATION:Tyr.1# citations: AUTHORS:Aaron Bunsen Lerner, Thomas B. Fitzpatrick, Evan Calkins, and William H. Summerson#TITLE:MAMMALIAN TYROSINASE: PREPARATION AND PROPERTIES#JOURNAL:J. Biol. Chem#YEAR:1949# AUTHORS:Pomerantz SH#TITLE:The tyrosine hydroxylase activity of mammalian tyrosinase#JOURNAL:J Biol Chem#YEAR:1968#PUBMED:5294951# GENE ASSOCIATION: (7299.1)
Tyrosine:dopa oxidase (dopaquinone producing 2)
Abbreviation: R_GLUt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: glu-L[c] + h[c] <==> glu-L[m] + h[m] Confidence Level: 4 NCD genes: LOCUS:_8604#TRANSCRIPT:1#ABBREVIATION:SLC25A12# LOCUS:_83733#TRANSCRIPT:1#ABBREVIATION:SLC25A18# LOCUS:_79751#TRANSCRIPT:1#ABBREVIATION:SLC25A22# LOCUS:_10165#TRANSCRIPT:1#ABBREVIATION:SLC25A13# proteins: NAME:Solute carrier family 25 member 12, mitochondrial #ABBREVIATION:Slc25a12.1-m# NAME: solute carrier family 25 member 13, mitochondrial #ABBREVIATION:Slc25a13.1-m# NAME:solute carrier family 25 member 22, mitochondrial #ABBREVIATION:Slc25a22.1-m# NAME:solute carrier family 25 member 18, mitochondrial #ABBREVIATION:Slc25a18.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (83733.1) or (8604.1) or (79751.1) or (10165.1)
L-glutamate reversible transport via proton symport, mitochondrial
Abbreviation: R_PHEtec Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: phe-L[e] <==> phe-L[c] Confidence Level: 4 SAB genes: LOCUS:_124935#TRANSCRIPT:1#ABBREVIATION:SLC43A2# LOCUS:_117247#TRANSCRIPT:1#ABBREVIATION:SLC16A10# proteins: NAME:solute carrier family 43, member 2#ABBREVIATION:Slc43a2.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 10#ABBREVIATION:Slc16a10.1-e# citations: AUTHORS:Bodoy S, Martin L, Zorzano A, Palacin M, Estevez R, Bertran J#TITLE:Identification of LAT4, a novel amino acid transporter with system L activity#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15659399# AUTHORS:Hediger MA, Romero MF, Peng JB, Rolfs A, Takanaga H, Bruford EA#TITLE:The ABCs of solute carriers: physiological, pathological and therapeutic implications of human membrane transport proteinsIntroduction#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14624363# AUTHORS:Kim do K, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H#TITLE:The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location#JOURNAL:Genomics#YEAR:2002#PUBMED:11827462# GENE ASSOCIATION: (124935.1) or (117247.1)
L-phenylalanine transport via diffusion (extracellular to cytosol)
Abbreviation: R_EX_dag_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dag_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GULNter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: guln[r] --> guln[c] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLXtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: glx[c] <==> glx[x] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP.#TITLE:Glycolate and glyoxylate metabolism in HepG2 cells.#YEAR:2004#PUBMED:15240345# GENE ASSOCIATION:
Abbreviation: R_EX_chsterol_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : chsterol <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
de-Fuc, reducing GlcNAc removed form of PA6 (w/o peptide linkage)_C76H123N5O57
Abbreviation: R_SIAASE Synonyms: _4 EC Number: 3.2.1.18 SUBSYSTEM: N-Glycan Degradation Equation: [c] : (2) h2o + s2l2n2m2mn --> (2) acnam + l2n2m2mn Confidence Level: 0 NCD genes: LOCUS:_4759#TRANSCRIPT:1#ABBREVIATION:NEU2# LOCUS:_129807#TRANSCRIPT:1#ABBREVIATION:NEU4# proteins: NAME:sialidase 2 (sk muscle)#ABBREVIATION:Neu2.1# NAME:sialidase 4 (liver)#ABBREVIATION:Neu4.1# citations: AUTHORS:Monti E, Preti A, Rossi E, Ballabio A, Borsani G.#TITLE:Cloning and characterization of NEU2, a human gene homologous to rodent soluble sialidases.#JOURNAL:Genomics#YEAR:1999#PUBMED:10191093# GENE ASSOCIATION: (4759.1) or (129807.1)
Abbreviation: R_FUT910g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucgalacglc13galacglcgal14acglcgalgluside_hs + gdpfuc --> fucfucgalacglc13galacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
(Gal)4 (Glc)1 (GlcNAc)3 (LFuc)2 (Cer)1_C84H145N4O50FULLRCO
(Gal)4 (Glc)1 (GlcNAc)3 (LFuc)1 (Cer)1_C78H135N4O46FULLRCO
chondroitin sulfate C (GalNAc6S-GlcA) proteoglycan_C45H66N2O45S3X
Abbreviation: R_FPGS7 Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [c] : 10fthf + (4) atp + (4) glu-L --> 10fthf5glu + (4) adp + (4) h + (4) pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_7DHCHSTEROLtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: _7dhchsterol[r] <==> 7dhchsterol[c] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: none GENE ASSOCIATION:
cholesterol precursor intracellular transport
Abbreviation: R_CSNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: csn[e] <==> csn[c] Confidence Level: 4 IT genes: LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Yao SY, Ng AM, Vickers MF, Sundaram M, Cass CE, Baldwin SA, Young JD.#TITLE:Functional and molecular characterization of nucleobase transport by recombinant human and rat equilibrative nucleoside transporters 1 and 2.#JOURNAL:J Biol Chem. #YEAR:2002#PUBMED:12006583# GENE ASSOCIATION: (3177.1)
cytosine transport via facilated diffusion
_1-alkyl 2-lysoglycerol 3-phosphocholine_C10H21O5NPFULLR
heparan sulfate, degradation product 15_C49H71N2O57S5
phosphatidylinositol 3-phosphate 4-kinase
Abbreviation: R_PI3P4K Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + pail3p_hs --> adp + h + pail34p_hs Confidence Level: 4 NCD FULLR corresponds to acyl/R groups in texts. NJ changed EC from 2.7.1.149 (pail5p) to 2.7.1.68 (pail4p) - NCD genes: LOCUS:_8394#TRANSCRIPT:1#ABBREVIATION:PIP5K1A#ECNUMBERS:2.7.1.68# LOCUS:_5297#TRANSCRIPT:1#ABBREVIATION:PIK4CA#ECNUMBERS:2.7.1.67,2.7.1.68# LOCUS:_23396#TRANSCRIPT:1#ABBREVIATION:PIP5K1C# LOCUS:_5298#TRANSCRIPT:1#ABBREVIATION:PIK4CB# LOCUS:_55361#TRANSCRIPT:1#ABBREVIATION:PI4KII# LOCUS:_8395#TRANSCRIPT:1#ABBREVIATION:PIP5K1B# proteins: NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, gamma #ABBREVIATION:Pip5k1c.1# NAME:phosphatidylinositol 4-kinase, catalytic, alpha polypeptide#ABBREVIATION:Pik4ca.1# NAME:phosphatidylinositol 4-kinase, catalytic, beta polypeptide#ABBREVIATION:Pik4cb.1# NAME:phosphtaidylinositol 4-kinase, type II#ABBREVIATION:Pi4kii.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, beta#ABBREVIATION:Pip5k1b.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, alpha#ABBREVIATION:Pip5k1a.1# citations: AUTHORS:Tolias KF, Cantley LC#TITLE:Pathways for phosphoinositide synthesis#JOURNAL:Chem Phys Lipids#YEAR:1999#PUBMED:10358929# AUTHORS:Zhang X, Loijens JC, Boronenkov IV, Parker GJ, Norris FA, Chen J, Thum O, Prestwich GD, Majerus PW, Anderson RA#TITLE:Phosphatidylinositol-4-phosphate 5-kinase isozymes catalyze the synthesis of 3-phosphate-containing phosphatidylinositol signaling molecules#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9211928# AUTHORS:Rameh LE, Tolias KF, Duckworth BC, Cantley LC#TITLE:A new pathway for synthesis of phosphatidylinositol-4,5-bisphosphate#JOURNAL:Nature#YEAR:1997#PUBMED:9367159# GENE ASSOCIATION: (55361.1) or (5297.1) or (8394.1) or (23396.1) or (8395.1) or (5298.1)
Abbreviation: R_HDCEAtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hdcea[e] <==> hdcea[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_LTC4CP Synonyms: _0 EC Number: 2.3.2.2 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : h2o + leuktrC4 <==> gly + leuktrF4 Confidence Level: 2 Converts leukotriene C4 (LTC4) to leukotriene F4 (LTF4). NJ genes: none proteins: none citations: AUTHORS:Reddanna P, Prabhu KS, Whelan J, Reddy CC.#TITLE:Carboxypeptidase A-catalyzed direct conversion of leukotriene C4 to leukotriene F4.#JOURNAL:Arch Biochem Biophys.#YEAR:2003#PUBMED:12729612# GENE ASSOCIATION:
Abbreviation: R_EX_mag_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : mag_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
monoacylglycerol 2 (homo sapiens) exchange
Abbreviation: R_GGT6 Synonyms: _0 EC Number: 2.3.2.2 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : glu-L + leuktrE4 <==> h2o + leuktrF4 Confidence Level: 2 Converts leukotriene E4 (LTE4) to leukotriene D4 (LTE4). NJ genes: none proteins: none citations: AUTHORS:Reddanna P, Prabhu KS, Whelan J, Reddy CC.#TITLE:Carboxypeptidase A-catalyzed direct conversion of leukotriene C4 to leukotriene F4.#JOURNAL:Arch Biochem Biophys.#YEAR:2003#PUBMED:12729612# GENE ASSOCIATION:
Abbreviation: R_LYStn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: lys-L[c] <==> lys-L[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate II (core 2-linked), degradation product 3_C58H94N5O46S2X
Abbreviation: R_NACHEXA20ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksii_core2_deg3 --> acgam + h + ksii_core2_deg5 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
keratan sulfate II (core 2-linked), degradation product 5_C50H82N4O38SX
Abbreviation: R_EX_ala_DASH_D_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ala-D <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SPODMe Synonyms: _1 EC Number: 1.15.1.1 SUBSYSTEM: ROS Detoxification Equation: [e] : (2) h + (2) o2s --> h2o2 + o2 Confidence Level: 4 JLR genes: LOCUS:_6649#TRANSCRIPT:1#ABBREVIATION:SOD3#ECNUMBERS:1.15.1.1# proteins: NAME:superoxide dismutase 3, extracellular#ABBREVIATION:Sod3.1-e# citations: AUTHORS:Fridovich I#TITLE:Superoxide anion radical (O2-.), superoxide dismutases, and related matters#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9228011# AUTHORS:Zelko IN, Mariani TJ, Folz RJ#TITLE:Superoxide dismutase multigene family: a comparison of the CuZn-SOD (SOD1), Mn-SOD (SOD2), and EC-SOD (SOD3) gene structures, evolution, and expression#JOURNAL:Free Radic Biol Med#YEAR:2002#PUBMED:12126755# AUTHORS:Hjalmarsson K, Marklund SL, Engstrom A, Edlund T#TITLE:Isolation and sequence of complementary DNA encoding human extracellular superoxide dismutase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1987#PUBMED:3476950# GENE ASSOCIATION: (6649.1)
Abbreviation: R_LNLCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lnlc[e] <==> lnlc[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Linoleic acid (n-C18:2) transport in via diffusion
Abbreviation: R_CSm Synonyms: _0 SUBSYSTEM: Citric Acid Cycle Equation: [m] : accoa + h2o + oaa --> cit + coa + h Confidence Level: 0 IF old EC number (4.1.3.7) is obsolete -- transferred to 2.3.3.1 NCD genes: LOCUS:_1431#TRANSCRIPT:1#ABBREVIATION:CS#ECNUMBERS:4.1.3.7# LOCUS:_1431#TRANSCRIPT:2#ABBREVIATION:CS#ECNUMBERS:4.1.3.7# proteins: NAME:citrate synthase, mitochondrial#ABBREVIATION:Cs.1-m# NAME:citrate synthase, mitochondrial#ABBREVIATION:Cs.2-m# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (1431.1) or (1431.2)
Abbreviation: R_ESTRONEGLCt Synonyms: _9 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + estroneglc[c] + h2o[c] --> adp[c] + estroneglc[e] + h[c] + pi[c] Confidence Level: 4 for bile acid secretion into bile NJ genes: LOCUS:_8714#TRANSCRIPT:1#ABBREVIATION:ABCC3# LOCUS:_8714#TRANSCRIPT:2#ABBREVIATION:ABCC3# LOCUS:_8714#TRANSCRIPT:3#ABBREVIATION:ABCC3# proteins: NAME:ABC, sub-family C (CFTR/MRP), member 3#ABBREVIATION:Abcc3.1# NAME:ABC, sub-family C, (CFTR/MRP), member 3#ABBREVIATION:Abcc3.2# NAME:ABC, sub-family C, (CFTR/MRP), member 3#ABBREVIATION:Abcc3.3# citations: AUTHORS:Kiuchi Y, Suzuki H, Hirohashi T, Tyson CA, Sugiyama Y.#TITLE:cDNA cloning and inducible expression of human multidrug resistance associated protein 3 (MRP3).#JOURNAL:FEBS Lett.#YEAR:1998#PUBMED:9738950# AUTHORS:Uchiumi T, Hinoshita E, Haga S, Nakamura T, Tanaka T, Toh S, Furukawa M, Kawabe T, Wada M, Kagotani K, Okumura K, Kohno K, Akiyama S, Kuwano M.#TITLE:Isolation of a novel human canalicular multispecific organic anion transporter, cMOAT2/MRP3, and its expression in cisplatin-resistant cancer cells with decreased ATP-dependent drug transport.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1998#PUBMED:9813153# AUTHORS:Scheffer GL, Kool M, de Haas M, de Vree JM, Pijnenborg AC, Bosman DK, Elferink RP, van der Valk P, Borst P, Scheper RJ.#TITLE:Tissue distribution and induction of human multidrug resistant protein 3.#JOURNAL:Lab Invest.#YEAR:2002#PUBMED:11850532# GENE ASSOCIATION: (8714.1) or (8714.3) or (8714.2)
dihomo-gamma-linolenyl carnitine_C27H47NO4
dihomo-gamma-linolenyl coenzyme A_C41H64N7O17P3S
Abbreviation: R_DLNLCGCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + dlnlcgcoa --> coa + dlnlcgcrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_PAIL_HStn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: pail_hs[c] <==> pail_hs[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
phosphatidylinositol nuclear transport (diffusion)
beta-D-Mannosyldiacetylchitobiosyldiphosphodolichol, human uterine homolog_C1245H2040N20O220P20
Abbreviation: R_ARACHDt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: arachd[e] <==> arachd[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
keratan sulfate I biosynthesis, precursor 5_C101H164N7O75SX
Abbreviation: R_FRUt1r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fru[e] <==> fru[c] Confidence Level: 4 NCD genes: LOCUS:_155184#TRANSCRIPT:1#ABBREVIATION:SLC2A7# LOCUS:_66035#TRANSCRIPT:1#ABBREVIATION:SLC2A11# LOCUS:_6514#TRANSCRIPT:1#ABBREVIATION:SLC2A2# LOCUS:_6518#TRANSCRIPT:1#ABBREVIATION:SLC2A5# LOCUS:_29988#TRANSCRIPT:1#ABBREVIATION:SLC2A8# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 2, extracellular#ABBREVIATION:Slc2a2.1-e# NAME:solute carrier family 2 (facilitated glucose/fructose transporter), member 5, extracellular#ABBREVIATION:Slc2a5.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 7, extracellular#ABBREVIATION:Slc2a7.1-e# NAME:solute carrier family 2, (facilitated glucose transporter) member 8, extracellular#ABBREVIATION:Slc2a8.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 11, extracellular#ABBREVIATION:Slc2a11.1-e# citations: AUTHORS:Hosokawa M, Thorens B#TITLE:Glucose release from GLUT2-null hepatocytes: characterization of a major and a minor pathway#JOURNAL:Am J Physiol Endocrinol Metab#YEAR:2002#PUBMED:11882499# AUTHORS:Guillam MT, Burcelin R, Thorens B#TITLE:Normal hepatic glucose production in the absence of GLUT2 reveals an alternative pathway for glucose release from hepatocytes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9770484# AUTHORS:Uldry M, Thorens B#TITLE:The SLC2 family of facilitated hexose and polyol transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750891# AUTHORS:Uldry M, Ibberson M, Hosokawa M, Thorens B#TITLE:GLUT2 is a high affinity glucosamine transporter#JOURNAL:FEBS Lett#YEAR:2002#PUBMED:12135767# AUTHORS:Fukumoto H, Seino S, Imura H, Seino Y, Eddy RL, Fukushima Y, Byers MG, Shows TB, Bell GI#TITLE:Sequence, tissue distribution, and chromosomal localization of mRNA encoding a human glucose transporter-like protein#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1988#PUBMED:3399500# AUTHORS:Johnson JH, Newgard CB, Milburn JL, Lodish HF, Thorens B#TITLE:The high Km glucose transporter of islets of Langerhans is functionally similar to the low affinity transporter of liver and has an identical primary sequence#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2182619# AUTHORS:Thorens B, Cheng ZQ, Brown D, Lodish HF#TITLE:Liver glucose transporter: a basolateral protein in hepatocytes and intestine and kidney cells#JOURNAL:Am J Physiol#YEAR:1990#PUBMED:1701966# AUTHORS:Burant CF, Takeda J, Brot-Laroche E, Bell GI, Davidson NO#TITLE:Fructose transporter in human spermatozoa and small intestine is GLUT5#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1634504# AUTHORS:Mantych GJ, James DE, Devaskar SU#TITLE:Jejunal/kidney glucose transporter isoform (Glut-5) is expressed in the human blood-brain barrier#JOURNAL:Endocrinology#YEAR:1993#PUBMED:8419132# AUTHORS:Kayano T, Burant CF, Fukumoto H, Gould GW, Fan YS, Eddy RL, Byers MG, Shows TB, Seino S, Bell GI#TITLE:Human facilitative glucose transporters.#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:1695905# AUTHORS:Li Q, Manolescu A, Ritzel M, Yao S, Slugoski M, Young JD, Chen XZ, Cheeseman CI#TITLE:Cloning and functional characterization of the human GLUT7 isoform SLC2A7 from the small intestine#JOURNAL:Am J Physiol Gastrointest Liver Physiol#YEAR:2004#PUBMED:15033637# AUTHORS:Joost HG, Thorens B#TITLE:The extended GLUT-family of sugar/polyol transport facilitators: nomenclature, sequence characteristics, and potential function of its novel members (review)#JOURNAL:Mol Membr Biol#YEAR:2001#PUBMED:11780753# AUTHORS:Doege H, Schurmann A, Bahrenberg G, Brauers A, Joost HG#TITLE:GLUT8, a novel member of the sugar transport facilitator family with glucose transport activity#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10821868# AUTHORS:Carayannopoulos MO, Chi MM, Cui Y, Pingsterhaus JM, McKnight RA, Mueckler M, Devaskar SU, Moley KH#TITLE:GLUT8 is a glucose transporter responsible for insulin-stimulated glucose uptake in the blastocyst#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10860996# AUTHORS:Doege H, Bocianski A, Scheepers A, Axer H, Eckel J, Joost HG, Schurmann A#TITLE:Characterization of human glucose transporter (GLUT) 11 (encoded by SLC2A11), a novel sugar-transport facilitator specifically expressed in heart and skeletal muscle#JOURNAL:Biochem J#YEAR:2001#PUBMED:11583593# GENE ASSOCIATION: (29988.1) or (6514.1) or (155184.1) or (6518.1) or (66035.1)
Dolichol diphosphate, human uterine homolog_C1025H1670O70P20
Electron transfer flavoprotein oxidized_R
Abbreviation: R_PRGNLONEtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: prgnlone[c] <==> prgnlone[r] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SMPD3l Synonyms: _4 SUBSYSTEM: Sphingolipid Metabolism Equation: [l] : h2o + sphmyln_hs --> cholp + crm_hs + h Confidence Level: 4 NJ genes: LOCUS:_55512#TRANSCRIPT:1#ABBREVIATION:SMPD3# LOCUS:_55512#TRANSCRIPT:2#ABBREVIATION:SMPD3# proteins: NAME:sphingomyelin phosphodiesterase 3, neutral membrane (neutral sphingomyelinase II) #ABBREVIATION:Smpd3.1# NAME:sphingomyelin phosphodiesterase 3, neutral membrane (neutral sphingomyelinase II) #ABBREVIATION:Smpd3.2# citations: AUTHORS:Hofmann K, Tomiuk S, Wolff G, Stoffel W.#TITLE:Cloning and characterization of the mammalian brain-specific, Mg2+-dependent neutral sphingomyelinase.#JOURNAL:Proc Natl Acad Sci#YEAR:2000#PUBMED:10823942# GENE ASSOCIATION: (55512.1) or (55512.2)
Abbreviation: R_GAO1 Synonyms: _1 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : accoa + gd3_hs --> coa + oagd3_hs Confidence Level: 4 for humans - seq predicted SLC transporters have been found to have O-acetylation activity on gangliosides NJ genes: LOCUS:_9197#TRANSCRIPT:1#ABBREVIATION:SLC33A1# proteins: NAME:acetyl-CoA transporter/O-acetylator#ABBREVIATION:Slc33a1.1# citations: AUTHORS:Kanamori A, Nakayama J, Fukuda MN, Stallcup WB, Sasaki K, Fukuda M, Hirabayashi Y#TITLE:Expression cloning and characterization of a cDNA encoding a novel membrane protein required for the formation of O-acetylated ganglioside: A putative acetyl-CoA-transporter#JOURNAL:Proc Natl Acad Sci#YEAR:1997# GENE ASSOCIATION: (9197.1)
Abbreviation: R_P4502C94 Synonyms: _5 SUBSYSTEM: Limonene and pinene degradation Equation: [c] : appnn + h + nadph + o2 --> apnnox + h2o + nadp Confidence Level: 4 assumed to take place on outer membrane NJ genes: LOCUS:_1557#TRANSCRIPT:1#ABBREVIATION:CYP2C19#ECNUMBERS:1.14.14.1# LOCUS:_1559#TRANSCRIPT:1#ABBREVIATION:CYP2C9#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 2, subfamily C, polypeptide 19#ABBREVIATION:Cyp2c19.1# NAME:cytochrome P450, family 2, subfamily C, polypeptide 9#ABBREVIATION:Cyp2c9.1# citations: AUTHORS:Rendic S.#TITLE:Summary of information on human CYP enzymes: human P450 metabolism data.#JOURNAL:Drug Metab Rev#YEAR:2002#PUBMED:11996015# AUTHORS:Miyazawa M, Shindo M, Shimada T.#TITLE:Metabolism of (+)- and (-)-limonenes to respective carveols and perillyl alcohols by CYP2C9 and CYP2C19 in human liver microsomes.#JOURNAL:Drug Metab Dispos#YEAR:2002#PUBMED:11950794# GENE ASSOCIATION: (1559.1) or (1557.1)
Abbreviation: R_DHORTS Synonyms: _0 EC Number: 3.5.2.3 SUBSYSTEM: Pyrimidine Biosynthesis Equation: [c] : dhor-S + h2o <==> cbasp + h Confidence Level: 4 genes: LOCUS:_790#TRANSCRIPT:1#ABBREVIATION:CAD1#ECNUMBERS:6.3.5.5,2.1.3.2,3.5.2.3# proteins: NAME:CAD protein (3.5.2.3, 2.1.3.2, 6.3.5.5)#ABBREVIATION:Cad1.1# citations: AUTHORS:Iwahana H, Fujimura M, Ii S, Kondo M, Moritani M, Takahashi Y, Yamaoka T, Yoshimoto K, Itakura M.#TITLE:Molecular cloning of a human cDNA encoding a trifunctional enzyme of carbamoyl-phosphate synthetase-aspartate transcarbamoylase-dihydroorotase in de Novo pyrimidine synthesis. #JOURNAL:Biochem Biophys Res Commun.#YEAR:1996#PUBMED:8619816# AUTHORS:Chen KC, Vannais DB, Jones C, Patterson D, Davidson JN.#TITLE:Mapping of the gene encoding the multifunctional protein carrying out the first three steps of pyrimidine biosynthesis to human chromosome 2. #JOURNAL: Hum Genet.#YEAR:1989#PUBMED:2565865# GENE ASSOCIATION: (790.1)
glucose-1,3-mannose oligosaccharide_C12H22O11
glucosyl-(alpha-D-mannosyl)7-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine, isoform C (protein)_C70H117N2O55X
Abbreviation: R_ENMAN4g Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : g1m7masnC + h2o --> glc1man + m6masnC Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# GENE ASSOCIATION:
Endomannosidase (m6masnC-producing), Golgi apparatus
alpha-Linolenic acid, C18:3, n-3_C18H29O2
Core 6 beta-GlcNAc-transferase A, Golgi apparatus
Abbreviation: R_CORE6GTg Synonyms: _8 EC Number: 2.4.1.102 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : Tn_antigen + uacgam --> core6 + h + udp Confidence Level: 0 NCD genes: LOCUS:_2650#TRANSCRIPT:1#ABBREVIATION:GCNT1#ECNUMBERS:2.4.1.102# proteins: NAME:glucosaminyl (N-acetyl) transferase 1, core 2 (beta-1,6-N-acetylglucosaminyltransferase), Golgi apparatus (ubiquitous)#ABBREVIATION:Gcnt1.1-g# citations: AUTHORS:Yeh JC, Ong E, Fukuda M#TITLE:Free Full Text Molecular cloning and expression of a novel beta-1, 6-N-acetylglucosaminyltransferase that forms core 2, core 4, and I branches#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9915862# GENE ASSOCIATION: (2650.1)
Abbreviation: R_FACOAL205 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + tmndnc <==> amp + ppi + tmndnccoa Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_IDPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: idp[c] <==> idp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DOGULNO2 Synonyms: _0 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : 23doguln + h2o --> eryth + h + oxa Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Simpson GL, Ortwerth BJ#TITLE:The non-oxidative degradation of ascorbic acid at physiological conditions#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10727845# GENE ASSOCIATION:
Abbreviation: R_EX_retfa_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : retfa <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
alcohol dehydrogenase (D-1,2-propanediol)
Abbreviation: R_ALCD21_D Synonyms: _0 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : 12ppd-R + nad --> h + lald-D + nadh Confidence Level: 2 NCD genes: LOCUS:_130#TRANSCRIPT:1#ABBREVIATION:ADH6#ECNUMBERS:1.1.1.1# LOCUS:_126#TRANSCRIPT:1#ABBREVIATION:ADH1C#ECNUMBERS:1.1.1.1# LOCUS:_125#TRANSCRIPT:1#ABBREVIATION:ADH1B#ECNUMBERS:1.1.1.1# LOCUS:_128#TRANSCRIPT:1#ABBREVIATION:ADH5#ECNUMBERS:1.1.1.1# LOCUS:_127#TRANSCRIPT:1#ABBREVIATION:ADH4#ECNUMBERS:1.1.1.1# LOCUS:_124#TRANSCRIPT:1#ABBREVIATION:ADH1A#ECNUMBERS:1.1.1.1# LOCUS:_131#TRANSCRIPT:1#ABBREVIATION:ADH7#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:1#ABBREVIATION:ZADH2# LOCUS:_137872#TRANSCRIPT:1#ABBREVIATION:ADHFE1#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:2#ABBREVIATION:ZADH2# proteins: NAME:alcohol dehydrogenase IV #ABBREVIATION:Adh4# NAME:alcohol dehydrogenase V#ABBREVIATION:Adh5# NAME:alcohol dehydrogenase 1A (class I), alpha polypeptide#ABBREVIATION:Adh1a.1# NAME:alcohol dehydrogenase IB (class I), beta polypeptide#ABBREVIATION:Adh1b.1# NAME:alcohol dehydrogenase 1C (class I), gamma polypeptide#ABBREVIATION:Adh1c.1# NAME:alcohol dehydrogenase 6 (class V)#ABBREVIATION:Adh6.1# NAME:alcohol dehydrogenase 7 (class IV), mu or sigma polypeptide#ABBREVIATION:Adh7.1# NAME:alcohol dehydrogenase, iron containing, 1 (liver)#ABBREVIATION:Adhfe1.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ab.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ac# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1bc# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.1# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.2# citations: AUTHORS:Ewaschuk JB, Naylor JM, Zello GA#TITLE:D-lactate in human and ruminant metabolism#JOURNAL:J Nutr#YEAR:2005#PUBMED:15987839# AUTHORS:Christopher MM, Eckfeldt JH, Eaton JW.#TITLE:Propylene glycol ingestion causes D-lactic acidosis#JOURNAL:Lab Invest.#YEAR:1990#PUBMED:2296157# GENE ASSOCIATION: (130.1) or (284273.2) or (126.1) or (131.1) or (124.1) or (125.1 and 124.1) or (125.1 and 126.1) or (137872.1) or (125.1) or (127.1) or (126.1 and 124.1) or (284273.1) or (128.1)
Abbreviation: R_APOC_DASH_LYS_DASH_BTNPm Synonyms: _0 SUBSYSTEM: Biotin Metabolism Equation: [m] : apoC-Lys_btn + h2o --> apoC + biocyt Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
proteolysis of apoC-Lys-btn, mitochondrial
V-type ATPase, H+ transporting, lysosomal
keratan sulfate I, degradation product 12_C174H279N12O153S9
N-acetylglucosamine-6-sulfatase, lysosomal
keratan sulfate I, degradation product 13_C174H280N12O150S8
Abbreviation: R_S6TASE13ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg12 <==> h + ksi_deg13 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
(2)[glucose-1,3]-mannose oligosaccharide_C18H32O16
Abbreviation: R_ACOAD9m Synonyms: _0 EC Number: 1.3.99.12 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : fad + ibcoa --> 2mp2coa + fadh2 Confidence Level: 4 MM genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_27034#TRANSCRIPT:1#ABBREVIATION:ACAD8# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# NAME:acyl-Coenzyme A dehydrogenase family, member 8#ABBREVIATION:Acad8.1-m# citations: AUTHORS:Andresen,B.S., Christensen,E., Corydon,T.J., Bross,P., etc.#TITLE:Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency....#YEAR:2000#PUBMED:11013134# GENE ASSOCIATION: (34.1) or (27034.1)
acyl-CoA dehydrogenase (isobutyryl-CoA), mitochondrial
Abbreviation: R_CITL Synonyms: _0 EC Number: 4.1.3.6 SUBSYSTEM: Citric Acid Cycle Equation: [c] : cit --> ac + oaa Confidence Level: 0 JLR genes: LOCUS:_171425#TRANSCRIPT:1#ABBREVIATION:CLYBL# LOCUS:_171425#TRANSCRIPT:2#ABBREVIATION:CLYBL# proteins: NAME:citrate lyase beta like#ABBREVIATION:Clybl.1# NAME:citrate lyase beta like#ABBREVIATION:Clybl.2# citations: AUTHORS:Morikawa J, Nishimura Y, Uchida A, Tanaka T#TITLE:Molecular cloning of novel mouse and human putative citrate lyase beta-subunit#JOURNAL:Biochem Biophys Res Commun#YEAR:2001#PUBMED:11741334# GENE ASSOCIATION: (171425.1) or (171425.2)
Abbreviation: R_P45027A12m Synonyms: _4 EC Number: 1.14.13.15 SUBSYSTEM: Bile Acid Biosynthesis Equation: [m] : nadp + xoltetrol --> h + nadph + thcholst Confidence Level: 4 from uniprot Russell - bile acid biosynthesis review paper in human recon citation db NJ genes: LOCUS:_1593#TRANSCRIPT:1#ABBREVIATION:CYP27A1# proteins: NAME:cytochrome P450, family 27, subfamily A, polypeptide 1#ABBREVIATION:Cyp27a1.1-m# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1593.1)
_5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol 27-hydroxylase
keratan sulfate II (core 4-linked), degradation product 3_C66H107N6O51S2X
keratan sulfate II (core 4-linked), degradation product 2_C78H127N6O61S2X
Abbreviation: R_GALASE20ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksii_core4_deg2 --> (2) gal + ksii_core4_deg3 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
chondroitin sulfate E (GalNAc4,6diS-GlcA), precursor 3_C37H52NO43S4X
Abbreviation: R_EX_prostge1_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : prostge1 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
glycine reversible transport via sodium and chloride symport (2:1:1)
Abbreviation: R_GLYt7_LPAREN_211_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[e] + gly[e] + (2) na1[e] <==> cl[c] + gly[c] + (2) na1[c] Confidence Level: 4 SAB genes: LOCUS:_6536#TRANSCRIPT:1#ABBREVIATION:SLC6A9# LOCUS:_6536#TRANSCRIPT:2#ABBREVIATION:SLC6A9# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, glycine), member 9#ABBREVIATION:Slc6a9.1# NAME:solute carrier family 6 (neurotransmitter transporter, glycine), member 9#ABBREVIATION:Slc6a9.2# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (6536.1) or (6536.2)
Abbreviation: R_EBASTINEOHte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ebastineoh[e] <==> ebastineoh[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LPS2e Synonyms: _1 EC Number: 3.1.1.3 SUBSYSTEM: Fatty Acid Metabolism Equation: [e] : dag_hs + h2o --> Rtotal + h + mag_hs Confidence Level: 2 carbonyl and extra H accounted for in R group NJ genes: LOCUS:_3990#TRANSCRIPT:1#ABBREVIATION:LIPC#ECNUMBERS:3.1.1.3# proteins: NAME:lipase, hepatic#ABBREVIATION:Lipc.1# citations: AUTHORS:Ben-Zeev O, Doolittle MH.#TITLE:Maturation of hepatic lipase. Formation of functional enzyme in the endoplasmic reticulum is the rate-limiting step in its secretion.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14630921# GENE ASSOCIATION: (3990.1)
Abbreviation: R_DADAe Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [e] : dad-2 + h + h2o --> din + nh4 Confidence Level: 4 adenosine deaminase has been found on cell surface in human (Gonzales-Gronow et al, JBC, 2004,279,20993-20998). IT genes: LOCUS:_100#TRANSCRIPT:1#ABBREVIATION:ADA#ECNUMBERS:3.5.4.4# proteins: NAME:adenosine deaminase #ABBREVIATION:Ada.1# citations: AUTHORS:Gonzalez-Gronow M, Hershfield MS, Arredondo-Vega FX, Pizzo SV.#TITLE:Cell surface adenosine deaminase binds and stimulates plasminogen activation on 1-LN human prostate cancer cells.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15016824# AUTHORS:Iwaki-Egawa S, Watanabe Y.#TITLE:Characterization and purification of adenosine deaminase 1 from human and chicken liver.#JOURNAL:Comp Biochem Physiol B Biochem Mol Biol.#YEAR:2002#PUBMED:12381379# GENE ASSOCIATION: (100.1)
phosphatidylinositol-3,5-bisphosphate (Homo sapiens)_C9H14O15P3FULLRCO2FULLR2CO2
Abbreviation: R_FAOXC182806m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (5) coa + (3) fad + (5) h2o + lnlccoa + (5) nad --> (5) accoa + (3) fadh2 + (5) h + (5) nadh + occoa Confidence Level: 4 c18:2,n-6 -> c8:0 - 5 rounds of beta ox, lose 2 degrees of unsat - only 3 fad oxidized 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_APOC_DASH_LYS_DASH_BTNP Synonyms: _0 SUBSYSTEM: Biotin Metabolism Equation: [c] : apoC-Lys_btn + h2o --> apoC + biocyt Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_EX_creat_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : creat <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CEPTE Synonyms: _2 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : cdpea + dag_hs --> cmp + h + pe_hs Confidence Level: 4 NJ genes: LOCUS:_10390#TRANSCRIPT:1#ABBREVIATION:CEPT1# proteins: NAME:choline/ethanolamine phosphotransferase#ABBREVIATION:Cept1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Henneberry AL, McMaster CR#TITLE:Cloning and expression of a human choline/ethanolaminephosphotransferase: synthesis of phosphatidylcholine and phosphatidylethanolamine#JOURNAL:Biochem J#YEAR:1999# GENE ASSOCIATION: (10390.1)
_5-Diphosphoinositol pentakisphosphate_C6H6O27P7
glycophosphatidylinositol (GPI) signal sequence (C-terminal peptide)_Y
Abbreviation: R_VACCCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + vacccoa --> coa + vacccrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
chondroitin sulfate C (GalNAc6S-GlcA), degradation product 3_C31H48NO32S2
chondroitin sulfate C (GalNAc6S-GlcA), degradation product 2_C37H55NO38S2
Abbreviation: R_GLCAASE5ly Synonyms: _3 EC Number: 3.2.1.31 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_c_deg2 + h2o --> cs_c_deg3 + glcur Confidence Level: 4 NCD genes: LOCUS:_2990#TRANSCRIPT:1#ABBREVIATION:GUSB#ECNUMBERS:3.2.1.31# proteins: NAME:glucuronidase, beta, lysosomal#ABBREVIATION:Gusb.1-l# citations: AUTHORS:Guise KS, Korneluk RG, Waye J, Lamhonwah AM, Quan F, Palmer R, Ganschow RE, Sly WS, Gravel RA#TITLE:Isolation and expression in Escherichia coli of a cDNA clone encoding human beta-glucuronidase#JOURNAL:Gene#YEAR:1985#PUBMED:3924735# AUTHORS:Ho YC, Ho LH, Ho KJ#TITLE:Human hepatic beta-glucuronidase: an enzyme kinetic study#JOURNAL:Enzyme#YEAR:1985#PUBMED:3987656# AUTHORS:Oshima A, Kyle JW, Miller RD, Hoffmann JW, Powell PP, Grubb JH, Sly WS, Tropak M, Guise KS, Gravel RA#TITLE:Cloning, sequencing, and expression of cDNA for human beta-glucuronidase#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3468507# GENE ASSOCIATION: (2990.1)
Abbreviation: R_EX_strch1_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : strch1 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
starch, structure 1 (1,6-{7[1,4-Glc], 4[1,4-Glc]}) exchange
Abbreviation: R_EX_dad_DASH_5_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dad-5 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(alpha-D-mannosyl)7-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine, isoform A (protein)_C64H107N2O50X
Abbreviation: R_NDPK9 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + idp <==> adp + itp Confidence Level: 4 NCD genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.2) or (10201.1) or (4831.1 and 4830.1) or (4832.1) or (4831.1 and 4830.2) or (29922.1)
Tetradecanoyl-CoA (n-C14:0CoA)_C35H58N7O17P3S
Abbreviation: R_ACACT7p Synonyms: _0 EC Number: 2.3.1.16 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : 3ohdcoa + coa --> accoa + tdcoa Confidence Level: 4 NCD genes: LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# proteins: NAME:acetyl-Coenzyme A acyltransferase 1#ABBREVIATION:Acaa1.1# citations: AUTHORS:Bout A, Franse MM, Collins J, Blonden L, Tager JM, Benne R.#TITLE:Characterization of the gene encoding human peroxisomal 3-oxoacyl-CoA thiolase (ACAA). No large DNA rearrangement in a thiolase-deficient patient.#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1679347# GENE ASSOCIATION: (30.1)
acetyl-CoA acyltransferase (tetradecanoyl-CoA), peroxisomal
Abbreviation: R_EX_Rtotal_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : Rtotal <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PIK3er Synonyms: _4 EC Number: 2.7.1.137 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [r] : atp + pail_hs --> adp + h + pail3p_hs Confidence Level: 4 NCD genes: LOCUS:_5287#TRANSCRIPT:1#ABBREVIATION:PIK3C2B#ECNUMBERS:2.7.1.137# proteins: NAME:phosphoinositide-3-kinase, class 2, beta polypeptide#ABBREVIATION:Pik3c2b.1-er# citations: AUTHORS:Arcaro A, Volinia S, Zvelebil MJ, Stein R, Watton SJ, Layton MJ, Gout I, Ahmadi K, Downward J, Waterfield MD#TITLE:Human phosphoinositide 3-kinase C2beta, the role of calcium and the C2 domain in enzyme activity#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9830063# GENE ASSOCIATION: (5287.1)
phosphatidylinositol 3-kinase, endoplasmic reticulum
Abbreviation: R_ETOHtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: etoh[c] <==> etoh[x] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
ethanol reversible peroxisomal transport
Abbreviation: R_LYStip Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: lys-L[c] --> lys-L[x] Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Yung-Feng Chang#TITLE:Lysine metabolism in the human and the monkey: Demonstration of pipecolic acid formation in the brain and other organs#YEAR:1982#PUBMED:6811962# GENE ASSOCIATION:
L-lysine transport, peroxisomal (irreversible)
R group 1 Coenzyme A homo sapiens_XCO2C21H31N7O15P3S
Abbreviation: R_PRFGS Synonyms: _0 EC Number: 6.3.5.3 SUBSYSTEM: IMP Biosynthesis Equation: [c] : atp + fgam + gln-L + h2o --> adp + fpram + glu-L + h + pi Confidence Level: 4 genes: LOCUS:_5198#TRANSCRIPT:1#ABBREVIATION:PFAS#ECNUMBERS:6.3.5.3# proteins: NAME:phosphoribosylformylglycinamidine synthase (FGAR amidotransferase) #ABBREVIATION:PfaS.1# citations: AUTHORS:Brodsky G, Barnes T, Bleskan J, Becker L, Cox M, Patterson D.#TITLE:The human GARS-AIRS-GART gene encodes two proteins which are differentially expressed during human brain development and temporally overexpressed in cerebellum of individuals with Down syndrome. #JOURNAL:Hum Mol Genet. #YEAR:1997#PUBMED:9328467# AUTHORS:Patterson D, Bleskan J, Gardiner K, Bowersox J.#TITLE:Human phosphoribosylformylglycineamide amidotransferase (FGARAT): regional mapping, complete coding sequence, isolation of a functional genomic clone, and DNA sequence analysis.#JOURNAL:Gene#YEAR:1999#PUBMED:10548741# GENE ASSOCIATION: (5198.1)
phosphoribosylformylglycinamidine synthase
Abbreviation: R_AVITE2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: avite2[e] --> avite2[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_ABTArm Synonyms: _0 EC Number: 2.6.1.19 SUBSYSTEM: Glutamate metabolism Equation: [m] : 4abut + akg <==> glu-L + sucsal Confidence Level: 4 kh 1/6/03 genes: LOCUS:_18#TRANSCRIPT:1#ABBREVIATION:ABAT#ECNUMBERS:2.6.19.4# LOCUS:_18#TRANSCRIPT:2#ABBREVIATION:ABAT#ECNUMBERS:2.6.19.4# proteins: NAME:_4-aminobutyrate aminotransferase, mitochondrial#ABBREVIATION:Abat.1-m# NAME:_4-aminobutyrate aminotransferase, mitochondrial#ABBREVIATION:Abat.2-m# citations: AUTHORS:Medina-Kauwe LK, Tobin AJ, De Meirleir L, Jaeken J, Jakobs C, Nyhan WL, Gibson KM.#TITLE:_4-Aminobutyrate aminotransferase (GABA-transaminase) deficiency.#YEAR:1999#PUBMED:10407778# AUTHORS:Osei,Y.D. , Churchich,J.E. #TITLE:Screening and sequence determination of a cDNA encoding the human brain 4-aminobutyrate aminotransferase.#YEAR:1995#PUBMED:7721088# GENE ASSOCIATION: (18.1) or (18.2)
_4-aminobutyrate transaminase, reversible (mitochondrial)
keratan sulfate I, degradation product 37_C62H104N4O46
Abbreviation: R_S6TASE21ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg36 <==> h + ksi_deg37 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
keratan sulfate I, degradation product 26_C110H179N7O93S4
(alpha-D-Mannosyl)8-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human liver homolog_C1780H2928N20O620P20
Abbreviation: R_DOLPMT4_Ler Synonyms: _2 EC Number: 2.4.1.130 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolmanp_L + (0.1) m7mpdol_L --> (0.1) dolp_L + h + (0.1) m8mpdol_L Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_LNLNCACPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + lnlncacoa --> coa + lnlncacrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_CAROtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: caro[e] <==> caro[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_O2tp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: o2[c] <==> o2[x] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DCYTD Synonyms: _0 EC Number: 3.5.4.14 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : dcyt + h + h2o --> duri + nh4 Confidence Level: 2 genes: LOCUS:_978#TRANSCRIPT:1#ABBREVIATION:CDA#ECNUMBERS:3.5.4.5# LOCUS:_57379#TRANSCRIPT:1#ABBREVIATION:AICDA# proteins: NAME:cytidine deaminase #ABBREVIATION:Cda.1# NAME:activation-induced cytidine deaminase #ABBREVIATION:Aicda.1# citations: AUTHORS:Kuhn K, Bertling WM, Emmrich F.#TITLE:Cloning of a functional cDNA for human cytidine deaminase (CDD) and its use as a marker of monocyte/macrophage differentiation.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1993#PUBMED:8422236# AUTHORS:Saccone S, Besati C, Andreozzi L, Della Valle G, Garattini E, Terao M.#TITLE:Assignment of the human cytidine deaminase (CDA) gene to chromosome 1 band p35-p36.2.#JOURNAL:Genomics#YEAR:1994#PUBMED:8001985# AUTHORS:Laliberte J, Momparler RL.#TITLE:Human cytidine deaminase: purification of enzyme, cloning, and expression of its complementary DNA.#JOURNAL:Cancer Res#YEAR:1994#PUBMED:7923172# AUTHORS:Ge Y, Jensen TL, Stout ML, Flatley RM, Grohar PJ, Ravindranath Y, Matherly LH, Taub JW. #TITLE:The role of cytidine deaminase and GATA1 mutations in the increased cytosine arabinoside sensitivity of Down syndrome myeloblasts and leukemia cell lines.#JOURNAL:Cancer Res. #YEAR:2004#PUBMED:14744791# AUTHORS:Brar SS, Watson M, Diaz M.#TITLE:Activation-induced cytosine deaminase (AID) is actively exported out of the nucleus but retained by the induction of DNA breaks.#JOURNAL:J Biol Chem.#YEAR:2004#PUBMED:15087440# GENE ASSOCIATION: (978.1) or (57379.1)
Abbreviation: R_SO4t4_3 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) na1[e] + so4[e] <==> (3) na1[c] + so4[c] Confidence Level: 4 NCD genes: LOCUS:_6561#TRANSCRIPT:1#ABBREVIATION:SLC13A1# proteins: NAME:solute carrier family 13 (sodium/sulfate symporters), member 1#ABBREVIATION:Slc13a1.1-e# citations: AUTHORS:Lee A, Beck L, Markovich D#TITLE:The human renal sodium sulfate cotransporter (SLC13A1; hNaSi-1) cDNA and gene: organization, chromosomal localization, and functional characterization#JOURNAL:Genomics#YEAR:2000#PUBMED:11161786# AUTHORS:Pajor AM#TITLE:Molecular properties of sodium/dicarboxylate cotransporters#JOURNAL:J Membr Biol#YEAR:2000#PUBMED:10811962# GENE ASSOCIATION: (6561.1)
Abbreviation: R_PIt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h[c] + pi[c] <==> h[m] + pi[m] Confidence Level: 4 Symport IF genes: LOCUS:_5250#TRANSCRIPT:1#ABBREVIATION:SLC25A3# LOCUS:_5250#TRANSCRIPT:2#ABBREVIATION:SLC25A3# LOCUS:_5250#TRANSCRIPT:3#ABBREVIATION:SLC25A3# LOCUS:_5250#TRANSCRIPT:4#ABBREVIATION:SLC25A3# proteins: NAME:Solute carrier family 25 (phosphate carrier) member 3, mitochondrial#ABBREVIATION:Slc25a3.1-m# NAME:Solute carrier family 25 (phosphate carrier) member 3, mitochondrial#ABBREVIATION:Slc25a3.2-m# NAME:Solute carrier family 25 (phosphate carrier) member 3, mitochondrial#ABBREVIATION:Slc25a3.3-m# NAME:Solute carrier family 25 (phosphate carrier) member 3, mitochondrial#ABBREVIATION:Slc25a3.4-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (5250.4) or (5250.1) or (5250.3) or (5250.2)
Abbreviation: R_LGNCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lgnc[e] <==> lgnc[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_RBK_D Synonyms: _0 EC Number: 2.7.1.47 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : atp + rbl-D --> adp + h + ru5p-D Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Huck JH, Roos B, Jakobs C, van der Knaap MS, Verhoeven NM#TITLE:Evaluation of pentitol metabolism in mammalian tissues provides new insight into disorders of human sugar metabolism#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15234337# GENE ASSOCIATION:
Diacylglycerol phosphate kinase (homo sapiens)
Abbreviation: R_DAGK_hs Synonyms: _0 EC Number: 2.7.1.107 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : atp + dag_hs <==> adp + h + pa_hs Confidence Level: 4 NJ genes: LOCUS:_1606#TRANSCRIPT:1#ABBREVIATION:DGKA#ECNUMBERS:2.7.1.107# LOCUS:_9162#TRANSCRIPT:1#ABBREVIATION:DGKI# LOCUS:_1609#TRANSCRIPT:1#ABBREVIATION:DGKQ#ECNUMBERS:2.7.1.107# LOCUS:_8525#TRANSCRIPT:1#ABBREVIATION:DGKZ# LOCUS:_8527#TRANSCRIPT:1#ABBREVIATION:DGKD# LOCUS:_1608#TRANSCRIPT:1#ABBREVIATION:DGKG#ECNUMBERS:2.7.1.107# LOCUS:_8526#TRANSCRIPT:1#ABBREVIATION:DGKE#ECNUMBERS:2.7.1.107# LOCUS:_160851#TRANSCRIPT:1#ABBREVIATION:DGKH# LOCUS:_1607#TRANSCRIPT:1#ABBREVIATION:DGKB#ECNUMBERS:2.7.1.107# LOCUS:_8525#TRANSCRIPT:2#ABBREVIATION:DGKZ# LOCUS:_8527#TRANSCRIPT:2#ABBREVIATION:DGKD# LOCUS:_160851#TRANSCRIPT:2#ABBREVIATION:DGKH# LOCUS:_1607#TRANSCRIPT:2#ABBREVIATION:DGKB#ECNUMBERS:2.7.1.107# LOCUS:_8525#TRANSCRIPT:3#ABBREVIATION:DGKZ# proteins: NAME:diacylglycerol kinase, epsilon#ABBREVIATION:Dgke.1# NAME:diacylglycerol kinase, zeta#ABBREVIATION:Dgkz.1# NAME:diacylglycerol kinase, zeta#ABBREVIATION:Dgkz.2# NAME:diacylglycerol kinase, zeta#ABBREVIATION:Dgkz.3# NAME:diacylglycerol kinase, theta#ABBREVIATION:Dgkq.1# NAME:diacylglycerol kinase, iota#ABBREVIATION:Dgki.1# NAME:diacylglycerol kinase, eta#ABBREVIATION:Dgkh.1# NAME:diacylglycerol kinase, eta#ABBREVIATION:Dgkh.2# NAME:diacylglycerol kinase, gamma#ABBREVIATION:Dgkg.1# NAME:diacylglycerol kinase, delta#ABBREVIATION:Dgkd.1# NAME:diacylglycerol kinase, delta#ABBREVIATION:Dgkd.2# NAME:diacylglycerol kinase, beta#ABBREVIATION:Dgkb.1# NAME:diacylglycerol kinase, beta#ABBREVIATION:Dgkb.2# NAME:diacylglycerol kinase, alpha#ABBREVIATION:Dgka.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Tang W, Bunting M, Zimmerman GA, McIntyre TM, Prescott SM#TITLE:Molecular cloning of a novel human diacylglycerol kinase highly selective for arachidonate-containing substrates#JOURNAL:Journal of Biological Chemistry#YEAR:1996# GENE ASSOCIATION: (9162.1) or (1607.1) or (8525.2) or (160851.1) or (1608.1) or (1607.2) or (8525.1) or (8527.1) or (1606.1) or (160851.2) or (8526.1) or (8525.3) or (1607.1) or (8527.2) or (1609.1)
Abbreviation: R_FAOXC2251836x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : (2) coa + dcsptn1coa + (2) h2o + (2) nad --> (2) accoa + (2) h + lnlncgcoa + (2) nadh Confidence Level: 4 _2 rounds of beta-ox, plus a loss of 2 degrees of desatuartion, so no fad is oxidized - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) peroxisomal (very long chain FA) c22:5,n-6 -> c18:3, n-6 - final '6' in abbreviation indicates omega6 FA 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (51.1 and 30.1 and 1962.1) or (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1)
cis-octadec-11-enoyl-[acyl-carrier protein] (n-C18:1)_C29H53N2O8PRS
Abbreviation: R_EX_adp Synonyms: _0 Equation: [e] : adp <==> Confidence Level: TV genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ICDHy Synonyms: _0 EC Number: 1.1.1.42 SUBSYSTEM: Citric Acid Cycle Equation: [c] : icit + nadp --> akg + co2 + nadph Confidence Level: 4 Two reactions (ICDH1 and ICDH2) for isocitrate dehydrogenase genes: LOCUS:_3417#TRANSCRIPT:1#ABBREVIATION:IDH1#ECNUMBERS:1.1.1.42# proteins: NAME:isocitrate dehydrogenase 1 (NADP+), soluble#ABBREVIATION:Idh1.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Geisbrecht BV, Gould SJ#TITLE:The human PICD gene encodes a cytoplasmic and peroxisomal NADP(+)-dependent isocitrate dehydrogenase#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10521434# GENE ASSOCIATION: (3417.1)
protein-linked serine or threonine residue (O-glycosylation site)_XH
Abbreviation: R_GALNTg Synonyms: _17 EC Number: 2.4.1.41 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : Ser/Thr + udpacgal --> Tn_antigen + h + udp Confidence Level: 0 NCD genes: LOCUS:_8693#TRANSCRIPT:1#ABBREVIATION:GALNT4# LOCUS:_11226#TRANSCRIPT:1#ABBREVIATION:GALNT6# LOCUS:_2589#TRANSCRIPT:1#ABBREVIATION:GALNT1# LOCUS:_26290#TRANSCRIPT:1#ABBREVIATION:GALNT8# LOCUS:_2590#TRANSCRIPT:1#ABBREVIATION:GALNT2#ECNUMBERS:2.4.1.41# LOCUS:_2591#TRANSCRIPT:1#ABBREVIATION:GALNT3# LOCUS:_50614#TRANSCRIPT:1#ABBREVIATION:GALNT9# LOCUS:_51809#TRANSCRIPT:1#ABBREVIATION:GALNT7# LOCUS:_55568#TRANSCRIPT:1#ABBREVIATION:GALNT10# LOCUS:_63917#TRANSCRIPT:1#ABBREVIATION:GALNT11# LOCUS:_79695#TRANSCRIPT:1#ABBREVIATION:GALNT12# LOCUS:_114805#TRANSCRIPT:1#ABBREVIATION:GALNT13# LOCUS:_79623#TRANSCRIPT:1#ABBREVIATION:GALNT14# LOCUS:_11227#TRANSCRIPT:1#ABBREVIATION:GALNT5# LOCUS:_117248#TRANSCRIPT:1#ABBREVIATION:GALNTL2# LOCUS:_55568#TRANSCRIPT:2#ABBREVIATION:GALNT10# proteins: NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 6 (GalNAc-T6), Golgi apparatus (placenta, trachea)#ABBREVIATION:Galnt6.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 1 (GalNAc-T1), Golgi apparatus (ubiquitous)#ABBREVIATION:Galnt1.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 2 (GalNAc-T2), Golgi apparatus (liver, skeletal muscle)#ABBREVIATION:Galnt2.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 3 (GalNAc-T3), Golgi apparatus (pancreas, testis)#ABBREVIATION:Galnt3.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 8 (GalNAc-T8), lg transcript, Golgi apparatus (ubiquitous)#ABBREVIATION:Galnt8lg.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 9 (GalNAc-T9), Golgi apparatus (brain)#ABBREVIATION:Galnt9.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 7 (GalNAc-T7), Golgi apparatus (spinal cord, trachea)#ABBREVIATION:Galnt7.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 10 (GalNAc-T10), Golgi apparatus (sm intestine, stomach, pancreas, ovary, thyroid gland, spleen)#ABBREVIATION:Galnt10.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 4 (GalNAc-T4), Golgi apparatus (stomach, sm intestine)#ABBREVIATION:Galnt4.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 11 (GalNAc-T11), Golgi apparatus (kidney)#ABBREVIATION:Galnt11.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 12 (GalNAc-T12), Golgi apparatus (stomach, sm intestine)#ABBREVIATION:Galnt12.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 13 (GalNAc-T13), Golgi apparatus (brain)#ABBREVIATION:Galnt13.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 14 (GalNAc-T14), Golgi apparatus (kidney)#ABBREVIATION:Galnt14.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 5 (GalNAc-T5), Golgi apparatus (sublingual gland, stomach, sm intestine, colon)#ABBREVIATION:Galnt5.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase-like 2 (GalNAc-T15), Golgi apparatus (sm intestine, spleen, placenta, brain, overy, uterus, mammary gland, stomach)#ABBREVIATION:Galntl2.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 8 (GalNAc-T8), sm transcript, Golgi apparatus (heart, kidney, liver, placenta) #ABBREVIATION:Galnt8sm.1-g# NAME:UDP-N-acetyl-alpha-D-galactosamine:polypeptide N-acetylgalactosaminyltransferase 10 (GalNAc-T10), Golgi apparatus (sm intestine, stomach, pancreas, ovary, thyroid gland, spleen)#ABBREVIATION:Galnt10.2-g# citations: AUTHORS:Cheng L, Tachibana K, Iwasaki H, Kameyama A, Zhang Y, Kubota T, Hiruma T, Tachibana K, Kudo T, Guo JM, Narimatsu H#TITLE:Characterization of a novel human UDP-GalNAc transferase, pp-GalNAc-T15#JOURNAL:FEBS Lett#YEAR:2004#PUBMED:15147861# AUTHORS:Ten Hagen KG, Hagen FK, Balys MM, Beres TM, Van Wuyckhuyse B, Tabak LA#TITLE:Cloning and expression of a novel, tissue specifically expressed member of the UDP-GalNAc:polypeptide N-acetylgalactosaminyltransferase family.#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9765313# AUTHORS:Wang H, Tachibana K, Zhang Y, Iwasaki H, Kameyama A, Cheng L, Guo J, Hiruma T, Togayachi A, Kudo T, Kikuchi N, Narimatsu H#TITLE:Cloning and characterization of a novel UDP-GalNAc:polypeptide N-acetylgalactosaminyltransferase, pp-GalNAc-T14.#JOURNAL:Biochem Biophys Res Commun#YEAR:2003#PUBMED:12507512# AUTHORS: Cheng L, Tachibana K, Zhang Y, Guo J, Kahori Tachibana K, Kameyama A, Wang H, Hiruma T, Iwasaki H, Togayachi A, Kudo T, Narimatsu H#TITLE:Characterization of a novel human UDP-GalNAc transferase, pp-GalNAc-T10#JOURNAL:FEBS Lett#YEAR:2002#PUBMED:12417297# AUTHORS:White KE, Lorenz B, Evans WE, Meitinger T, Strom TM, Econs MJ#TITLE:Molecular cloning of a novel human UDP-GalNAc:polypeptide N-acetylgalactosaminyltransferase, GalNAc-T8, and analysis as a candidate autosomal dominant hypophosphatemic rickets (ADHR) gene.#JOURNAL:Gene#YEAR:2000#PUBMED:10767557# AUTHORS:Ten Hagen KG, Fritz TA, Tabak LA.#TITLE:All in the family: the UDP-GalNAc:polypeptide N-acetylgalactosaminyltransferases#JOURNAL:Glycobiology#YEAR:2003#PUBMED:12634319# GENE ASSOCIATION: (26290.1) or (79695.1) or (2589.1) or (50614.1) or (8693.1) or (55568.2) or (114805.1) or (11226.1) or (117248.1) or (11227.1) or (55568.1) or (2590.1) or (51809.1) or (26290.1) or (79623.1) or (63917.1) or (2591.1)
Abbreviation: R_SPHS1Pte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: sphs1p[c] <==> sphs1p[e] Confidence Level: 2 unknown mechanism, however sphs1p is known to have intracellular and extracellular activity and signal transduction functions (PMID: 10751414). NJ genes: none proteins: none citations: AUTHORS:Liu H, Sugiura M, Nava VE, Edsall LC, Kono K, Poulton S, Milstien S, Kohama T, Spiegel S.#TITLE:Molecular cloning and functional characterization of a novel mammalian sphingosine kinase type 2 isoform.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10751414# GENE ASSOCIATION:
Abbreviation: R_ESTSULT Synonyms: _2 EC Number: 2.8.2.4 SUBSYSTEM: Steroid Metabolism Equation: [c] : estrone + paps --> estrones + h + pap Confidence Level: 4 NJ genes: LOCUS:_6783#TRANSCRIPT:1#ABBREVIATION:SULT1E1# LOCUS:_6817#TRANSCRIPT:1#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:2#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:3#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:4#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:5#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# proteins: NAME:sulfotransferase family 1E, estrogen#ABBREVIATION:Sult1e1.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.2# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.3# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.4# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.5# citations: AUTHORS:Aksoy IA, Wood TC, Weinshilboum R.#TITLE:Human liver estrogen sulfotransferase: identification by cDNA cloning and expression.#JOURNAL:Biochem Biophys Res Commun#YEAR:1994#PUBMED:8185618# AUTHORS:Glatt H, Boeing H, Engelke CE, Ma L, Kuhlow A, Pabel U, Pomplun D, Teubner W, Meinl W.#TITLE:Human cytosolic sulphotransferases: genetics, characteristics, toxicological aspects.#JOURNAL:Mutat Res#YEAR:2001#PUBMED:11535246# GENE ASSOCIATION: (6783.1) or (6817.4) or (6817.1) or (6817.3) or (6817.2) or (6817.5)
Abbreviation: R_DNDPt52m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dctp[c] + dudp[m] --> dctp[m] + dudp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EICOSTETt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: eicostet[e] <==> eicostet[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_MCPST Synonyms: _2 EC Number: 2.8.1.2 SUBSYSTEM: Cysteine Metabolism Equation: [c] : cyan + mercppyr --> h + pyr + tcynt Confidence Level: 4 MM AMF genes: LOCUS:_4357#TRANSCRIPT:1#ABBREVIATION:MPST# proteins: NAME:mercaptopyruvate sulfurtransferase #ABBREVIATION:Mpst.1# citations: none GENE ASSOCIATION: (4357.1)
Abbreviation: R_ARTFR32 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + odecoa[c] --> (1.125) R3coa_hs[c] + fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MCDm Synonyms: _0 EC Number: 4.1.1.9 SUBSYSTEM: Fatty Acid Metabolism Equation: [m] : h + malcoa --> accoa + co2 Confidence Level: 4 TDV (9/14/2004) added "m" to denote compartmentalization - NCD genes: LOCUS:_23417#TRANSCRIPT:1#ABBREVIATION:MLYCD#ECNUMBERS:4.1.1.9# proteins: NAME:malonyl-CoA decarboxylase#ABBREVIATION:Mlycd.1-m# citations: AUTHORS:Kerner J, Hoppel CL.#TITLE:Radiochemical malonyl-CoA decarboxylase assay: activity and subcellular distribution in heart and skeletal muscle.#JOURNAL:Anal Biochem#YEAR:2002#PUBMED:12123667# AUTHORS:Wightman PJ, Santer R, Ribes A, Dougherty F, McGill N, Thorburn DR, FitzPatrick DR#TITLE:MLYCD mutation analysis: evidence for protein mistargeting as a cause of MLYCD deficiency#JOURNAL:Hum Mutat#YEAR:2003#PUBMED:12955715# GENE ASSOCIATION: (23417.1)
Malonyl-CoA Decarboxylase, mitochondrial
Abbreviation: R_ADA Synonyms: _0 EC Number: 3.5.4.4 SUBSYSTEM: Purine Catabolism Equation: [c] : adn + h + h2o --> ins + nh4 Confidence Level: 4 Added EC- JLR genes: LOCUS:_100#TRANSCRIPT:1#ABBREVIATION:ADA#ECNUMBERS:3.5.4.4# proteins: NAME:adenosine deaminase #ABBREVIATION:Ada.1# citations: AUTHORS:Gonzalez-Gronow M, Hershfield MS, Arredondo-Vega FX, Pizzo SV.#TITLE:Cell surface adenosine deaminase binds and stimulates plasminogen activation on 1-LN human prostate cancer cells.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15016824# AUTHORS:Iwaki-Egawa S, Watanabe Y.#TITLE:Characterization and purification of adenosine deaminase 1 from human and chicken liver.#JOURNAL:Comp Biochem Physiol B Biochem Mol Biol.#YEAR:2002#PUBMED:12381379# GENE ASSOCIATION: (100.1)
chondroitin sulfate E (GalNAc4,6diS-GlcA), degradation product 5_C31H47NO35S3
chondroitin sulfate E (GalNAc4,6diS-GlcA), degradation product 4_C31H46NO38S4
Abbreviation: R_S4TASE5ly Synonyms: _5 EC Number: 3.1.6.12 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e_deg4 + h2o <==> cs_e_deg5 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_411#TRANSCRIPT:1#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# LOCUS:_411#TRANSCRIPT:2#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# proteins: NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.1-l# NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.2-l# citations: AUTHORS:Schuchman EH, Jackson CE, Desnick RJ#TITLE:Human arylsulfatase B: MOPAC cloning, nucleotide sequence of a full-length cDNA, and regions of amino acid identity with arylsulfatases A and C#JOURNAL:Genomics#YEAR:1990#PUBMED:1968043# AUTHORS:Peters C, Schmidt B, Rommerskirch W, Rupp K, Zuhlsdorf M, Vingron M, Meyer HE, Pohlmann R, von Figura K#TITLE:Phylogenetic conservation of arylsulfatases. cDNA cloning and expression of human arylsulfatase B.#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2303452# GENE ASSOCIATION: (411.1) or (411.2)
Abbreviation: R_EX_eaflatoxin_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : eaflatoxin <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(alpha-D-Glucosyl)3-(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine (protein)_C88H147N2O70X
Abbreviation: R_CYANt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cyan[e] <==> cyan[c] Confidence Level: 2 assumed to be readily diffusible MM genes: none proteins: none citations: none GENE ASSOCIATION:
Cyanide transport via diffusion (extracellular to cytosol)
Abbreviation: R_EX_digalsgalside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : digalsgalside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_btn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : btn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MTHFCm Synonyms: _0 EC Number: 3.5.4.9 SUBSYSTEM: Folate Metabolism Equation: [m] : h2o + methf <==> 10fthf + h Confidence Level: 4 NCD genes: LOCUS:_10797#TRANSCRIPT:1#ABBREVIATION:MTHFD2#ECNUMBERS:1.5.1.15,3.5.4.9# LOCUS:_25902#TRANSCRIPT:1#ABBREVIATION:MTHFD1L# proteins: NAME:methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1-like, mitochondrial#ABBREVIATION:Mthfd1l-m# NAME:methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2, methenyltetrahydrofolate cyclohydrolase, mitochondrial#ABBREVIATION:Mthfd2.1-m# citations: AUTHORS:Prasannan P, Pike S, Peng K, Shane B, Appling DR#TITLE:Human mitochondrial C1-tetrahydrofolate synthase: gene structure, tissue distribution of the mRNA, and immunolocalization in Chinese hamster ovary calls#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12937168# GENE ASSOCIATION: (25902.1) or (10797.1)
methenyltetrahydrifikate cyclohydrolase, mitochondrial
Abbreviation: R_EX_3mlda_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 3mlda <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
lysophosphatidic acid (homo sapiens)_C3H6O5PFULLRCO2
Abbreviation: R_AGPAT1 Synonyms: _4 EC Number: 2.3.1.51 SUBSYSTEM: Triacylglycerol Synthesis Equation: [c] : Rtotal2coa + alpa_hs --> coa + pa_hs Confidence Level: 4 FULLR corresponds to acyl/R groups in texts. NJ genes: LOCUS:_56895#TRANSCRIPT:1#ABBREVIATION:AGPAT4#ECNUMBERS:2.3.1.51# LOCUS:_56894#TRANSCRIPT:1#ABBREVIATION:AGPAT3#ECNUMBERS:2.3.1.51# LOCUS:_10555#TRANSCRIPT:1#ABBREVIATION:AGPAT2#ECNUMBERS:2.3.1.51# LOCUS:_10554#TRANSCRIPT:1#ABBREVIATION:AGPAT1#ECNUMBERS:2.3.1.51# LOCUS:_55326#TRANSCRIPT:1#ABBREVIATION:AGPAT5#ECNUMBERS:2.3.1.51# LOCUS:_56894#TRANSCRIPT:2#ABBREVIATION:AGPAT3#ECNUMBERS:2.3.1.51# LOCUS:_137964#TRANSCRIPT:1#ABBREVIATION:AGPAT6# proteins: NAME:_1-acylglycerol-3-phosphate O-acyltransferase 1#ABBREVIATION:Agpat1.1# NAME:_1-acylglycerol-3-phosphate O-acyltransferase 2#ABBREVIATION:Agpat2.1# NAME:_1-acylglycerol-3-phosphate O-acyltransferase 3#ABBREVIATION:Agpat3.1# NAME:_1-acylglycerol-3-phosphate O-acyltransferase 4#ABBREVIATION:Agpat4.1# NAME:_1-acylglycerol-3-phosphate O-acyltransferase 5#ABBREVIATION:Agpat5.1# NAME:_1-acylglycerol-3-phosphate O-acyltransferase 3#ABBREVIATION:Agpat3.2# NAME:_1-acylglycerol-3-phosphate O-acyltransferase 3#ABBREVIATION:Agpat6.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Coleman RA, Lee DP#TITLE:Enzymes of triacylglycerol synthesis and their regulation#JOURNAL:Prog in Lipid Research#YEAR:2004# GENE ASSOCIATION: (10555.1) or (137964.1) or (56895.1) or (56894.2) or (56894.1) or (55326.1) or (10554.1)
_1-acylglycerol-3-phosphate O-acyltransferase 1
Abbreviation: R_TSTSTERONESULT Synonyms: _0 EC Number: 2.8.2.2 SUBSYSTEM: Steroid Metabolism Equation: [c] : paps + tststerone --> h + pap + tststerones Confidence Level: 4 NJ genes: LOCUS:_6822#TRANSCRIPT:1#ABBREVIATION:SULT2A1#ECNUMBERS:2.8.2.2# proteins: NAME:sulfotransferase family, cytosolic, 2A, dehydroepiandrosterone#ABBREVIATION:Sult2a1.1# citations: AUTHORS:Otterness DM, Wieben ED, Wood TC, Watson WG, Madden BJ, McCormick DJ, Weinshilboum RM.#TITLE:Human liver dehydroepiandrosterone sulfotransferase: molecular cloning and expression of cDNA.#JOURNAL:Mol Pharmacol#YEAR:1992#PUBMED:1588921# AUTHORS:Chatterjee B, Echchgadda I, Song CS#TITLE:Vitamin D receptor regulation of the steroid/bile acid sulfotransferase SULT2A1#JOURNAL:Methods Enzymol#YEAR:2005#PUBMED:16399349# GENE ASSOCIATION: (6822.1) or (6822.1)
Abbreviation: R_EBP1r Synonyms: _4 EC Number: 5.3.3.5 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : zymst --> chlstol Confidence Level: 4 involved in lanosterol -> cholesterol pathway NJ genes: LOCUS:_10682#TRANSCRIPT:1#ABBREVIATION:EBP#ECNUMBERS:5.3.3.5# proteins: NAME:emopamil binding protein (sterol isomerase)#ABBREVIATION:Ebp.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# AUTHORS:Braverman N, Lin P, Moebius FF, Obie C, Moser A, Glossmann H, Wilcox WR, Rimoin DL, Smith M, Kratz L, Kelley RI, Valle D#TITLE:Mutations in the gene encoding 3-beta-hydroxysteroid delta8, delta7- isomerase cause X-linked dominant Conradi-Hunermann Syndrome#JOURNAL:Nature Genetics#YEAR:1999# GENE ASSOCIATION: (10682.1)
Abbreviation: R_PItx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: pi[c] <==> pi[x] Confidence Level: 1 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LEUt5m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: leu-L[c] <==> leu-L[m] Confidence Level: 4 MM genes: none proteins: none citations: AUTHORS:Porter RK.#TITLE:Mammalian mitochondrial inner membrane cationic and neutral amino acid carriers.#YEAR:2000#PUBMED:11004451# GENE ASSOCIATION:
Abbreviation: R_CO2tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: co2[c] <==> co2[m] Confidence Level: 4 IF genes: none proteins: none citations: AUTHORS:Balboni E, Lehninger AL.#TITLE:Entry and exit pathways of CO2 in rat liver mitochondria respiring in a bicarbonate buffer system.#JOURNAL:J Biol Chem#YEAR:1968#PUBMED:3081508# GENE ASSOCIATION:
CO2 transport (diffusion), mitochondrial
Abbreviation: R_GLUTCOADHm Synonyms: _1 EC Number: 1.3.99.7 SUBSYSTEM: Tryptophan metabolism Equation: [m] : fad + glutcoa + h --> b2coa + co2 + fadh2 Confidence Level: 2 SAB use of FAD is not certain genes: LOCUS:_2639#TRANSCRIPT:1#ABBREVIATION:GCDH#ECNUMBERS:1.3.99.7# LOCUS:_2639#TRANSCRIPT:2#ABBREVIATION:GCDH# proteins: NAME:glutaryl-Coenzyme A dehydrogenase#ABBREVIATION:Gcdh.1-m# NAME:glutaryl-Coenzyme A dehydrogenase#ABBREVIATION:Gcdh.2-m# citations: none GENE ASSOCIATION: (2639.1) or (2639.2)
glutaryl-CoA dehydrogenase (mitochondria)
Abbreviation: R_ST8SIA54g Synonyms: _13 EC Number: 2.4.99.8 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gm1b_hs --> cmp + gd1c_hs + h Confidence Level: 4 NJ genes: LOCUS:_29906#TRANSCRIPT:1#ABBREVIATION:SIAT8E# proteins: NAME:ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 5 #ABBREVIATION:St8sia5.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Harduin-Lepers A, Vallejo-Ruiz V, Krzewinski-Recchi MA, Smyn-Petit B, Julien S, Delannoy P#TITLE:The human sialyltransferase family#JOURNAL:Biochimie#YEAR:2001# AUTHORS:Kim YJ, Kim KS, Do S, Kim CH, Kim SK, Lee YC.#TITLE:Molecular cloning and expression of human alpha2,8-sialyltransferase (hST8Sia V).#JOURNAL:Biochem Biophys Res Commun.#YEAR:1997#PUBMED:9199191# GENE ASSOCIATION: (29906.1)
Abbreviation: R_HMGCOARr Synonyms: _0 EC Number: 1.1.1.34 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : (2) h + hmgcoa + (2) nadph --> coa + mev-R + (2) nadp Confidence Level: 4 NCD ER version - NJ genes: LOCUS:_3156#TRANSCRIPT:1#ABBREVIATION:HMGCR#ECNUMBERS:1.1.1.88# proteins: NAME:_3-hydroxy-3-methylglutaryl-Coenzyme A reductase#ABBREVIATION:Hmgcr.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# AUTHORS:Luskey KL, Stevens B.#TITLE:Human 3-hydroxy-3-methylglutaryl coenzyme A reductase. Conserved domains responsible for catalytic activity and sterol-regulated degradation.#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2991281# GENE ASSOCIATION: (3156.1)
omega hydroxy dodecanoate (n-C12:0)_C12H23O3
Abbreviation: R_KSItly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: ksi[e] --> ksi[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
keratan sulfate I transport, extracellular to lysosome
Abbreviation: R_HOXG Synonyms: _0 EC Number: 1.14.99.3 SUBSYSTEM: Heme Degradation Equation: [c] : (5) h + (3) nadph + (3) o2 + pheme --> biliverd + co + fe2 + (3) h2o + (3) nadp Confidence Level: 0 TDV (4/28/2004) There are conflicting data on how many moles of NADPH consumed per mole of Biliverdine (Tenhunen 1969, Ryter, 2000) genes: LOCUS:_3162#TRANSCRIPT:1#ABBREVIATION:HMOX1#ECNUMBERS:1.14.99.3# LOCUS:_3163#TRANSCRIPT:1#ABBREVIATION:HMOX2#ECNUMBERS:1.14.99.3# proteins: NAME:heme oxygenase (decycling) 1#ABBREVIATION:Hmox1.1# NAME:Heme oxygenase (decycling) 2 #ABBREVIATION:Hmox2.1# citations: none GENE ASSOCIATION: (3162.1) or (3163.1)
Abbreviation: R_CSPG_Ctly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: cspg_c[e] --> cspg_c[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate C transport, extracellular to lysosome
Abbreviation: R_EBP2r Synonyms: _4 EC Number: 5.3.3.5 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : zymstnl --> lthstrl Confidence Level: 4 involved in lanosterol -> cholesterol pathway NJ genes: LOCUS:_10682#TRANSCRIPT:1#ABBREVIATION:EBP#ECNUMBERS:5.3.3.5# proteins: NAME:emopamil binding protein (sterol isomerase)#ABBREVIATION:Ebp.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# AUTHORS:Braverman N, Lin P, Moebius FF, Obie C, Moser A, Glossmann H, Wilcox WR, Rimoin DL, Smith M, Kratz L, Kelley RI, Valle D#TITLE:Mutations in the gene encoding 3-beta-hydroxysteroid delta8, delta7- isomerase cause X-linked dominant Conradi-Hunermann Syndrome#JOURNAL:Nature Genetics#YEAR:1999# GENE ASSOCIATION: (10682.1)
Abbreviation: R_EX_ribflv_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ribflv <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ADNK1m Synonyms: _0 EC Number: 2.7.1.20 SUBSYSTEM: Nucleotides Equation: [m] : adn + atp --> adp + amp + h Confidence Level: 1 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACOAD8m Synonyms: _0 EC Number: 1.3.99.10 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : fad + ivcoa --> 3mb2coa + fadh2 Confidence Level: 4 MM genes: LOCUS:_3712#TRANSCRIPT:1#ABBREVIATION:IVD#ECNUMBERS:1.3.99.10# proteins: NAME:isovaleryl Coenzyme A dehydrogenase, mitochondrial #ABBREVIATION:Ivd.1-m# citations: AUTHORS:Tiffany,K.A., Roberts,D.L., Wang,M., Paschke,R., Mohsen,A.W., Vockley,J., Kim,J.-J.P., #TITLE:Structure of human isovaleryl-CoA dehydrogenase at 2.6-A resolution: structural basis for substrate specificity. .#YEAR:1997#PUBMED:9214289# AUTHORS:Vockley J, Nagao M, Parimoo B, Tanaka K. #TITLE:The variant human isovaleryl-CoA dehydrogenase gene responsible...#YEAR:1992# GENE ASSOCIATION: (3712.1)
acyl-CoA dehydrogenase (isovaleryl-CoA), mitochondrial
saccharopine dehydrogenase (NADP, L-lysine forming), mitochondrial
Abbreviation: R_SACCD3m Synonyms: _0 EC Number: 1.5.1.8 SUBSYSTEM: Lysine Metabolism Equation: [m] : akg + h + lys-L + nadph --> h2o + nadp + saccrp-L Confidence Level: 4 MM genes: LOCUS:_10157#TRANSCRIPT:1#ABBREVIATION:AASS# proteins: NAME:aminoadipate-semialdehyde synthase, mitochondrial #ABBREVIATION:Aass.1-m# citations: AUTHORS:Sacksteder,K.A., Biery,B.J., Morrell,J.C., Goodman,B.K., Geisbrecht,B.V., Cox,R.P., Gould,S.J., Geraghty,M.T., #TITLE:Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia.#YEAR:2000#PUBMED:10775527# AUTHORS:Cox, RP#TITLE:Errors of lysine metabolism#JOURNAL:The Metabolic and Molecular Bases of Inherited Disease, 8th ed#YEAR:2001# GENE ASSOCIATION: (10157.1)
Abbreviation: R_A_MANASE Synonyms: _6 EC Number: 3.2.1.24 SUBSYSTEM: N-Glycan Degradation Equation: [c] : (2) h2o + m2mn --> (2) man + mn Confidence Level: 0 NCD genes: LOCUS:_4123#TRANSCRIPT:1#ABBREVIATION:MAN2C1#ECNUMBERS:3.2.1.24# proteins: NAME:mannosidase, alpha, class 2C, member 1#ABBREVIATION:Man2c1.1# citations: none GENE ASSOCIATION: (4123.1)
Abbreviation: R_EX_txa2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : txa2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_hista_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hista <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_1D-myo-Inositol 1,3-bisphosphate_C6H10O12P2
Abbreviation: R_MI13PP Synonyms: _5 EC Number: 3.1.3.64 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi13p --> mi1p-D + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# GENE ASSOCIATION:
Abbreviation: R_ST6GALNAC31 Synonyms: _4 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gm1b_hs --> cmp + gd1alpha_hs + h Confidence Level: 4 NJ genes: LOCUS:_30815#TRANSCRIPT:1#ABBREVIATION:SIAT7F# LOCUS:_256435#TRANSCRIPT:1#ABBREVIATION:SIAT7C# LOCUS:_81849#TRANSCRIPT:1#ABBREVIATION:ST6GALNAC5# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 6 #ABBREVIATION:St6galnac6.1# NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide #ABBREVIATION:St6galnac3.1# NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 5 #ABBREVIATION:St6galnac5.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Harduin-Lepers A, Vallejo-Ruiz V, Krzewinski-Recchi MA, Smyn-Petit B, Julien S, Delannoy P#TITLE:The human sialyltransferase family#JOURNAL:Biochimie#YEAR:2001# GENE ASSOCIATION: (256435.1) or (30815.1) or (81849.1)
Alpha-N-acetylgalactosaminide alpha-2,6-sialyltransferase 3
Abbreviation: R_MLTG1 Synonyms: _0 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : h2o + malttr --> glc-D + malt Confidence Level: 4 JLR genes: LOCUS:_2595#TRANSCRIPT:1#ABBREVIATION:GANC#ECNUMBERS:3.2.1.20# proteins: NAME:alpha glucosidase; neutral C#ABBREVIATION:Ganc.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Hirschhorn R, Huie ML, Kasper JS#TITLE:Computer assisted cloning of human neutral alpha-glucosidase C (GANC): a new paralog in the glycosyl hydrolase gene family 31#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2002#PUBMED:12370436# GENE ASSOCIATION: (2595.1)
Abbreviation: R_EX_cspg_c_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cspg_c <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate C (GalNAc6S-GlcA) proteoglycan exchange
GT1aalpha (homo sapiens)_C77H127N5O46FULLRCO
chondroitin sulfate B / dermatan sulfate (IdoA2S-GalNAc4S), degradation product 3_C37H55NO38S2
Abbreviation: R_S2TASE3ly Synonyms: _12 EC Number: 3.1.6.13 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_b_deg2 + h2o --> cs_b_deg3 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_3423#TRANSCRIPT:1#ABBREVIATION:IDS#ECNUMBERS:3.1.6.13# LOCUS:_3423#TRANSCRIPT:2#ABBREVIATION:IDS#ECNUMBERS:3.1.6.13# proteins: NAME:iduronate 2-sulfatase (Hunter syndrome), lysosome#ABBREVIATION:Ids.1-l# NAME:iduronate 2-sulfatase (Hunter syndrome), lysosome#ABBREVIATION:Ids.2-l# citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Wilson PJ, Meaney CA, Hopwood JJ, Morris CP#TITLE:Sequence of the human iduronate 2-sulfatase (IDS) gene#JOURNAL:Genomics#YEAR:1993#PUBMED:8244397# AUTHORS:Malmgren H, Carlberg BM, Pettersson U, Bondeson ML#TITLE:dentification of an alternative transcript from the human iduronate-2-sulfatase (IDS) gene#JOURNAL:Genomics#YEAR:1995#PUBMED:8530090# AUTHORS:Wilson PJ, Morris CP, Anson DS, Occhiodoro T, Bielicki J, Clements PR, Hopwood JJ#TITLE:Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990#PUBMED:2122463# GENE ASSOCIATION: (3423.2) or (3423.1)
Abbreviation: R_PYDX5Ptm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: pydx5p[c] <==> pydx5p[m] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Lui A, Lumeng L, Li TK.#TITLE:Metabolism of vitamin B6 in rat liver mitochondria.#JOURNAL:J Biol Chem.#YEAR:1981#PUBMED:6263901# AUTHORS:Lui A, Lumeng L, Li TK.#TITLE:Transport of pyridoxine and pyridoxal 5'-phosphate in isolated rat liver mitochondria.#JOURNAL:J Biol Chem.#YEAR:1982#PUBMED:7174673# GENE ASSOCIATION:
Pyridoxal 5'-phosphate transport via diffusion, mitochondrial
Abbreviation: R_EX_asp_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : asp-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_N2M2NMASNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: n2m2nmasn[g] --> n2m2nmasn[e] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
n2m2nmasn transport, Golgi to extracellular
Abbreviation: R_G6PDH2r Synonyms: _0 EC Number: 1.1.1.49 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : g6p + nadp <==> 6pgl + h + nadph Confidence Level: 0 JLR - a reversible reaction that is otherwise identical to ZWF genes: LOCUS:_2539#TRANSCRIPT:1#ABBREVIATION:G6PD#ECNUMBERS:1.1.1.49# proteins: NAME:glucose-6-phosphate dehydrogenase#ABBREVIATION:G6pd.1# citations: AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (2539.1)
Abbreviation: R_DOLPMT_U Synonyms: _0 EC Number: 2.4.1.83 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dolp_U + gdpmann --> (0.1) dolmanp_U + gdp Confidence Level: 0 NCD genes: LOCUS:_8813#TRANSCRIPT:1#ABBREVIATION:DPM1#ECNUMBERS:2.4.1.83# LOCUS:_8818#TRANSCRIPT:1#ABBREVIATION:DPM2# LOCUS:_54344#TRANSCRIPT:1#ABBREVIATION:DPM3# LOCUS:_8818#TRANSCRIPT:2#ABBREVIATION:DPM2# LOCUS:_54344#TRANSCRIPT:2#ABBREVIATION:DPM3# proteins: NAME:dolichyl-phosphate mannosyltransferase (ubiquitous)#ABBREVIATION:Dpm111.1# NAME:dolichyl-phosphate mannosyltransferase (ubiquitous)#ABBREVIATION:Dpm112.1# NAME:dolichyl-phosphate mannosyltransferase (ubiquitous)#ABBREVIATION:Dpm121.1# NAME:dolichyl-phosphate mannosyltransferase (ubiquitous)#ABBREVIATION:Dpm122.1# citations: AUTHORS:Manos EJ, Kim ML, Kassis J, Chang PY, Wells A, Jones DA.#TITLE:Dolichol-phosphate-mannose-3 (DPM3)/prostin-1 is a novel phospholipase C-gamma regulated gene negatively associated with prostate tumor invasion.#JOURNAL:Oncogene#YEAR:2001#PUBMED:11420690# GENE ASSOCIATION: (8818.1 and 54344.1 and 8813.1) or (54344.1 and 8818.2 and 8813.1) or (54344.2 and 8818.1 and 8813.1) or (54344.2 and 8818.2 and 8813.1)
Dolichyl-phosphate D-mannosyltransferase (uterus)
Abbreviation: R_TCHOLAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hco3[c] + tchola[e] <==> hco3[e] + tchola[c] Confidence Level: 4 NJ genes: LOCUS:_28234#TRANSCRIPT:1#ABBREVIATION:SLCO1B3# LOCUS:_6579#TRANSCRIPT:1#ABBREVIATION:SLCO1A2# LOCUS:_28231#TRANSCRIPT:1#ABBREVIATION:SLCO4A1# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1A2#ABBREVIATION:Slco1a2.1# NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# NAME:solute carrier organic anion transporter family, member 1B3#ABBREVIATION:Slco1b3.1# NAME:solute carrier organic anion transporter family, member 4A1#ABBREVIATION:Slco4a1.1# citations: AUTHORS:Kullak-Ublick GA, Beuers U, Meier PJ, Domdey H, Paumgartner G.#TITLE:Assignment of the human organic anion transporting polypeptide (OATP) gene to chromosome 12p12 by fluorescence in situ hybridization.#JOURNAL:J Hepatol.#YEAR:1996#PUBMED:9007731# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (28231.1) or (6579.1) or (28234.1) or (10599.1)
taurocholate transport via bicarbonate countertransport
omega hydroxy tetradecanoate (n-C14:0)_C14H27O3
Abbreviation: R_EX_whttdca_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : whttdca <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
omega hydroxy tetradecanoate (n-C14:0) exchange
Abbreviation: R_KDNH Synonyms: _0 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : h2o + kdnp --> kdn + pi Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
_2-keto-3deoxy-D-glycero-D-galactononic acid phosphohydrolase
Abbreviation: R_PI3P3Pn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail3p_hs --> pail_hs + pi Confidence Level: 2 EC 3.1.3.- NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
phosphatidylinositol-3-phosphate 3-phosphatase, nuclear
(alpha-D-Glucosyl)-(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine (protein)_C76H127N2O60X
(alpha-D-Glucosyl)2-(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine (protein)_C82H137N2O65X
Abbreviation: R_MG2er Synonyms: _1 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : g2m8masn + h2o --> g1m8masn + glc-D Confidence Level: 0 EC-3.2.1.- NCD genes: none proteins: none citations: none GENE ASSOCIATION:
mannosyl-oligosaccharide glucosidase, endoplasmic reticulum
Abbreviation: R_AIRCr Synonyms: _0 EC Number: 4.1.1.21 SUBSYSTEM: IMP Biosynthesis Equation: [c] : air + co2 <==> 5aizc + h Confidence Level: 4 NCD genes: LOCUS:_10606#TRANSCRIPT:1#ABBREVIATION:PAICS#ECNUMBERS:6.3.2.6,4.1.1.21# proteins: NAME:phosphoribosylaminoimidazole carboxylase, phosphoribosylaminoimidazole succinocarboxamide synthetase #ABBREVIATION:PaicS.1# citations: AUTHORS:Brayton KA, Chen Z, Zhou G, Nagy PL, Gavalas A, Trent JM, Deaven LL, Dixon JE, Zalkin H. #TITLE:Two genes for de novo purine nucleotide synthesis on human chromosome 4 are closely linked and divergently transcribed.#JOURNAL:J Biol Chem. #YEAR:1994#PUBMED:8106516# AUTHORS:Minet M, Lacroute F.#TITLE:Cloning and sequencing of a human cDNA coding for a multifunctional polypeptide of the purine pathway by complementation of the ade2-101 mutant in Saccharomyces cerevisiae.#JOURNAL:Curr Genet.#YEAR:1990#PUBMED:2253271# AUTHORS:Barton JW, Hart IM, Patterson D.#TITLE:Mapping of a locus correcting lack of phosphoribosylaminoimidazole carboxylase activity in Chinese hamster ovary cell Ade-D mutants to human chromosome 4. #JOURNAL:Genomics#YEAR:1991#PUBMED:2004782# GENE ASSOCIATION: (10606.1)
phosphoribosylaminoimidazole carboxylase
keratan sulfate II biosynthesis, precursor 6_C61H98N5O46SX
Abbreviation: R_G14T4g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre6 + udpgal --> h + ksii_core2_pre7 + udp Confidence Level: 4 Keratan sulfate II biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
mannosyl-3-(phosphoethanolaminyl-mannosyl)-glucosaminyl-acylphosphatidylinositol (M4A)_C61H116N4O43P4FULLRCO2FULLR2CO2
Abbreviation: R_GULLACter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: gullac[c] --> gullac[r] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Banhegyi G, Mandl J#TITLE:The hepatic glycogenoreticular system#JOURNAL:Pathol Oncol Res#YEAR:2001#PUBMED:11458272# GENE ASSOCIATION:
gulonolactone endoplasmic reticular transport
phosphoethanolaminyl-mannosyl-glucosylaminyl-acylphosphatidylinositol (H5)_C39H74N2O22P2FULLRCO2FULLR2CO2
Abbreviation: R_H2ETer Synonyms: _1 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : mgacpail_hs + pe_hs --> dag_hs + emgacpail_hs Confidence Level: 2 NCD genes: LOCUS:_23556#TRANSCRIPT:1#ABBREVIATION:PIGN# LOCUS:_23556#TRANSCRIPT:2#ABBREVIATION:PIGN# proteins: NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.1-r# NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.2-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Hong Y, Maeda Y, Watanabe R, Ohishi K, Mishkind M, Riezman H, Kinoshita T#TITLE:Pig-n, a mammalian homologue of yeast Mcd4p, is involved in transferring phosphoethanolamine to the first mannose of the glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10574991# GENE ASSOCIATION: (23556.1) or (23556.2)
H2 phosphoethanolaminyl transferase, endoplasmic reticulum
(Gal)3 (Glc)1 (GlcNAc)2 (LFuc)3 (Cer)1_C76H132N3O44FULLRCO
Abbreviation: R_HIStiDF Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: his-L[e] --> his-L[c] Confidence Level: 4 SAB genes: LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# GENE ASSOCIATION: (55089.1)
L-histidine transport via diffusion (extracellular to cytosol)
phosphatidylinositol 4-phosphate 5-kinase, nuclear
Abbreviation: R_PI4P5Kn Synonyms: _5 EC Number: 2.7.1.68 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + pail4p_hs --> adp + h + pail45p_hs Confidence Level: 2 Notice that contrary to some standards in texts, KEGG, etc, the Rtotal group accounts for the whole fatty acyl CoA (so the CHO only accounts for the core backbone), manner in which Rtotal was produced accounts for O and H balances. Formation of the Rtotal group (rxn flux)accounted for mass balance for CoA NJ changed EC from 2.7.1.149 (pail5p) to 2.7.1.68 (pail4p) - NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
Abbreviation: R_CERT2gt Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gluside_hs[c] <==> gluside_hs[g] Confidence Level: 2 non-vesicular intracellular transport of glucocerebroside genes: LOCUS:_10087#TRANSCRIPT:1#ABBREVIATION:COL4A3BP# proteins: NAME:collagen, type IV, alpha 3 (Goodpasture antigen) binding protein#ABBREVIATION:Col4a3bp.1# citations: AUTHORS:Kumagai K, Yasuda S, Okemoto K, Nishijima M, Kobayashi S, Hanada K.#TITLE:CERT mediates intermembrane transfer of various molecular species of ceramides.#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15596449# AUTHORS:Hanada K, Kumagai K, Yasuda S, Miura Y, Kawano M, Fukasawa M, Nishijima M.#TITLE:Molecular machinery for non-vesicular trafficking of ceramide.#JOURNAL:Nature#YEAR:2003#PUBMED:14685229# AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# AUTHORS:van Meer G, Wolthoorn J, Degroote S.#TITLE:The fate and function of glycosphingolipid glucosylceramide.#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:2003#PUBMED:12803919# GENE ASSOCIATION: (10087.1)
_1-alkyl 2-acylglycerol 3-phosphate_C5H9O6PFULLRFULLR2CO
Abbreviation: R_EX_ocdcea_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ocdcea <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_10FTHF7GLUtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _10fthf7glu[m] --> 10fthf7glu[c] Confidence Level: 2 mechanism is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_7-glutamyl-10FTHF transport, mitochondrial
testicular 17-beta-hydroxysteroid dehydrogenase
Abbreviation: R_HSD17B7r Synonyms: _1 EC Number: 1.1.1.62 SUBSYSTEM: Steroid Metabolism Equation: [r] : estrone + h + nadph --> estradiol + nadp Confidence Level: 4 microsomal NADP as cofactor HSD type 7 recheck/confirm comparmentalization - type 1: cytosol, types 2,3: microsomes NJ genes: LOCUS:_51478#TRANSCRIPT:1#ABBREVIATION:HSD17B7#ECNUMBERS:1.1.1.62# proteins: NAME:hydroxysteroid (17-beta) dehydrogenase 7#ABBREVIATION:Hsd17b7.1# citations: AUTHORS:Leenders F, Dolez V, Begue A, Moller G, Gloeckner JC, deLaunoit Y, Adamski J#TITLE:Structure of the gene for the human 17-beta-hydroxysteroid dehydrogenase type IV#JOURNAL:Mammalian Genome#YEAR:1998# AUTHORS:Adamski J, Leenders F Carstensen JF, Kaumann M, Markus MM, Husen B, Tesdorpf JG, Seedor U, deLaunoit Y, Jakob F#TITLE:Steroids, fatty acyl-CoA, and sterols are substrates of 80-kDa multifunctional protein#JOURNAL:Steroids#YEAR:1997# AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (51478.1)
Abbreviation: R_NRVNCCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + nrvnccoa --> coa + nrvnccrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
dihydroceramide (homo sapiens)_C18H38NO2FULLRCO
Abbreviation: R_DSAT Synonyms: _0 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : Rtotalcoa + sphgn --> coa + dhcrm_hs + h Confidence Level: 2 CoA transfer not explicitly in rxn (accounted for in Rtotal rxn -> ARTF) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION:
mannosyl-3-(phosphoethanolaminyl-mannosyl)-glucosaminyl-acylphosphatidylinositol-Protein (M4A)_C61H116N4O43P4FULLRCO2FULLR2CO2X
galactocerebroside (homo sapiens)_C24H46NO7FULLRCO
Abbreviation: R_GALSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: galside_hs[c] <==> galside_hs[g] Confidence Level: 1 unknown mechanism for transport - not known if it is energy dependent or not, recycling and IC transport is important to get it to other areas of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
galactocerebroside intracellular transport
Abbreviation: R_C161CPT12 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + hdd2coa --> coa + hdd2crn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (126129.1) or (1375.3) or (1375.1) or (1375.2) or (1374.1) or (1375.4)
Abbreviation: R_NBAHH_ir Synonyms: _0 EC Number: 3.4.13.3 SUBSYSTEM: beta-Alanine metabolism Equation: [c] : carn + h2o --> ala-B + his-L Confidence Level: 1 NAME Nalpha-(beta-alanyl)-L-histidine hydrolase DEFINITION Carnosine + H2O <=> beta-Alanine + L-Histidine SAB made irreversible for human genes: LOCUS:_84735#TRANSCRIPT:1#ABBREVIATION:CNDP1#ECNUMBERS:3.4.13.20# proteins: NAME:carnosine dipeptidase 1 (metallopeptidase M20 family)#ABBREVIATION:Cndp1.1# citations: none GENE ASSOCIATION: (84735.1)
Nalpha-(beta-alanyl)-L-histidine hydrolase IR
Abbreviation: R_COQ3m Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 2dpmhobq + amet --> ahcys + h + q10 Confidence Level: 4 _2.1.1.114 IT genes: LOCUS:_51805#TRANSCRIPT:1#ABBREVIATION:COQ3# proteins: NAME:coenzyme Q3 homolog, methyltransferase (yeast) #ABBREVIATION:Coq3.1-m# citations: AUTHORS:Kang DC, Takeshige K, Minakami S.#TITLE:An intermediate of ubiquinone biosynthesis exists in the microsomal fraction of HepG2 cells. #JOURNAL:J Biochem (Tokyo).#YEAR:1990#PUBMED:1965190# AUTHORS:Nambudiri AM, Ranganathan S, Rudney H#TITLE:The role of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in the regulation of ubiquinone synthesis in human fibroblasts. #JOURNAL:J Biol Chem.#YEAR:1980#PUBMED:7380842# AUTHORS:Szkopinska A.#TITLE:Ubiquinone. Biosynthesis of quinone ring and its isoprenoid side chain. Intracellular localization.#JOURNAL:Acta Biochim Pol. #YEAR:2000#PUBMED:11051212# AUTHORS:Jonassen T, Clarke CF.#TITLE:Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis. #JOURNAL:J Biol Chem.#YEAR:2000#PUBMED:10777520# GENE ASSOCIATION: (51805.1)
Abbreviation: R_CEPTC Synonyms: _1 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : cdpchol + dag_hs --> cmp + h + pchol_hs Confidence Level: 4 NJ genes: LOCUS:_56994#TRANSCRIPT:1#ABBREVIATION:CHPT1# LOCUS:_10390#TRANSCRIPT:1#ABBREVIATION:CEPT1# proteins: NAME:choline phosphotransferase 1 #ABBREVIATION:Chpt1.1# NAME:choline/ethanolamine phosphotransferase#ABBREVIATION:Cept1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Henneberry AL, McMaster CR#TITLE:Cloning and expression of a human choline/ethanolaminephosphotransferase: synthesis of phosphatidylcholine and phosphatidylethanolamine#JOURNAL:Biochem J#YEAR:1999# GENE ASSOCIATION: (10390.1) or (56994.1)
Abbreviation: R_DM_hretn_LPAREN_n_RPAREN_ Synonyms: _0 Equation: [n] : hretn --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_fucfuc132galacglcgal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucfuc132galacglcgal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_COAtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: coa[c] <==> coa[m] Confidence Level: 4 TV May not a "real" transporter, use in heart mitochondria model only genes: LOCUS:_8034#TRANSCRIPT:1#ABBREVIATION:SLC25A16# proteins: NAME:Solute carrier family 25 member 16, mitochondrial#ABBREVIATION:Slc25a16.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (8034.1)
keratan sulfate I biosynthesis, precursor 3_C93H152N6O67X
Abbreviation: R_ELAIDCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + od2coa --> coa + elaidcrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_IMPD Synonyms: _0 EC Number: 1.1.1.205 SUBSYSTEM: Nucleotides Equation: [c] : h2o + imp + nad --> h + nadh + xmp Confidence Level: 4 genes: LOCUS:_3615#TRANSCRIPT:1#ABBREVIATION:IMPDH2#ECNUMBERS:1.1.1.205# LOCUS:_3614#TRANSCRIPT:1#ABBREVIATION:IMPDH1#ECNUMBERS:1.1.1.205# LOCUS:_3614#TRANSCRIPT:2#ABBREVIATION:IMPDH1#ECNUMBERS:1.1.1.205# proteins: NAME:IMP (inosine monophosphate) dehydrogenase 1 #ABBREVIATION:ImpDH1.1# NAME:IMP (inosine monophosphate) dehydrogenase 1 #ABBREVIATION:ImpDH1.2# NAME:IMP (inosine monophosphate) dehydrogenase 2 #ABBREVIATION:ImpDH2.1# citations: AUTHORS:Natsumeda Y, Ohno S, Kawasaki H, Konno Y, Weber G, Suzuki K.#TITLE:Two distinct cDNAs for human IMP dehydrogenase.#JOURNAL:J Biol Chem.#YEAR:1990# AUTHORS:Gu JJ, Kaiser-Rogers K, Rao K, Mitchell BS.#TITLE:Assignment of the human type I IMP dehydrogenase gene (IMPDH1) to chromosome 7q31.3-q32). #JOURNAL:Genomics#YEAR:1994# AUTHORS:Xiang B, Taylor JC, Markham GD. #TITLE:Monovalent cation activation and kinetic mechanism of inosine 5'-monophosphate dehydrogenase. #JOURNAL:J Biol Chem. #YEAR:1996# GENE ASSOCIATION: (3614.1) or (3615.1) or (3614.2)
Abbreviation: R_PRPPS Synonyms: _0 EC Number: 2.7.6.1 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : atp + r5p <==> amp + h + prpp Confidence Level: 0 genes: LOCUS:_5634#TRANSCRIPT:1#ABBREVIATION:PRPS2#ECNUMBERS:2.4.2.17# LOCUS:_5631#TRANSCRIPT:1#ABBREVIATION:PRPS1#ECNUMBERS:2.4.2.17# LOCUS:_221823#TRANSCRIPT:1#ABBREVIATION:PRPS1L1# LOCUS:_221823#TRANSCRIPT:2#ABBREVIATION:PRPS1L1# proteins: NAME:phosphoribosyl pyrophosphate synthetase 1#ABBREVIATION:Prps1.1# NAME:phosphoribosyl pyrophosphate synthetase 2#ABBREVIATION:Prps2.1# NAME:phosphoribosyl pyrophosphate synthetase 1-like 1 (testis)#ABBREVIATION:Prps1l1.1# NAME:phosphoribosyl pyrophosphate synthetase 1-like 1 (testis)#ABBREVIATION:Prps1l1.2# citations: AUTHORS:Taira M, Iizasa T, Shimada H, Kudoh J, Shimizu N, Tatibana M.#TITLE:A human testis-specific mRNA for phosphoribosylpyrophosphate synthetase that initiates from a non-AUG codon.#JOURNAL:J Biol Chem.#YEAR:1990#PUBMED:2168892# AUTHORS:Becker MA, Kim M#TITLE:Regulation of purine synthesis de novo in human fibroblasts by purine nucleotides and phosphoribosylpyrophosphate.#JOURNAL:J Biol Chem. #YEAR:1987#PUBMED:2444588# GENE ASSOCIATION: (221823.2) or (5631.1) or (221823.1) or (5634.1)
Abbreviation: R_DNDPt28m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[c] + dgdp[m] --> dcdp[m] + dgdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_hco3_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hco3 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_oh1 Synonyms: _0 Equation: [e] : oh1 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6TASE25ly Synonyms: _7 EC Number: 3.1.6.4 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core4_deg1 <==> h + ksii_core4_deg2 + so4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
galactose-6-sulfate sulfatase, lysosomal
keratan sulfate II (core 4-linked), degradation product 1_C78H126N6O64S3X
Abbreviation: R_GALNACT5g Synonyms: _6 EC Number: 2.4.1.175 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_e_pre3 + uacgam --> cs_e_pre4 + h + udp Confidence Level: 4 NCD genes: LOCUS:_22856#TRANSCRIPT:1#ABBREVIATION:CHSY1# LOCUS:_55454#TRANSCRIPT:1#ABBREVIATION:GALNACT-2# LOCUS:_55790#TRANSCRIPT:1#ABBREVIATION:ChGn# LOCUS:_79586#TRANSCRIPT:1#ABBREVIATION:CHPF# LOCUS:_337876#TRANSCRIPT:1#ABBREVIATION:CSS3# proteins: NAME:chondroitin sulfate GalNAcT-2, Golgi apparatus#ABBREVIATION:Galnact-2.1-g# NAME:chondroitin beta1,4 N-acetylgalactosaminyltransferase, Golgi apparatus#ABBREVIATION:Chgn.1-g# NAME:carbohydrate (chondroitin) synthase 1, Golgi apparatus#ABBREVIATION:Chsy1.1-g# NAME:chondroitin polymerizing factor, Golgi apparatus#ABBREVIATION:Chpf.1-g# NAME:chondroitin sulfate synthase 3, Golgi apparatus#ABBREVIATION:Css3.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Sato T, Gotoh M, Kiyohara K, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Togayachi A, Kudo T, Asada M, Watanabe H, Imamura T, Kimata K, Narimatsu H#TITLE:Differential roles of two N-acetylgalactosaminyltransferases, CSGalNAcT-1, and a novel enzyme, CSGalNAcT-2. Initiation and elongation in synthesis of chondroitin sulfate#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12446672# AUTHORS:Uyama T, Kitagawa H, Tamura Ji J, Sugahara K#TITLE:Molecular cloning and expression of human chondroitin N-acetylgalactosaminyltransferase: the key enzyme for chain initiation and elongation of chondroitin/dermatan sulfate on the protein linkage region tetrasaccharide shared by heparin/heparan sulfate#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11788602# AUTHORS:Gotoh M, Sato T, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Zhang Y, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Nishihara S, Watanabe H, Kimata K, Narimatsu H#TITLE:Enzymatic synthesis of chondroitin with a novel chondroitin sulfate N-acetylgalactosaminyltransferase that transfers N-acetylgalactosamine to glucuronic acid in initiation and elongation of chondroitin sulfate synthesis#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12163485# AUTHORS:Kitagawa H, Uyama T, Sugahara K#TITLE:Molecular cloning and expression of a human chondroitin synthase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11514575# AUTHORS:Kitagawa H, Izumikawa T, Uyama T, Sugahara K#TITLE:Molecular cloning of a chondroitin polymerizing factor that cooperates with chondroitin synthase for chondroitin polymerization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12716890# AUTHORS:Yada T, Sato T, Kaseyama H, Gotoh M, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-3. Molecular cloning and characterization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12907687# AUTHORS:Yada T, Gotoh M, Sato T, Shionyu M, Go M, Kaseyama H, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12761225# GENE ASSOCIATION: (55790.1) or (79586.1) and (22856.1) or (55454.1) or (337876.1)
chondroitin sulfate E (GalNAc4,6diS-GlcA), precursor 4_C45H65N2O48S4X
Abbreviation: R_STCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: stcoa[c] <==> stcoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ADPT Synonyms: _0 EC Number: 2.4.2.7 SUBSYSTEM: Salvage Pathway Equation: [c] : ade + prpp --> amp + ppi Confidence Level: 4 genes: LOCUS:_353#TRANSCRIPT:1#ABBREVIATION:APRT#ECNUMBERS:2.4.2.7# proteins: NAME:adenine phosphoribosyltransferase #ABBREVIATION:AprT.1# citations: AUTHORS:Silva M, Silva CH, Iulek J, Thiemann OH.#TITLE: Three-dimensional structure of human adenine phosphoribosyltransferase and its relation to DHA-urolithiasis. #JOURNAL:Biochemistry#YEAR:2004#PUBMED:15196008# AUTHORS:Crespillo J, Llorente P, Argomaniz L, Montero C.#TITLE:APRT from erythrocytes of HGPRT deficient patients: kinetic, regulatory and thermostability properties. #JOURNAL:Mol Cell Biochem#YEAR:2003#PUBMED:14674717# GENE ASSOCIATION: (353.1)
Abbreviation: R_PHYHx Synonyms: _4 EC Number: 1.14.11.18 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : akg + o2 + phytcoa --> co2 + phyt2ohcoa + succ Confidence Level: 4 NJ genes: LOCUS:_5264#TRANSCRIPT:1#ABBREVIATION:PHYH# proteins: NAME:phytanoyl-CoA hydroxylase (Refsum disease)#ABBREVIATION:Phyh.1# citations: AUTHORS:Jansen GA, Ofman R, Ferdinandusse S, Ijlst L, Muijsers AO, Skjeldal OH, Stokke O, Jakobs C, Besley GT, Wraith JE, Wanders RJ.#TITLE:Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene.#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9326940# AUTHORS:Jansen GA, Mihalik SJ, Watkins PA, Moser HW, Jakobs C, Denis S, Wanders RJ.#TITLE:Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid alpha-oxidation in humans.#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8954107# GENE ASSOCIATION: (5264.1)
Abbreviation: R_FATP6t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: adrn[c] + na1[c] <==> adrn[e] + na1[e] Confidence Level: 4 electroneutral transport NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# LOCUS:_10998#TRANSCRIPT:1#ABBREVIATION:SLC27A5# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 5#ABBREVIATION:Slc27a5.1# NAME:solute carrier family 27 (fatty acid transporter), member 2#ABBREVIATION:Slc27a2.1# citations: AUTHORS:Steinberg SJ, Wang SJ, McGuinness MC, Watkins PA.#TITLE:Human liver-specific very-long-chain acyl-coenzyme A synthetase: cDNA cloning and characterization of a second enzymatically active protein.#JOURNAL:Mol Genet Metab.#YEAR:1999#PUBMED:10479480# AUTHORS:Stahl A.#TITLE:A current review of fatty acid transport proteins (SLC27).#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12856180# AUTHORS:Pohl J, Ring A, Hermann T, Stremmel W.#TITLE:Role of FATP in parenchymal cell fatty acid uptake.#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15522816# GENE ASSOCIATION: (10998.1) or (11001.1)
Abbreviation: R_EX_5mthf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 5mthf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FTHFL Synonyms: _0 EC Number: 6.3.4.3 SUBSYSTEM: Folate Metabolism Equation: [c] : atp + for + thf <==> 10fthf + adp + pi Confidence Level: 4 genes: LOCUS:_4522#TRANSCRIPT:1#ABBREVIATION:MTHFD1#ECNUMBERS:1.5.1.5,3.5.4.9,6.3.4.3# LOCUS:_286297#TRANSCRIPT:1#ABBREVIATION:LOC286297# proteins: NAME:methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1, methenyltetrahydrofolate cyclohydrolase, formyltetrahydrofolate synthetase#ABBREVIATION:Mthfd1.1# NAME:hypothetical protein LOC286297#ABBREVIATION:Loc286297.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (4522.1) or (286297.1)
Abbreviation: R_NDPK2m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + udp <==> adp + utp Confidence Level: 2 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:UDP), mitochondrial
Abbreviation: R_FUMm Synonyms: _0 EC Number: 4.2.1.2 SUBSYSTEM: Citric Acid Cycle Equation: [m] : fum + h2o <==> mal-L Confidence Level: 0 IF genes: LOCUS:_2271#TRANSCRIPT:1#ABBREVIATION:FH#ECNUMBERS:4.2.1.2# proteins: NAME:fumarate hydratase, mitochondrial#ABBREVIATION:Fh.1-m# citations: none GENE ASSOCIATION: (2271.1)
chondroitin sulfate D (GlcNAc6S-GlcA2S), degradation product 1_C45H66N2O49S4
Abbreviation: R_TTDCAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ttdca[e] --> ttdca[c] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Tetradecanoate (n-C14:0) transport in via uniport
Abbreviation: R_DGAT Synonyms: _0 SUBSYSTEM: Triacylglycerol Synthesis Equation: [c] : Rtotal3coa + dag_hs --> coa + tag_hs Confidence Level: 4 FULLR corresponds to acyl/R groups in texts. NJ genes: LOCUS:_8694#TRANSCRIPT:1#ABBREVIATION:DGAT1#ECNUMBERS:2.3.1.20# LOCUS:_84649#TRANSCRIPT:1#ABBREVIATION:DGAT2# proteins: NAME:diacylglycerol O-acyltransferase#ABBREVIATION:Dgat1.1# NAME:diacylglycerol O-acyltransferase homolog 2#ABBREVIATION:Dgat2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Coleman RA, Lee DP#TITLE:Enzymes of triacylglycerol synthesis and their regulation#JOURNAL:Prog in Lipid Research#YEAR:2004# AUTHORS:Cases S, Stone SJ, Zhou P, Yen E, Tow B, Lardizabal KD, Voelker T, Farese RV Jr.#TITLE:Cloning of DGAT2, a second mammalian diacylglycerol acyltransferase, and related family members.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11481335# GENE ASSOCIATION: (8694.1) or (84649.1)
Abbreviation: R_7DHFtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _7dhf[c] --> 7dhf[l] Confidence Level: 2 carrier-mediated transport - but which and how is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
Abbreviation: R_CHAT Synonyms: _3 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : accoa + chol <==> ach + coa Confidence Level: 4 rev by uniprot NJ genes: LOCUS:_1103#TRANSCRIPT:1#ABBREVIATION:CHAT#ECNUMBERS:2.3.1.6# LOCUS:_1103#TRANSCRIPT:4#ABBREVIATION:CHAT#ECNUMBERS:2.3.1.6# LOCUS:_1103#TRANSCRIPT:2#ABBREVIATION:CHAT#ECNUMBERS:2.3.1.6# LOCUS:_1103#TRANSCRIPT:3#ABBREVIATION:CHAT#ECNUMBERS:2.3.1.6# proteins: NAME:choline acetyltransferase#ABBREVIATION:Chat.1# NAME:choline acetyltransferase#ABBREVIATION:Chat.2# NAME:choline acetyltransferase#ABBREVIATION:Chat.3# NAME:choline acetyltransferase#ABBREVIATION:Chat.4# citations: AUTHORS:Dobransky T, Davis WL, Xiao GH, Rylett RJ.#TITLE:Expression, purification and characterization of recombinant human choline acetyltransferase: phosphorylation of the enzyme regulates catalytic activity.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10861222# GENE ASSOCIATION: (1103.4) or (1103.2) or (1103.3) or (1103.1)
Gal-GlcNAc-Gal globoside (homo sapiens)_C64H112N3O37FULLRCO
Abbreviation: R_PHETA1 Synonyms: _5 EC Number: 2.6.1.58 SUBSYSTEM: Phenylalanine metabolism Equation: [c] : akg + phe-L <==> glu-L + phpyr Confidence Level: 2 genes: LOCUS:_2805#TRANSCRIPT:1#ABBREVIATION:GOT1#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase #ABBREVIATION:Got1.1# citations: AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# GENE ASSOCIATION: (2805.1)
Abbreviation: R_STS4r Synonyms: _0 EC Number: 3.1.6.2 SUBSYSTEM: Steroid Metabolism Equation: [r] : h2o + prgnlones --> h + prgnlone + so4 Confidence Level: 4 NJ genes: LOCUS:_412#TRANSCRIPT:1#ABBREVIATION:STS#ECNUMBERS:3.1.6.2# proteins: NAME:steroid sulfatase (microsomal), arylsulfatase C#ABBREVIATION:Sts.1# citations: AUTHORS:Stein C, Hille A, Seidel J, Rijnbout S, Waheed A, Schmidt B, Geuze H, von Figura K.#TITLE:Cloning and expression of human steroid-sulfatase. Membrane topology, glycosylation, and subcellular distribution in BHK-21 cells.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2668275# AUTHORS:Migeon BR, Shapiro LJ, Norum RA, Mohandas T, Axelman J, Dabora RL.#TITLE:Abstract Differential expression of steroid sulphatase locus on active and inactive human X chromosome.#JOURNAL:Nature#YEAR:1982#PUBMED:6957717# GENE ASSOCIATION: (412.1)
Iodide:hydrogen-peroxide oxidoreductase 4
Abbreviation: R_IDHPOXOX4 Synonyms: _0 EC Number: 1.11.1.8 SUBSYSTEM: Tyrosine metabolism Equation: [c] : (2) 35diotyr + h2o2 --> 2amac + (2) h2o + thyox-L Confidence Level: 4 SAB genes: LOCUS:_7173#TRANSCRIPT:1#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:2#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:5#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:4#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:3#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# proteins: NAME:thyroid peroxidase#ABBREVIATION:Tpo.1# NAME:thyroid peroxidase#ABBREVIATION:Tpo.2# NAME:thyroid peroxidase#ABBREVIATION:Tpo.3# NAME:thyroid peroxidase#ABBREVIATION:Tpo.4# NAME:thyroid peroxidase#ABBREVIATION:Tpo.5# citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# AUTHORS:Nakamura M, Yamazaki I, Kotani T, Ohtaki S#TITLE:Thyroid peroxidase selects the mechanism of either 1- or 2-electron oxidation of phenols, depending on their substituents#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2997169# AUTHORS:Kimura S, Kotani T, McBride OW, Umeki K, Hirai K, Nakayama T, Ohtaki S#TITLE:Human thyroid peroxidase: complete cDNA and protein sequence, chromosome mapping, and identification of two alternately spliced mRNAs#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1987#PUBMED:3475693# AUTHORS:Magnusson RP, Chazenbalk GD, Gestautas J, Seto P, Filetti S, DeGroot LJ, Rapoport B. #TITLE:Molecular cloning of the complementary deoxyribonucleic acid for human thyroid peroxidase.#JOURNAL:Mol Endocrinol#YEAR:1987#PUBMED:3153466# GENE ASSOCIATION: (7173.4) or (7173.1) or (7173.3) or (7173.2) or (7173.5)
Abbreviation: R_DM_dctp_LPAREN_n_RPAREN_ Synonyms: _0 Equation: [n] : dctp --> Confidence Level: IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RETH2 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : 11-cis-retfa + h2o --> Rtotal2 + h + retinol-cis-11 Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
keratan sulfate II (core 2-linked), degradation product 4_C58H95N5O43SX
Abbreviation: R_ENMAN2g Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : g2m8masn + h2o --> glc2man + m7masnA Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# AUTHORS:Roth J, Ziak M, Zuber C.#TITLE:The role of glucosidase II and endomannosidase in glucose trimming of asparagine-linked oligosaccharides.#JOURNAL:Biochimie#YEAR:2003#PUBMED:12770767# GENE ASSOCIATION:
Endomannosidase (glc2man-producing), Golgi apparatus
chondroitin sulfate D (GlcNAc6S-GlcA2S), degradation product 2_C37H53NO44S4
Abbreviation: R_FUMtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: fum[c] + pi[m] <==> fum[m] + pi[c] Confidence Level: 4 TDV genes: LOCUS:_1468#TRANSCRIPT:1#ABBREVIATION:SLC25A10# proteins: NAME:Solute carrier family 25 (dicarboxylate transporter) member 10, mitochondrial #ABBREVIATION:Slc25a10.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# AUTHORS:Passarella S, Atlante A, Barile M, Quagliariello E.#TITLE:Anion transport in rat brain mitochondria: fumarate uptake via the dicarboxylate carrier. #JOURNAL:Neurochem Res.#YEAR:1987#PUBMED:3587497# GENE ASSOCIATION: (1468.1)
Abbreviation: R_DM_yvite_LPAREN_c_RPAREN_ Synonyms: _0 Equation: [c] : yvite --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PROt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + pro-L[e] --> na1[c] + pro-L[c] Confidence Level: 4 JLR genes: LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# GENE ASSOCIATION: (55089.1) or (81539.1) or (54407.1)
Abbreviation: R_GGH_DASH_5THFl Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [l] : 5thf + (4) h2o --> (4) glu-L + thf Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) - last step from 5-glutamyl to monoglutamyl is in one step IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-l# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (5THF), lysosomal
Abbreviation: R_MEOHt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: meoh[e] <==> meoh[c] Confidence Level: 2 genes: none proteins: none citations: none GENE ASSOCIATION:
_1 acyl phosphoglycerol_C6H13O7PFULLRCO2
Abbreviation: R_EX_1glyc_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 1glyc_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GACMTRc Synonyms: _5 EC Number: 2.1.1.2 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : amet + gudac <==> ahcys + creat + h Confidence Level: 4 SAB c genes: LOCUS:_2593#TRANSCRIPT:1#ABBREVIATION:GAMT#ECNUMBERS:2.1.1.2# LOCUS:_2593#TRANSCRIPT:2#ABBREVIATION:GAMT#ECNUMBERS:2.1.1.2# proteins: NAME:guanidinoacetate N-methyltransferase#ABBREVIATION:Gamt.1# NAME:guanidinoacetate N-methyltransferase#ABBREVIATION:Gamt.2# citations: AUTHORS:Pegg AE, Nagarajan S, Naficy S, Ganem B#TITLE:Role of unsaturated derivatives of spermidine as substrates for spermine synthase and in supporting growth of SV-3T3 cells#JOURNAL:Biochem J#YEAR:1991#PUBMED:2001229# GENE ASSOCIATION: (2593.1) or (2593.2)
guanidinoacetate N-methyltransferase (c)
Abbreviation: R_UMPK6n Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : dctp + ump <==> dcdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_VALt5m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: val-L[c] <==> val-L[m] Confidence Level: 4 MM genes: none proteins: none citations: AUTHORS:Porter RK.#TITLE:Mammalian mitochondrial inner membrane cationic and neutral amino acid carriers.#YEAR:2000#PUBMED:11004451# GENE ASSOCIATION:
Valine reversible mitochondrial transport
Abbreviation: R_BUTt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: but[c] + h[c] <==> but[m] + h[m] Confidence Level: 0 NCD genes: LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1 #ABBREVIATION:Slc16a1.1-m# citations: none GENE ASSOCIATION: (6566.1)
Butyrate mitochondrial transport via proton symport, reversible
Abbreviation: R_NDPK8n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + dadp <==> adp + datp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:dADP), nuclear
Abbreviation: R_EX_nadp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : nadp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Nicotinamide adenine dinucleotide phosphate exchange
Abbreviation: R_O2tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: o2[c] <==> o2[m] Confidence Level: 1 IF genes: none proteins: none citations: AUTHORS:Koyama T, Kinjo M, Araiso T.#TITLE:Oxygen diffusion through mitochondrial membranes.#JOURNAL:Adv Exp Med Biol#YEAR:1989#PUBMED:2551143# GENE ASSOCIATION:
Abbreviation: R_SEAHCYSHYD Synonyms: _0 EC Number: 3.3.1.1 SUBSYSTEM: Selenoamino acid metabolism Equation: [c] : h2o + seahcys --> adn + selhcys Confidence Level: 1 SAB genes: LOCUS:_191#TRANSCRIPT:1#ABBREVIATION:AHCY#ECNUMBERS:3.3.1.1# LOCUS:_10768#TRANSCRIPT:1#ABBREVIATION:AHCYL1# LOCUS:_10768#TRANSCRIPT:2#ABBREVIATION:AHCYL1# LOCUS:_23382#TRANSCRIPT:1#ABBREVIATION:AHCYL2# proteins: NAME:S-adenosylhomocysteine hydrolase #ABBREVIATION:Ahcy.1# NAME:S-adenosylhomocysteine hydrolase-like 1 #ABBREVIATION:Ahcyl1.1# NAME:S-adenosylhomocysteine hydrolase-like 1#ABBREVIATION:Ahcyl1.2# NAME:KIAA0828 protein#ABBREVIATION:Kiaa0828.1# citations: none GENE ASSOCIATION: (23382.1) or (191.1) or (10768.1) or (10768.2)
Abbreviation: R_LCADi Synonyms: _0 EC Number: 1.2.1.21 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : h2o + lald-L + nad --> (2) h + lac-L + nadh Confidence Level: 2 JLR- added irreversible form of LCAD genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Ewaschuk JB, Naylor JM, Zello GA#TITLE:D-lactate in human and ruminant metabolism#JOURNAL:J Nutr#YEAR:2005#PUBMED:15987839# AUTHORS:Christopher MM, Eckfeldt JH, Eaton JW.#TITLE:Propylene glycol ingestion causes D-lactic acidosis#JOURNAL:Lab Invest.#YEAR:1990#PUBMED:2296157# AUTHORS:Ray S, Ray M.#TITLE:Oxidation of lactaldehyde by cytosolic aldehyde dehydrogenase and inhibition of cytosolic and mitochondrial aldehyde dehydrogenase by metabolites.#JOURNAL:Biochim Biophys Acta.#YEAR:1984#PUBMED:6487654# GENE ASSOCIATION: (218.1) or (223.1) or (8854.2) or (224.1) or (222.1) or (8854.1) or (221.1) or (216.1) or (8854.3) or (501.1) or (220.1)
Abbreviation: R_FALDH Synonyms: _0 EC Number: 1.2.1.1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : fald + gthrd + nad <==> Sfglutth + h + nadh Confidence Level: 4 NCD genes: LOCUS:_128#TRANSCRIPT:1#ABBREVIATION:ADH5#ECNUMBERS:1.1.1.1# proteins: NAME:alcohol dehydrogenase V#ABBREVIATION:Adh5# citations: AUTHORS:Engeland K, Hoog JO, Holmquist B, Estonius M, Jornvall H, Vallee BL#TITLE:Mutation of Arg-115 of human class III alcohol dehydrogenase: a binding site required for formaldehyde dehydrogenase activity and fatty acid activation#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1993#PUBMED:8460164# AUTHORS:Uotila L, Koivusalo M#TITLE:Purification and properties of S-formylglutathione hydrolase from human liver#JOURNAL:J Biol Chem#YEAR:1974#PUBMED:4436331# GENE ASSOCIATION: (128.1)
Abbreviation: R_MAOX Synonyms: _0 EC Number: 1.4.3.6 SUBSYSTEM: Tyrosine metabolism Equation: [c] : h2o + mma + o2 --> fald + h2o2 + nh4 Confidence Level: 2 SAB genes: LOCUS:_314#TRANSCRIPT:1#ABBREVIATION:AOC2#ECNUMBERS:1.4.3.6# LOCUS:_8639#TRANSCRIPT:1#ABBREVIATION:AOC3#ECNUMBERS:1.4.3.6# LOCUS:_26#TRANSCRIPT:1#ABBREVIATION:ABP1#ECNUMBERS:1.4.3.6# LOCUS:_314#TRANSCRIPT:2#ABBREVIATION:AOC2# proteins: NAME:amine oxidase, copper containing 3#ABBREVIATION:Aoc3.1# NAME:amiloride binding protein 1 (amine oxidase (copper-containing))#ABBREVIATION:Abp1.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.2# citations: AUTHORS:Buffoni F, Ignesti G#TITLE:The copper-containing amine oxidases: biochemical aspects and functional role#JOURNAL:Mol Genet Metab#YEAR:2000#PUBMED:11136547# AUTHORS:Conklin DJ, Cowley HR, Wiechmann RJ, Johnson GH, Trent MB, Boor PJ#TITLE:Vasoactive effects of methylamine in isolated human blood vessels: role of semicarbazide-sensitive amine oxidase, formaldehyde, and hydrogen peroxide#JOURNAL:Am J Physiol Heart Circ Physiol#YEAR:2004#PUBMED:14715500# AUTHORS:Yu PH, Lai CT, Zuo DM#TITLE:Formation of formaldehyde from adrenaline in vivo; a potential risk factor for stress-related angiopathy#JOURNAL:Neurochem Res#YEAR:1997#PUBMED:9131641# GENE ASSOCIATION: (8639.1) or (26.1) or (314.2) or (314.1)
Abbreviation: R_EX_thr_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : thr-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_THRS Synonyms: _0 EC Number: 4.2.3.1 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : h2o + phom --> pi + thr-L Confidence Level: 2 genes: LOCUS:_79896#TRANSCRIPT:1#ABBREVIATION:THNSL1# proteins: NAME:threonine synthase-like 1 #ABBREVIATION:Thnsl1.1# citations: none GENE ASSOCIATION: (79896.1)
Abbreviation: R_M4MPDOL_Lter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: m4mpdol_L[c] --> m4mpdol_L[r] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_GALFUC12GAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: galfuc12gal14acglcgalgluside_hs[e] <==> galfuc12gal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CSPG_Btly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: cspg_b[e] --> cspg_b[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate B transport, extracellular to lysosome
Abbreviation: R_EBASTINEOHtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: ebastineoh[r] <==> ebastineoh[c] Confidence Level: 0 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I, degradation product 29_C96H157N6O80S3
Abbreviation: R_GALASE11ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg29 --> gal + ksi_deg30 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_FPGS9 Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [c] : 10fthf6glu + atp + glu-L --> 10fthf7glu + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Dopamine secretion via secretory vesicle (ATP driven)
Abbreviation: R_DOPAVESSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) atp[c] + (3) dopa[c] + (2) h2o[c] --> (2) adp[c] + (3) dopa[e] + (2) h[c] + (2) pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of dopa from the cell. It leaves out the vesicle because that is not a compartment. ATP:H ratio is assumed as 1:3 for vacuolar ATPase and substrate:h ratio is 1:2. This gives a 3:2 ratio for substrate:ATP genes: LOCUS:_6571#TRANSCRIPT:1#ABBREVIATION:SLC18A2# LOCUS:_6570#TRANSCRIPT:1#ABBREVIATION:SLC18A1# proteins: NAME:solute carrier family 18 (vesicular monoamine), member 1#ABBREVIATION:Slc18a1.1# NAME:solute carrier family 18 (vesicular monoamine), member 2#ABBREVIATION:Slc18a2.1# citations: AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# AUTHORS:Eiden LE, Schafer MK, Weihe E, Schutz B#TITLE:The vesicular amine transporter family (SLC18): amine/proton antiporters required for vesicular accumulation and regulated exocytotic secretion of monoamines and acetylcholine#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12827358# GENE ASSOCIATION: (6570.1) or (6571.1)
Abbreviation: R_RDH2 Synonyms: _0 EC Number: 1.1.1.105 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : nad + retinol-9-cis <==> h + nadh + retinal-cis-9 Confidence Level: 4 IT genes: LOCUS:_5959#TRANSCRIPT:1#ABBREVIATION:RDH5#ECNUMBERS:1.1.1.105# proteins: NAME:retinol dehydrogenase 5 (11-cis and 9-cis) #ABBREVIATION:Rdh5.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Haeseleer F, Jang GF, Imanishi Y, Driessen CA, Matsumura M, Nelson PS, Palczewski K.#TITLE:Dual-substrate specificity short chain retinol dehydrogenases from the vertebrate retina.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12226107# AUTHORS:Simon A, Hellman U, Wernstedt C, Eriksson U. #TITLE:The retinal pigment epithelial-specific 11-cis retinol dehydrogenase belongs to the family of short chain alcohol dehydrogenases. #JOURNAL:J Biol Chem.#YEAR:1995#PUBMED:7836368# AUTHORS:Mertz JR, Shang E, Piantedosi R, Wei S, Wolgemuth DJ, Blaner WS. #TITLE:Identification and characterization of a stereospecific human enzyme that catalyzes 9-cis-retinol oxidation. A possible role in 9-cis-retinoic acid formation.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9115228# GENE ASSOCIATION: (5959.1)
heparan sulfate, degradation product 18_C43H66N2O45S3
Abbreviation: R_EX_cys_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cys-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
nicotinamide-nucleotide adenylyltransferase, mitochondrial
Abbreviation: R_NMNATm Synonyms: _0 EC Number: 2.7.7.1 SUBSYSTEM: NAD Metabolism Equation: [m] : atp + h + nmn --> nad + ppi Confidence Level: 4 NCD genes: LOCUS:_349565#TRANSCRIPT:1#ABBREVIATION:NMNAT3# proteins: NAME:nicotinamide nucleotide adenylyltransferase 3 #ABBREVIATION:Nmnat3.1-m# citations: AUTHORS:Zhang X, Kurnasov OV, Karthikeyan S, Grishin NV, Osterman AL, Zhang H. #TITLE:Structural characterization of a human cytosolic NMN/NaMN adenylyltransferase and implication in human NAD biosynthesis.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:12574164# GENE ASSOCIATION: (349565.1)
Abbreviation: R_CO2tp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: co2[c] <==> co2[x] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GALt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gal[e] + na1[e] <==> gal[c] + na1[c] Confidence Level: 4 NCD genes: LOCUS:_200010#TRANSCRIPT:1#ABBREVIATION:SLC5A9# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 9, extracellular#ABBREVIATION:Slc5a9.1-e# citations: AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# AUTHORS:Tazawa S, Yamato T, Fujikura H, Hiratochi M, Itoh F, Tomae M, Takemura Y, Maruyama H, Sugiyama T, Wakamatsu A, Isogai T, Isaji M#TITLE:SLC5A9/SGLT4, a new Na+-dependent glucose transporter, is an essential transporter for mannose, 1,5-anhydro-D-glucitol, and fructose#JOURNAL:Life Sci#YEAR:2005#PUBMED:15607332# GENE ASSOCIATION: (200010.1)
Abbreviation: R_UGT1A7r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Steroid Metabolism Equation: [r] : ahandrostan + udpglcur --> ahandrostanglc + udp Confidence Level: 4 NJ genes: LOCUS:_54575#TRANSCRIPT:1#ABBREVIATION:UGT1A10# LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A10#ABBREVIATION:Ugt1a10.1# NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# citations: AUTHORS:Basu NK, Kubota S, Meselhy MR, Ciotti M, Chowdhury B, Hartori M, Owens IS.#TITLE:Free Full Text Gastrointestinally distributed UDP-glucuronosyltransferase 1A10, which metabolizes estrogens and nonsteroidal anti-inflammatory drugs, depends upon phosphorylation.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117964# AUTHORS:Mackenzie PI, Owens IS, Burchell B, Bock KW, Bairoch A, Belanger A, Fournel-Gigleux S, Green M, Hum DW, Iyanagi T, Lancet D, Louisot P, Magdalou J, Chowdhury JR, Ritter JK, Schachter H, Tephly TR, Tipton KF, Nebert DW.#TITLE:The UDP glycosyltransferase gene superfamily: recommended nomenclature update based on evolutionary divergence.#JOURNAL:Pharmacogenetics#YEAR:1997#PUBMED:9295054# GENE ASSOCIATION: (54575.1) or (54576.1)
Abbreviation: R_EX_25hvitd3_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 25hvitd3 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_13DAMPPOX Synonyms: _0 EC Number: 1.4.3.6 SUBSYSTEM: beta-Alanine metabolism Equation: [c] : 13dampp + h2o + o2 --> bamppald + h2o2 + nh4 Confidence Level: 1 SAB genes: LOCUS:_314#TRANSCRIPT:1#ABBREVIATION:AOC2#ECNUMBERS:1.4.3.6# LOCUS:_8639#TRANSCRIPT:1#ABBREVIATION:AOC3#ECNUMBERS:1.4.3.6# LOCUS:_26#TRANSCRIPT:1#ABBREVIATION:ABP1#ECNUMBERS:1.4.3.6# LOCUS:_314#TRANSCRIPT:2#ABBREVIATION:AOC2# proteins: NAME:amine oxidase, copper containing 3#ABBREVIATION:Aoc3.1# NAME:amiloride binding protein 1 (amine oxidase (copper-containing))#ABBREVIATION:Abp1.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.2# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# GENE ASSOCIATION: (8639.1) or (26.1) or (314.2) or (314.1)
_1,3-Diaminopropane:oxygen oxidoreductase (deaminating)
Abbreviation: R_NDPK7 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + dcdp <==> adp + dctp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.2) or (10201.1) or (4831.1 and 4830.1) or (4832.1) or (4831.1 and 4830.2) or (29922.1)
nucleoside-diphosphate kinase (ATP:dCDP)
Abbreviation: R_NTD2m Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [m] : h2o + ump --> pi + uri Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_56953#TRANSCRIPT:1#ABBREVIATION:NT5M#ECNUMBERS:3.1.3.5# proteins: NAME:_5',3'-nucleotidase, mitochondrial #ABBREVIATION:Nt5M.1-m# citations: AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION: (56953.1)
Abbreviation: R_TRIODTHYt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: triodthy[e] <==> triodthy[c] Confidence Level: 4 NJ genes: LOCUS:_6567#TRANSCRIPT:1#ABBREVIATION:SLC16A2# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 2#ABBREVIATION:Slc16a2.1-e# citations: AUTHORS:Friesema EC, Ganguly S, Abdalla A, Manning Fox JE, Halestrap AP, Visser TJ#TITLE:Identification of monocarboxylate transporter 8 as a specific thyroid hormone transporter#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12871948# AUTHORS:Lafreniere RG, Carrel L, Willard HF.#TITLE:A novel transmembrane transporter encoded by the XPCT gene in Xq13.2.#JOURNAL:Hum Mol Genet#YEAR:1994#PUBMED:7981683# GENE ASSOCIATION: (6567.1)
Abbreviation: R_ACGAMK Synonyms: _0 EC Number: 2.7.1.59 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acgam + atp --> acgam6p + adp + h Confidence Level: 4 genes: LOCUS:_55577#TRANSCRIPT:1#ABBREVIATION:NAGK#ECNUMBERS:2.7.1.59# proteins: NAME:N-acetylglucosamine kinase#ABBREVIATION:Nagk.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Hinderlich S, Berger M, Schwarzkopf M, Effertz K, Reutter W#TITLE:Molecular cloning and characterization of murine and human N-acetylglucosamine kinase#JOURNAL:Eur J Biochem#YEAR:2000#PUBMED:10824116# GENE ASSOCIATION: (55577.1)
Abbreviation: R_T2M26DCOAHLx Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: Limonene and pinene degradation Equation: [x] : h2o + t2m26dcoa <==> 3h26dm5coa Confidence Level: 1 SAB genes: LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_1891#TRANSCRIPT:1#ABBREVIATION:ECH1# proteins: NAME:enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase (x)#ABBREVIATION:Ehhadh.1-x# NAME:enoyl Coenzyme A hydratase 1, peroxisomal #ABBREVIATION:Ech1.1-x# citations: none GENE ASSOCIATION: (1891.1) or (1962.1)
trans-2-Methyl-5-isopropylhexa-2,5-dienoyl-CoA hydro-lyase (x)
Abbreviation: R_TTDCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + ttdcrn --> crn + tdcoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_DOLK_U Synonyms: _0 EC Number: 2.7.1.108 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : ctp + (0.1) dolichol_U --> cdp + (0.1) dolp_U + h Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LNLNCGCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + lnlncgcoa --> coa + lnlncgcrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Protein N6,N6,N6-trimethyl-L-lysine_C3H10X
Abbreviation: R_LYSMTF3n Synonyms: _7 EC Number: 2.1.1.43 SUBSYSTEM: Lysine Metabolism Equation: [n] : Ndmelys + amet --> Ntmelys + ahcys Confidence Level: 4 MM genes: LOCUS:_79723#TRANSCRIPT:1#ABBREVIATION:SUV39H2# LOCUS:_10919#TRANSCRIPT:1#ABBREVIATION:BAT8# LOCUS:_9869#TRANSCRIPT:1#ABBREVIATION:SETDB1# LOCUS:_6839#TRANSCRIPT:1#ABBREVIATION:SUV39H1# LOCUS:_83852#TRANSCRIPT:1#ABBREVIATION:SETDB2# LOCUS:_58508#TRANSCRIPT:1#ABBREVIATION:MLL3# LOCUS:_58508#TRANSCRIPT:2#ABBREVIATION:MLL3# LOCUS:_10919#TRANSCRIPT:2#ABBREVIATION:BAT8# LOCUS:_84444#TRANSCRIPT:1#ABBREVIATION:DOT1L# LOCUS:_80854#TRANSCRIPT:1#ABBREVIATION:SET7# LOCUS:_79813#TRANSCRIPT:1#ABBREVIATION:EHMT1# LOCUS:_387893#TRANSCRIPT:1#ABBREVIATION:SET8# proteins: NAME:suppressor of variegation 3-9 homolog 1 (Drosophila), nuclear #ABBREVIATION:Suv39h1.1-n# NAME:SET domain, bifurcated 1, nuclear #ABBREVIATION:Setdb1.1-n# NAME:myeloid/lymphoid or mixed-lineage leukemia 3, nuclear#ABBREVIATION:Mll3.1-n# NAME:myeloid/lymphoid or mixed-lineage leukemia 3, nuclear#ABBREVIATION:Mll3.2-n# NAME:DOT1-like, histone H3 methyltransferase (S. cerevisiae), nuclear #ABBREVIATION:Dot1l.1-n# NAME:SET domain-containing protein 7, nuclear #ABBREVIATION:Set7.1-n# NAME:HLA-B associated transcript 8, nuclear#ABBREVIATION:Bat8.1-n# NAME:HLA-B associated transcript 8, nuclear#ABBREVIATION:Bat8.2-n# NAME:SET domain, bifurcated 2, nuclear #ABBREVIATION:Setdb2.1-n# NAME:suppressor of variegation 3-9 homolog 2 (Drosophila), nuclear #ABBREVIATION:Suv39h2.1-n# NAME:euchromatic histone methyltransferase 1, nuclear #ABBREVIATION:Ehmt1.1-n# NAME:PR/SET domain containing protein 8, nuclear #ABBREVIATION:Set8.1-n# citations: none GENE ASSOCIATION: (387893.1) or (58508.2) or (83852.1) or (80854.1) or (9869.1) or (84444.1) or (10919.2) or (6839.1) or (10919.1) or (79723.1) or (58508.1) or (79813.1)
histone-lysine N-methyltransferase, nuclear
Abbreviation: R_ACt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ac[e] + h[e] <==> ac[c] + h[c] Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Colleen Smith, Allan Marks, Michael Lieberman#TITLE:Marks' Basic Medical Biochemistry#YEAR:2004# AUTHORS:Waniewski RA, Martin DL.#TITLE:Preferential utilization of acetate by astrocytes is attributable to transport.#JOURNAL: J Neurosci#YEAR:1998#PUBMED:9651205# GENE ASSOCIATION:
acetate reversible transport via proton symport
Abbreviation: R_FTHFCL Synonyms: _0 EC Number: 6.3.3.2 SUBSYSTEM: Folate Metabolism Equation: [c] : 5fthf + atp --> adp + methf + pi Confidence Level: 4 NCD genes: LOCUS:_10588#TRANSCRIPT:1#ABBREVIATION:MTHFS#ECNUMBERS:6.3.3.2# proteins: NAME:_5,10-methenyltetrahydrofolate synthetase (5-formyltetrahydrofolate cyclo-ligase) #ABBREVIATION:Mthfs1.1# citations: AUTHORS:Anguera MC, Suh JR, Ghandour H, Nasrallah IM, Selhub J, Stover PJ.#TITLE:Methenyltetrahydrofolate synthetase regulates folate turnover and accumulation.#JOURNAL: J Biol Chem#YEAR:2003#PUBMED:12764149# GENE ASSOCIATION: (10588.1)
_5-formethyltetrahydrofolate cyclo-ligase
Abbreviation: R_5HXKYNDCL Synonyms: _0 EC Number: 4.1.1.28 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 5hxkyn + h --> 5hxkynam + co2 Confidence Level: 1 SAB genes: LOCUS:_1644#TRANSCRIPT:1#ABBREVIATION:DDC#ECNUMBERS:4.1.1.28# proteins: NAME:aromatic-L-amino-acid decarboxylase#ABBREVIATION:Ddc.1# citations: none GENE ASSOCIATION: (1644.1)
Abbreviation: R_SELNPS Synonyms: _0 EC Number: 2.7.9.3 SUBSYSTEM: Selenoamino acid metabolism Equation: [c] : atp + h2o + seln --> amp + pi + selnp Confidence Level: 4 JLR genes: LOCUS:_22928#TRANSCRIPT:1#ABBREVIATION:SEPHS2# LOCUS:_22929#TRANSCRIPT:1#ABBREVIATION:SEPHS1# proteins: NAME:selenophosphate synthetase 1#ABBREVIATION:Sephs1.1# NAME:selenophosphate synthetase 2#ABBREVIATION:Sephs2.1# citations: AUTHORS:Stadtman TC#TITLE:Selenocysteine#JOURNAL:Annu Rev Biochem#YEAR:1996#PUBMED:8811175# AUTHORS:Esaki N, Nakamura T, Tanaka H, Soda K#TITLE:Selenocysteine lyase, a novel enzyme that specifically acts on selenocysteine. Mammalian distribution and purification and properties of pig liver enzyme#JOURNAL:J Biol Chem#YEAR:1982#PUBMED:6461656# GENE ASSOCIATION: (22929.1) or (22928.1)
beta-Aminopropion aldehyde:NAD+ oxidoreductase (m)
Abbreviation: R_BAMPPALDOXm Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: beta-Alanine metabolism Equation: [m] : bamppald + h2o + nad --> ala-B + (2) h + nadh Confidence Level: 1 SAB genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: none GENE ASSOCIATION: (8659.2) or (217.1) or (8659.1) or (219.1)
Abbreviation: R_EX_estriolglc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : estriolglc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
heparan sulfate, degradation product 3_C81H117N5O97S10
Abbreviation: R_ACYP Synonyms: _0 EC Number: 3.6.1.7 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : 13dpg + h2o --> 3pg + h + pi Confidence Level: 2 Reaction from KEGG: an acylphosphate + H2O -> a carboxylate + phosphate genes: LOCUS:_97#TRANSCRIPT:1#ABBREVIATION:ACYP1#ECNUMBERS:3.6.1.7# LOCUS:_98#TRANSCRIPT:1#ABBREVIATION:ACYP2#ECNUMBERS:3.6.1.7# LOCUS:_112611#TRANSCRIPT:1#ABBREVIATION:RWDD2# LOCUS:_97#TRANSCRIPT:2#ABBREVIATION:ACYP1#ECNUMBERS:3.6.1.7# proteins: NAME:acylphosphatase 2, muscle type (muscle)#ABBREVIATION:Acyp2.1# NAME:acylphosphatase 1, erythrocyte (common) type (erythrocyte)#ABBREVIATION:Acyp1.1# NAME:ayclphosphatase 1, erythrocyte (common) type (erythrocyte)#ABBREVIATION:Acyp1.2# NAME:RWD domain containing 2#ABBREVIATION:Rwdd2.1# citations: AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# GENE ASSOCIATION: (112611.1) or (98.1) or (97.2) or (97.1)
phosphatidyl-CMP: glycerophosphate phosphatidyltransferase
Abbreviation: R_PGPPT Synonyms: _0 EC Number: 2.7.8.11 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : cdpdag_hs + glyc3p --> cmp + h + pgp_hs Confidence Level: 2 NJ genes: LOCUS:_9489#TRANSCRIPT:1#ABBREVIATION:PGS1#ECNUMBERS:2.7.8.5# proteins: NAME:phosphatidylglycerophosphate synthase#ABBREVIATION:Pgs1.1# citations: AUTHORS:Schlame M, Rua D, Greenberg ML.#TITLE:The biosynthesis and functional role of cardiolipin.#JOURNAL:Prog Lipid Res.#YEAR:2000#PUBMED:10799718# GENE ASSOCIATION: (9489.1)
Abbreviation: R_25HVITD3t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _25hvitd3[c] --> 25hvitd3[e] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_25-hydroxyvitamin D3 transport from cytoplasm
Abbreviation: R_GLCt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glc-D[e] + na1[e] <==> glc-D[c] + na1[c] Confidence Level: 4 NCD genes: LOCUS:_6524#TRANSCRIPT:1#ABBREVIATION:SLC5A2# LOCUS:_159963#TRANSCRIPT:1#ABBREVIATION:SLC5A12# LOCUS:_6526#TRANSCRIPT:1#ABBREVIATION:SLC5A3# LOCUS:_200010#TRANSCRIPT:1#ABBREVIATION:SLC5A9# LOCUS:_125206#TRANSCRIPT:1#ABBREVIATION:SLC5A10# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 2, extracellular#ABBREVIATION:Slc5a2.1-e# NAME:solute carrier family 5 (inositol transporters), member 3, extracellular#ABBREVIATION:Slc5a3.1-e# NAME:solute carrier family 5 (sodium/glucose cotransporter), member 9, extracellular#ABBREVIATION:Slc5a9.1-e# NAME:SLC5A10 solute carrier family 5 (sodium/glucose cotransporter), member 10#ABBREVIATION:Slc5a10.1-e# NAME:solute carrier family 5 (sodium/glucose cotransporter), member 12#ABBREVIATION:Slc5a12.1-e# citations: AUTHORS:Kanai Y, Lee WS, You G, Brown D, Hediger MA#TITLE:The human kidney low affinity Na+/glucose cotransporter SGLT2. Delineation of the major renal reabsorptive mechanism for D-glucose#JOURNAL:J Clin Invest#YEAR:1994#PUBMED:8282810# AUTHORS:Wells RG, Pajor AM, Kanai Y, Turk E, Wright EM, Hediger MA#TITLE:Cloning of a human kidney cDNA with similarity to the sodium-glucose cotransporter#JOURNAL:Am J Physiol#YEAR:1992#PUBMED:1415574# AUTHORS:Wright EM#TITLE:Renal Na(+)-glucose cotransporters#JOURNAL:Am J Physiol Renal Physiol#YEAR:2001#PUBMED:11133510# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# AUTHORS:van den Heuvel LP, Assink K, Willemsen M, Monnens L#TITLE:Autosomal recessive renal glucosuria attributable to a mutation in the sodium glucose cotransporter (SGLT2).#JOURNAL:Hum Genet#YEAR:2002#PUBMED:12436245# AUTHORS:Kwon HM, Yamauchi A, Uchida S, Preston AS, Garcia-Perez A, Burg MB, Handler JS#TITLE:Cloning of the cDNa for a Na+/myo-inositol cotransporter, a hypertonicity stress protein#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1372904# AUTHORS:Berry GT, Mallee JJ, Kwon HM, Rim JS, Mulla WR, Muenke M, Spinner NB#TITLE:The human osmoregulatory Na+/myo-inositol cotransporter gene (SLC5A3): molecular cloning and localization to chromosome 21#JOURNAL:Genomics#YEAR:1995#PUBMED:7789985# AUTHORS:Hager K, Hazama A, Kwon HM, Loo DD, Handler JS, Wright EM#TITLE:Kinetics and specificity of the renal Na+/myo-inositol cotransporter expressed in Xenopus oocytes#JOURNAL:J Membr Biol#YEAR:1995#PUBMED:7537337# AUTHORS:Wright EM, Loo DD, Hirayama BA, Turk E#TITLE:Surprising versatility of Na+-glucose cotransporters: SLC5#JOURNAL:Physiology (Bethesda)#YEAR:2004#PUBMED:15546855# GENE ASSOCIATION: (6526.1) or (159963.1) or (6524.1) or (125206.1) or (200010.1)
Abbreviation: R_EX_arachd_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : arachd <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_O16G2e Synonyms: _0 EC Number: 3.2.1.10 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [e] : h2o + strch2 --> glc-D + malt Confidence Level: 0 NCD genes: LOCUS:_6476#TRANSCRIPT:1#ABBREVIATION:SI#ECNUMBERS:3.2.1.48,3.2.1.10# proteins: NAME:sucrase-isomaltase (alpha-glucosidase), extracellular (sm intestine)#ABBREVIATION:Si.1-e# citations: none GENE ASSOCIATION: (6476.1)
oligo-1,6-glucosidase (strch2 ->strch3), extracellular
Abbreviation: R_DKMPPD Synonyms: _0 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : dkmpp + h2o + o2 --> 2kmb + for + (2) h + pi Confidence Level: 2 JLR- based on B. subtilis and K. pneumoniae reactions (not sure about the h2o and h) genes: none proteins: none citations: AUTHORS:Myers RW, Abeles RH#TITLE:Conversion of 5-S-ethyl-5-thio-D-ribose to ethionine in Klebsiella pneumoniae. Basis for the selective toxicity of 5-S-ethyl-5-thio-D-ribose#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2543672# GENE ASSOCIATION:
_2,3-diketo-5-methylthio-1-phosphopentane degradation reaction
Abbreviation: R_EX_11_DASH_cis_DASH_retfa_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 11-cis-retfa <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DM_dttp_LPAREN_m_RPAREN_ Synonyms: _0 Equation: [m] : dttp --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DHPM2 Synonyms: _0 EC Number: 3.5.2.2 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : 56dthm + h2o --> 3uib + h Confidence Level: 2 Also acts on dihydrothymine and hydantoin genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION:
Abbreviation: R_ACtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: ac[c] <==> ac[g] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Wolfe AJ.#TITLE:The acetate switch#JOURNAL:Microbiol Mol Biol Rev#YEAR:2005#PUBMED:15755952# GENE ASSOCIATION:
Abbreviation: R_LNELDCCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + lneldccoa --> coa + lneldccrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_CYTK7n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : cmp + utp <==> cdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_DM_13_DASH_cis_DASH_retn_LPAREN_n_RPAREN_ Synonyms: _0 Equation: [n] : 13-cis-retn --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PIK3n Synonyms: _4 EC Number: 2.7.1.137 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + pail_hs --> adp + h + pail3p_hs Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
_5alpha-Dihydrotestosterone sulfate_C19H29O5S
Abbreviation: R_5ADTSTSTERONESULT Synonyms: _0 EC Number: 2.8.2.2 SUBSYSTEM: Steroid Metabolism Equation: [c] : 5adtststerone + paps --> 5adtststerones + h + pap Confidence Level: 4 NJ genes: LOCUS:_6822#TRANSCRIPT:1#ABBREVIATION:SULT2A1#ECNUMBERS:2.8.2.2# proteins: NAME:sulfotransferase family, cytosolic, 2A, dehydroepiandrosterone#ABBREVIATION:Sult2a1.1# citations: AUTHORS:Otterness DM, Wieben ED, Wood TC, Watson WG, Madden BJ, McCormick DJ, Weinshilboum RM.#TITLE:Human liver dehydroepiandrosterone sulfotransferase: molecular cloning and expression of cDNA.#JOURNAL:Mol Pharmacol#YEAR:1992#PUBMED:1588921# AUTHORS:Chatterjee B, Echchgadda I, Song CS#TITLE:Vitamin D receptor regulation of the steroid/bile acid sulfotransferase SULT2A1#JOURNAL:Methods Enzymol#YEAR:2005#PUBMED:16399349# GENE ASSOCIATION: (6822.1)
_5alpha-Dihydrotestosterone sulfotransferase
Abbreviation: R_PGDI Synonyms: _8 EC Number: 5.3.99.2 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : prostgh2 <==> prostgd2 Confidence Level: 4 noted on RER, but also cytoplasmic structures NJ genes: LOCUS:_5730#TRANSCRIPT:1#ABBREVIATION:PTGDS#ECNUMBERS:5.3.99.2# LOCUS:_27306#TRANSCRIPT:1#ABBREVIATION:PGDS#ECNUMBERS:5.3.99.2# proteins: NAME:prostaglandin D2 synthase#ABBREVIATION:Ptgds.1# NAME:prostaglandin D2 synthase, hematopoietic#ABBREVIATION:Pgds.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Yamashima T, Sakuda K, Tohma Y, Yamashita J, Oda H, Irikura D, Eguchi N, Beuckmann CT, Kanaoka Y, Urade Y, Hayaishi O#TITLE:Free Full Text Prostaglandin D synthase (beta-trace) in human arachnoid and meningioma cells: roles as a cell marker or in cerebrospinal fluid absorption, tumorigenesis, and calcification process.#JOURNAL:J Neurosci#YEAR:1997#PUBMED:9065498# AUTHORS:Mahmud I, Ueda N, Yamaguchi H, Yamashita R, Yamamoto S, Kanaoka Y, Urade Y, Hayaishi O.#TITLE:Prostaglandin D synthase in human megakaryoblastic cells.#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9353279# GENE ASSOCIATION: (5730.1) or (27306.1)
Prostaglandin-H2 D-isomerase [Precursor]
Abbreviation: R_EX_o2s_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : o2s <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
cytochrome P450, family 7, subfamily A, polypeptide 1
Abbreviation: R_P4507A1r Synonyms: _7 EC Number: 1.14.13.17 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : chsterol + h + nadph + o2 --> h2o + nadp + xol7a Confidence Level: 4 genes: LOCUS:_1581#TRANSCRIPT:1#ABBREVIATION:CYP7A1#ECNUMBERS:1.14.13.17# proteins: NAME:cytochrome P450, family 7, subfamily A, polypeptide 1#ABBREVIATION:Cyp7a1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1581.1)
Abbreviation: R_EX_bilirub_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : bilirub <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TXA2tr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: txa2[c] <==> txa2[r] Confidence Level: 2 unknown mechanism for transport - known to be secreted from cells - unknown mechanism or pathway, TXA2tr -> TXA2te to get to EC space NJ genes: none proteins: none citations: TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# GENE ASSOCIATION:
Beta-galactoside alpha-2,3-sialyltransferase, Golgi apparatus
galactosylgloboside (homo sapiens)_C50H89N2O27FULLRCO
Abbreviation: R_SIAT4Bg Synonyms: _8 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + galgbside_hs --> acngalgbside_hs + cmp + h Confidence Level: 4 NJ genes: LOCUS:_6483#TRANSCRIPT:1#ABBREVIATION:SIAT4B#ECNUMBERS:2.4.99.4# proteins: NAME:sialyltransferase 4B, Golgi apparatus#ABBREVIATION:Siat4b.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Giordanengo V, Bannwarth S, Laffont C, Van Miegem V, Harduin-Lepers A, Delannoy P, Lefebvre JC. #TITLE:Cloning and expression of cDNA for a human Gal(beta1-3)GalNAc alpha2,3-sialyltransferase from the CEM T-cell line. #JOURNAL:Eur J Biochem#YEAR:1997#PUBMED:9266697# GENE ASSOCIATION: (6483.1)
Abbreviation: R_GGH_DASH_6DHFe Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [e] : 6dhf + h2o --> 5dhf + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-e# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (6DHF), extracellular
Abbreviation: R_NTD10 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [c] : h2o + xmp --> pi + xtsn Confidence Level: 2 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_22978#TRANSCRIPT:1#ABBREVIATION:NT5C2# proteins: NAME:_5'-nucleotidase, cytosolic II #ABBREVIATION:Nt5C2.1# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# GENE ASSOCIATION: (22978.1)
Abbreviation: R_CORE2GTg Synonyms: _8 EC Number: 2.4.1.102 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : T_antigen + uacgam --> core2 + h + udp Confidence Level: 0 NCD genes: LOCUS:_2650#TRANSCRIPT:1#ABBREVIATION:GCNT1#ECNUMBERS:2.4.1.102# LOCUS:_9245#TRANSCRIPT:1#ABBREVIATION:GCNT3# LOCUS:_51301#TRANSCRIPT:1#ABBREVIATION:C2GNT3# proteins: NAME:glucosaminyl (N-acetyl) transferase 1, core 2 (beta-1,6-N-acetylglucosaminyltransferase), Golgi apparatus (ubiquitous)#ABBREVIATION:Gcnt1.1-g# NAME:glucosaminyl (N-acetyl) transferase 3, mucin type, Golgi apparatus (colon, sm intestine, stomach, thyroid, testis, prostate, kidney, pancreas)#ABBREVIATION:Gcnt3.1-g# NAME:Core 2 beta 1,6,N-acetylglucosaminyl transferase 3, Golgi appratus (thymus)#ABBREVIATION:C2gnt3.1g# citations: AUTHORS:Schwientek T, Yeh JC, Levery SB, Keck B, Merkx G, van Kessel AG, Fukuda M, Clausen H#TITLE:Control of O-glycan branch formation. Molecular cloning and characterization of a novel thymus-associated core 2 beta1, 6-n-acetylglucosaminyltransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10753916# AUTHORS:Yeh JC, Ong E, Fukuda M#TITLE:Free Full Text Molecular cloning and expression of a novel beta-1, 6-N-acetylglucosaminyltransferase that forms core 2, core 4, and I branches#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9915862# GENE ASSOCIATION: (2650.1) or (51301.1) or (9245.1)
Core 2 acetylglucosaminyltransferase, Golgi apparatus
Abbreviation: R_4MTOLBUTAMIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _4mtolbutamide[e] <==> 4mtolbutamide[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FMNAT Synonyms: _0 EC Number: 2.7.7.2 SUBSYSTEM: Riboflavin Metabolism Equation: [c] : atp + fmn + h --> fad + ppi Confidence Level: 2 genes: LOCUS:_80308#TRANSCRIPT:1#ABBREVIATION:FADS#ECNUMBERS:2.7.7.2# LOCUS:_80308#TRANSCRIPT:2#ABBREVIATION:FADS#ECNUMBERS:2.7.7.2# proteins: NAME:FAD-synthetase#ABBREVIATION:FadS.1# NAME:FAD-synthetase#ABBREVIATION:FadS.2# citations: none GENE ASSOCIATION: (80308.1) or (80308.2)
Abbreviation: R_DUMPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: dump[c] <==> dump[n] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
(phosphoethanolaminyl-dimannosyl),(phosphoethanolaminyl)-mannosyl-glucosaminyl-acylphosphatidylinositol (H7)_C53H100N3O35P3FULLRCO2FULLR2CO2
({[(mannosyl),(phosphoethanolaminyl)]-dimannosyl},{phosphoethanolaminyl})-mannosyl-glucosaminyl-acylphosphatidylinositol (M4B)_C59H110N3O40P3FULLRCO2FULLR2CO2
H7 mannosyltransferase, endoplasmic reticulum
Abbreviation: R_H7MTer_L Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_L + em2emgacpail_hs --> (0.1) dolp_L + h + mem2emgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_80235#TRANSCRIPT:1#ABBREVIATION:SMP3# proteins: NAME:SMP3 mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Smp3.1-r# citations: AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (80235.1)
chondroitin sulfate E (GalNAc4,6diS-GlcA), degradation product 2_C45H66N2O49S4
Abbreviation: R_ST6GALNAC21 Synonyms: _5 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : acngalgbside_hs + cmpacna --> acn13acngalgbside_hs + cmp + h Confidence Level: 2 NJ genes: LOCUS:_10610#TRANSCRIPT:1#ABBREVIATION:SIAT7B#ECNUMBERS:2.4.99.7# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 2#ABBREVIATION:St6galnac2.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10610.1)
phosphatidylinositol-5-phosphate 4-kinase, nuclear
Abbreviation: R_PI5P4Kn Synonyms: _0 EC Number: 2.7.1.149 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + pail5p_hs --> adp + h + pail45p_hs Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
Abbreviation: R_PROSTGE1t3 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + prostge1[c] --> adp[c] + h[c] + pi[c] + prostge1[e] Confidence Level: 4 IT genes: LOCUS:_10257#TRANSCRIPT:1#ABBREVIATION:ABCC4# proteins: NAME:ATP-binding cassette, sub-family C (CFTR/MRP), member 4 #ABBREVIATION:Abcc4.1-e# citations: AUTHORS:Chen ZS, Lee K, Kruh GD.#TITLE:Transport of cyclic nucleotides and estradiol 17-beta-D-glucuronide by multidrug resistance protein 4. Resistance to 6-mercaptopurine and 6-thioguanine.#JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11447229# AUTHORS:Reid G, Wielinga P, Zelcer N, van der Heijden I, Kuil A, de Haas M, Wijnholds J, Borst P. #TITLE:The human multidrug resistance protein MRP4 functions as a prostaglandin efflux transporter and is inhibited by nonsteroidal antiinflammatory drugs.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2003#PUBMED:12835412# AUTHORS:Wielinga PR, van der Heijden I, Reid G, Beijnen JH, Wijnholds J, Borst P. #TITLE:Characterization of the MRP4- and MRP5-mediated transport of cyclic nucleotides from intact cells. #JOURNAL: J Biol Chem. #YEAR:2003#PUBMED:12637526# GENE ASSOCIATION: (10257.1)
prostaglandine E1 transport (ATP-dependent)
Abbreviation: R_G14T12g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre20 + udpgal --> h + ksi_pre21 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
keratan sulfate I biosynthesis, precursor 20_C171H274N12O140S6X
Abbreviation: R_G3M8MASNterg Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: g3m8masn[r] --> g3m8masn[g] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# GENE ASSOCIATION:
g3m8masn transport from endoplasmic reticulum to Golgi apparatus
Abbreviation: R_CRNtim Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: crn[m] --> crn[c] Confidence Level: 4 NCD genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (788.1)
L-carnitine transport out of mitochondria via diffusion
_1D-myo-Inositol 1,4,5-trisphosphate_C6H9O15P3
Abbreviation: R_DOGULND2 Synonyms: _0 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : 23doguln + h2o --> co2 + xylnt Confidence Level: 2 EC-4.1.1.- NCD genes: none proteins: none citations: AUTHORS:Banhegyi G, Braun L, Csala M, Puskas F, Mandl J#TITLE:Ascorbate metabolism and its regulation in animals#JOURNAL:Free Radic Biol Med#YEAR:1997#PUBMED:9296457# GENE ASSOCIATION:
_2,3-dioxo-L-gulonate decarboxylase (L-xylonate-forming)
Abbreviation: R_PPNCL3 Synonyms: _0 EC Number: 6.3.2.5 SUBSYSTEM: CoA Biosynthesis Equation: [c] : 4ppan + atp + cys-L --> 4ppcys + amp + h + ppi Confidence Level: 4 _01-24-05 based on Manoj et al, 2003, 11(8),Structure, 927-936, that found ATP and not CTP for the human phosphopantothenate-cysteine ligase IT genes: LOCUS:_79717#TRANSCRIPT:1#ABBREVIATION:PPCS#ECNUMBERS:6.3.2.5# proteins: NAME:Phosphopantothenate-cysteine ligase#ABBREVIATION:Dfp2.1# citations: AUTHORS:Daugherty M, Polanuyer B, Farrell M, Scholle M, Lykidis A, de Crecy-Lagard V, Osterman A. #TITLE:Complete reconstitution of the human coenzyme A biosynthetic pathway via comparative genomics.#JOURNAL:J Biol Chem.#YEAR:2002#PUBMED:11923312# AUTHORS:Manoj N, Strauss E, Begley TP, Ealick SE. #TITLE:Structure of human phosphopantothenoylcysteine synthetase at 2.3 A resolution.#JOURNAL:Structure (Camb).#YEAR:2003#PUBMED:12906824# GENE ASSOCIATION: (79717.1)
Abbreviation: R_ARTFR44 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (2) fadh2[m] + lneldccoa[c] --> (1.125) R4coa_hs[c] + (2) fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_VITD3tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: vitd3[m] --> vitd3[c] Confidence Level: 2 I am not sure how vitamin D3 is transported in cytosol based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_PIt7 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) na1[e] + pi[e] <==> (3) na1[c] + pi[c] Confidence Level: 4 SMP genes: LOCUS:_6569#TRANSCRIPT:1#ABBREVIATION:SLC34A1# LOCUS:_10568#TRANSCRIPT:1#ABBREVIATION:SLC34A2# proteins: NAME:solute carrier family 34 (sodium phosphate), member 1, extracellular #ABBREVIATION:Slc34a1.1-e# NAME:solute carrier family 34 (sodium phosphate), member 2, extracellular #ABBREVIATION:Slc34a2.1-e# citations: AUTHORS:Murer H, Forster I, Biber J.#TITLE:The sodium phosphate cotransporter family SLC34. #YEAR:2004#PUBMED:12750889# GENE ASSOCIATION: (6569.1) or (10568.1)
phosphate transport in/out via three Na+ symporter
Cyanide sulfurtransferase, mitochondrial
Abbreviation: R_CYANSTm Synonyms: _0 EC Number: 2.8.1.1 SUBSYSTEM: Cysteine Metabolism Equation: [m] : cyan + tsul --> h + so3 + tcynt Confidence Level: 4 MM genes: LOCUS:_7263#TRANSCRIPT:1#ABBREVIATION:TST#ECNUMBERS:2.8.1.1# proteins: NAME:thiosulfate sulfurtransferase (rhodanese), mitochondrial #ABBREVIATION:Tst.1-m# citations: AUTHORS:Horowitz PM, Butler M, McClure GD Jr.#TITLE:Reducing sugars can induce the oxidative inactivation of rhodanese.#YEAR:1992#PUBMED:1429701# AUTHORS:Aita N, Ishii K, Akamatsu Y, Ogasawara Y, Tanabe S.#TITLE:Cloning and expression of human liver rhodanese cDNA.#YEAR:1997#PUBMED:9070219# GENE ASSOCIATION: (7263.1)
Abbreviation: R_FPGS8 Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [c] : 10fthf5glu + atp + glu-L --> 10fthf6glu + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_OAGD3te Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: oagd3_hs[c] <==> oagd3_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_NDPK6 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + dudp <==> adp + dutp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.2) or (10201.1) or (4831.1 and 4830.1) or (4832.1) or (4831.1 and 4830.2) or (29922.1)
nucleoside-diphosphate kinase (ATP:dUDP)
Abbreviation: R_P4504F122r Synonyms: _1 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : h + leuktrB4 + nadph + o2 --> h2o + leuktrB4woh + nadp Confidence Level: 4 omega hydroxylation of leukotriene B4 - see CYP4F3 NJ genes: LOCUS:_4051#TRANSCRIPT:1#ABBREVIATION:CYP4F3#ECNUMBERS:1.14.13.30# LOCUS:_8529#TRANSCRIPT:1#ABBREVIATION:CYP4F2#ECNUMBERS:1.14.13.30# LOCUS:_66002#TRANSCRIPT:1#ABBREVIATION:CYP4F12# proteins: NAME:cytochrome P450, family 4, subfamily F, polypeptide 12#ABBREVIATION:Cyp4f12.1# NAME:cytochrome P450, family 4, subfamily F, polypeptide 3#ABBREVIATION:Cyp4f3.1# NAME:cytochrome P450, family 4, subfamily F, polypeptide 2#ABBREVIATION:Cyp4f2.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS: Kikuta Y, Kusunose E, Endo K, Yamamoto S, Sogawa K, Fujii-Kuriyama Y, Kusunose M.#TITLE:A novel form of cytochrome P-450 family 4 in human polymorphonuclear leukocytes. cDNA cloning and expression of leukotriene B4 omega-hydroxylase.#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8486631# AUTHORS:Kikuta Y, Kusunose E, Kondo T, Yamamoto S, Kinoshita H, Kusunose M.#TITLE:Cloning and expression of a novel form of leukotriene B4 omega-hydroxylase from human liver.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8026587# AUTHORS:Le Quere V, Plee-Gautier E, Potin P, Madec S, Salaun JP.#TITLE:Human CYP4F3s are the main catalysts in the oxidation of fatty acid epoxides.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15145985# GENE ASSOCIATION: (4051.1) or (8529.1) or (66002.1)
Abbreviation: R_DNDPt11m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[c] + dgdp[m] --> dadp[m] + dgdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_4ABUTtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _4abut[c] <==> 4abut[m] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Medina-Kauwe LK, Tobin AJ, De Meirleir L, Jaeken J, Jakobs C, Nyhan WL, Gibson KM.#TITLE:_4-Aminobutyrate aminotransferase (GABA-transaminase) deficiency.#YEAR:1999#PUBMED:10407778# GENE ASSOCIATION:
_4-aminobutanoate mitochondrial transport via diffusion
Abbreviation: R_HSD17B2r Synonyms: _1 EC Number: 1.1.1.62 SUBSYSTEM: Steroid Metabolism Equation: [r] : andrstndn + h + nadh --> nad + tststerone Confidence Level: 4 microsomal NAD as cofactor HSD type 2 recheck/confirm comparmentalization - type 1: cytosol, types 2,3: microsomes NJ genes: LOCUS:_3294#TRANSCRIPT:1#ABBREVIATION:HSD17B2# proteins: NAME:hydroxysteroid (17-beta) dehydrogenase 2#ABBREVIATION:Hsd17b2.1# citations: AUTHORS:Wu L, Einstein M, Geissler WM, Chan HK, Elliston KO, Andersson S.#TITLE:Expression cloning and characterization of human 17 beta-hydroxysteroid dehydrogenase type 2, a microsomal enzyme possessing 20 alpha-hydroxysteroid dehydrogenase activity.#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8099587# GENE ASSOCIATION: (3294.1)
Abbreviation: R_ACGAMtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: acgam[l] --> acgam[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Rome LH, Hill DF#TITLE:Lysosomal degradation of glycoproteins and glycosaminoglycans. Efflux and recycling of sulphate and N-acetylhexosamines#JOURNAL:Biochem J#YEAR:1986#PUBMED:3753439# AUTHORS:Jonas AJ, Speller RJ, Conrad PB, Dubinsky WP#TITLE:Transport of N-acetyl-D-glucosamine and N-acetyl-D-galactosamine by rat liver lysosomes#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2784441# GENE ASSOCIATION:
Abbreviation: R_FATP2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[c] + ocdcea[c] <==> na1[e] + ocdcea[e] Confidence Level: 4 electroneutral transport NJ genes: LOCUS:_376497#TRANSCRIPT:1#ABBREVIATION:SLC27A1# LOCUS:_376497#TRANSCRIPT:2#ABBREVIATION:SLC27A1# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 1#ABBREVIATION:Slc27a1hs.1# NAME:solute carrier family 27 (fatty acid transporter), member 1#ABBREVIATION:Slc27a1hs.2# citations: AUTHORS:Stahl A.#TITLE:A current review of fatty acid transport proteins (SLC27).#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12856180# AUTHORS:Pohl J, Ring A, Hermann T, Stremmel W.#TITLE:Role of FATP in parenchymal cell fatty acid uptake.#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15522816# GENE ASSOCIATION: (376497.1) or (376497.2)
Abbreviation: R_PMTCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: pmtcoa[c] <==> pmtcoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Hydroxylation of 3-Decaprenyl-4-hydroxybenzoate (NADH)
Abbreviation: R_3DPHBH1 Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 3dphb + h + nadh + o2 --> 3dpdhb + h2o + nad Confidence Level: 2 Used to make Ubiquinone-10 enzyme has not been identified yet. IT genes: none proteins: none citations: AUTHORS:Szkopinska A.#TITLE:Ubiquinone. Biosynthesis of quinone ring and its isoprenoid side chain. Intracellular localization.#JOURNAL:Acta Biochim Pol. #YEAR:2000#PUBMED:11051212# GENE ASSOCIATION:
Abbreviation: R_NACHEX25ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core2_deg9 --> acgam + f1a Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3073.1) or (3074.1)
keratan sulfate II biosynthesis, precursor 2_C39H63N3O28X
Abbreviation: R_G14T2g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre1 + udpgal --> h + ksii_core2_pre2 + udp Confidence Level: 4 Keratan sulfate II biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
Abbreviation: R_3HAO Synonyms: _4 EC Number: 1.13.11.6 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 3hanthrn + o2 --> cmusa + h Confidence Level: 4 NCD genes: LOCUS:_23498#TRANSCRIPT:1#ABBREVIATION:HAAO#ECNUMBERS:1.13.11.6# proteins: NAME:_3-hydroxyanthranilate 3,4-dioxygenase#ABBREVIATION:Haao.1# citations: AUTHORS:Malherbe P, Kohler C, Da Prada M, Lang G, Kiefer V, Schwarcz R, Lahm HW, Cesura AM#TITLE:Molecular cloning and functional expression of human 3-hydroxyanthranilic-acid dioxygenase#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:7514594# GENE ASSOCIATION: (23498.1)
Abbreviation: R_DNDPt37m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dtdp[m] + dutp[c] --> dtdp[c] + dutp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
keratan sulfate I, degradation product 23_C124H201N8O106S5
Abbreviation: R_SBPP3er Synonyms: _0 SUBSYSTEM: Sphingolipid Metabolism Equation: [r] : h2o + sphs1p --> pi + sphings Confidence Level: 4 NCD genes: LOCUS:_81537#TRANSCRIPT:1#ABBREVIATION:SGPP1# proteins: NAME:sphingosine-1-phosphate phosphatase 1 #ABBREVIATION:Sgpp1.1# citations: AUTHORS:Johnson KR, Johnson KY, Becker KP, Bielawski J, Mao C, Obeid LM.#TITLE:Role of human sphingosine-1-phosphate phosphatase 1 in the regulation of intra- and extracellular sphingosine-1-phosphate levels and cell viability.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12815058# GENE ASSOCIATION: (81537.1)
sphingoid base-phosphate phosphatase (sphinganine 1-phosphatase), endoplasmic reticulum
Abbreviation: R_PUNP1 Synonyms: _0 EC Number: 2.4.2.1 SUBSYSTEM: Nucleotides Equation: [c] : adn + pi <==> ade + r1p Confidence Level: 4 Specificity not completely determined. Can also catalyse ribosyltransferase reactions of the type catalysed by EC 2.4.2.5, nucleoside ribosyltransferase genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# citations: AUTHORS:Erion MD, Stoeckler JD, Guida WC, Walter RL, Ealick SE.#TITLE:Purine nucleoside phosphorylase. 2. Catalytic mechanism. #JOURNAL:Biochemistry#YEAR:1997#PUBMED:9305963# AUTHORS:Williams SR, Goddard JM, Martin DW Jr. #TITLE:Human purine nucleoside phosphorylase cDNA sequence and genomic clone characterization.#JOURNAL:Nucleic Acids Res.#YEAR:1984#PUBMED:6087295# GENE ASSOCIATION: (4860.1)
purine-nucleoside phosphorylase (Adenosine)
Abbreviation: R_BTNt3i Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + btn[e] + h2o[c] + (2) na1[e] --> adp[c] + btn[c] + h[c] + (2) na1[c] + pi[c] Confidence Level: 4 _01-25-05 based on Wang et al., 1999, JBC, 274(21), 14875-14888. IT 03-07-05:replaced IT: now based on Balamurugan et al, 2005, Am J Physiol Renal Physiol, 288, F823-831,2005 genes: LOCUS:_8884#TRANSCRIPT:1#ABBREVIATION:SMVT#ECNUMBERS:TC,2.A.21.1.1# proteins: NAME:sodium-dependent vitamin transporter (biotin, pantothenate,lipoate) #ABBREVIATION:SmvT.1# citations: AUTHORS:Prasad PD, Wang H, Kekuda R, Fujita T, Fei YJ, Devoe LD, Leibach FH, Ganapathy V.#TITLE:Cloning and functional expression of a cDNA encoding a mammalian sodium-dependent vitamin transporter mediating the uptake of pantothenate, biotin, and lipoate.#JOURNAL: J Biol Chem.#YEAR:1998#PUBMED:9516450# AUTHORS:Balamurugan K, Ortiz A, Said HM.#TITLE:Biotin uptake by human intestinal and liver epithelial cells: role of the SMVT system.#JOURNAL:Am J Physiol Gastrointest Liver Physiol. #YEAR:2003#PUBMED:12646417# AUTHORS:Prasad PD, Wang H, Huang W, Fei YJ, Leibach FH, Devoe LD, Ganapathy V.#TITLE:Molecular and functional characterization of the intestinal Na+-dependent multivitamin transporter. #JOURNAL:Arch Biochem Biophys.#YEAR:1999#PUBMED:10334869# AUTHORS:Wang H, Huang W, Fei YJ, Xia H, Yang-Feng TL, Leibach FH, Devoe LD, Ganapathy V, Prasad PD.#TITLE:Human placental Na+-dependent multivitamin transporter. Cloning, functional expression, gene structure, and chromosomal localization.#JOURNAL: J Biol Chem. #YEAR:1999#PUBMED:10329687# AUTHORS:Balamurugan K, Vaziri ND, Said HM.#TITLE:Biotin uptake by human proximal tubular epithelial cells: Cellular and molecular aspects. #JOURNAL:Am J Physiol Renal Physiol. #YEAR:2005#PUBMED:15561972# GENE ASSOCIATION: (8884.1)
Gal-Gal-Gal-Gal-Glc-Cer (homo sapiens)_C48H86NO27FULLRCO
Abbreviation: R_PCHOL_HStg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: pchol_hs[c] <==> pchol_hs[g] Confidence Level: 2 for assumed reversible, non-energy dep scramblase mechanism (PMID: 10856717). NJ genes: none proteins: none citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# GENE ASSOCIATION:
Abbreviation: R_PAFS Synonyms: _2 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : accoa + ak2lgchol_hs --> coa + paf_hs Confidence Level: 0 NJ genes: none proteins: none citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION:
alkyl glycerol phosphocholine acetyl transferase
Abbreviation: R_T2M26DCOAHLm Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: Limonene and pinene degradation Equation: [m] : h2o + t2m26dcoa <==> 3h26dm5coa Confidence Level: 1 SAB genes: LOCUS:_3030#TRANSCRIPT:1#ABBREVIATION:HADHA#ECNUMBERS:4.2.1.17,1.1.1.35# LOCUS:_3032#TRANSCRIPT:1#ABBREVIATION:HADHB# LOCUS:_549#TRANSCRIPT:1#ABBREVIATION:AUH# proteins: NAME:AU RNA binding protein/enoyl-Coenzyme A hydratase (mito)#ABBREVIATION:Auh.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit #ABBREVIATION:Hadha.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit #ABBREVIATION:Hadhb.1-m# citations: none GENE ASSOCIATION: (549.1) or (3030.1) and (3032.1)
trans-2-Methyl-5-isopropylhexa-2,5-dienoyl-CoA hydro-lyase (m)
Abbreviation: R_BILIRED Synonyms: _0 EC Number: 1.3.1.24 SUBSYSTEM: Heme Degradation Equation: [c] : biliverd + h + nadph --> bilirub + nadp Confidence Level: 0 TDV genes: LOCUS:_645#TRANSCRIPT:1#ABBREVIATION:BLVRB#ECNUMBERS:1.3.1.24# LOCUS:_644#TRANSCRIPT:1#ABBREVIATION:BLVRA#ECNUMBERS:1.3.1.24# proteins: NAME:biliverdin reductase A#ABBREVIATION:Blvra.1# NAME:biliverdin reductase B (flavin reductase (NADPH)) #ABBREVIATION:Blvrdb.1# citations: AUTHORS:Baranano DE, Rao M, Ferris CD, Snyder SH.#TITLE:Biliverdin reductase: a major physiologic cytoprotectant. #JOURNAL:Proc Natl Acad Sci U S A#YEAR:2002#PUBMED:12456881# AUTHORS:Chikuba K, Yubisui T, Shirabe K, Takeshita M.#TITLE:Cloning and nucleotide sequence of a cDNA of the human erythrocyte NADPH-flavin reductase. #JOURNAL:Biochem Biophys Res Commun. #YEAR:1994#PUBMED:8117274# AUTHORS:Saito F, Yamaguchi T, Komuro A, Tobe T, Ikeuchi T, Tomita M, Nakajima H. #TITLE:Mapping of the newly identified biliverdin-IX beta reductase gene (BLVRB) to human chromosome 19q13.13-->q13.2 by fluorescence in situ hybridization.#JOURNAL:Cytogenet Cell Genet#YEAR:1995#PUBMED:7656592# GENE ASSOCIATION: (645.1) or (644.1)
keratan sulfate I, degradation product 24_C118H191N8O101S5
keratan sulfate II biosynthesis, precursor 3_C47H76N4O33X
Abbreviation: R_G14T3g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre3 + udpgal --> h + ksii_core2_pre4 + udp Confidence Level: 4 Keratan sulfate II biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
keratan sulfate II biosynthesis, precursor 4_C53H86N4O38X
Abbreviation: R_ESTRONESt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: estrones[e] + (2) na1[e] --> estrones[c] + (2) na1[c] Confidence Level: 4 electrogenic uptake NJ genes: LOCUS:_6554#TRANSCRIPT:1#ABBREVIATION:SLC10A1# proteins: NAME:solute carrier family 10 (sodium/bile acid cotransporter family), member 1#ABBREVIATION:Slc10a1.1# citations: AUTHORS:Hagenbuch B, Dawson P.#TITLE:The sodium bile salt cotransport family SLC10.#JOURNAL:Pflugers Arch.#YEAR:2004#PUBMED:12851823# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:Molecular cloning, chromosomal localization, and functional characterization of a human liver Na+/bile acid cotransporter.#JOURNAL:J Clin Invest.#YEAR:1994#PUBMED:8132774# GENE ASSOCIATION: (6554.1)
estrone-3-sulfate transport via sodium cotransport
Abbreviation: R_DNDPt4m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: datp[c] + dudp[m] --> datp[m] + dudp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_acngalacglcgal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acngalacglcgal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S2TASE4ly Synonyms: _1 EC Number: 3.1.6.18 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_d_deg2 + h2o --> cs_d_deg3 + h + so4 Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Shaklee PN, Glaser JH, Conrad HE#TITLE:A sulfatase specific for glucuronic acid 2-sulfate residues in glycosaminoglycans#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:4019466# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver glucuronate 2-sulphatase. Purification, characterization and catalytic properties#JOURNAL:Biochem J#YEAR:1989#PUBMED:2497731# GENE ASSOCIATION:
Abbreviation: R_PGLer Synonyms: _0 EC Number: 3.1.1.31 SUBSYSTEM: Pentose Phosphate Pathway Equation: [r] : 6pgl + h2o --> 6pgc + h Confidence Level: 4 SMP Net reaction same as described in Lehringer 334 NCD genes: LOCUS:_9563#TRANSCRIPT:1#ABBREVIATION:H6PD#ECNUMBERS:1.1.1.47# proteins: NAME:hexose-6-phosphate dehydrogenase, endoplasmic reticulum#ABBREVIATION:H6pd.1-r# citations: AUTHORS:Mason PJ, Stevens D, Diez A, Knight SW, Scopes DA, Vulliamy TJ.#TITLE:Human hexose-6-phosphate dehydrogenase (glucose 1-dehydrogenase) encoded at 1p36: coding sequence and expression.#JOURNAL: Blood Cells Mol Dis#YEAR:1999#PUBMED:10349511# AUTHORS:Clarke JL, Mason PJ#TITLE:Murine hexose-6-phosphate dehydrogenase: a bifunctional enzyme with broad substrate specificity and 6-phosphogluconolactonase activity.#JOURNAL:Arch Biochem Biophys#YEAR:2003#PUBMED:12831846# AUTHORS:Barash V, Erlich T, Bashan N.#TITLE:Microsomal hexose-6-phosphate and 6-phosphogluconate dehydrogenases in extrahepatic tissues: human placenta and pig kidney cortex.#JOURNAL:Biochem Int.#YEAR:1990#PUBMED:2156506# GENE ASSOCIATION: (9563.1)
_6-phosphogluconolactonase, endoplasmic reticulum
_1-alpha-Vitamin D-24,25-hydroxylase (D2)
Abbreviation: R_1a_COMMA_24_COMMA_25VITD2Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : 2425dhvitd2 + h + nadph + o2 --> 1a2425thvitd2 + h2o + nadp Confidence Level: 2 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.196 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_DNDPt21m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[m] + dtdp[c] --> adp[c] + dtdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_Lcystin_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : Lcystin <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ORETNtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: oretn[c] <==> oretn[n] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_EX_tcynt_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tcynt <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_5MTHFt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _5mthf[e] <==> 5mthf[c] Confidence Level: 4 IT genes: LOCUS:_2348#TRANSCRIPT:1#ABBREVIATION:FOLR1# LOCUS:_2351#TRANSCRIPT:1#ABBREVIATION:FOLR2L# LOCUS:_2352#TRANSCRIPT:1#ABBREVIATION:FOLR3# LOCUS:_2348#TRANSCRIPT:3#ABBREVIATION:FOLR1# LOCUS:_2348#TRANSCRIPT:2#ABBREVIATION:FOLR1# LOCUS:_2348#TRANSCRIPT:5#ABBREVIATION:FOLR1# LOCUS:_2348#TRANSCRIPT:4#ABBREVIATION:FOLR1# LOCUS:_2348#TRANSCRIPT:6#ABBREVIATION:FOLR1# proteins: NAME:folate receptor 1 (adult) #ABBREVIATION:Folr1.1# NAME:folate receptor 1 (adult) #ABBREVIATION:Folr1.2# NAME:folate receptor 1 (adult) #ABBREVIATION:Folr1.3# NAME:folate receptor 1 (adult) #ABBREVIATION:Folr1.4# NAME:folate receptor 1 (adult) #ABBREVIATION:Folr1.5# NAME:folate receptor 1 (adult) #ABBREVIATION:Folr1.6# NAME:folate receptor 2 (fetal) like #ABBREVIATION:Folr2.1# NAME:folate receptor 3 (gamma) #ABBREVIATION:Folr3.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (2352.1) or (2348.6) or (2348.4) or (2348.1) or (2348.3) or (2351.1) or (2348.2) or (2348.5)
_5-Methyltetrahydrofolate transport via receptor binding and protolysis
glycophosphatidylinositol (GPI) anchor precursor (3-(phosphoethanolaminyl-mannosyl)-glucosaminyl-acylphosphatidylinositol) (H8)_C55H106N4O38P4FULLRCO2FULLR2CO2
H8 mannosyltransferase, endoplasmic reticulum
Abbreviation: R_H8MTer_U Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_U + gpi_hs --> (0.1) dolp_U + h + m(em)3gacpail_hs Confidence Level: 4 NCD genes: LOCUS:_80235#TRANSCRIPT:1#ABBREVIATION:SMP3# proteins: NAME:SMP3 mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Smp3.1-r# citations: AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (80235.1)
stearoyl-CoA desaturase (n-C18:0CoA -> n-C18:1CoA)
Abbreviation: R_DESAT18_3 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + nadh + o2 + stcoa --> (2) h2o + nad + odecoa Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_6319#TRANSCRIPT:1#ABBREVIATION:SCD# proteins: NAME:stearoyl-CoA desaturase#ABBREVIATION:Scd.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Zhang L, Ge L, Parimoo S, Stenn K, Prouty SM#TITLE:Human stearoyl-CoA desaturase: alternative transcripts generated from a single gene by usage of tandem polyadenylation sites#JOURNAL:Biochem J#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# AUTHORS:Wang Y, Kurdi-Haidar B, Oram JF.#TITLE:LXR-mediated activation of macrophage stearoyl-CoA desaturase generates unsaturated fatty acids that destabilize ABCA1.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:14967823# GENE ASSOCIATION: (6319.1)
Abbreviation: R_AHC Synonyms: _0 EC Number: 3.3.1.1 SUBSYSTEM: Methionine Metabolism Equation: [c] : ahcys + h2o <==> adn + hcys-L Confidence Level: 4 genes: LOCUS:_191#TRANSCRIPT:1#ABBREVIATION:AHCY#ECNUMBERS:3.3.1.1# LOCUS:_10768#TRANSCRIPT:1#ABBREVIATION:AHCYL1# LOCUS:_10768#TRANSCRIPT:2#ABBREVIATION:AHCYL1# proteins: NAME:S-adenosylhomocysteine hydrolase #ABBREVIATION:Ahcy.1# NAME:S-adenosylhomocysteine hydrolase-like 1 #ABBREVIATION:Ahcyl1.1# NAME:S-adenosylhomocysteine hydrolase-like 1#ABBREVIATION:Ahcyl1.2# citations: AUTHORS:Yang X, Hu Y, Yin DH, Turner MA, Wang M, Borchardt RT, Howell PL, Kuczera K, Schowen RL.#TITLE:Catalytic strategy of S-adenosyl-L-homocysteine hydrolase: transition-state stabilization and the avoidance of abortive reactions.#YEAR:2003#PUBMED:12590576# AUTHORS:Coulter-Karis DE, Hershfield MS.#TITLE:Sequence of full length cDNA for human S-adenosylhomocysteine hydrolase.#YEAR:1989#PUBMED:2596825# GENE ASSOCIATION: (191.1) or (10768.2) or (10768.1)
Abbreviation: R_DESAT18_9 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + lnlccoa + nadh + o2 --> (2) h2o + lnlncgcoa + nad Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_9415#TRANSCRIPT:1#ABBREVIATION:FADS2#ECNUMBERS:1.14.99.25# proteins: NAME:fatty acid desaturase 2#ABBREVIATION:Fads2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Cho HP, Nakamura MT, Clarke SD#TITLE:Cloning, expression, and nutritional regulation of the mammalian delta-6 desaturase#JOURNAL:The Journal of Biological Chemistry#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION: (9415.1)
((N-acetyl-D-glucosaminyl)3-(alpha-D-mannosyl)2-beta-D-mannosyl-diacetylchitobiosyl)-L-asparagine (protein)_C58H96N5O40X
Abbreviation: R_M13N4Tg Synonyms: _5 EC Number: 2.4.1.145 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : n2m2masn + uacgam --> h + n3m2masn + udp Confidence Level: 0 NCD genes: LOCUS:_11320#TRANSCRIPT:1#ABBREVIATION:MGAT4A#ECNUMBERS:2.4.1.145# LOCUS:_11282#TRANSCRIPT:1#ABBREVIATION:MGAT4B#ECNUMBERS:2.4.1.145# LOCUS:_11282#TRANSCRIPT:2#ABBREVIATION:MGAT4B#ECNUMBERS:2.4.1.145# proteins: NAME:mannosyl (alpha-1,3-)-glycoprotein beta-1,4-N-acetylglucosaminyltransferase, isoenzyme B, Golgi apparatus (ubiquitous)#ABBREVIATION:Mgat4b.1-g# NAME:mannosyl (alpha-1,3-)-glycoprotein beta-1,4-N-acetylglucosaminyltransferase, isoenzyme A, Golgi apparatus (ubiquitous)#ABBREVIATION:Mgat4a.1-g# NAME:mannosyl (alpha-1,3-)-glycoprotein beta-1,4-N-acetylglucosaminyltransferase, isoenzyme B, Golgi apparatus (ubiquitous)#ABBREVIATION:Mgat4b.2-g# citations: AUTHORS:Yoshida A, Minowa MT, Takamatsu S, Hara T, Oguri S, Ikenaga H, Takeuchi M.#TITLE:Tissue specific expression and chromosomal mapping of a human UDP-N-acetylglucosamine: alpha1,3-d-mannoside beta1, 4-N-acetylglucosaminyltransferase.#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10024668# AUTHORS:Yoshida A, Minowa MT, Takamatsu S, Hara T, Ikenaga H, Takeuchi M.#TITLE:A novel second isoenzyme of the human UDP-N-acetylglucosamine:alpha1,3-D-mannoside beta1,4-N-acetylglucosaminyltransferase family: cDNA cloning, expression, and chromosomal assignment.#JOURNAL:Glycoconj J#YEAR:1998#PUBMED:10372966# GENE ASSOCIATION: (11320.1) or (11282.2) or (11282.1)
((N-acetyl-D-glucosaminyl)2-(alpha-D-mannosyl)2-beta-D-mannosyl-diacetylchitobiosyl)-L-asparagine (protein)_C50H83N4O35X
alpha-1,3-mannosyl-glycoprotein 4-beta-N-acetylglucosaminyltransferase
Abbreviation: R_EX_34dhphe_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 34dhphe <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
glycophosphatidylinositol (GPI)-anchored protein precursor_XY
Abbreviation: R_H7_APOS_TAer Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : em2emgacpail_hs + pre_prot --> em2emgacpail_prot_hs + gpi_sig Confidence Level: 4 EC 2.6.-.- NCD genes: LOCUS:_10026#TRANSCRIPT:1#ABBREVIATION:PIGK# LOCUS:_8733#TRANSCRIPT:1#ABBREVIATION:GPAA1# LOCUS:_94005#TRANSCRIPT:1#ABBREVIATION:PIGS# LOCUS:_128869#TRANSCRIPT:1#ABBREVIATION:CDC91L1# LOCUS:_51604#TRANSCRIPT:1#ABBREVIATION:PIGT# proteins: NAME:GPAA1P anchor attachment protein 1 homolog (yeast), endoplasmic reticulum#ABBREVIATION:Gpaa1.1-r# NAME:phosphatidylinositol glycan, class K, endoplasmic reticulum#ABBREVIATION:Pigk.1-r# NAME:phosphatidylinositol glycan, class S, endoplasmic reticulum#ABBREVIATION:Pigs.1-r# NAME:phosphatidylinositol glycan, class T, endoplasmic reticulum#ABBREVIATION:Pigt.1-r# NAME:CDC91 cell division cycle 91-like 1 (S. cerevisiae), endoplasmic reticulum#ABBREVIATION:Cdc91l1.1-r# citations: AUTHORS:Shishioh N, Hong Y, Ohishi K, Ashida H, Maeda Y, Kinoshita T#TITLE:GPI7 is the second partner of PIG-F and involved in modification of glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15632136# AUTHORS:Hiroi Y, Komuro I, Chen R, Hosoda T, Mizuno T, Kudoh S, Georgescu SP, Medof ME, Yazaki Y#TITLE:Molecular cloning of human homolog of yeast GAA1 which is required for attachment of glycosylphosphatidylinositols to proteins#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9468317# AUTHORS:Yu J, Nagarajan S, Knez JJ, Udenfriend S, Chen R, Medof ME#TITLE:The affected gene underlying the class K glycosylphosphatidylinositol (GPI) surface protein defect codes for the GPI transamidase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9356492# AUTHORS:Meyer U, Benghezal M, Imhof I, Conzelmann A#TITLE:Active site determination of Gpi8p, a caspase-related enzyme required for glycosylphosphatidylinositol anchor addition to proteins#JOURNAL:Biochemistry#YEAR:2000#PUBMED:10727241# AUTHORS:Ohishi K, Inoue N, Kinoshita T#TITLE:PIG-S and PIG-T, essential for GPI anchor attachment to proteins, form a complex with GAA1 and GPI8#JOURNAL:EMBO J#YEAR:2001#PUBMED:11483512# AUTHORS:Hong Y, Ohishi K, Kang JY, Tanaka S, Inoue N, Nishimura J, Maeda Y, Kinoshita T#TITLE:Human PIG-U and yeast Cdc91p are the fifth subunit of GPI transamidase that attaches GPI-anchors to proteins#JOURNAL:Mol Biol Cell#YEAR:2003#PUBMED:12802054# GENE ASSOCIATION: (10026.1) and (8733.1) and (128869.1) and (94005.1) and (51604.1)
Abbreviation: R_DORNOp Synonyms: _0 EC Number: 1.4.3.3 SUBSYSTEM: D-arg and D-orn metabolism Equation: [x] : h2o + o2 + orn-D --> 5a2opntn + h2o2 + nh4 Confidence Level: 4 SAB genes: LOCUS:_1610#TRANSCRIPT:1#ABBREVIATION:DAO#ECNUMBERS:1.4.3.3# proteins: NAME:D-amino-acid oxidase#ABBREVIATION:Dao.1-x# citations: AUTHORS:Vanoni MA, Cosma A, Mazzeo D, Mattevi A, Todone F, Curti B#TITLE:Limited proteolysis and X-ray crystallography reveal the origin of substrate specificity and of the rate-limiting product release during oxidation of D-amino acids catalyzed by mammalian D-amino acid oxidase#JOURNAL:Biochemistry#YEAR:1997#PUBMED:9153402# AUTHORS:D'Aniello A, Vetere A, Petrucelli L#TITLE:Further study on the specificity of D-amino acid oxidase and D-aspartate oxidase and time course for complete oxidation of D-amino acids#JOURNAL:Comp Biochem Physiol B#YEAR:1993#PUBMED:8103425# GENE ASSOCIATION: (1610.1)
Abbreviation: R_DTMPK Synonyms: _0 EC Number: 2.7.4.9 SUBSYSTEM: Nucleotides Equation: [c] : atp + dtmp <==> adp + dtdp Confidence Level: 4 genes: LOCUS:_1841#TRANSCRIPT:1#ABBREVIATION:DTYMK#ECNUMBERS:2.7.4.9# proteins: NAME:deoxythymidylate kinase (thymidylate kinase) #ABBREVIATION:Dtymk.1# citations: AUTHORS:Su JY, Sclafani RA.#TITLE:Molecular cloning and expression of the human deoxythymidylate kinase gene in yeast.#JOURNAL:Nucleic Acids Res.#YEAR:1991#PUBMED:2017365# AUTHORS:Huang SH, Tang A, Drisco B, Zhang SQ, Seeger R, Li C, Jong A.#TITLE:Human dTMP kinase: gene expression and enzymatic activity coinciding with cell cycle progression and cell growth.#JOURNAL:DNA Cell Biol.#YEAR:1994#PUBMED:8024690# GENE ASSOCIATION: (1841.1)
Abbreviation: R_RETNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: retn[c] <==> retn[e] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Hodam JR, Creek KE.#TITLE:Uptake and metabolism of [3H]retinoic acid delivered to human foreskin keratinocytes either bound to serum albumin or added directly to the culture medium.#JOURNAL:Biochim Biophys Acta.#YEAR:1996#PUBMED:8630327# GENE ASSOCIATION:
Abbreviation: R_ARTPLM3m Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [m] : Rtotal3coa <==> pmtcoa Confidence Level: 0 cytosolic conversion of pooled FA into palmitate (so subsequent transport into mit w/ FA oxidation can occur). Since Carbon stoichiometry normalized wrt C16 ratio between Rgroupcoa and pmtcoa is 1:1 HR fatty acid metabolism - R groups = acyl groups, Rtotal, Rtotal2, Rtotal3 correspond to three possible acyl groups added to glycerol R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate II (core 4-linked)_C89H142N7O72S3X
Abbreviation: R_EX_ksii_core4_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ksii_core4 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate II (core 4-linked) exchange
Abbreviation: R_FATP8t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lgnc[c] + na1[c] <==> lgnc[e] + na1[e] Confidence Level: 4 electroneutral transport NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# LOCUS:_10998#TRANSCRIPT:1#ABBREVIATION:SLC27A5# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 5#ABBREVIATION:Slc27a5.1# NAME:solute carrier family 27 (fatty acid transporter), member 2#ABBREVIATION:Slc27a2.1# citations: AUTHORS:Steinberg SJ, Wang SJ, McGuinness MC, Watkins PA.#TITLE:Human liver-specific very-long-chain acyl-coenzyme A synthetase: cDNA cloning and characterization of a second enzymatically active protein.#JOURNAL:Mol Genet Metab.#YEAR:1999#PUBMED:10479480# AUTHORS:Stahl A.#TITLE:A current review of fatty acid transport proteins (SLC27).#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12856180# AUTHORS:Pohl J, Ring A, Hermann T, Stremmel W.#TITLE:Role of FATP in parenchymal cell fatty acid uptake.#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15522816# GENE ASSOCIATION: (10998.1) or (11001.1)
Abbreviation: R_AHCYStr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: ahcys[c] <==> ahcys[r] Confidence Level: 1 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
S-Adenosyl-L-homocysteine intracellular diffusion
Abbreviation: R_EX_glyb_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : glyb <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GUACYC Synonyms: _3 EC Number: 4.6.1.2 SUBSYSTEM: Nucleotides Equation: [c] : gtp --> 35cgmp + ppi Confidence Level: 4 IT genes: LOCUS:_2984#TRANSCRIPT:1#ABBREVIATION:GUCY2C# LOCUS:_2977#TRANSCRIPT:1#ABBREVIATION:GUCY1A2# LOCUS:_2986#TRANSCRIPT:1#ABBREVIATION:GUCY2F#ECNUMBERS:4.6.1.2# LOCUS:_2983#TRANSCRIPT:1#ABBREVIATION:GUCY1B3# LOCUS:_2982#TRANSCRIPT:1#ABBREVIATION:GUCY1A3# LOCUS:_2974#TRANSCRIPT:1#ABBREVIATION:GUCY1B2#ECNUMBERS:4.6.1.2# LOCUS:_4882#TRANSCRIPT:1#ABBREVIATION:NPR2# LOCUS:_4881#TRANSCRIPT:1#ABBREVIATION:NPR1# LOCUS:_3000#TRANSCRIPT:1#ABBREVIATION:GUCY2D# LOCUS:_4882#TRANSCRIPT:2#ABBREVIATION:NPR2# proteins: NAME:natriuretic peptide receptor A/guanylate cyclase A (atrionatriuretic peptide receptor A) #ABBREVIATION:Npr1.1# NAME:natriuretic peptide receptor B/guanylate cyclase B (atrionatriuretic peptide receptor B) #ABBREVIATION:Npr2.1# NAME:natriuretic peptide receptor B/guanylate cyclase B (atrionatriuretic peptide receptor B) #ABBREVIATION:Npr2.2# NAME:guanylate cyclase 1, soluble #ABBREVIATION:Gucy1A2B2.1# NAME:guanylate cyclase 1, soluble #ABBREVIATION:Gucy1A3B3.1# NAME:guanylate cyclase 1, soluble #ABBREVIATION:Gucy1A3B2.1# NAME:guanylate cyclase 1, soluble #ABBREVIATION:Gucy1A2B3.1# NAME:guanylate cyclase 2C (heat stable enterotoxin receptor) #ABBREVIATION:Gucy2C.1# NAME:guanylate cyclase 2D, membrane (retina-specific) #ABBREVIATION:Gucy2D.1# NAME:guanylate cyclase 2F, retinal #ABBREVIATION:Gucy2F.1# citations: AUTHORS:Pardhasaradhi K, Kutty RK, Gentleman S, Krishna G.#TITLE:Expression of mRNA for atrial natriuretic peptide receptor guanylate cyclase (ANPRA) in human retina. #JOURNAL:Cell Mol Neurobiol. #YEAR:1994#PUBMED:7954658# AUTHORS:Arden KC, Viars CS, Weiss S, Argentin S, Nemer M.#TITLE:Localization of the human B-type natriuretic peptide precursor (NPPB) gene to chromosome 1p36. #JOURNAL:Genomics. #YEAR:1995#PUBMED:7601467# AUTHORS:Koptides M, Mean R, Stavrou C, Pierides A, Demetriou K, Nakayama T, Hildebrandt F, Fuchshuber A, Deltas CC. #TITLE:Novel NPR1 polymorphic variants and its exclusion as a candidate gene for medullary cystic kidney disease (ADMCKD) type 1. #JOURNAL:Mol Cell Probes#YEAR:2001#PUBMED:11851379# AUTHORS:Potthast R, Abbey-Hosch SE, Antos LK, Marchant JS, Kuhn M, Potter LR. #TITLE:Calcium-dependent dephosphorylation mediates the hyperosmotic and lysophosphatidic acid-dependent inhibition of natriuretic peptide receptor-B/guanylyl cyclase-B. #JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15371450# AUTHORS:Malterer A, Gupta G, Danziger RS.#TITLE:Assignment of GUCY1B2, the human homologue of a candidate gene for hypertension, to chromosome bands 13q14.2-->q14.3 by in situ hybridization.#JOURNAL:Cytogenet Cell Genet.#YEAR:1999#PUBMED:10449911# AUTHORS:Behrends S, Vehse K.#TITLE:The beta(2) subunit of soluble guanylyl cyclase contains a human-specific frameshift and is expressed in gastric carcinoma.#JOURNAL:Biochem Biophys Res Commun.#YEAR:2000#PUBMED:10777682# AUTHORS:Zabel U, Weeger M, La M, Schmidt HH. #TITLE:Human soluble guanylate cyclase: functional expression and revised isoenzyme family.#JOURNAL:Biochem J. #YEAR:1998#PUBMED:9742212# AUTHORS:Giuili G, Scholl U, Bulle F, Guellaen G.#TITLE:Molecular cloning of the cDNAs coding for the two subunits of soluble guanylyl cyclase from human brain.#JOURNAL:FEBS Lett. #YEAR:1992#PUBMED:1352257# AUTHORS:Sugawara T, Holt JA, Driscoll D, Strauss JF 3rd, Lin D, Miller WL, Patterson D, Clancy KP, Hart IM, Clark BJ, et al. #TITLE:Human steroidogenic acute regulatory protein: functional activity in COS-1 cells, tissue-specific expression, and mapping of the structural gene to 8p11.2 and a pseudogene to chromosome 13.#JOURNAL:Proc Natl Acad Sci U S A. #YEAR:1995#PUBMED:7761400# AUTHORS:Ghanekar Y, Chandrashaker A, Tatu U, Visweswariah SS.#TITLE:Glycosylation of the receptor guanylate cyclase C: role in ligand binding and catalytic activity.#JOURNAL:Biochem J. #YEAR:2004#PUBMED:14748740# AUTHORS:Yang RB, Fulle HJ, Garbers DL.#TITLE:Chromosomal localization and genomic organization of genes encoding guanylyl cyclase receptors expressed in olfactory sensory neurons and retina. #JOURNAL:Genomics#YEAR:1996#PUBMED:8838319# AUTHORS:Lowe DG, Dizhoor AM, Liu K, Gu Q, Spencer M, Laura R, Lu L, Hurley JB.#TITLE:Cloning and expression of a second photoreceptor-specific membrane retina guanylyl cyclase (RetGC), RetGC-2. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1995#PUBMED:7777544# AUTHORS:Payne AM, Morris AG, Downes SM, Johnson S, Bird AC, Moore AT, Bhattacharya SS, Hunt DM.#TITLE:Clustering and frequency of mutations in the retinal guanylate cyclase (GUCY2D) gene in patients with dominant cone-rod dystrophies.#JOURNAL:J Med Genet. #YEAR:2001#PUBMED:11565546# AUTHORS:Kelsell RE, Evans K, Gregory CY, Moore AT, Bird AC, Hunt DM.#TITLE:Localisation of a gene for dominant cone-rod dystrophy (CORD6) to chromosome 17p. #JOURNAL:Hum Mol Genet.#YEAR:1997#PUBMED:9097965# GENE ASSOCIATION: (2977.1 and 2983.1) or (4882.2) or (2982.1 and 2974.1) or (2986.1) or (4882.1) or (2984.1) or (4881.1) or (2977.1 and 2974.1) or (3000.1) or (2982.1 and 2983.1)
Abbreviation: R_HAS2 Synonyms: _0 EC Number: 2.4.1.212 SUBSYSTEM: Hyaluronan Metabolism Equation: ha_pre1[e] + uacgam[c] + udpglcur[c] --> (2) h[c] + ha[e] + (2) udp[c] Confidence Level: 4 NCD genes: LOCUS:_3036#TRANSCRIPT:1#ABBREVIATION:HAS1# LOCUS:_3037#TRANSCRIPT:1#ABBREVIATION:HAS2# LOCUS:_3038#TRANSCRIPT:1#ABBREVIATION:HAS3#ECNUMBERS:2.4.1.-# LOCUS:_3038#TRANSCRIPT:2#ABBREVIATION:HAS3# proteins: NAME:hyaluronan synthase 1#ABBREVIATION:Has1.1# NAME:hyaluronan synthase 2#ABBREVIATION:Has2.1# NAME:hyaluronan synthase 3#ABBREVIATION:Has3.1# NAME:hyaluronan synthase 3#ABBREVIATION:Has3.2# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Itano N, Kimata K.#TITLE:Molecular cloning of human hyaluronan synthase#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8651928# AUTHORS:Shyjan AM, Heldin P, Butcher EC, Yoshino T, Briskin MJ#TITLE:Functional cloning of the cDNA for a human hyaluronan synthase#JOURNAL:J Biol Chem.#YEAR:1996#PUBMED:8798544# AUTHORS:Watanabe K, Yamaguchi Y#TITLE:Molecular identification of a putative human hyaluronan synthase#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8798477# AUTHORS:Spicer AP, Olson JS, McDonald JA#TITLE:Molecular cloning and characterization of a cDNA encoding the third putative mammalian hyaluronan synthase#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9083017# AUTHORS:Itano N, Kimata K#TITLE:Mammalian hyaluronan synthases#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512858# GENE ASSOCIATION: (3038.2) or (3036.1) or (3038.1) or (3037.1)
Abbreviation: R_LALDO2x Synonyms: _0 EC Number: 1.1.1.21 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : h + mthgxl + nadh --> lald-D + nad Confidence Level: 4 AMF genes: LOCUS:_8574#TRANSCRIPT:1#ABBREVIATION:AKR7A2# proteins: NAME:aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)#ABBREVIATION:Akr7a2.1# citations: AUTHORS:Kelly VP, Sherratt PJ, Crouch DH, Hayes JD#TITLE:Novel homodimeric and heterodimeric rat gamma-hydroxybutyrate synthases that associate with the Golgi apparatus define a distinct subclass of aldo-keto reductase 7 family proteins.#JOURNAL:Biochem J#YEAR:2002#PUBMED:12071861# AUTHORS:Praml C, Savelyeva L, Perri P, Schwab M.#TITLE:Cloning of the human aflatoxin B1-aldehyde reductase gene at 1p35-1p36.1 in a region frequently altered in human tumor cells.#JOURNAL:Cancer Res#YEAR:1998#PUBMED:9823300# GENE ASSOCIATION: (8574.1)
Abbreviation: R_DNDPt39m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgdp[m] + dutp[c] --> dgdp[c] + dutp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
_2-Oxoadipate:lipoamde 2-oxidoreductase(decarboxylating and acceptor-succinylating) (mitochondria)
Abbreviation: R_2OXOADOXm Synonyms: _0 EC Number: 1.2.4.2 SUBSYSTEM: Lysine Metabolism Equation: [m] : 2oxoadp + coa + nad --> co2 + glutcoa + nadh Confidence Level: 2 SAB genes: LOCUS:_4967#TRANSCRIPT:1#ABBREVIATION:OGDH#ECNUMBERS:1.2.4.2# LOCUS:_8050#TRANSCRIPT:1#ABBREVIATION:PDHX#ECNUMBERS:2.3.1.12# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_1743#TRANSCRIPT:1#ABBREVIATION:DLST#ECNUMBERS:2.3.1.61# LOCUS:_4967#TRANSCRIPT:2#ABBREVIATION:OGDH# proteins: NAME:dihydrolipoamide dehydrogenase (pyruvate dehydrogenase, E3 component), mitochondrial#ABBREVIATION:Dld/Pdhx.1-m# NAME: oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide), mitochondrial#ABBREVIATION:Ogdh.1-m# NAME:oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide), mitochondrial#ABBREVIATION:Ogdh.2-m# NAME:dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex), mitochondrial#ABBREVIATION:Dlst.1-m# citations: AUTHORS:Cox, RP#TITLE:Errors of lysine metabolism#JOURNAL:The Metabolic and Molecular Bases of Inherited Disease, 8th ed#YEAR:2001# GENE ASSOCIATION: (4967.1) and (1738.1 and 8050.1) and (1743.1) or (4967.2) and (1738.1 and 8050.1) and (1743.1)
Abbreviation: R_EX_xyl_DASH_D_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : xyl-D <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_5ADTSTSTERONEGLCtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: _5adtststeroneglc[c] <==> 5adtststeroneglc[r] Confidence Level: 2 unknown mechanism, however glucuronidated metabolites need to get out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_6HTSTSTERONEtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: _6htststerone[c] <==> 6htststerone[r] Confidence Level: 2 unknown mechanism, inactivated testosterone transport to get out of cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PI4PLCn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail4p_hs --> dag_hs + h + mi14p Confidence Level: 4 NCD genes: LOCUS:_23236#TRANSCRIPT:1#ABBREVIATION:PLCB1#ECNUMBERS:3.1.4.11# LOCUS:_23236#TRANSCRIPT:2#ABBREVIATION:PLCB1# proteins: NAME:phospholipase C, beta 1 (phosphoinositide-specific), nucleus#ABBREVIATION:Plcb1.2-n# NAME:phospholipase C, beta 1 (phosphoinositide-specific), nucleus#ABBREVIATION:Plcb1.1-n# citations: AUTHORS:Peruzzi D, Aluigi M, Manzoli L, Billi AM, Di Giorgio FP, Morleo M, Martelli AM, Cocco L#TITLE:Molecular characterization of the human PLC beta1 gene#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12213492# AUTHORS:Caricasole A, Sala C, Roncarati R, Formenti E, Terstappen GC#TITLE:Cloning and characterization of the human phosphoinositide-specific phospholipase C-beta 1 (PLC beta 1)#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:11118617# AUTHORS:Peruzzi D, Calabrese G, Faenza I, Manzoli L, Matteucci A, Gianfrancesco F, Billi AM, Stuppia L, Palka G, Cocco L#TITLE:Identification and chromosomal localisation by fluorescence in situ hybridisation of human gene of phosphoinositide-specific phospholipase C beta(1)#JOURNAL:Biochim Biophy Acta#YEAR:2000#PUBMED:10760467# GENE ASSOCIATION: (23236.2) or (23236.1)
phosphatidylinositol 4-phosphate phospholipase C, nucleus
Abbreviation: R_DM_ethamp_LPAREN_r_RPAREN_ Synonyms: _0 Equation: [r] : ethamp --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MICITDr Synonyms: _0 EC Number: 4.2.1.99 SUBSYSTEM: Propanoate Metabolism Equation: [c] : 2mcacn + h2o <==> micit Confidence Level: 2 JLR NCD genes: none proteins: none citations: AUTHORS:Beach RL, Aogaichi T, Plaut GW.#TITLE:Identification of D-threo-alpha-methylisocitrate as stereochemically specific substrate for bovine heart aconitase and inhibitor of TPN-linked isocitrate dehydrogenase.#JOURNAL:J Biol Chem#YEAR:1977#PUBMED:856801# AUTHORS:Lauble H, Stout CD#TITLE:Steric and conformational features of the aconitase mechanism.#JOURNAL:Proteins#YEAR:1995#PUBMED:7675781# GENE ASSOCIATION:
Abbreviation: R_LNS14DM Synonyms: _2 EC Number: 1.14.14.1 SUBSYSTEM: Cholesterol Metabolism Equation: [c] : (2) h + lanost + (3) nadph + (3) o2 --> 44mctr + for + (4) h2o + (3) nadp Confidence Level: 4 NCD genes: LOCUS:_11283#TRANSCRIPT:1#ABBREVIATION:CYP4F8# proteins: NAME:cytochrome P450, family 4, subfamily F, polypeptide 8#ABBREVIATION:Cyp4f8.1# citations: AUTHORS:Bylund J, Finnstrom N, Oliw EH.#TITLE:Gene expression of a novel cytochrome P450 of the CYP4F subfamily in human seminal vesicles.#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10405341# GENE ASSOCIATION: (11283.1)
cytochrome P450 lanosterol 14-alpha-demethylase (NADP)
Abbreviation: R_CSBPASEly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cspg_b + h2o --> Ser-Gly/Ala-X-Gly + cs_b Confidence Level: 1 Note that this reaction occurs in the endosome during endocytosis, but has been compartmentalized as lysosomal. NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
chondroitin sulfate B proteoglycan protease, lysosome (endosome)
Abbreviation: R_BZt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: bz[e] <==> bz[c] Confidence Level: 1 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
(alpha-D-mannosyl)7-beta-D-mannosyl-diacetylchitobiosyl-L-asparagine, isoform C (protein)_C64H107N2O50X
Abbreviation: R_MM7Cbg Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m7masnC --> m6masnC + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
Abbreviation: R_LNLNCGCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + lnlncgcrn --> crn + lnlncgcoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_DM_avite1_LPAREN_c_RPAREN_ Synonyms: _0 Equation: [c] : avite1 --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NTMELYStner Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: Ntmelys[n] --> Ntmelys[r] Confidence Level: 1 MM genes: none proteins: none citations: none GENE ASSOCIATION:
protein trimethyl lysine transport (nucleus to ER)
Abbreviation: R_PNTKm Synonyms: _0 EC Number: 2.7.1.33 SUBSYSTEM: CoA Biosynthesis Equation: [m] : atp + pnto-R --> 4ppan + adp + h Confidence Level: 4 IT based on In Hoertnagel et al, 2003, Human Mol. genetics, 12(3), 321-327, genes: LOCUS:_80025#TRANSCRIPT:5#ABBREVIATION:PANK2#ECNUMBERS:2.7.1.33# proteins: NAME:Pantothenate kinase 2 (mitochondrial)#ABBREVIATION:PanK2.5# citations: AUTHORS:Daugherty M, Polanuyer B, Farrell M, Scholle M, Lykidis A, de Crecy-Lagard V, Osterman A. #TITLE:Complete reconstitution of the human coenzyme A biosynthetic pathway via comparative genomics.#JOURNAL:J Biol Chem.#YEAR:2002#PUBMED:11923312# AUTHORS:Zhou B, Westaway SK, Levinson B, Johnson MA, Gitschier J, Hayflick SJ. #TITLE:A novel pantothenate kinase gene (PANK2) is defective in Hallervorden-Spatz syndrome.#JOURNAL:Nat Genet. #YEAR:2001#PUBMED:11479594# AUTHORS:Hortnagel K, Prokisch H, Meitinger T. #TITLE:An isoform of hPANK2, deficient in pantothenate kinase-associated neurodegeneration, localizes to mitochondria.#JOURNAL:Hum Mol Genet#YEAR:2003#PUBMED:12554685# GENE ASSOCIATION: (80025.5)
alpha-N-acetylglucosaminyltransferase II
Abbreviation: R_GLCNACT3g Synonyms: _3 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre4 + uacgam --> h + hs_pre5 + udp Confidence Level: 4 EC 2.4.1.224 NCD genes: LOCUS:_2131#TRANSCRIPT:1#ABBREVIATION:EXT1# LOCUS:_2132#TRANSCRIPT:1#ABBREVIATION:EXT2#ECNUMBERS:2.4.1.-# LOCUS:_2137#TRANSCRIPT:1#ABBREVIATION:EXTL3#ECNUMBERS:2.4.1.-# LOCUS:_2134#TRANSCRIPT:1#ABBREVIATION:EXTL1# LOCUS:_2132#TRANSCRIPT:2#ABBREVIATION:EXT2# proteins: NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.1-g# NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.2-g# NAME:exostoses (multiple)-like 1, Golgi apparatus#ABBREVIATION:Extl1.1-g# NAME:exostoses (multiple)-like 3, Golgi apparatus#ABBREVIATION:Extl3.1-g# citations: AUTHORS:Saito T, Seki N, Yamauchi M, Tsuji S, Hayashi A, Kozuma S, Hori T#TITLE:Structure, chromosomal location, and expression profile of EXTR1 and EXTR2, new members of the multiple exostoses gene family#JOURNAL:Biochem Biophys Res Commun#YEAR:1998#PUBMED:9473480# AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Kim BT, Kitagawa H, Tamura J, Saito T, Kusche-Gullberg M, Lindahl U, Sugahara K#TITLE:Human tumor suppressor EXT gene family members EXTL1 and EXTL3 encode alpha 1,4- N-acetylglucosaminyltransferases that likely are involved in heparan sulfate/ heparin biosynthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390981# AUTHORS:Van Hul W, Wuyts W, Hendrickx J, Speleman F, Wauters J, De Boulle K, Van Roy N, Bossuyt P, Willems PJ#TITLE:Identification of a third EXT-like gene (EXTL3) belonging to the EXT gene family#JOURNAL:Genomics#YEAR:1998#PUBMED:9479495# AUTHORS:McCormick C, Duncan G, Goutsos KT, Tufaro F#TITLE:The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10639137# AUTHORS:Kobayashi S, Morimoto K, Shimizu T, Takahashi M, Kurosawa H, Shirasawa T#TITLE:Association of EXT1 and EXT2, hereditary multiple exostoses gene products, in Golgi apparatus#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679296# AUTHORS:Ahn J, Ludecke HJ, Lindow S, Horton WA, Lee B, Wagner MJ, Horsthemke B, Wells DE#TITLE:Cloning of the putative tumour suppressor gene for hereditary multiple exostoses (EXT1)#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7550340# AUTHORS:McCormick C, Leduc Y, Martindale D, Mattison K, Esford LE, Dyer AP, Tufaro F#TITLE:The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate#JOURNAL:Nat Genet#YEAR:1998#PUBMED:9620772# AUTHORS:Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K#TITLE:The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9756849# AUTHORS:Stickens D, Clines G, Burbee D, Ramos P, Thomas S, Hogue D, Hecht JT, Lovett M, Evans GA#TITLE:The EXT2 multiple exostoses gene defines a family of putative tumour suppressor genes#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8782816# AUTHORS:Wise CA, Clines GA, Massa H, Trask BJ, Lovett M#TITLE:Identification and localization of the gene for EXTL, a third member of the multiple exostoses gene family#JOURNAL:Genome Res#YEAR:1997#PUBMED:9037597# GENE ASSOCIATION: (2131.1 and 2132.2) or (2137.1) or (2131.1 and 2132.1) or (2134.1)
heparan sulfate, precursor 4_C51H76N2O42X
Abbreviation: R_PI45P5P Synonyms: _0 EC Number: 3.1.3.36 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail45p_hs --> pail4p_hs + pi Confidence Level: 4 NCD genes: LOCUS:_8867#TRANSCRIPT:1#ABBREVIATION:SYNJ1#ECNUMBERS:3.1.3.56# LOCUS:_3633#TRANSCRIPT:1#ABBREVIATION:INPP5B#ECNUMBERS:3.1.3.56# LOCUS:_4952#TRANSCRIPT:1#ABBREVIATION:OCRL# LOCUS:_56623#TRANSCRIPT:1#ABBREVIATION:INPP5E# LOCUS:_8871#TRANSCRIPT:1#ABBREVIATION:SYNJ2# LOCUS:_4952#TRANSCRIPT:2#ABBREVIATION:OCRL# LOCUS:_8867#TRANSCRIPT:2#ABBREVIATION:SYNJ1# proteins: NAME:oculocerebrorenal syndrome of Lowe#ABBREVIATION:Ocrl.1# NAME:oculocerebrorenal syndrome of Lowe#ABBREVIATION:Ocrl.2# NAME:synaptojanin 2#ABBREVIATION:Synj2.1# NAME:synaptojanin 1#ABBREVIATION:Synj1.1# NAME:inositol polyphosphate-5-phosphatase, 75kDa#ABBREVIATION:Inpp5b.1# NAME:inositol polyphosphate-5-phosphatase, 72 kDa#ABBREVIATION:Inpp5e.1# NAME:synaptojanin 1#ABBREVIATION:Synj1.2# citations: AUTHORS:Zhang X, Jefferson AB, Auethavekiat V, Majerus PW#TITLE:The protein deficient in Lowe syndrome is a phosphatidylinositol-4,5-bisphosphate 5-phosphatase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1995#PUBMED:7761412# AUTHORS:Faucherre A, Desbois P, Satre V, Lunardi J, Dorseuil O, Gacon G#TITLE:Lowe syndrome protein OCRL1 interacts with Rac GTPase in the trans-Golgi network.#JOURNAL:Hum Mol Genet#YEAR:2003#PUBMED:12915445# AUTHORS:Jefferson AB, Majerus PW#TITLE:Properties of type II inositol polyphosphate 5-phosphatase#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7721860# AUTHORS:Kisseleva MV, Wilson MP, Majerus PW#TITLE:The isolation and characterization of a cDNA encoding phospholipid-specific inositol polyphosphate 5-phosphatase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10764818# GENE ASSOCIATION: (8867.1) or (56623.1) or (4952.1) or (8871.1) or (8867.2) or (4952.2) or (3633.1)
phosphatidylinositol-4,5-bisphosphate 5-phosphatase
Abbreviation: R_CYTK13n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : datp + dcmp <==> dadp + dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_ARTFR213 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: c226coa[c] + (3) fadh2[m] + (3) h[m] + (3) nadph[m] --> (1.375) R2coa_hs[c] + (3) fad[m] + (3) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LRAT1 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : pchol_hs + retinol-cis-11 --> 11-cis-retfa + lpchol_hs Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Lecithin retinol acyltransferase (11-cis)
Abbreviation: R_HRETNtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: hretn[c] <==> hretn[n] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
_4-hydroxyretinoic acid transport, Nuclear
Gal-Gal-Gal-Glc-Cer (homo sapiens)_C42H76NO22FULLRCO
Abbreviation: R_B3GNT32g Synonyms: _9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : galthcrm_hs + udpgal --> galgalthcrm_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Zhou D, Dinter A, Gutierrez Gallego R, Kamerling JP, Vliegenthart JF, Berger EG, Hennet T. #TITLE:A beta-1,3-N-acetylglucosaminyltransferase with poly-N-acetyllactosamine synthase activity is structurally related to beta-1,3-galactosyltransferases. #JOURNAL:Proc Natl Acad Sci#YEAR:1999# GENE ASSOCIATION: (10331.1)
Abbreviation: R_EX_prostgd2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : prostgd2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_G6PPer Synonyms: _0 EC Number: 3.1.3.9 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [r] : g6p + h2o --> glc-D + pi Confidence Level: 4 NCD genes: LOCUS:_2538#TRANSCRIPT:1#ABBREVIATION:G6PC#ECNUMBERS:3.1.3.9# LOCUS:_92579#TRANSCRIPT:1#ABBREVIATION:G6PC3# LOCUS:_57818#TRANSCRIPT:1#ABBREVIATION:G6PC2# proteins: NAME:glucose-6-phosphatase, catalytic (glycogen storage disease type I, von Gierke disease), endoplasmic reticulum (liver, kidney, intestine)#ABBREVIATION:G6pc.1-r# NAME:glucose-6-phosphatase, catalytic, 2, endoplasmic reticulum (pancreatic B islets)#ABBREVIATION:G6pc2.1-r# NAME:glucose 6 phosphatase, catalytic, 3, endoplasmic reticulum (ubiquitous)#ABBREVIATION:G6pc3.1-r# citations: AUTHORS:Pan CJ, Lei KJ, Annabi B, Hemrika W, Chou JY#TITLE:Transmembrane topology of glucose-6-phosphatase.#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9497333# AUTHORS:Nordlie, R.C. and Sukalski, K.A.#TITLE:The Enzymes of Biological Membranes#YEAR:1985# AUTHORS:Lei KJ, Shelly LL, Pan CJ, Sidbury JB, Chou JY#TITLE:Mutations in the glucose-6-phosphatase gene that cause glycogen storage disease type 1a#JOURNAL:Science#YEAR:1993#PUBMED:8211187# AUTHORS:Shelly LL, Lei KJ, Pan CJ, Sakata SF, Ruppert S, Schutz G, Chou JY.#TITLE:Isolation of the gene for murine glucose-6-phosphatase, the enzyme deficient in glycogen storage disease type 1A#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8407995# AUTHORS:Shieh JJ, Pan CJ, Mansfield BC, Chou JY#TITLE:The islet-specific glucose-6-phosphatase-related protein, implicated in diabetes, is a glycoprotein embedded in the endoplasmic reticulum membrane#JOURNAL:FEBS Lett#YEAR:2004#PUBMED:15044018# AUTHORS:Arden SD, Zahn T, Steegers S, Webb S, Bergman B, O'Brien RM, Hutton JC.#TITLE:Molecular cloning of a pancreatic islet-specific glucose-6-phosphatase catalytic subunit-related protein.#JOURNAL:Diabetes#YEAR:1999#PUBMED:10078553# AUTHORS:Martin CC, Bischof LJ, Bergman B, Hornbuckle LA, Hilliker C, Frigeri C, Wahl D, Svitek CA, Wong R, Goldman JK, Oeser JK, Lepretre F, Froguel P, O'Brien RM, Hutton JC.#TITLE:Cloning and characterization of the human and rat islet-specific glucose-6-phosphatase catalytic subunit-related protein (IGRP) genes.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11297555# AUTHORS:Martin CC, Oeser JK, Svitek CA, Hunter SI, Hutton JC, O'Brien RM#TITLE:Identification and characterization of a human cDNA and gene encoding a ubiquitously expressed glucose-6-phosphatase catalytic subunit-related protein.#JOURNAL:J Mol Endocrinol#YEAR:2002#PUBMED:12370122# AUTHORS:Shieh JJ, Pan CJ, Mansfield BC, Chou JY.#TITLE:A glucose-6-phosphate hydrolase, widely expressed outside the liver, can explain age-dependent resolution of hypoglycemia in glycogen storage disease type Ia.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:13129915# AUTHORS:Guionie O, Clottes E, Stafford K, Burchell A. #TITLE:Identification and characterisation of a new human glucose-6-phosphatase isoform#JOURNAL:FEBS Lett#YEAR:2003#PUBMED:12965222# GENE ASSOCIATION: (57818.1) or (2538.1) or (92579.1)
glucose-6-phosphate phosphatase, edoplasmic reticular
Abbreviation: R_DADA Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [c] : dad-2 + h + h2o --> din + nh4 Confidence Level: 4 JLR genes: LOCUS:_100#TRANSCRIPT:1#ABBREVIATION:ADA#ECNUMBERS:3.5.4.4# proteins: NAME:adenosine deaminase #ABBREVIATION:Ada.1# citations: AUTHORS:Gonzalez-Gronow M, Hershfield MS, Arredondo-Vega FX, Pizzo SV.#TITLE:Cell surface adenosine deaminase binds and stimulates plasminogen activation on 1-LN human prostate cancer cells.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15016824# AUTHORS:Iwaki-Egawa S, Watanabe Y.#TITLE:Characterization and purification of adenosine deaminase 1 from human and chicken liver.#JOURNAL:Comp Biochem Physiol B Biochem Mol Biol.#YEAR:2002#PUBMED:12381379# GENE ASSOCIATION: (100.1)
Abbreviation: R_11DOCRTSLtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: _11docrtsl[c] <==> 11docrtsl[r] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
_11-deoxycortisol intracellular transport
Abbreviation: R_NACHEXA22ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (3) h2o + ksii_core4_deg3 --> (2) acgam + h + ksii_core2_deg5 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_EX_mercplaccys_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : mercplaccys <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_3-mercaptolactate-cysteine disulfide exchange
Abbreviation: R_SUCD1m Synonyms: _0 EC Number: 1.3.99.1 SUBSYSTEM: Citric Acid Cycle Equation: [m] : fad + succ <==> fadh2 + fum Confidence Level: 0 NCD genes: LOCUS:_6392#TRANSCRIPT:1#ABBREVIATION:SDHD#ECNUMBERS:1.3.5.1# LOCUS:_6391#TRANSCRIPT:1#ABBREVIATION:SDHC#ECNUMBERS:1.3.5.1# LOCUS:_6390#TRANSCRIPT:1#ABBREVIATION:SDHB#ECNUMBERS:1.3.99.1# LOCUS:_6389#TRANSCRIPT:1#ABBREVIATION:SDHA# proteins: NAME:respiratory complex II, succinate dehydrogenase, mitochondrial#ABBREVIATION:Sdh-m# citations: none GENE ASSOCIATION: (6389.1 and 6392.1 and 6391.1 and 6390.1)
Cytochrome P450 11A1, mitochondrial [Precursor]
Abbreviation: R_P45011A1m Synonyms: _4 EC Number: 1.14.15.6 SUBSYSTEM: Steroid Metabolism Equation: [m] : chsterol + h + nadph + (2) o2 --> 4mptnl + (2) h2o + nadp + prgnlone Confidence Level: 4 reaction sequence mx steps, grouped into single reaction uses adrenal ferredoxin NJ genes: LOCUS:_1583#TRANSCRIPT:1#ABBREVIATION:CYP11A1#ECNUMBERS:1.14.15.67# proteins: NAME:cytochrome P450, family 11, subfamily A, polypeptide 1#ABBREVIATION:Cyp11a1.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# AUTHORS:Sakaki T, Inouye K.#TITLE:Practical application of mammalian cytochrome P450.#JOURNAL:J Biosci Bioeng#YEAR:2000#PUBMED:16232916# GENE ASSOCIATION: (1583.1)
Abbreviation: R_A4GNT1g Synonyms: _5 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : T_antigen + uacgam --> gncore1 + h + udp Confidence Level: 0 EC 2.4.1.- NCD genes: LOCUS:_51146#TRANSCRIPT:1#ABBREVIATION:A4GNT# proteins: NAME:alpha-1,4-N-acetylglucosaminyltransferase, Golgi apparatus (stomach, pancreas)#ABBREVIATION:A4gnt.1-g# citations: AUTHORS:Nakayama J, Yeh JC, Misra AK, Ito S, Katsuyama T, Fukuda M.#TITLE:Expression cloning of a human alpha1, 4-N-acetylglucosaminyltransferase that forms GlcNAcalpha1-->4Galbeta-->R, a glycan specifically expressed in the gastric gland mucous cell-type mucin#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1999#PUBMED:10430883# GENE ASSOCIATION: (51146.1)
Abbreviation: R_CATm Synonyms: _4 EC Number: 1.11.1.6 SUBSYSTEM: ROS Detoxification Equation: [m] : (2) h2o2 --> (2) h2o + o2 Confidence Level: 4 TV genes: LOCUS:_847#TRANSCRIPT:1#ABBREVIATION:CAT#ECNUMBERS:1.11.1.6# proteins: NAME:catalase, mitochondrial#ABBREVIATION:Cat.1-m# citations: AUTHORS:Radi R, Turrens JF, Chang LY, Bush KM, Crapo JD, Freeman BA.#TITLE:Detection of catalase in rat heart mitochondria.#JOURNAL:J Biol Chem#YEAR:1991#PUBMED:1657986# AUTHORS:Quan F, Korneluk RG, Tropak MB, Gravel RA.#TITLE:Isolation and characterization of the human catalase gene.#JOURNAL:Nucleic Acids Res#YEAR:1986#PUBMED:3755525# GENE ASSOCIATION: (847.1)
Abbreviation: R_EX_sph1p_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : sph1p <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CLPNDCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: clpndcrn[c] --> clpndcrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_6DHFtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _6dhf[m] --> 6dhf[c] Confidence Level: 2 unknown mechanism. IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_6-glutamyl-DHF transport,m mitochondrial
Abbreviation: R_EX_aprgstrn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : aprgstrn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ME2 Synonyms: _6 EC Number: 1.1.1.40 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : mal-L + nadp --> co2 + nadph + pyr Confidence Level: 0 genes: LOCUS:_4199#TRANSCRIPT:1#ABBREVIATION:ME1#ECNUMBERS:1.1.1.40# proteins: NAME:malic enzyme 1, NADP(+)-dependent, cytosolic#ABBREVIATION:Me1.1# citations: AUTHORS:Loeber G, Infante AA, Maurer-Fogy I, Krystek E, Dworkin MB.#TITLE:Human NAD(+)-dependent mitochondrial malic enzyme. cDNA cloning, primary structure, and expression in Escherichia coli.#JOURNAL:J Biol Chem.#YEAR:1991#PUBMED:1993674# GENE ASSOCIATION: (4199.1)
Abbreviation: R_FAOXC183803m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (5) coa + (2) fad + (5) h2o + lnlncacoa + (5) nad --> (5) accoa + (2) fadh2 + (5) h + (5) nadh + occoa Confidence Level: 4 c18:3, n-3 -> c8:0 - 5 rounds of beta ox, lose 3 degrees of unsat - only 2 fad oxidized 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_P4503A7r Synonyms: _2 SUBSYSTEM: Steroid Metabolism Equation: [r] : h + nadph + o2 + tststerone --> 6htststerone + h2o + nadp Confidence Level: 4 _3 beta hydroxylation of testosterone NJ genes: LOCUS:_1551#TRANSCRIPT:1#ABBREVIATION:CYP3A7# proteins: NAME:cytochrome P450, family 3, subfamily A, polypeptide 7#ABBREVIATION:Cyp3a7.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Komori M, Nishio K, Ohi H, Kitada M, Kamataki T.#TITLE:Molecular cloning and sequence analysis of cDNA containing the entire coding region for human fetal liver cytochrome P-450.#JOURNAL:J Biochem (Tokyo)#YEAR:1989#PUBMED:2722762# GENE ASSOCIATION: (1551.1)
Abbreviation: R_FBP Synonyms: _0 EC Number: 3.1.3.11 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : fdp + h2o --> f6p + pi Confidence Level: 0 SMP genes: LOCUS:_8789#TRANSCRIPT:1#ABBREVIATION:FBP2#ECNUMBERS:2.7.1.11# LOCUS:_2203#TRANSCRIPT:1#ABBREVIATION:FBP1#ECNUMBERS:3.1.3.11# proteins: NAME:fructose-1,6-bisphosphatase 1 (liver)#ABBREVIATION:Fbp1.1# NAME:fructose-1,6-bisphosphatase 2 (muscle)#ABBREVIATION:Fbp2.1# citations: AUTHORS:Tillmann H, Eschrich K#TITLE:Isolation and characterization of an allelic cDNA for human muscle fructose-1,6-bisphosphatase#JOURNAL:Gene#YEAR:1998#PUBMED:9678974# GENE ASSOCIATION: (2203.1) or (8789.1)
Abbreviation: R_INOSTO Synonyms: _4 EC Number: 1.13.99.1 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : inost + o2 --> glcur + h + h2o Confidence Level: 4 NCD genes: LOCUS:_55586#TRANSCRIPT:1#ABBREVIATION:ALDRL6# proteins: NAME:aldehyde reductase (aldose reductase) like 6#ABBREVIATION:Aldrl6.1# citations: AUTHORS:Arner RJ, Prabhu KS, Reddy CC.#TITLE:Molecular cloning, expression, and characterization of myo-inositol oxygenase from mouse, rat, and human kidney#JOURNAL:Biochem Biophys Res Commun#YEAR:2004#PUBMED:15504367# GENE ASSOCIATION: (55586.1)
Abbreviation: R_TYR3MO2 Synonyms: _3 EC Number: 1.14.16.2 SUBSYSTEM: Tyrosine metabolism Equation: [c] : o2 + thbpt + tyr-L --> 34dhphe + thbpt4acam Confidence Level: 4 SAB unlumped form based on PMID 10727395 genes: LOCUS:_7054#TRANSCRIPT:1#ABBREVIATION:TH#ECNUMBERS:1.14.16.2# LOCUS:_7054#TRANSCRIPT:3#ABBREVIATION:TH#ECNUMBERS:1.14.16.2# LOCUS:_7054#TRANSCRIPT:2#ABBREVIATION:TH#ECNUMBERS:1.14.16.2# proteins: NAME:tyrosine hydroxylase#ABBREVIATION:Th.1# NAME:tyrosine hydroxylase#ABBREVIATION:Th.2# NAME:tyrosine hydroxylase#ABBREVIATION:Th.3# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# AUTHORS:Bodeau-Pean S, Ravassard P, Neuner-Jehle M, Faucheux B, Mallet J, Dumas S#TITLE:A human tyrosine hydroxylase isoform associated with progressive supranuclear palsy shows altered enzymatic activity#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9920892# AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (7054.1) or (7054.3) or (7054.2)
docosa-4,7,10,13,16-pentaenoic acid (n-6)_C22H33O2
Abbreviation: R_PPCOAOm Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Propanoate Metabolism Equation: [m] : fad + ppcoa --> fadh2 + prpncoa Confidence Level: 4 NCD genes: LOCUS:_84129#TRANSCRIPT:1#ABBREVIATION:FLJ12592#ECNUMBERS:1.3.99.3# LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# LOCUS:_36#TRANSCRIPT:1#ABBREVIATION:ACADSB#ECNUMBERS:1.3.99.-# LOCUS:_27034#TRANSCRIPT:1#ABBREVIATION:ACAD8# LOCUS:_80724#TRANSCRIPT:1#ABBREVIATION:ACAD10# LOCUS:_28976#TRANSCRIPT:1#ABBREVIATION:ACAD9# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# NAME:acyl-Coenzyme A dehydrogenase, short/branched chain#ABBREVIATION:Acadsb.1-m# NAME:acyl-Coenzyme A dehydrogenase family, member 8#ABBREVIATION:Acad8.1-m# NAME:acyl-Coenzyme A dehydrogenase family, member 9, mitochondrial#ABBREVIATION:Acad9.1-m# NAME:acyl-Coenzyme A dehydrogenase family, member 10, mitochondrial#ABBREVIATION:Acad10.1-m# NAME:putative acyl-CoA dehydrogenase#ABBREVIATION:Flj12592.1-m# citations: AUTHORS:Kelly DP, Kim JJ, Billadello JJ, Hainline BE, Chu TW, Strauss AW#TITLE:Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3035565# AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# AUTHORS:Kelly CL, Hinsdale ME, Wood PA#TITLE:Cloning and characterization of the mouse short-chain acyl-CoA dehydrogenase cDNA#JOURNAL:Genomics#YEAR:1993#PUBMED:8276399# AUTHORS:Rozen R, Vockley J, Zhou L, Milos R, Willard J, Fu K, Vicanek C, Low-Nang L, Torban E, Fournier B#TITLE:Isolation and expression of a cDNA encoding the precursor for a novel member (ACADSB) of the acyl-CoA dehydrogenase gene family#JOURNAL:Genomics#YEAR:1994#PUBMED:7698750# AUTHORS:Nguyen TV, Andresen BS, Corydon TJ, Ghisla S, Abd-El Razik N, Mohsen AW, Cederbaum SD, Roe DS, Roe CR, Lench NJ, Vockley J#TITLE:Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans#JOURNAL:Mol Genet Metab#YEAR:2002#PUBMED:12359132# AUTHORS:Zhang J, Zhang W, Zou D, Chen G, Wan T, Zhang M, Cao X#TITLE:Cloning and functional characterization of ACAD-9, a novel member of human acyl-CoA dehydrogenase family#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12359260# AUTHORS:Ye X, Ji C, Zhou C, Zeng L, Gu S, Ying K, Xie Y, Mao Y#TITLE:Cloning and characterization of a human cDNA ACAD10 mapped to chromosome 12q24.1#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560374# GENE ASSOCIATION: (80724.1) or (27034.1) or (36.1) or (35.1) or (84129.1) or (34.1) or (28976.1)
Propanoyl-CoA:FAD 2,3-oxidoreductase, mitochondrial
heparan sulfate, degradation product 16_C49H72N2O54S4
Abbreviation: R_S2TASE1ly Synonyms: _12 EC Number: 3.1.6.13 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg15 --> h + hs_deg16 + so4 Confidence Level: 4 NCD genes: LOCUS:_3423#TRANSCRIPT:1#ABBREVIATION:IDS#ECNUMBERS:3.1.6.13# LOCUS:_3423#TRANSCRIPT:2#ABBREVIATION:IDS#ECNUMBERS:3.1.6.13# proteins: NAME:iduronate 2-sulfatase (Hunter syndrome), lysosome#ABBREVIATION:Ids.1-l# NAME:iduronate 2-sulfatase (Hunter syndrome), lysosome#ABBREVIATION:Ids.2-l# citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Wilson PJ, Meaney CA, Hopwood JJ, Morris CP#TITLE:Sequence of the human iduronate 2-sulfatase (IDS) gene#JOURNAL:Genomics#YEAR:1993#PUBMED:8244397# AUTHORS:Malmgren H, Carlberg BM, Pettersson U, Bondeson ML#TITLE:dentification of an alternative transcript from the human iduronate-2-sulfatase (IDS) gene#JOURNAL:Genomics#YEAR:1995#PUBMED:8530090# AUTHORS:Wilson PJ, Morris CP, Anson DS, Occhiodoro T, Bielicki J, Clements PR, Hopwood JJ#TITLE:Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990#PUBMED:2122463# GENE ASSOCIATION: (3423.1) or (3423.2)
Abbreviation: R_FUT17g Synonyms: _4 EC Number: 2.4.1.69 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacgalfucgalacglcgal14acglcgalgluside_hs + gdpfuc --> fucgalacgalfucgalacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2523#TRANSCRIPT:1#ABBREVIATION:FUT1#ECNUMBERS:2.4.1.69# proteins: NAME:fucosyltransferase 1#ABBREVIATION:Fut1.1# citations: AUTHORS:Larsen RD, Ernst LK, Nair RP, Lowe JB.#TITLE:Molecular cloning, sequence, and expression of a human GDP-L-fucose:beta-D-galactoside 2-alpha-L-fucosyltransferase cDNA that can form the H blood group antigen.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990#PUBMED:2118655# GENE ASSOCIATION: (2523.1)
(Gal)4 (GalNAc)1 (Glc)1 (GlcNAc)2 (LFuc)2 (Cer)1_C84H145N4O50FULLRCO
(Gal)4 (GalNAc)1 (Glc)1 (GlcNAc)2 (LFuc)1 (Cer)1_C78H135N4O46FULLRCO
Abbreviation: R_FAOXC1811603m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (5) coa + (4) fad + (5) h2o + (5) nad + odecoa --> (5) accoa + (4) fadh2 + (5) h + (5) nadh + occoa Confidence Level: 4 _5 rounds of beta ox, 1 loss of degree of unsat -> 4 fad oxidized c18:1 -> c8:0 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_CYTK1n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : atp + cmp <==> adp + cdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_H7MTer_U Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_U + em2emgacpail_hs --> (0.1) dolp_U + h + mem2emgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_80235#TRANSCRIPT:1#ABBREVIATION:SMP3# proteins: NAME:SMP3 mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Smp3.1-r# citations: AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (80235.1)
Abbreviation: R_METt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: met-L[e] + na1[e] --> met-L[c] + na1[c] Confidence Level: 4 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# LOCUS:_6584#TRANSCRIPT:1#ABBREVIATION:SLC22A5# proteins: NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# NAME:solute carrier family 22 (organic cation transporter), member 5#ABBREVIATION:Slc22a5.1# citations: AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# GENE ASSOCIATION: (81539.1) or (11254.1) or (54407.1) or (6584.1)
L-methionine transport in via sodium symport
keratan sulfate II (core 2-linked), degradation product 2_C70H114N5O56S2X
Abbreviation: R_GALASE16ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksii_core2_deg2 --> (2) gal + ksii_core2_deg3 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_5ADTSTSTERONEGLCte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _5adtststeroneglc[c] + atp[c] + h2o[c] --> 5adtststeroneglc[e] + adp[c] + h[c] + pi[c] Confidence Level: 2 presumed MDR transporter NJ genes: LOCUS:_4363#TRANSCRIPT:1#ABBREVIATION:ABCC1.1# proteins: NAME:ATP-binding cassette, subfamily C (CFTR/MRP), member 1#ABBREVIATION:ABCC1.1# citations: none GENE ASSOCIATION: (4363.1)
Abbreviation: R_CHOLtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: chol[c] <==> chol[n] Confidence Level: 2 it genes: none proteins: none citations: none GENE ASSOCIATION:
Choline transport, nuclear through pores
Abbreviation: R_HSD17B8r Synonyms: _1 EC Number: 1.1.1.62 SUBSYSTEM: Steroid Metabolism Equation: [r] : estrone + h + nadh --> estradiol + nad Confidence Level: 4 microsomal NAD as cofactor HSD type 2 recheck/confirm comparmentalization - type 1: cytosol, types 2,3: microsomes NJ genes: LOCUS:_3294#TRANSCRIPT:1#ABBREVIATION:HSD17B2# proteins: NAME:hydroxysteroid (17-beta) dehydrogenase 2#ABBREVIATION:Hsd17b2.1# citations: AUTHORS:Wu L, Einstein M, Geissler WM, Chan HK, Elliston KO, Andersson S.#TITLE:Expression cloning and characterization of human 17 beta-hydroxysteroid dehydrogenase type 2, a microsomal enzyme possessing 20 alpha-hydroxysteroid dehydrogenase activity.#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8099587# GENE ASSOCIATION: (3294.1)
Abbreviation: R_S23Tg Synonyms: _0 EC Number: 2.4.99.4 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : T_antigen + cmpacna --> cmp + h + sT_antigen Confidence Level: 4 NCD genes: LOCUS:_6482#TRANSCRIPT:1#ABBREVIATION:SIAT4A#ECNUMBERS:2.4.99.4# LOCUS:_6482#TRANSCRIPT:2#ABBREVIATION:SIAT4A#ECNUMBERS:2.4.99.4# proteins: NAME:sialyltransferase 4A (beta-galactoside alpha-2,3-sialyltransferase), Golgi apparatus (placenta, liver, sk muscle)#ABBREVIATION:Siat4a.1-g# NAME:_3-sialyltransferase), Golgi apparatus (placenta, liver, sk muscle)#ABBREVIATION:Siat4a.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Kitagawa H, Paulson JC#TITLE:Differential expression of five sialyltransferase genes in human tissues#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8027041# AUTHORS:Shang J, Qiu R, Wang J, Liu J, Zhou R, Ding H, Yang S, Zhang S, Jin C#TITLE:Molecular cloning and expression of Galbeta1,3GalNAc alpha2, 3-sialyltransferase from human fetal liver#JOURNAL:Eur J Biochem#YEAR:1999#PUBMED:10504389# GENE ASSOCIATION: (6482.2) or (6482.1)
beta-galactoside alpha-2,3-sialyltransferase (T antigen)
Abbreviation: R_HACD9m Synonyms: _0 EC Number: 1.1.1.35 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 3hmbcoa + nad <==> 2maacoa + h + nadh Confidence Level: 4 MM genes: LOCUS:_3028#TRANSCRIPT:1#ABBREVIATION:HADH2# proteins: NAME:hydroxyacyl-Coenzyme A dehydrogenase, type II#ABBREVIATION:Hadh2.1-m# citations: AUTHORS:Sweetman, L, Williams, JC#TITLE:Branched chain organic acidurias#JOURNAL:The Metabolic and Molecular Bases of Inherited Disease, 8th ed (Scriver CR, et al., editors)#YEAR:2001# GENE ASSOCIATION: (3028.1)
_3-hydroxyacyl-CoA dehydrogenase (2-Methylacetoacetyl-CoA), mitochondrial
Abbreviation: R_PETOHMm_hs Synonyms: _0 EC Number: 2.1.1.17 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [m] : (3) amet + pe_hs --> (3) ahcys + (3) h + pchol_hs Confidence Level: 2 for human Catalyzes three sequential methylation of phosphatidylethanolamine (PE) by AdoMet, thus producing phosphatidylcholine (PC). [Swiss-prot] NJ genes: LOCUS:_10400#TRANSCRIPT:1#ABBREVIATION:PEMT#ECNUMBERS:2.1.1.17# LOCUS:_10400#TRANSCRIPT:2#ABBREVIATION:PEMT#ECNUMBERS:2.1.1.17# LOCUS:_10400#TRANSCRIPT:3#ABBREVIATION:PEMT#ECNUMBERS:2.1.1.17# proteins: NAME:phosphatidylethanolamine N-methyltransferase #ABBREVIATION:Pemt.1# NAME:phosphatidylethanolamine N-methyltransferase #ABBREVIATION:Pemt.2# NAME:phosphatidylethanolamine N-methyltransferase #ABBREVIATION:Pemt.3# citations: AUTHORS:Walkey CJ, Shields DJ, Vance DE.#TITLE:Identification of three novel cDNAs for human phosphatidylethanolamine N-methyltransferase and localization of the human gene on chromosome 17p11.2.#JOURNAL:Biochim Biophys Acta.#YEAR:1999#PUBMED:9989271# GENE ASSOCIATION: (10400.2) or (10400.1) or (10400.3)
phosphatidylethanolamine N-methyltransferase
Abbreviation: R_GLNSERNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gln-L[e] + na1[e] + ser-L[c] --> gln-L[c] + na1[c] + ser-L[e] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-serine/L-glutamine Na-dependent exchange (Gln-L in)
Abbreviation: R_EX_tyr_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tyr-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ORNDC Synonyms: _0 EC Number: 4.1.1.17 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : h + orn --> co2 + ptrc Confidence Level: 4 JLR- added proton to balance genes: LOCUS:_4953#TRANSCRIPT:1#ABBREVIATION:ODC1#ECNUMBERS:4.1.1.17# proteins: NAME:ornithine decarboxylase 1#ABBREVIATION:Odc1.1# citations: AUTHORS:Zhu MY, Iyo A, Piletz JE, Regunathan S#TITLE:Expression of human arginine decarboxylase, the biosynthetic enzyme for agmatine#JOURNAL:Biochim Biophys Acta#YEAR:2004# AUTHORS:Hsieh JT, Denning MF, Heidel SM, Verma AK.#TITLE:Expression of human chromosome 2 ornithine decarboxylase gene in ornithine decarboxylase-deficient Chinese hamster ovary cells#JOURNAL:Cancer Res#YEAR:1990#PUBMED:2317811# GENE ASSOCIATION: (4953.1)
Abbreviation: R_BAAT2x Synonyms: _1 EC Number: 2.3.1.65 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : cholcoa + taur --> coa + tchola Confidence Level: 4 glycine or taurine as substrates, produces glycocholic or taurocholic acids respectively NJ genes: LOCUS:_570#TRANSCRIPT:1#ABBREVIATION:BAAT#ECNUMBERS:2.3.1.65# proteins: NAME:bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase)#ABBREVIATION:Baat.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Falany CN, Johnson MR, Barnes S, Diasio RB#TITLE:Glycine and taurine conugation of bile acids by a single enzyme#JOURNAL:Journal of Biological Chemistry#YEAR:1994# GENE ASSOCIATION: (570.1)
bile acid Coenzyme A: amino acid N-acyltransferase
keratan sulfate I biosynthesis, precursor 6_C107H174N7O80SX
Abbreviation: R_DHDPBMTm Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 3dpdhb + amet --> 3dpdhb_me + ahcys + h Confidence Level: 4 Used to make Ubiquinone-10 EC 2.1.1.114 IT genes: LOCUS:_51805#TRANSCRIPT:1#ABBREVIATION:COQ3# proteins: NAME:coenzyme Q3 homolog, methyltransferase (yeast) #ABBREVIATION:Coq3.1-m# citations: AUTHORS:Kang DC, Takeshige K, Minakami S.#TITLE:An intermediate of ubiquinone biosynthesis exists in the microsomal fraction of HepG2 cells. #JOURNAL:J Biochem (Tokyo).#YEAR:1990#PUBMED:1965190# AUTHORS:Nambudiri AM, Ranganathan S, Rudney H#TITLE:The role of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in the regulation of ubiquinone synthesis in human fibroblasts. #JOURNAL:J Biol Chem.#YEAR:1980#PUBMED:7380842# AUTHORS:Jonassen T, Clarke CF.#TITLE:Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis. #JOURNAL:J Biol Chem.#YEAR:2000#PUBMED:10777520# GENE ASSOCIATION: (51805.1)
dihydroxydecaprenylbenzoate methyltransferase
Abbreviation: R_Uritl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: uri[l] <==> uri[c] Confidence Level: 4 IT genes: LOCUS:_55315#TRANSCRIPT:1#ABBREVIATION:SLC29A3# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 3 #ABBREVIATION:Slc29a3.1-l# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Pisoni RL, Thoene JG.#TITLE:Detection and characterization of a nucleoside transport system in human fibroblast lysosomes.#JOURNAL:J Biol Chem.#YEAR:1989#PUBMED:2925670# AUTHORS:Baldwin SA, Yao SY, Hyde RJ, Ng AM, Foppolo S, Barnes K, Ritzel MW, Cass CE, Young JD.#TITLE:Functional characterization of novel human and mouse equilibrative nucleoside transporters (hENT3 and mENT3) located in intracellular membranes.#JOURNAL:J Biol Chem. #YEAR:2005#PUBMED:15701636# GENE ASSOCIATION: (55315.1)
uridine facilated transport from lysosome
Abbreviation: R_EX_tettet6_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tettet6 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SARCStex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: sarcs[e] --> sarcs[c] Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Glorieux FH, Scriver CR, Delvin E, Mohyuddin F.#TITLE:Transport and metabolism of sarcosine in hypersarcosinemic and normal phenotypes.#YEAR:1971#PUBMED:5096515# GENE ASSOCIATION:
Sarcosine transport (extracellular to cytosol)
Abbreviation: R_H2Otm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h2o[c] <==> h2o[m] Confidence Level: 4 IF genes: LOCUS:_343#TRANSCRIPT:1#ABBREVIATION:AQP8# proteins: NAME:Aquaporin 8 (mitochondrial) #ABBREVIATION:Aqp8.1-m# citations: AUTHORS:Calamita G, Ferri D, Gena P, Liquori GE, Cavalier A, Thomas D, Svelto M.#TITLE:The inner mitochondrial membrane has aquaporin-8 water channels and is highly permeable to water. #JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15749715# GENE ASSOCIATION: (343.1)
Abbreviation: R_ST3GAL22g Synonyms: _7 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gm1_hs --> cmp + gd1a_hs + h Confidence Level: 4 NJ genes: LOCUS:_6483#TRANSCRIPT:1#ABBREVIATION:SIAT4B#ECNUMBERS:2.4.99.4# proteins: NAME:sialyltransferase 4B, Golgi apparatus#ABBREVIATION:Siat4b.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Giordanengo V, Bannwarth S, Laffont C, Van Miegem V, Harduin-Lepers A, Delannoy P, Lefebvre JC. #TITLE:Cloning and expression of cDNA for a human Gal(beta1-3)GalNAc alpha2,3-sialyltransferase from the CEM T-cell line. #JOURNAL:Eur J Biochem#YEAR:1997#PUBMED:9266697# GENE ASSOCIATION: (6483.1)
CMP-N-acetylneuraminate-beta-galactosamide-alpha-2,3-sialyltransferase, Golgi apparatus
chondroitin sulfate C (GalNAc6S-GlcA), precursor 2_C37H54NO37S2X
heparan sulfate, degradation product 4_C73H104N4O92S10
Abbreviation: R_GLCNACASE1ly Synonyms: _4 EC Number: 3.2.1.50 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg3 --> acgam + hs_deg4 Confidence Level: 4 NCD genes: LOCUS:_4669#TRANSCRIPT:1#ABBREVIATION:NAGLU#ECNUMBERS:3.2.1.50# proteins: NAME:N-acetylglucosaminidase, alpha- (Sanfilippo disease IIIB), lysosome#ABBREVIATION:Naglu.1-l# citations: AUTHORS:Weber B, Blanch L, Clements PR, Scott HS, Hopwood JJ#TITLE:Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B)#JOURNAL:Hum Mol Genet#YEAR:1996#PUBMED:8776591# AUTHORS:Sasaki T, Sukegawa K, Masue M, Fukuda S, Tomatsu S, Orii T#TITLE:Purification and partial characterization of alpha-N-acetylglucosaminidase from human liver#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1783617# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION: (4669.1)
alpha-N-acetylglucosaminidase, lysosomal
Abbreviation: R_NDPK9n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + idp <==> adp + itp Confidence Level: 4 NCD genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:IDP), nuclear
Abbreviation: R_GPIAT Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [c] : gpail_hs + pmtcoa --> coa + gacpail_hs Confidence Level: 4 EC 2.3.-.- NCD genes: LOCUS:_284098#TRANSCRIPT:1#ABBREVIATION:PIGW# proteins: NAME:phosphatidylinositol glycan, class W#ABBREVIATION:Pigw.1# citations: AUTHORS:Murakami Y, Siripanyapinyo U, Hong Y, Kang JY, Ishihara S, Nakakuma H, Maeda Y, Kinoshita T#TITLE:PIG-W is critical for inositol acylation but not for flipping of glycosylphosphatidylinositol-anchor#JOURNAL:Mol Biol Cell#YEAR:2003#PUBMED:14517336# GENE ASSOCIATION: (284098.1)
glucosaminylphosphatidyl inositol acetyltransferase
Abbreviation: R_DNDPt44m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + dttp[c] --> atp[c] + dttp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_P45011B21m Synonyms: _3 EC Number: 1.14.15.4 SUBSYSTEM: Steroid Metabolism Equation: [m] : 11docrtstrn + h + nadph + (2) o2 --> aldstrn + (2) h2o + nadp Confidence Level: 4 Payne and Hales (2004) Mol Bio of Steroidogenic Enzymes 25(6):947-970 H2O added for balancing grouped reaction - actually 2 step (2 intermediates), but enzyme carries them all out sequentially NJ genes: LOCUS:_1585#TRANSCRIPT:1#ABBREVIATION:CYP11B2#ECNUMBERS:1.14.15.4# proteins: NAME:cytochrome P450, family 11, subfamily B, polypeptide 2#ABBREVIATION:Cyp11b2.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (1585.1)
Abbreviation: R_PTDCACRNCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + ptdcacrn --> crn + ptdcacoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# AUTHORS:Fraser F, Padovese R, Zammit VA.#TITLE:Distinct kinetics of carnitine palmitoyltransferase i in contact sites and outer membranes of rat liver mitochondria.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11274214# GENE ASSOCIATION: (1376.1)
pentadecanoate transport into the mitochondria
Abbreviation: R_TRDR2 Synonyms: _0 EC Number: 1.8.1.9 SUBSYSTEM: Miscellaneous Equation: [c] : h + nadph + q10 --> nadp + q10h2 Confidence Level: 4 Xia et al, 2003, JBC, 2141-2146 genes: LOCUS:_7296#TRANSCRIPT:1#ABBREVIATION:TXNRD1# LOCUS:_7296#TRANSCRIPT:4#ABBREVIATION:TXNRD1# LOCUS:_7296#TRANSCRIPT:2#ABBREVIATION:TXNRD1# LOCUS:_7296#TRANSCRIPT:3#ABBREVIATION:TXNRD1# proteins: NAME:thioredoxin reductase 1 #ABBREVIATION:Txnrd1.1# NAME:thioredoxin reductase 1#ABBREVIATION:Txnrd1.2# NAME:thioredoxin reductase 1#ABBREVIATION:Txnrd1.3# NAME:thioredoxin reductase 1#ABBREVIATION:Txnrd1.4# citations: AUTHORS:Tamura T, Stadtman TC.#TITLE:A new selenoprotein from human lung adenocarcinoma cells: purification, properties, and thioredoxin reductase activity. #JOURNAL:Proc Natl Acad Sci U S A. #YEAR:1996#PUBMED:8577704# AUTHORS:Koishi R, Kawashima I, Yoshimura C, Sugawara M, Serizawa N.#TITLE:Cloning and characterization of a novel oxidoreductase KDRF from a human bone marrow-derived stromal cell line KM-102. #JOURNAL:J Biol Chem. #YEAR:1997#PUBMED:8999974# AUTHORS:Xia L, Nordman T, Olsson JM, Damdimopoulos A, Bjorkhem-Bergman L, Nalvarte I, Eriksson LC, Arner ES, Spyrou G, Bjornstedt M.#TITLE:The mammalian cytosolic selenoenzyme thioredoxin reductase reduces ubiquinone. A novel mechanism for defense against oxidative stress.#JOURNAL:J Biol Chem.#YEAR:2003#PUBMED:12435734# GENE ASSOCIATION: (7296.1) or (7296.4) or (7296.2) or (7296.3)
Thioredoxin (ubiquinone 10) reductase (NADPH)
Abbreviation: R_EX_retinol_DASH_cis_DASH_11_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : retinol-cis-11 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NTD5m Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [m] : dtmp + h2o --> pi + thymd Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_56953#TRANSCRIPT:1#ABBREVIATION:NT5M#ECNUMBERS:3.1.3.5# proteins: NAME:_5',3'-nucleotidase, mitochondrial #ABBREVIATION:Nt5M.1-m# citations: AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION: (56953.1)
Abbreviation: R_EX_idp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : idp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_BTNDe Synonyms: _0 EC Number: 3.5.1.12 SUBSYSTEM: Biotin Metabolism Equation: [e] : biocyt + h2o --> btn + lys-L Confidence Level: 4 Also acts on biotin esters IT genes: LOCUS:_686#TRANSCRIPT:1#ABBREVIATION:BTD#ECNUMBERS:3.5.1.12# proteins: NAME:biotinidase #ABBREVIATION:Btd.1-e# citations: AUTHORS:Cole H, Weremowicz S, Morton CC, Wolf B.#TITLE:Localization of serum biotinidase (BTD) to human chromosome 3 in band p25. #JOURNAL:Genomics#YEAR:1994#PUBMED:8001986# AUTHORS:Cole H, Reynolds TR, Lockyer JM, Buck GA, Denson T, Spence JE, Hymes J, Wolf B. #TITLE:Human serum biotinidase. cDNA cloning, sequence, and characterization.#JOURNAL:J Biol Chem. #YEAR:1994#PUBMED:7509806# AUTHORS:Pacheco-Alvarez D, Solorzano-Vargas RS, Del Rio AL.#TITLE:Biotin in metabolism and its relationship to human disease.#JOURNAL:Arch Med Res. #YEAR:2002#PUBMED:12459313# GENE ASSOCIATION: (686.1)
Abbreviation: R_Rtotaltl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: Rtotal[c] <==> Rtotal[l] Confidence Level: 1 unknown mechanism for transport - carnitine transporters not found, unknown mechanism intracellularly, however there must be a mechanism for FA transport NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_VACCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: vacc[e] <==> vacc[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_M16NTg Synonyms: _8 EC Number: 2.4.1.143 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : nm2masn + uacgam --> h + n2m2masn + udp Confidence Level: 0 NCD genes: LOCUS:_4247#TRANSCRIPT:1#ABBREVIATION:MGAT2#ECNUMBERS:2.4.1.143# proteins: NAME:mannosyl (alpha-1,6-)-glycoprotein beta-1,2-N-acetylglucosaminyltransferase, Golgi apparatus (sm intestine, liver, stomach, kidney, colon)#ABBREVIATION:Mgat2.1-g# citations: AUTHORS:Yen CL, Farese RV Jr.#TITLE:MGAT2, a monoacylglycerol acyltransferase expressed in the small intestine#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12621063# GENE ASSOCIATION: (4247.1)
alpha-1,6-mannosyl-glycoprotein 2-beta-N-acetylglucosaminyltransferase
Abbreviation: R_NDPK3 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + cdp <==> adp + ctp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.2) or (10201.1) or (4831.1 and 4830.1) or (4832.1) or (4831.1 and 4830.2) or (29922.1)
keratan sulfate I biosynthesis, precursor 36_C247H394N17O210S11X
Abbreviation: R_S6T15g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre36 + paps --> h + ksi + pap Confidence Level: 4 NCD genes: LOCUS:_8534#TRANSCRIPT:1#ABBREVIATION:CHST1# LOCUS:_9469#TRANSCRIPT:1#ABBREVIATION:CHST3# proteins: NAME:carbohydrate (keratan sulfate Gal-6) sulfotransferase 1, Golgi#ABBREVIATION:Chst1.1-g# NAME:carbohydrate (chondroitin 6) sulfotransferase 3, Golgi#ABBREVIATION:Chst3.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Habuchi O, Hirahara Y, Uchimura K, Fukuta M#TITLE:Enzymatic sulfation of galactose residue of keratan sulfate by chondroitin 6-sulfotransferase#JOURNAL:Glycobiology#YEAR:1996#PUBMED:8991509# AUTHORS:Fukuta M, Kobayashi Y, Uchimura K, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of human chondroitin 6-sulfotransferase#JOURNAL:Biochim Biophys Acta#YEAR:1998#PUBMED:9714738# AUTHORS:Fukuta M, Inazawa J, Torii T, Tsuzuki K, Shimada E, Habuchi O#TITLE:Molecular cloning and characterization of human keratan sulfate Gal-6-sulfotransferase#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405439# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (8534.1) or (9469.1)
Abbreviation: R_EX_pydxn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pydxn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I, degradation product 11_C180H289N12O158S9
Abbreviation: R_GALASE5ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg11 --> gal + ksi_deg12 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_ADKd Synonyms: _0 EC Number: 2.7.4.3 SUBSYSTEM: Nucleotides Equation: [c] : damp + datp <==> (2) dadp Confidence Level: 4 Inorganic triphosphate can also act as donor This form was added for Staph based on KEGG evidence (SAB) genes: LOCUS:_26289#TRANSCRIPT:1#ABBREVIATION:AK5# LOCUS:_26289#TRANSCRIPT:2#ABBREVIATION:AK5# proteins: NAME:adenylate kinase 5 #ABBREVIATION:Ak5.1# NAME:adenylate kinase 5 #ABBREVIATION:Ak5.2# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Identification of a novel human adenylate kinase. cDNA cloning, expression analysis, chromosome localization and characterization of the recombinant protein. #JOURNAL:Eur J Biochem. #YEAR:1999#PUBMED:10215863# GENE ASSOCIATION: (26289.2) or (26289.1)
Abbreviation: R_TALA Synonyms: _0 EC Number: 2.2.1.2 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : g3p + s7p <==> e4p + f6p Confidence Level: 0 SMP genes: LOCUS:_6888#TRANSCRIPT:1#ABBREVIATION:TALDO1# proteins: NAME:transaldolase 1#ABBREVIATION:Taldo1.1# citations: none GENE ASSOCIATION: (6888.1)
Abbreviation: R_GTHOm Synonyms: _0 EC Number: 1.8.1.7 SUBSYSTEM: Glutamate metabolism Equation: [m] : gthox + h + nadph --> (2) gthrd + nadp Confidence Level: 2 NCD, TV This EC (1.6.4.2) is obsolete. The new EC is 1.8.1.7. http://www.genome.ad.jp/dbget-bin/www_bget?ec:1.6.4.2 genes: LOCUS:_2936#TRANSCRIPT:1#ABBREVIATION:GSR#ECNUMBERS:1.6.4.2# proteins: NAME:glutathione reductase (mitochondrial)#ABBREVIATION:Gsr.1-m# citations: AUTHORS:Kelner MJ, Montoya MA.#TITLE:Structural organization of the human glutathione reductase gene: determination of correct cDNA sequence and identification of a mitochondrial leader sequence. #JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10708558# AUTHORS:Tramer F, Caponecchia L, Sgro P, Martinelli M, Sandri G, Panfili E, Lenzi A, Gandini L.#TITLE:Native specific activity of glutathione peroxidase (GPx-1), phospholipid hydroperoxide glutathione peroxidase (PHGPx) and glutathione reductase (GR) does not differ between normo- and hypomotile human sperm samples.#JOURNAL:Int J Androl.#YEAR:2004#PUBMED:15149466# GENE ASSOCIATION: (2936.1)
Abbreviation: R_ACNGALACGLCGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acngalacglcgal14acglcgalgluside_hs[e] <==> acngalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I biosynthesis, precursor 26_C199H318N14O166S8X
Abbreviation: R_G14T14g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre26 + udpgal --> h + ksi_pre27 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (8704.2) or (8704.1) or (2683.1) or (9334.1)
keratan sulfate I biosynthesis, precursor 27_C205H328N14O171S8X
glycogen, structure 1 (glycogenin-11[1,4-Glc])_C66H111O56X
Abbreviation: R_PI4P3K Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + pail4p_hs --> adp + h + pail34p_hs Confidence Level: 4 NCD Notice that contrary to some standards in texts, KEGG, etc, the Rtotal group accounts for the whole fatty acyl CoA (so the CHO only accounts for the core backbone), manner in which Rtotal was produced accounts for O and H balances. Formation of the Rtotal group (rxn flux)accounted for mass balance for CoA NJ changed EC from 2.7.1.149 (pail5p) to 2.7.1.68 (pail4p) - NCD genes: LOCUS:_5291#TRANSCRIPT:1#ABBREVIATION:PIK3CB#ECNUMBERS:2.7.1.137# LOCUS:_5290#TRANSCRIPT:1#ABBREVIATION:PIK3CA#ECNUMBERS:2.7.1.137# LOCUS:_5287#TRANSCRIPT:1#ABBREVIATION:PIK3C2B#ECNUMBERS:2.7.1.137# LOCUS:_5293#TRANSCRIPT:1#ABBREVIATION:PIK3CD#ECNUMBERS:2.7.1.137# LOCUS:_5286#TRANSCRIPT:1#ABBREVIATION:PIK3C2A#ECNUMBERS:2.7.1.137# LOCUS:_5294#TRANSCRIPT:1#ABBREVIATION:PIK3CG#ECNUMBERS:2.7.1.137# LOCUS:_23533#TRANSCRIPT:1#ABBREVIATION:P101-PI3K# LOCUS:_5288#TRANSCRIPT:1#ABBREVIATION:PIK3C2G#ECNUMBERS:2.7.1.137# LOCUS:_5295#TRANSCRIPT:1#ABBREVIATION:PIK3R1# LOCUS:_8503#TRANSCRIPT:1#ABBREVIATION:PIK3R3# LOCUS:_5296#TRANSCRIPT:1#ABBREVIATION:PIK3R2# LOCUS:_5295#TRANSCRIPT:2#ABBREVIATION:PIK3R1# LOCUS:_5295#TRANSCRIPT:3#ABBREVIATION:PIK3R1# LOCUS:_10870#TRANSCRIPT:1#ABBREVIATION:HCST# proteins: NAME:phosphoinositide-3-kinase (p110a, p85a)#ABBREVIATION:Pik3car1.1# NAME:phosphoinositide-3-kinase (p110a, p55a)#ABBREVIATION:Pik3car1.2# NAME:phosphoinositide-3-kinase (p110a, p50a)#ABBREVIATION:Pik3car.3# NAME:phosphoinositide-3-kinase (p110a, p85b)#ABBREVIATION:Pik3car2.1# NAME:phosphoinositide-3-kinase (p110a, p55c)#ABBREVIATION:Pik3car3.1# NAME:phosphoinositide-3-kinase (p110b, p85a)#ABBREVIATION:Pik3cbr1.1# NAME:phosphoinositide-3-kinase (p110b, p55a)#ABBREVIATION:Pik3cbr1.2# NAME:phosphoinositide-3-kinase (p110b, p50a)#ABBREVIATION:Pik3cbr1.3# NAME:phosphoinositide-3-kinase (p110b, p85b)#ABBREVIATION:Pik3cbr2.1# NAME:phosphoinositide-3-kinase (p110b, p55c)#ABBREVIATION:Pik3cbr3.1# NAME:phosphoinositide-3-kinase (p110c, p85a)#ABBREVIATION:Pik3cdr1.1# NAME:phosphoinositide-3-kinase (p110c, p55a)#ABBREVIATION:Pik3cdr1.2# NAME:phosphoinositide-3-kinase (p110c, p50a)#ABBREVIATION:Pik3cdr1.3# NAME:phosphoinositide-3-kinase (p110c, p85b)#ABBREVIATION:Pik3cdr2.1# NAME:phosphoinositide-3-kinase (p110c, p55c)#ABBREVIATION:Pik3cdr3.1# NAME:phosphoinositide-3-kinase (PIK3cg, p101)#ABBREVIATION:Pik3cgr5.1# NAME:phosphoinositide-3-kinase (PIK3CG, p87 adapter protein)#ABBREVIATION:Pik3cgap.1# NAME:phosphoinositide-3-kinase, class 2, alpha polypeptide#ABBREVIATION:Pik3c2a.1# NAME:phosphoinositide-3-kinase, class 2, beta polypeptide#ABBREVIATION:Pik3c2b.1# NAME:phosphoinositide-3-kinase, class 2, gamma polypeptide#ABBREVIATION:Pik3c2g.1# citations: AUTHORS:Volinia S, Hiles I, Ormondroyd E, Nizetic D, Antonacci R, Rocchi M, Waterfield MD#TITLE:Molecular cloning, cDNA sequence, and chromosomal localization of the human phosphatidylinositol 3-kinase p110 alpha (PIK3CA) gene#JOURNAL:Genomics#YEAR:1994#PUBMED:7713498# AUTHORS:Hu P, Mondino A, Skolnik EY, Schlessinger J#TITLE:Cloning of a novel, ubiquitously expressed human phosphatidylinositol 3-kinase and identification of its binding site on p85#JOURNAL:Mol Cell Biol#YEAR:1993#PUBMED:8246984# AUTHORS:Vanhaesebroeck B, Welham MJ, Kotani K, Stein R, Warne PH, Zvelebil MJ, Higashi K, Volinia S, Downward J, Waterfield MD#TITLE:P110delta, a novel phosphoinositide 3-kinase in leukocytes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9113989# AUTHORS:Stoyanov B, Volinia S, Hanck T, Rubio I, Loubtchenkov M, Malek D, Stoyanova S, Vanhaesebroeck B, Dhand R, Nurnberg B, et al#TITLE:Cloning and characterization of a G protein-activated human phosphoinositide-3 kinase#JOURNAL:Science#YEAR:1995#PUBMED:7624799# AUTHORS:Domin J, Pages F, Volinia S, Rittenhouse SE, Zvelebil MJ, Stein RC, Waterfield MD#TITLE:Cloning of a human phosphoinositide 3-kinase with a C2 domain that displays reduced sensitivity to the inhibitor wortmannin#JOURNAL:Biochem J#YEAR:1997#PUBMED:9337861# AUTHORS:Arcaro A, Volinia S, Zvelebil MJ, Stein R, Watton SJ, Layton MJ, Gout I, Ahmadi K, Downward J, Waterfield MD#TITLE:Human phosphoinositide 3-kinase C2beta, the role of calcium and the C2 domain in enzyme activity#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9830063# AUTHORS:Rozycka M, Lu YJ, Brown RA, Lau MR, Shipley JM, Fry MJ#TITLE:cDNA cloning of a third human C2-domain-containing class II phosphoinositide 3-kinase, PI3K-C2gamma, and chromosomal assignment of this gene (PIK3C2G) to 12p12#JOURNAL:Genomics#YEAR:1998#PUBMED:9878262# GENE ASSOCIATION: (5291.1 and 5295.3) or (5296.1 and 5290.1) or (5293.1 and 5296.1) or (5287.1) or (5286.1) or (5293.1 and 8503.1) or (5293.1 and 5295.3) or (8503.1 and 5291.1) or (5295.2 and 5290.1) or (5288.1) or (5291.1 and 5295.1) or (5296.1 and 5291.1) or (23533.1 and 5294.1) or (5293.1 and 5295.1) or (5295.2 and 5293.1) or (5290.1 and 8503.1) or (5290.1 and 5295.3) or (10870.1 and 5294.1) or (5295.2 and 5291.1) or (5290.1 and 5295.1)
phosphatidylinositol 4-phosphate 3-kinase
Abbreviation: R_DOLPMT_L Synonyms: _0 EC Number: 2.4.1.83 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dolp_L + gdpmann --> (0.1) dolmanp_L + gdp Confidence Level: 0 NCD genes: LOCUS:_8813#TRANSCRIPT:1#ABBREVIATION:DPM1#ECNUMBERS:2.4.1.83# LOCUS:_8818#TRANSCRIPT:1#ABBREVIATION:DPM2# LOCUS:_54344#TRANSCRIPT:1#ABBREVIATION:DPM3# LOCUS:_8818#TRANSCRIPT:2#ABBREVIATION:DPM2# LOCUS:_54344#TRANSCRIPT:2#ABBREVIATION:DPM3# proteins: NAME:dolichyl-phosphate mannosyltransferase (ubiquitous)#ABBREVIATION:Dpm111.1# NAME:dolichyl-phosphate mannosyltransferase (ubiquitous)#ABBREVIATION:Dpm112.1# NAME:dolichyl-phosphate mannosyltransferase (ubiquitous)#ABBREVIATION:Dpm121.1# NAME:dolichyl-phosphate mannosyltransferase (ubiquitous)#ABBREVIATION:Dpm122.1# citations: AUTHORS:Manos EJ, Kim ML, Kassis J, Chang PY, Wells A, Jones DA.#TITLE:Dolichol-phosphate-mannose-3 (DPM3)/prostin-1 is a novel phospholipase C-gamma regulated gene negatively associated with prostate tumor invasion.#JOURNAL:Oncogene#YEAR:2001#PUBMED:11420690# GENE ASSOCIATION: (8818.1 and 54344.1 and 8813.1) or (54344.1 and 8818.2 and 8813.1) or (54344.2 and 8818.1 and 8813.1) or (54344.2 and 8818.2 and 8813.1)
Dolichyl-phosphate D-mannosyltransferase (liver)
Abbreviation: R_DNDPt13m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + dadp[c] --> atp[c] + dadp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_S6TASE1ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs <==> h + hs_deg1 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_4HOXPACDOX_LPAREN_NADP_RPAREN_ Synonyms: _0 EC Number: 1.2.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 4hoxpacd + h2o + nadp <==> 4hphac + (2) h + nadph Confidence Level: 2 SAB genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Shimamura M, Kamada S, Hayashi T, Naruse H, Iida Y.#TITLE:Sensitive determination of tyrosine metabolites, p-hydroxyphenylacetic acid, 4-hydroxy-3-methoxyphenyl-acetic acid and 4-hydroxy-3-methoxymandelic acid, by gas chromatography-negative-ion chemical-ionization mass spectrometry.#JOURNAL:J Chromatogr#YEAR:1986#PUBMED:3753983# GENE ASSOCIATION: (222.1) or (220.1) or (221.1) or (218.1)
_4-Hydroxyphenylacetaldehyde:NADP+ oxidoreductase
Abbreviation: R_ADNtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adn[c] <==> adn[m] Confidence Level: 4 IT genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-m# citations: AUTHORS:Lai Y, Tse CM, Unadkat JD.#TITLE:Mitochondrial expression of the human equilibrative nucleoside transporter 1 (hENT1) results in enhanced mitochondrial toxicity of antiviral drugs.#JOURNAL:J Biol Chem. #YEAR:2004#PUBMED:14607828# AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (2030.1)
adenosine facilated transport in mitochondria
keratan sulfate I, degradation product 9_C188H301N13O166S10
Abbreviation: R_EX_avite1_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : avite1 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_limnen_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : limnen <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I, degradation product 21_C132H213N9O114S6
Diphosphoinositol tetrakisphosphate_C6H7O24P6
Abbreviation: R_42A12BOOX Synonyms: _0 EC Number: 1.4.3.6 SUBSYSTEM: Tyrosine metabolism Equation: [c] : dopa + h2o + o2 --> 34dhpac + h2o2 + nh4 Confidence Level: 4 SAB genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# LOCUS:_314#TRANSCRIPT:1#ABBREVIATION:AOC2#ECNUMBERS:1.4.3.6# LOCUS:_8639#TRANSCRIPT:1#ABBREVIATION:AOC3#ECNUMBERS:1.4.3.6# LOCUS:_26#TRANSCRIPT:1#ABBREVIATION:ABP1#ECNUMBERS:1.4.3.6# LOCUS:_314#TRANSCRIPT:2#ABBREVIATION:AOC2# proteins: NAME:amine oxidase, copper containing 3#ABBREVIATION:Aoc3.1# NAME:amiloride binding protein 1 (amine oxidase (copper-containing))#ABBREVIATION:Abp1.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.2# NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (4129.1) or (8639.1) or (26.1) or (314.2) or (314.1) or (4128.1)
_4-(2-Aminoethyl)-1,2-benzenediol:oxygen oxidoreductase(deaminating)(flavin-containing)
Abbreviation: R_MI1456PKn Synonyms: _0 EC Number: 2.7.1.151 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + mi1456p --> adp + h + mi13456p Confidence Level: 4 NCD genes: LOCUS:_253430#TRANSCRIPT:1#ABBREVIATION:IPMK# proteins: NAME:inositol polyphosphate multikinase, nucleus#ABBREVIATION:Ipmk.1-n# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:Chang SC, Miller AL, Feng Y, Wente SR, Majerus PW. #TITLE:The human homolog of the rat inositol phosphate multikinase is an inositol 1,3,4,6-tetrakisphosphate 5-kinase#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12223481# AUTHORS:Nalaskowski MM, Deschermeier C, Fanick W, Mayr GW#TITLE:The human homologue of yeast ArgRIII protein is an inositol phosphate multikinase with predominantly nuclear localization#JOURNAL:Biochem J#YEAR:2002#PUBMED:12027805# GENE ASSOCIATION: (253430.1)
inositol-1,4,5,6- tetrakisphosphate 3-kinase, nucleus
Abbreviation: R_EX_ha_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ha <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FA140ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + myrsACP <==> ACP + h + ttdca Confidence Level: 4 NCD rxn is now charge balanced - NCD genes: LOCUS:_55301#TRANSCRIPT:1#ABBREVIATION:THEDC1# proteins: NAME:s-acyl fatty acid synthase thioesterase#ABBREVIATION:Thedc1.1# citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION: (55301.1)
B mannosyltransferase, endoplasmic reticulum
Abbreviation: R_BMTer_L Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_L + memgacpail_hs --> (0.1) dolp_L + h + m2emgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_9488#TRANSCRIPT:1#ABBREVIATION:PIGB# proteins: NAME:phosphatidylinositol glycan, class B, endoplasmic reticulum#ABBREVIATION:Pigb.1-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Takahashi M, Inoue N, Ohishi K, Maeda Y, Nakamura N, Endo Y, Fujita T, Takeda J, Kinoshita T#TITLE:PIG-B, a membrane protein of the endoplasmic reticulum with a large lumenal domain, is involved in transferring the third mannose of the GPI anchor#JOURNAL:EMBO J#YEAR:1996#PUBMED:8861954# GENE ASSOCIATION: (9488.1)
(dimannosyl),(phosphoethanolaminyl)-mannosyl-glucosylaminyl-acylphosphatidylinositol (H6)_C51H94N2O32P2FULLRCO2FULLR2CO2
Abbreviation: R_ESTRIOLGLCte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + estriolglc[c] + h2o[c] --> adp[c] + estriolglc[e] + h[c] + pi[c] Confidence Level: 2 for MDR transport mechanism NJ genes: LOCUS:_4363#TRANSCRIPT:1#ABBREVIATION:ABCC1.1# proteins: NAME:ATP-binding cassette, subfamily C (CFTR/MRP), member 1#ABBREVIATION:ABCC1.1# citations: none GENE ASSOCIATION: (4363.1)
Abbreviation: R_RETNGLCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: retnglc[c] --> retnglc[e] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_C226CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : c226crn + coa --> c226coa + crn Confidence Level: 4 TDV genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# AUTHORS:Fraser F, Padovese R, Zammit VA.#TITLE:Distinct kinetics of carnitine palmitoyltransferase i in contact sites and outer membranes of rat liver mitochondria.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11274214# GENE ASSOCIATION: (1376.1)
Abbreviation: R_LPS Synonyms: _1 EC Number: 3.1.1.3 SUBSYSTEM: Triacylglycerol Synthesis Equation: [c] : h2o + tag_hs --> Rtotal3 + dag_hs + h Confidence Level: 4 carbonyl and extra H accounted for in R group NJ genes: LOCUS:_4023#TRANSCRIPT:1#ABBREVIATION:LPL#ECNUMBERS:3.1.1.34# proteins: NAME:lipoprotein lipase#ABBREVIATION:Lpl.1# citations: AUTHORS:Emi M, Wilson DE, Iverius PH, Wu L, Hata A, Hegele R, Williams RR, Lalouel JM.#TITLE:Missense mutation (Gly----Glu188) of human lipoprotein lipase imparting functional deficiency.#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:1969408# GENE ASSOCIATION: (4023.1)
Abbreviation: R_CYSGLYex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[c] + gly[e] --> cys-L[e] + gly[c] Confidence Level: 4 SAB genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_23428#TRANSCRIPT:1#ABBREVIATION:SLC7A8# LOCUS:_23428#TRANSCRIPT:2#ABBREVIATION:SLC7A8# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 8#ABBREVIATION:Slc7a8.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 8#ABBREVIATION:Slc7a8.2# citations: AUTHORS:Pineda M, Fernandez E, Torrents D, Estevez R, Lopez C, Camps M, Lloberas J, Zorzano A, Palacin M#TITLE:Identification of a membrane protein, LAT-2, that Co-expresses with 4F2 heavy chain, an L-type amino acid transport activity with broad specificity for small and large zwitterionic amino acids#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10391915# AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (6520.1) and (23428.1) or (6520.1) and (23428.2)
keratan sulfate I biosynthesis, precursor 29_C213H340N15O179S9X
Abbreviation: R_G14T15g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre29 + udpgal --> h + ksi_pre30 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
Abbreviation: R_CLPNDCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : clpndcrn + coa --> clpndcoa + crn Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_EICOSTETCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + eicostetcrn --> crn + eicostetcoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_THRGLNexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gln-L[c] + thr-L[e] <==> gln-L[e] + thr-L[c] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_PI34P3Pn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail34p_hs --> pail4p_hs + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
phosphatidylinositol-3,4-bisphosphate 3-phosphatase, nuclear
chondroitin sulfate D (GlcNAc6S-GlcA2S), free chain_C45H65N2O52S5
N-acetylgalactosamine-6-sulfatase, lysosomal
Abbreviation: R_S6TASE6ly Synonyms: _7 EC Number: 3.1.6.4 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_d + h2o <==> cs_d_deg1 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_FPGS3m Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [m] : 6thf + atp + glu-L --> 7thf + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
folylpolyglutamate synthetase, mitochondrial
Abbreviation: R_ABO7g Synonyms: _10 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucgalacgalfucgalacglcgal14acglcgalgluside_hs + udpacgal --> acgalfucgalacgalfucgalacglcgal14acglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_28#TRANSCRIPT:1#ABBREVIATION:ABO# proteins: NAME:ABO blood group#ABBREVIATION:Abo.1# citations: AUTHORS:Lloyd KO.#TITLE:The chemistry and immunochemistry of blood group A, B, H, and Lewis antigens: past, present and future.#JOURNAL:Glycoconj J#YEAR:2000#PUBMED:11421346# AUTHORS:Yamamoto F.-I., Hakomori S.-I.;#TITLE:Sugar-nucleotide donor specificity of histo-blood group A and B transferases is based on amino acid substitutions#JOURNAL:J. Biol. Chem#YEAR:1990#PUBMED:2121736# AUTHORS:Patenaude S.I., Seto N.O.L., Borisova S.N., Szpacenko A., Marcus S.L., Palcic M.M., Evans S.V.;#TITLE:The structural basis for specificity in human ABO(H) blood group biosynthesis#JOURNAL:Nat. Struct. Biol#YEAR:2002#PUBMED:12198488# GENE ASSOCIATION: (28.1)
Abbreviation: R_ACRNtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: acrn[c] --> acrn[m] Confidence Level: 4 NCD genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# AUTHORS:Pande SV, Parvin R.#TITLE:Characterization of carnitine acylcarnitine translocase system of heart mitochondria.#JOURNAL:J Biol Chem#YEAR:1976#PUBMED:977593# GENE ASSOCIATION: (788.1)
O-acetylcarnintine transport into mitochondria via diffusion
Abbreviation: R_CYSTA Synonyms: _0 EC Number: 2.6.1.3 SUBSYSTEM: Cysteine Metabolism Equation: [c] : akg + cys-L <==> glu-L + mercppyr Confidence Level: 2 MM genes: LOCUS:_2805#TRANSCRIPT:1#ABBREVIATION:GOT1#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase #ABBREVIATION:Got1.1# citations: AUTHORS:Stipanuk MH#TITLE:Sulfur amino acid metabolism: pathways for production and removal of homocysteine and cysteine#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189131# AUTHORS:Akagi R.#TITLE:Purification and characterization of cysteine aminotransferase from rat liver cytosol. #YEAR:1982#PUBMED:7113743# GENE ASSOCIATION: (2805.1)
chondroitin sulfate A (GalNAc4S-GlcA), degradation product 5_C23H36O24S
chondroitin sulfate A (GalNAc4S-GlcA), degradation product 4_C31H49NO29S
Abbreviation: R_NACHEX2ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_a_deg4 + h2o --> acgal + cs_a_deg5 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3073.1) or (3074.1)
Abbreviation: R_AG13T12g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre24 + uacgam --> h + ksi_pre25 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
keratan sulfate I biosynthesis, precursor 25_C199H319N14O163S7X
Abbreviation: R_FATP5t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: arachd[c] + na1[c] <==> arachd[e] + na1[e] Confidence Level: 4 electroneutral transport NJ genes: LOCUS:_10998#TRANSCRIPT:1#ABBREVIATION:SLC27A5# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 5#ABBREVIATION:Slc27a5.1# citations: AUTHORS:Steinberg SJ, Wang SJ, McGuinness MC, Watkins PA.#TITLE:Human liver-specific very-long-chain acyl-coenzyme A synthetase: cDNA cloning and characterization of a second enzymatically active protein.#JOURNAL:Mol Genet Metab.#YEAR:1999#PUBMED:10479480# AUTHORS:Stahl A.#TITLE:A current review of fatty acid transport proteins (SLC27).#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12856180# AUTHORS:Pohl J, Ring A, Hermann T, Stremmel W.#TITLE:Role of FATP in parenchymal cell fatty acid uptake.#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15522816# GENE ASSOCIATION: (10998.1)
Abbreviation: R_ILEt5m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: ile-L[c] <==> ile-L[m] Confidence Level: 4 MM genes: none proteins: none citations: AUTHORS:Porter RK.#TITLE:Mammalian mitochondrial inner membrane cationic and neutral amino acid carriers.#YEAR:2000#PUBMED:11004451# GENE ASSOCIATION:
Abbreviation: R_41R2A1H12BOOX Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Tyrosine metabolism Equation: [c] : h2o + nrpphr + o2 --> 34dhmald + h2o2 + nh4 Confidence Level: 4 SAB genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# proteins: NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (4128.1) or (4129.1)
_4-[(1R)-2-Amino-1-hydroxyethyl]-1,2-benzenediol:oxygen oxidoreductase(deaminating)(flavin-containing)
glycine reversible transport via sodium and chloride symport (3:1:1)
Abbreviation: R_GLYt7_LPAREN_311_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[e] + gly[e] + (3) na1[e] <==> cl[c] + gly[c] + (3) na1[c] Confidence Level: 4 SAB genes: LOCUS:_9152#TRANSCRIPT:1#ABBREVIATION:SLC6A5# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, glycine), member 5#ABBREVIATION:Slc6a5.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (9152.1)
Abbreviation: R_S6T19g Synonyms: _3 EC Number: 2.8.2.17 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_pre + (2) paps --> cs_c,d,e_pre1 + (2) h + (2) pap Confidence Level: 4 NCD genes: LOCUS:_56548#TRANSCRIPT:1#ABBREVIATION:CHST7# LOCUS:_9469#TRANSCRIPT:1#ABBREVIATION:CHST3# proteins: NAME:carbohydrate (chondroitin 6) sulfotransferase 3, Golgi#ABBREVIATION:Chst3.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 7, Golgi apparatus#ABBREVIATION:Chst7.1-g# citations: AUTHORS:Habuchi O, Hirahara Y, Uchimura K, Fukuta M#TITLE:Enzymatic sulfation of galactose residue of keratan sulfate by chondroitin 6-sulfotransferase#JOURNAL:Glycobiology#YEAR:1996#PUBMED:8991509# AUTHORS:Fukuta M, Kobayashi Y, Uchimura K, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of human chondroitin 6-sulfotransferase#JOURNAL:Biochim Biophys Acta#YEAR:1998#PUBMED:9714738# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Tsutsumi K, Shimakawa H, Kitagawa H, Sugahara K#TITLE:Functional expression and genomic structure of human chondroitin 6-sulfotransferase#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9883891# AUTHORS:Kitagawa H, Fujita M, Ito N, Sugahara K#TITLE:Molecular cloning and expression of a novel chondroitin 6-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781596# AUTHORS:Bhakta S, Bartes A, Bowman KG, Kao WM, Polsky I, Lee JK, Cook BN, Bruehl RE, Rosen SD, Bertozzi CR, Hemmerich S#TITLE:Sulfation of N-acetylglucosamine by chondroitin 6-sulfotransferase 2 (GST-5)#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10956661# GENE ASSOCIATION: (9469.1) or (56548.1)
chondroitin sulfate C (GalNAc6S-GlcA) and D (GlcNAc6S-GlcA2S), precursor 1_C31H47NO31S2X
chondroitin sulfate precursor (GalNAc-GlcA-(Gal)2-Xyl-L-Ser (protein))_C31H49NO25X
chondroitin 6-sulfotransferase, Golgi apparatus
Abbreviation: R_ACCOAC Synonyms: _0 EC Number: 6.4.1.2 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : accoa + atp + hco3 --> adp + h + malcoa + pi Confidence Level: 4 A biotinyl-protein. Step 1: biotin + ATP + HCO3 -> carboxy-biotin + ADP + Pi. Step 2: carboxy-biotin + ACCOA -> MALCOA bacteria have separate enzymes for each step and a biotin-carrier protein; in yeast a single protein carries out all activities NCD genes: LOCUS:_31#TRANSCRIPT:1#ABBREVIATION:ACACA#ECNUMBERS:6.4.1.2# LOCUS:_32#TRANSCRIPT:1#ABBREVIATION:ACACB# LOCUS:_31#TRANSCRIPT:2#ABBREVIATION:ACACA#ECNUMBERS:6.4.1.2# LOCUS:_31#TRANSCRIPT:3#ABBREVIATION:ACACA#ECNUMBERS:6.4.1.2# LOCUS:_31#TRANSCRIPT:4#ABBREVIATION:ACACA#ECNUMBERS:6.4.1.2# LOCUS:_31#TRANSCRIPT:5#ABBREVIATION:ACACA#ECNUMBERS:6.4.1.2# proteins: NAME:acetyl-Coenzyme A carboxylase alpha#ABBREVIATION:Acaca.1# NAME:acetyl-Coenzyme A carboxylase alpha#ABBREVIATION:AcacAB# NAME:acetyl-Coenzyme A carboxylase alpha#ABBREVIATION:Acaca2.1# NAME:acetyl-Coenzyme A carboxylase alpha#ABBREVIATION:Acaca3.1# NAME:acetyl-Coenzyme A carboxylase alpha#ABBREVIATION:Acaca4.1# NAME:acetyl-Coenzyme A carboxylase alpha#ABBREVIATION:Acaca5.1# NAME:acetyl-Coenzyme A carboxylase#ABBREVIATION:AcacA2B# NAME:acetyl-Coenzyme A carboxylase#ABBREVIATION:AcacA3B# NAME:acetyl-Coenzyme A carboxylase#ABBREVIATION:AcacA4B# NAME:acetyl-Coenzyme A carboxylase#ABBREVIATION:AcacA5B# citations: none GENE ASSOCIATION: (31.2 and 32.1) or (31.1) or (32.1 and 31.3) or (32.1 and 31.5) or (31.4) or (31.4 and 32.1) or (31.2) or (31.3) or (31.1 and 32.1) or (31.5)
fatty acid 9-cis-retinol_C20H29OFULLR2CO
Abbreviation: R_RETFAt1 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _9-cis-retfa[c] --> 9-cis-retfa[e] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_XYLt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: xyl-D[e] <==> xyl-D[c] Confidence Level: 4 NCD genes: LOCUS:_6515#TRANSCRIPT:1#ABBREVIATION:SLC2A3# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 3, extracellular#ABBREVIATION:Slc2a3.1-e# citations: AUTHORS:Uldry M, Thorens B#TITLE:The SLC2 family of facilitated hexose and polyol transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750891# AUTHORS:Haber RS, Weinstein SP, O'Boyle E, Morgello S#TITLE:Tissue distribution of the human GLUT3 glucose transporte#JOURNAL:Endocrinology#YEAR:1993#PUBMED:8504756# AUTHORS:Heijnen HF, Oorschot V, Sixma JJ, Slot JW, James DE#TITLE:Thrombin stimulates glucose transport in human platelets via the translocation of the glucose transporter GLUT-3 from alpha-granules to the cell surface#JOURNAL:J Cell Biol#YEAR:1997#PUBMED:9230074# AUTHORS:Kayano T, Fukumoto H, Eddy RL, Fan YS, Byers MG, Shows TB, Bell GI#TITLE:Evidence for a family of human glucose transporter-like proteins. Sequence and gene localization of a protein expressed in fetal skeletal muscle and other tissues#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3170580# AUTHORS:Stuart CA, Wen G, Jiang J#TITLE:GLUT3 protein and mRNA in autopsy muscle specimens#JOURNAL:Metabolism#YEAR:1999#PUBMED:10421229# GENE ASSOCIATION: (6515.1)
Abbreviation: R_EX_pi_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pi <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ADPRDPm Synonyms: _0 EC Number: 3.6.1.13 SUBSYSTEM: Nucleotides Equation: [m] : adprib + h2o --> amp + (2) h + r5p Confidence Level: 4 IT genes: LOCUS:_53343#TRANSCRIPT:1#ABBREVIATION:NUDT9# LOCUS:_53343#TRANSCRIPT:2#ABBREVIATION:NUDT9# LOCUS:_53343#TRANSCRIPT:3#ABBREVIATION:NUDT9# proteins: NAME:nudix (nucleoside diphosphate linked moiety X)-type motif 9 #ABBREVIATION:NudT9.2# NAME:nudix (nucleoside diphosphate linked moiety X)-type motif 9 #ABBREVIATION:NudT9.1# NAME:nudix (nucleoside diphosphate linked moiety X)-type motif 9 #ABBREVIATION:NudT9.3# citations: AUTHORS:Perraud AL, Shen B, Dunn CA, Rippe K, Smith MK, Bessman MJ, Stoddard BL, Scharenberg AM.#TITLE:NUDT9, a member of the Nudix hydrolase family, is an evolutionarily conserved mitochondrial ADP-ribose pyrophosphatase.#JOURNAL: J Biol Chem. #YEAR:2003#PUBMED:12427752# AUTHORS:Shen BW, Perraud AL, Scharenberg A, Stoddard BL.#TITLE:The crystal structure and mutational analysis of human NUDT9.#JOURNAL: J Mol Biol#YEAR:2003#PUBMED:12948489# AUTHORS:Kuhn FJ, Luckhoff A.#TITLE: Sites of the NUDT9-H domain critical for ADP-ribose activation of the cation channel TRPM2. #JOURNAL:J Biol Chem.#YEAR:2005#PUBMED:15347676# GENE ASSOCIATION: (53343.1) or (53343.3) or (53343.2)
Abbreviation: R_CREATtmdiffir Synonyms: _0 SUBSYSTEM: Urea cycle/amino group metabolism Equation: creat[c] --> creat[m] Confidence Level: 2 SAB genes: none proteins: none citations: AUTHORS:Bessman SP, Carpenter CL#TITLE:The creatine-creatine phosphate energy shuttle#JOURNAL:Annu Rev Biochem#YEAR:1985#PUBMED:3896131# AUTHORS:Speer O, Neukomm LJ, Murphy RM, Zanolla E, Schlattner U, Henry H, Snow RJ, Wallimann T#TITLE:Creatine transporters: a reappraisal#JOURNAL:Mol Cell Biochem#YEAR:2004#PUBMED:14977199# TITLE:Biochemical pathways : an atlas of biochemistry and molecular biology #YEAR:1999# GENE ASSOCIATION:
Creatine transport to/from mitochondria via diffusion
heparan-glucosaminide N-acetyltransferase, lysosomal
heparan sulfate, degradation product 8_C69H100N4O81S8
Abbreviation: R_HSAT2ly Synonyms: _1 EC Number: 2.3.1.78 SUBSYSTEM: Heparan sulfate degradation Equation: accoa[c] + hs_deg7[l] --> coa[c] + h[l] + hs_deg8[l] Confidence Level: 4 The proton is located in the lysosome since it comes from oxidation of the NH3+ group in glucosamine. This reaction assumes that the other proton liberated from glucosamine reduces coa in the cytosol. NCD genes: none proteins: none citations: AUTHORS:Ausseil J, Loredo-Osti JC, Verner A, Darmond-Zwaig C, Maire I, Poorthuis B, van Diggelen OP, Hudson TJ, Fujiwara TM, Morgan K, Pshezhetsky AV#TITLE:Localisation of a gene for mucopolysaccharidosis IIIC to the pericentromeric region of chromosome 8#JOURNAL:J Med Genet#YEAR:2004#PUBMED:15591281# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
heparan sulfate, degradation product 7_C67H99N4O80S8
Abbreviation: R_CHTNASEe Synonyms: _0 EC Number: 3.2.1.14 SUBSYSTEM: Aminosugar Metabolism Equation: [e] : chtn + (2) h2o --> (3) acgam Confidence Level: 4 NCD genes: LOCUS:_27159#TRANSCRIPT:2#ABBREVIATION:CHIA# proteins: NAME:eosinophil chemotactic cytokine, extracellular#ABBREVIATION:Chia.2-e# citations: AUTHORS:Boot RG, Blommaart EF, Swart E, Ghauharali-van der Vlugt K, Bijl N, Moe C, Place A, Aerts JM#TITLE:Identification of a novel acidic mammalian chitinase distinct from chitotriosidase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11085997# GENE ASSOCIATION: (27159.2)
Abbreviation: R_ADRNCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : adrncrn + coa --> adrncoa + crn Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_APPNNte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: appnn[e] <==> appnn[c] Confidence Level: 2 unknown mechanism, xenobiotic transport (original and metabolized) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NABTNO Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : h2o + n4abutn + nad --> 4aabutn + (2) h + nadh Confidence Level: 1 NCD genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# citations: none GENE ASSOCIATION: (8854.2) or (501.1) or (216.1) or (224.1) or (8854.3) or (223.1) or (8854.1)
N4-Acetylaminobutanal:NAD+ oxidoreductase
Abbreviation: R_GT1Atg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gt1a_hs[c] <==> gt1a_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_CRMPte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: crmp_hs[c] <==> crmp_hs[e] Confidence Level: 2 unknown mechanism for transport, may be vesicular/caveolar/other ... intracellular transport and extracellular export mechanisms must exist - see for example: PMID: 15585321 NJ genes: none proteins: none citations: AUTHORS:Baumruker T, Bornancin F, Billich A.#TITLE:The role of sphingosine and ceramide kinases in inflammatory responses.#JOURNAL:Immunol Lett.#YEAR:2005#PUBMED:15585321# GENE ASSOCIATION:
Abbreviation: R_MGSA Synonyms: _0 EC Number: 4.2.3.3 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : dhap --> mthgxl + pi Confidence Level: 4 Previously EC 4.2.99.11 this rxn represents the enzymatic conversion or non-enzymatic fragmentation of dhap to mthgxl - NCD genes: none proteins: none citations: AUTHORS:Vander Jagt DL, Robinson B, Taylor KK, Hunsaker LA.#TITLE:Reduction of trioses by NADPH-dependent aldo-keto reductases. Aldose reductase, methylglyoxal, and diabetic complications#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1537826# AUTHORS:Thornalley PJ#TITLE:Pharmacology of methylglyoxal: formation, modification of proteins and nucleic acids, and enzymatic detoxification--a role in pathogenesis and antiproliferative chemotherapy.#JOURNAL:Gen Pharmacol#YEAR:1996#PUBMED:8853285# AUTHORS:Beisswenger BG, Delucia EM, Lapoint N, Sanford RJ, Beisswenger PJ.#TITLE:Ketosis leads to increased methylglyoxal production on the atkins diet#JOURNAL:Ann N Y Acad Sci#YEAR:2005#PUBMED:16037240# GENE ASSOCIATION:
Abbreviation: R_5FTHFt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _5fthf[e] + oh1[c] <==> 5fthf[c] + oh1[e] Confidence Level: 4 IT genes: LOCUS:_6573#TRANSCRIPT:1#ABBREVIATION:SLC19A1# LOCUS:_6573#TRANSCRIPT:2#ABBREVIATION:SLC19A1# proteins: NAME:solute carrier family 19 (folate transporter), member 1 #ABBREVIATION:Slc19a1.1# NAME:solute carrier family 19 (folate transporter), member 1#ABBREVIATION:Slc19a1.2# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Ganapathy V, Smith SB, Prasad PD.#TITLE:SLC19: the folate/thiamine transporter family.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:14770311# AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION: (6573.1) or (6573.2)
_5-formyltetrahydrofolate transport via anion exchange
Abbreviation: R_AHEXASEly Synonyms: _13 EC Number: 3.2.1.52 SUBSYSTEM: N-Glycan Degradation Equation: [l] : (2) h2o + n2m2mn --> (2) acgam + m2mn Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3074.1) or (3073.1 and 3074.1)
Abbreviation: R_TCHOLAte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tchola[e] <==> tchola[c] Confidence Level: 2 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_COUMARINte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: coumarin[e] <==> coumarin[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TRYPTAOX Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Tryptophan metabolism Equation: [c] : h2o + o2 + trypta --> h2o2 + id3acald + nh4 Confidence Level: 4 SAB genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# LOCUS:_314#TRANSCRIPT:1#ABBREVIATION:AOC2#ECNUMBERS:1.4.3.6# LOCUS:_26#TRANSCRIPT:1#ABBREVIATION:ABP1#ECNUMBERS:1.4.3.6# LOCUS:_314#TRANSCRIPT:2#ABBREVIATION:AOC2# proteins: NAME:amiloride binding protein 1 (amine oxidase (copper-containing))#ABBREVIATION:Abp1.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.2# NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# AUTHORS:Kitahama K, Ikemoto K, Jouvet A, Nagatsu I, Sakamoto N, Pearson J#TITLE:Aromatic L-amino acid decarboxylase- and tyrosine hydroxylase-immunohistochemistry in the adult human hypothalamus#JOURNAL:J Chem Neuroanat#YEAR:1998#PUBMED:9924972# GENE ASSOCIATION: (314.1) or (4128.1) or (26.1) or (4129.1) or (314.2)
Tryptamine:oxygen oxidoreductase(deaminating)(flavin-containing)
Abbreviation: R_CLOXAtex2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) cl[e] + oxa[c] --> (2) cl[c] + oxa[e] Confidence Level: 2 SAB genes: LOCUS:_65010#TRANSCRIPT:1#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:2#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:3#ABBREVIATION:SLC26A6# proteins: NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.1# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.2# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.3# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (65010.1) or (65010.3) or (65010.2)
chloride transport via oxalate countertransport (2:1)
Abbreviation: R_LEUTA Synonyms: _0 EC Number: 2.6.1.6 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [c] : akg + leu-L <==> 4mop + glu-L Confidence Level: 4 _2.6.1.42 genes: LOCUS:_586#TRANSCRIPT:1#ABBREVIATION:BCAT1#ECNUMBERS:2.6.1.26# proteins: NAME:branched chain aminotransferase 1, cytosolic #ABBREVIATION:Bcat1.1# citations: AUTHORS:Bledsoe RK, Dawson PA, Hutson SM.#TITLE:Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm). #YEAR:1997#PUBMED:9165094# AUTHORS:Naylor SL, Shows TB.#TITLE:Branched-chain aminotransferase deficiency in Chinese hamster cells complemented by two independent genes on human chromosomes 12 and 19. #YEAR:1980#PUBMED:6933702# GENE ASSOCIATION: (586.1)
Abbreviation: R_SOAT11 Synonyms: _9 SUBSYSTEM: Cholesterol Metabolism Equation: [c] : R1coa_hs + chsterol --> coa + xolest_hs Confidence Level: 4 SOAT11 and SOAT12 are templates, customized rxns can be formed particular ester moieties. NJ genes: LOCUS:_6646#TRANSCRIPT:1#ABBREVIATION:SOAT1#ECNUMBERS:2.3.1.26# proteins: NAME:sterol O-acetyltransferase (acyl-Coenzyme A: cholesterol acyltransferase) 1#ABBREVIATION:Soat1.1# citations: AUTHORS:Guo ZY, Chang CC, Lu X, Chen J, Li BL, Chang TY.#TITLE:The disulfide linkage and the free sulfhydryl accessibility of acyl-coenzyme A:cholesterol acyltransferase 1 as studied by using mPEG5000-maleimide.#JOURNAL:Biochemistry#YEAR:2005#PUBMED:15850387# AUTHORS:Chang CC, Huh HY, Cadigan KM, Chang TY.#TITLE:Molecular cloning and functional expression of human acyl-coenzyme A:cholesterol acyltransferase cDNA in mutant Chinese hamster ovary cells.#JOURNAL:J Biol Chem.#YEAR:1993#PUBMED:8407899# GENE ASSOCIATION: (6646.1)
Abbreviation: R_EX_lnlncg_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lnlncg <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate II boisynthesis, precursor 2_C47H76N4O33X
Abbreviation: R_AG13T16g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core4_pre2 + uacgam --> h + ksii_core4_pre3 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (79369.1) or (10678.1) or (11041.1) or (284208.1) or (374907.1) or (93010.1) or (374907.2) or (10678.2) or (10331.1)
keratan sulfate II biosynthesis, precursor 3_C55H89N5O38X
Abbreviation: R_G5SADs Synonyms: _0 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : glu5sa --> 1pyr5c + h + h2o Confidence Level: 2 Needed in L-proline biosynthesis genes: none proteins: none citations: AUTHORS:Hu CA, Lin WW, Obie C, Valle D#TITLE:Molecular enzymology of mammalian Delta1-pyrroline-5-carboxylate synthase. Alternative splice donor utilization generates isoforms with different sensitivity to ornithine inhibition#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10037775# GENE ASSOCIATION:
L-glutamate 5-semialdehyde dehydratase (spontaneous)
Abbreviation: R_C180CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + stcrn --> crn + stcoa Confidence Level: 4 TV genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# GENE ASSOCIATION: (1376.1)
Abbreviation: R_LDH_D Synonyms: _0 EC Number: 1.1.1.28 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : lac-D + nad <==> h + nadh + pyr Confidence Level: 2 See also LDL_L genes: LOCUS:_197257#TRANSCRIPT:1#ABBREVIATION:LDHD# LOCUS:_197257#TRANSCRIPT:2#ABBREVIATION:LDHD# proteins: NAME:lactate dehydrogenase D#ABBREVIATION:Ldhd.1# NAME:lactate dehydrogenase D#ABBREVIATION:Ldhd.2# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# GENE ASSOCIATION: (197257.1) or (197257.2)
Abbreviation: R_THP2Ctp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: thp2c[c] <==> thp2c[x] Confidence Level: 1 MM genes: none proteins: none citations: none GENE ASSOCIATION:
_2,3,4,5-Tetrahydropyridine-2-carboxylate transport, peroxisomal
N,N'-Diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1185H1940N20O170P20
Abbreviation: R_QUILSYN Synonyms: _0 SUBSYSTEM: Tryptophan metabolism Equation: [c] : cmusa --> h + h2o + quln Confidence Level: 4 SAB mechanism is unclear non-enzymatic genes: none proteins: none citations: AUTHORS:Fukuoka S, Ishiguro K, Yanagihara K, Tanabe A, Egashira Y, Sanada H, Shibata K#TITLE:Identification and expression of a cDNA encoding human alpha-amino-beta-carboxymuconate-epsilon-semialdehyde decarboxylase (ACMSD). A key enzyme for the tryptophan-niacine pathway and "quinolinate hypothesis"#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12140278# GENE ASSOCIATION:
Abbreviation: R_P45011B11m Synonyms: _3 EC Number: 1.14.15.4 SUBSYSTEM: Steroid Metabolism Equation: [m] : 11docrtstrn + h + nadph + o2 --> crtstrn + h2o + nadp Confidence Level: 4 Payne and Hales (2004) Mol Bio of Steroidogenic Enzymes 25(6):947-970 H2O added for balancing NJ genes: LOCUS:_1584#TRANSCRIPT:1#ABBREVIATION:CYP11B1#ECNUMBERS:1.14.15.4# proteins: NAME:cytochrome P450, family 11, subfamily B, polypeptide 1#ABBREVIATION:Cyp11b1.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (1584.1)
Abbreviation: R_ESTRIOLtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: estriol[r] <==> estriol[c] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_THYMt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: thym[e] <==> thym[c] Confidence Level: 4 IT genes: LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Yao SY, Ng AM, Vickers MF, Sundaram M, Cass CE, Baldwin SA, Young JD.#TITLE:Functional and molecular characterization of nucleobase transport by recombinant human and rat equilibrative nucleoside transporters 1 and 2.#JOURNAL:J Biol Chem. #YEAR:2002#PUBMED:12006583# GENE ASSOCIATION: (3177.1)
thymine reversible transport via facilated diffusion
Abbreviation: R_RTOTALCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: Rtotalcrn[c] --> Rtotalcrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (788.1)
Abbreviation: R_NDPK8m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + dadp <==> adp + datp Confidence Level: 2 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:dADP), mitochondrial
Abbreviation: R_ALASERNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + na1[e] + ser-L[c] --> ala-L[c] + na1[c] + ser-L[e] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
L-alanine/L-serine Na-dependent exchange (Ala-L in)
Abbreviation: R_LPS3 Synonyms: _1 EC Number: 3.1.1.23 SUBSYSTEM: Triacylglycerol Synthesis Equation: [c] : h2o + mag_hs --> Rtotal2 + glyc + h Confidence Level: 4 NJ genes: LOCUS:_11343#TRANSCRIPT:1#ABBREVIATION:MGLL# LOCUS:_11343#TRANSCRIPT:2#ABBREVIATION:MGLL# proteins: NAME:monoglyceride lipase#ABBREVIATION:Mgll.1# NAME:monoglyceride lipase#ABBREVIATION:Mgll.2# citations: AUTHORS:Wall EM, Cao J, Chen N, Buller RM, Upton C.#TITLE:A novel poxvirus gene and its human homolog are similar to an E. coli lysophospholipase.#JOURNAL:Virus Res#YEAR:1997#PUBMED:9495531# GENE ASSOCIATION: (11343.1) or (11343.2)
Abbreviation: R_CYSTSERex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: Lcystin[e] + ser-L[c] --> Lcystin[c] + ser-L[e] Confidence Level: 4 SAB see PMID 14770310 genes: LOCUS:_6519#TRANSCRIPT:1#ABBREVIATION:SLC3A1# LOCUS:_11136#TRANSCRIPT:1#ABBREVIATION:SLC7A9# proteins: NAME:solute carrier family (amino acid transporter), extracellular#ABBREVIATION:Slc3a1/7a9-e# citations: none GENE ASSOCIATION: (6519.1 and 11136.1)
L-cystine/glycine exchanger (cystine in)
glucose-6-phosphate dehydrogenase, endoplasmic reticulum
Abbreviation: R_G6PDH1rer Synonyms: _0 SUBSYSTEM: Pentose Phosphate Pathway Equation: [r] : g6p + nad <==> 6pgl + h + nadh Confidence Level: 4 tv, jp NCD genes: LOCUS:_9563#TRANSCRIPT:1#ABBREVIATION:H6PD#ECNUMBERS:1.1.1.47# proteins: NAME:hexose-6-phosphate dehydrogenase, endoplasmic reticulum#ABBREVIATION:H6pd.1-r# citations: AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Mason PJ, Stevens D, Diez A, Knight SW, Scopes DA, Vulliamy TJ.#TITLE:Human hexose-6-phosphate dehydrogenase (glucose 1-dehydrogenase) encoded at 1p36: coding sequence and expression.#JOURNAL: Blood Cells Mol Dis#YEAR:1999#PUBMED:10349511# AUTHORS:Clarke JL, Mason PJ#TITLE:Murine hexose-6-phosphate dehydrogenase: a bifunctional enzyme with broad substrate specificity and 6-phosphogluconolactonase activity.#JOURNAL:Arch Biochem Biophys#YEAR:2003#PUBMED:12831846# AUTHORS:Barash V, Erlich T, Bashan N.#TITLE:Microsomal hexose-6-phosphate and 6-phosphogluconate dehydrogenases in extrahepatic tissues: human placenta and pig kidney cortex.#JOURNAL:Biochem Int.#YEAR:1990#PUBMED:2156506# GENE ASSOCIATION: (9563.1)
Abbreviation: R_PHEACGLNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: pheacgln[c] <==> pheacgln[e] Confidence Level: 0 SAB genes: none proteins: none citations: AUTHORS:Vanholder RC, Glorieux G, De Smet R, De Deyn PP#TITLE:Low water-soluble uremic toxins#JOURNAL:Adv Ren Replace Ther#YEAR:2003#PUBMED:14681857# GENE ASSOCIATION:
PHEACGLN extracellular transport via diffusion
Abbreviation: R_ACOX22x Synonyms: _0 EC Number: 1.3.3.6 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : dhcholoylcoa + fadh2 + (0.5) o2 --> fad + thcholoylcoa Confidence Level: 2 noted in uniprot to have FAD as cofactor - further reaction specific lit evidence not found to support or contradict, see Baumgart et al PNAS (1996) 93: 13748-13753 analog as per KEGG added NJ genes: LOCUS:_8309#TRANSCRIPT:1#ABBREVIATION:ACOX2#ECNUMBERS:1.3.3.6# proteins: NAME:acel-Coenzyme A oxidase 2, branched chain#ABBREVIATION:Acox2.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Baumgartner MR, Almashanu S, Suormala T, Obie C, Cole RN, Packman S, Baumgartner ER, Valle D. #TITLE:The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency.#YEAR:2001#PUBMED:11181649# GENE ASSOCIATION: (8309.1)
acyl-Coenzyme A oxidase 2, branched chain
Abbreviation: R_ARACHt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: arach[e] <==> arach[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_EX_lnlc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lnlc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HSD11B2r Synonyms: _4 SUBSYSTEM: Steroid Metabolism Equation: [r] : cortsn + h + nadh <==> crtsl + nad Confidence Level: 4 NJ genes: LOCUS:_3291#TRANSCRIPT:1#ABBREVIATION:HSD11B2# proteins: NAME:hydroxysteroid (11-beta) dehydrogenase 2#ABBREVIATION:Hsd11b2.1# citations: AUTHORS:Yau JL, Seckl JR.#TITLE:_11beta-hydroxysteroid dehydrogenase type I in the brain; thickening the glucocorticoid soup.#JOURNAL:Mol Psychiatry#YEAR:2001#PUBMED:11673786# AUTHORS:Albiston AL, Obeyesekere VR, Smith RE, Krozowski ZS.#TITLE:Cloning and tissue distribution of the human 11 beta-hydroxysteroid dehydrogenase type 2 enzyme.#JOURNAL:Mol Cell Endocrinol#YEAR:1994#PUBMED:7859916# AUTHORS:Tomlinson JW, Walker EA, Bujalska IJ, Draper N, Lavery GG, Cooper MS, Hewison M, Stewart PM.#TITLE:_11beta-hydroxysteroid dehydrogenase type 1: a tissue-specific regulator of glucocorticoid response.#JOURNAL:Endocr Rev#YEAR:2004#PUBMED:15466942# GENE ASSOCIATION: (3291.1)
_11-beta-hydroxysteroid dehydrogenase type 2
Abbreviation: R_PRO1xm Synonyms: _0 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : nad + pro-L --> 1pyr5c + (2) h + nadh Confidence Level: 2 NCD genes: LOCUS:_5625#TRANSCRIPT:1#ABBREVIATION:PRODH# proteins: NAME:proline dehydrogenase (oxidase) 1#ABBREVIATION:Prodh.1-m# citations: AUTHORS:Gogos JA, Santha M, Takacs Z, Beck KD, Luine V, Lucas LR, Nadler JV, Karayiorgou M.#TITLE:The gene encoding proline dehydrogenase modulates sensorimotor gating in mice. #JOURNAL:Nat Genet#YEAR:1999#PUBMED:10192398# GENE ASSOCIATION: (5625.1)
Abbreviation: R_TETPENT3t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tetpent3[e] <==> tetpent3[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
heparan sulfate, precursor 9_C87H129N5O69X
GlcNAc N-deacetylase / N-sulfotransferase
heparan sulfate, precursor 10_C79H121N5O77S4X
Abbreviation: R_GLCNACDASg Synonyms: _0 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : (4) h2o + hs_pre9 + (4) paps --> (4) ac + (4) h + hs_pre10 + (4) pap Confidence Level: 4 NCD genes: LOCUS:_9348#TRANSCRIPT:1#ABBREVIATION:NDST3# LOCUS:_8509#TRANSCRIPT:1#ABBREVIATION:NDST2# LOCUS:_64579#TRANSCRIPT:1#ABBREVIATION:NDST4# LOCUS:_3340#TRANSCRIPT:1#ABBREVIATION:NDST1#ECNUMBERS:3.1.1.-,2.8.2.-# proteins: NAME:N-deacetylase/N-sulfotransferase (heparan glucosaminyl) 1, Golgi apparatus#ABBREVIATION:Ndst1.1-g# NAME:N-deacetylase/N-sulfotransferase (heparan glucosaminyl) 2, Golgi apparatus#ABBREVIATION:Ndst2.1-g# NAME:N-deacetylase/N-sulfotransferase (heparan glucosaminyl) 3, Golgi apparatus#ABBREVIATION:Ndst3.1-g# NAME:N-deacetylase/N-sulfotransferase (heparan glucosaminyl) 4, Golgi apparatus#ABBREVIATION:Ndst4.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Dixon J, Loftus SK, Gladwin AJ, Scambler PJ, Wasmuth JJ, Dixon MJ#TITLE:Cloning of the human heparan sulfate-N-deacetylase/N-sulfotransferase gene from the Treacher Collins syndrome candidate region at 5q32-q33.1#JOURNAL:Genomics#YEAR:1995#PUBMED:7601448# AUTHORS:Humphries DE, Sullivan BM, Aleixo MD, Stow JL#TITLE:Localization of human heparan glucosaminyl N-deacetylase/N-sulphotransferase to the trans-Golgi network.#JOURNAL:Biochem J#YEAR:1997#PUBMED:9230113# AUTHORS:Humphries DE, Lanciotti J, Karlinsky JB#TITLE:cDNA cloning, genomic organization and chromosomal localization of human heparan glucosaminyl N-deacetylase/N-sulphotransferase-2#JOURNAL:Biochem J#YEAR:1998#PUBMED:9601056# AUTHORS:van den Born J, Pikas DS, Pisa BJ, Eriksson I, Kjellen L, Berden JH#TITLE:Antibody-based assay for N-deacetylase activity of heparan sulfate/heparin N-deacetylase/N-sulfotransferase (NDST): novel characteristics of NDST-1 and -2#JOURNAL:Glycobiology#YEAR:2003#PUBMED:12634318# AUTHORS:Aikawa J, Esko JD#TITLE:Molecular cloning and expression of a third member of the heparan sulfate/heparin GlcNAc N-deacetylase/ N-sulfotransferase family#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9915799# AUTHORS:Aikawa J, Grobe K, Tsujimoto M, Esko JD#TITLE:Multiple isozymes of heparan sulfate/heparin GlcNAc N-deacetylase/GlcN N-sulfotransferase. Structure and activity of the fourth member, NDST4#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11087757# GENE ASSOCIATION: (64579.1) or (3340.1) or (9348.1) or (8509.1)
Abbreviation: R_H2CO3D Synonyms: _0 EC Number: 4.2.1.1 SUBSYSTEM: Miscellaneous Equation: [c] : co2 + h2o <==> h2co3 Confidence Level: 0 tv genes: LOCUS:_23632#TRANSCRIPT:1#ABBREVIATION:CA14# LOCUS:_760#TRANSCRIPT:1#ABBREVIATION:CA2#ECNUMBERS:4.2.1.1# LOCUS:_762#TRANSCRIPT:1#ABBREVIATION:CA4#ECNUMBERS:4.2.1.1# LOCUS:_766#TRANSCRIPT:1#ABBREVIATION:CA7#ECNUMBERS:4.2.1.1# LOCUS:_768#TRANSCRIPT:1#ABBREVIATION:CA9#ECNUMBERS:4.2.1.1# LOCUS:_771#TRANSCRIPT:1#ABBREVIATION:CA12# LOCUS:_761#TRANSCRIPT:1#ABBREVIATION:CA3#ECNUMBERS:4.2.1.1# LOCUS:_759#TRANSCRIPT:1#ABBREVIATION:CA1#ECNUMBERS:4.2.1.1# LOCUS:_377677#TRANSCRIPT:1#ABBREVIATION:CA13# LOCUS:_771#TRANSCRIPT:2#ABBREVIATION:CA12# LOCUS:_766#TRANSCRIPT:2#ABBREVIATION:CA7# proteins: NAME:carbonic anhydrase I, cytosolic#ABBREVIATION:Ca1.1# NAME:Carbonic anhydrase II, cytosolic #ABBREVIATION:Ca2.1# NAME:Carbonic anhydrase III (muscle, adipose), cytosolic #ABBREVIATION:Ca3.1# NAME:Carbonic anhydrase IV, cytosolic #ABBREVIATION:Ca4.1# NAME:Carbonic anhydrase VII (salivary glands), cytosolic#ABBREVIATION:Ca7.1# NAME:Carbonic anhydrase VII (salivary gland), cytosolic#ABBREVIATION:Ca7.2# NAME:Carbonic anhydrase IX#ABBREVIATION:Ca9.1# NAME:Carbonic anhydrase XII #ABBREVIATION:Ca12.1# NAME:Carbonic anhydrase XII #ABBREVIATION:Ca12.2# NAME:Carbonic anhydrase XIV#ABBREVIATION:Ca14.1# NAME:Carbonic anhydrase XIII, cytosolic#ABBREVIATION:Ca13.1# citations: AUTHORS:Sly WS, Hu PY#TITLE:Human carbonic anhydrases and carbonic anhydrase deficiencies#JOURNAL:Annu Rev Biochem#YEAR:1995#PUBMED:7574487# AUTHORS:Wingo T, Tu C, Laipis PJ, Silverman DN.#TITLE:The catalytic properties of human carbonic anhydrase IX#JOURNAL:Biochem Biophys Res Commun#YEAR:2001#PUBMED:11676494# AUTHORS:Parkkila S, Parkkila AK, Rajaniemi H, Shah GN, Grubb JH, Waheed A, Sly WS.#TITLE:Expression of membrane-associated carbonic anhydrase XIV on neurons and axons in mouse and human brain#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11172051# AUTHORS:Taniuchi K, Nishimori I, Takeuchi T, Fujikawa-Adachi K, Ohtsuki Y, Onishi S.#TITLE:Developmental expression of carbonic anhydrase-related proteins VIII, X, and XI in the human brain.#JOURNAL:Neuroscience#YEAR:2002# AUTHORS:Parkkila S, Parkkila AK, Saarnio J, Kivela J, Karttunen TJ, Kaunisto K, Waheed A, Sly WS, Tureci O, Virtanen I, Rajaniemi H.#TITLE:Expression of the membrane-associated carbonic anhydrase isozyme XII in the human kidney and renal tumors#JOURNAL:J Histochem Cytochem#YEAR:2000#PUBMED:11101628# AUTHORS:Fleming RE, Parkkila S, Parkkila AK, Rajaniemi H, Waheed A, Sly WS.#TITLE:Carbonic anhydrase IV expression in rat and human gastrointestinal tract regional, cellular, and subcellular localization.#JOURNAL:J Clin Invest#YEAR:1995# AUTHORS:Lehtonen J, Shen B, Vihinen M, Casini A, Scozzafava A, Supuran CT, Parkkila AK, Saarnio J, Kivela AJ, Waheed A, Sly WS, Parkkila S.#TITLE:Characterization of CA XIII, a novel member of the carbonic anhydrase isozyme family.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14600151# GENE ASSOCIATION: (761.1) or (771.1) or (377677.1) or (762.1) or (766.2) or (23632.1) or (766.1) or (771.2) or (759.1) or (768.1) or (760.1)
Abbreviation: R_THRGLYexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gly[c] + thr-L[e] <==> gly[e] + thr-L[c] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_CLOHtex2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) cl[e] + oh1[c] --> (2) cl[c] + oh1[e] Confidence Level: 2 SAB genes: LOCUS:_65010#TRANSCRIPT:1#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:2#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:3#ABBREVIATION:SLC26A6# proteins: NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.1# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.2# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.3# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (65010.1) or (65010.3) or (65010.2)
chloride transport via hydroxide countertransport (2:1)
Abbreviation: R_EX_aqcobal_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : aqcobal <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PROSTGF2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: prostgf2[e] <==> prostgf2[c] Confidence Level: 4 electrogenic, Na indepdendent & rev NJ genes: LOCUS:_6580#TRANSCRIPT:1#ABBREVIATION:SLC22A1# LOCUS:_6582#TRANSCRIPT:1#ABBREVIATION:SLC22A2# LOCUS:_6582#TRANSCRIPT:2#ABBREVIATION:SLC22A2# proteins: NAME:solute carrier family 22 (organic cation transporter), member 1#ABBREVIATION:Slc22a1.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.2# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# AUTHORS:Koepsell H, Schmitt BM, Gorboulev V.#TITLE:Organic cation transporters.#JOURNAL:Rev Physiol Biochem Pharmacol#YEAR:2003#PUBMED:12827517# AUTHORS:Zhang L, Dresser MJ, Gray AT, Yost SC, Terashita S, Giacomini KM.#TITLE:Cloning and functional expression of a human liver organic cation transporter.#JOURNAL:Mol Pharmacol. #YEAR:1997#PUBMED:9187257# GENE ASSOCIATION: (6582.2) or (6582.1) or (6580.1)
Abbreviation: R_NDPK7m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + dcdp <==> adp + dctp Confidence Level: 2 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:dCDP), mitochondrial
Abbreviation: R_EX_whhdca_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : whhdca <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
omega hydroxy hexadecanoate (n-C16:0) exchange
Abbreviation: R_FAOXC1811602m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (5) coa + (4) fad + (5) h2o + (5) nad + vacccoa --> (5) accoa + (4) fadh2 + (5) h + (5) nadh + occoa Confidence Level: 4 _5 rounds of beta ox, 1 loss of degree of unsat -> 4 fad oxidized c18:1 -> c8:0 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_EX_acald_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acald <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PCHOLPr_hs Synonyms: _2 EC Number: 3.1.4.4 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [r] : h2o + pchol_hs --> chol + h + pa_hs Confidence Level: 4 NJ genes: LOCUS:_5337#TRANSCRIPT:1#ABBREVIATION:PLD1#ECNUMBERS:3.1.4.4# proteins: NAME:phospholipase D1, phosphatidylcholine-specific#ABBREVIATION:Pld1.1# citations: AUTHORS:Cases S, Stone SJ, Zhou P, Yen E, Tow B, Lardizabal KD, Voelker T, Farese RV Jr.#TITLE:Cloning of DGAT2, a second mammalian diacylglycerol acyltransferase, and related family members.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11481335# AUTHORS:Hammond SM, Altshuller YM, Sung TC, Rudge SA, Rose K, Engebrecht J, Morris AJ, Frohman MA.#TITLE:Human ADP-ribosylation factor-activated phosphatidylcholine-specific phospholipase D defines a new and highly conserved gene family.#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:8530346# GENE ASSOCIATION: (5337.1)
Abbreviation: R_ACOX2x Synonyms: _0 EC Number: 1.3.3.6 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : cholcoas + fadh2 + o2 --> cholcoads + fad + (2) h2o Confidence Level: 4 noted in uniprot to have FAD as cofactor - further reaction specific lit evidence not found to support or contradict, see Baumgart et al PNAS (1996) 93: 13748-13753 NJ genes: LOCUS:_8309#TRANSCRIPT:1#ABBREVIATION:ACOX2#ECNUMBERS:1.3.3.6# proteins: NAME:acel-Coenzyme A oxidase 2, branched chain#ABBREVIATION:Acox2.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Baumgart E, Vanhooren JCT, Fransen M, Marynen P, Puype M, Vandekerckhove J, Leunissen JAM, Fahimi HD, Mannaerts GP, Veldhoven PP#TITLE:Molecular characterization of the human peroxisomal branched-chain acyl-CoA oxidase#JOURNAL:Proc Natl Acad Sci#YEAR:1996# GENE ASSOCIATION: (8309.1)
Abbreviation: R_CYANtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: cyan[c] <==> cyan[m] Confidence Level: 0 assumed to be readily diffusible MM genes: none proteins: none citations: none GENE ASSOCIATION:
Cyanide transport via diffusion (mitochondrial)
Abbreviation: R_LTD4DP Synonyms: _0 EC Number: 2.3.2.2 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : h2o + leuktrD4 <==> gly + leuktrE4 Confidence Level: 4 Converts leukotriene D4 (LTD4) to leukotriene E4 (LTE4). unknown compartmentalization, cytosolic by default NJ genes: none proteins: none citations: AUTHORS:Leff AR.#TITLE:Regulation of leukotrienes in the management of asthma: biology and clinical therapy.#JOURNAL:Annu Rev Med#YEAR:2001#PUBMED:11160764# AUTHORS:Hammarstrom S, Orning L, Bernstrom K.#TITLE:Metabolism of leukotrienes.#JOURNAL:Mol Cell Biochem.#YEAR:1985#PUBMED:3001504# GENE ASSOCIATION:
Abbreviation: R_SPHMDAc Synonyms: _0 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : h2o + sphmyln_hs --> Rtotal + spc_hs Confidence Level: 0 limited details on mechanism, localization, etc. See PMID: 12069827 NJ genes: none proteins: none citations: AUTHORS:Meyer zu Heringdorf D, Himmel HM, Jakobs KH.#TITLE:Sphingosylphosphorylcholine-biological functions and mechanisms of action.#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12069827# GENE ASSOCIATION:
Abbreviation: R_ICDHyrm Synonyms: _0 EC Number: 1.1.1.42 SUBSYSTEM: Citric Acid Cycle Equation: [m] : icit + nadp <==> akg + co2 + nadph Confidence Level: 4 The reaction may be reversible. Strathern shows the reaction as irreversible. IF genes: LOCUS:_3418#TRANSCRIPT:1#ABBREVIATION:IDH2#ECNUMBERS:1.1.1.42# proteins: NAME:isocitrate dehydrogenase 2 (NADP+), mitochondrial#ABBREVIATION:Idh2.1-m# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Sazanov LA, Jackson JB.#TITLE:Proton-translocating transhydrogenase and NAD- and NADP-linked isocitrate dehydrogenases operate in a substrate cycle which contributes to fine regulation of the tricarboxylic acid cycle activity in mitochondria.#JOURNAL:FEBS Lett#YEAR:1994#PUBMED:8187868# AUTHORS:Comte B, Vincent G, Bouchard B, Benderdour M, Des Rosiers C.#TITLE:Reverse flux through cardiac NADP(+)-isocitrate dehydrogenase under normoxia and ischemia.#JOURNAL:Am J Physiol Heart Circ Physiol#YEAR:2002#PUBMED:12234803# GENE ASSOCIATION: (3418.1)
Abbreviation: R_G6PDA Synonyms: _0 EC Number: 3.5.99.6 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : gam6p + h2o --> f6p + nh4 Confidence Level: 4 Previously EC 5.3.1.10 genes: LOCUS:_10007#TRANSCRIPT:1#ABBREVIATION:GNPI#ECNUMBERS:3.5.99.6# LOCUS:_132789#TRANSCRIPT:1#ABBREVIATION:GNPDA2#ECNUMBERS:3.5.99.6# proteins: NAME:glucosamine-6-phosphate deaminase 1#ABBREVIATION:Gnpda1.1# NAME:glucosamine-6-phosphate deaminase 2 #ABBREVIATION:Gnpda2.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shevchenko V, Hogben M, Ekong R, Parrington J, Lai FA#TITLE:The human glucosamine-6-phosphate deaminase gene: cDNA cloning and expression, genomic organization and chromosomal localization#JOURNAL:Gene#YEAR:1998#PUBMED:9714720# GENE ASSOCIATION: (10007.1) or (132789.1)
Abbreviation: R_CYTK5 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : ctp + dcmp <==> cdp + dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_EX_10fthf6glu_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 10fthf6glu <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_10-formyltetrahydrofolate-[Glu](6) exchange
Abbreviation: R_AG13T17g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core4_pre4 + uacgam --> h + ksii_core4_pre5 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (10678.2) or (11041.1) or (10678.1) or (374907.2) or (93010.1) or (374907.1) or (284208.1) or (79369.1) or (10331.1)
keratan sulfate II biosynthesis, precursor 4_C61H99N5O43X
keratan sulfate II biosynthesis, precursor 5_C69H112N6O48X
Abbreviation: R_CYSGLUexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[e] + gln-L[c] <==> cys-L[c] + gln-L[e] Confidence Level: 2 SAB genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (6520.1) and (56301.1)
L-cysteine/L-glutamine reversible exchanger
Abbreviation: R_MCOATA Synonyms: _2 EC Number: 2.3.1.39 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : ACP + malcoa <==> coa + malACP Confidence Level: 4 tv genes: LOCUS:_2194#TRANSCRIPT:1#ABBREVIATION:FASN#ECNUMBERS:2.3.1.85# proteins: NAME:fatty acid synthase#ABBREVIATION:Fasn.1# citations: AUTHORS:Chakravarty B, Gu Z, Chirala SS, Wakil SJ, Quiocho FA.#TITLE:Human fatty acid synthase: structure and substrate selectivity of the thioesterase domain.#YEAR:2004#PUBMED:15507492# AUTHORS:Brink J, Ludtke SJ, Yang CY, Gu ZW, Wakil SJ, Chiu W.#TITLE:Quaternary structure of human fatty acid synthase by electron cryomicroscopy.#JOURNAL:Proc Natl Acad Sci#YEAR:2002#PUBMED:11756679# GENE ASSOCIATION: (2194.1)
Abbreviation: R_ST6GALNAC27 Synonyms: _5 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gt1c_hs --> cmp + gq1c_hs + h Confidence Level: 2 NJ genes: LOCUS:_10610#TRANSCRIPT:1#ABBREVIATION:SIAT7B#ECNUMBERS:2.4.99.7# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 2#ABBREVIATION:St6galnac2.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10610.1)
Abbreviation: R_IZPN Synonyms: _0 EC Number: 3.5.2.7 SUBSYSTEM: Histidine Metabolism Equation: [c] : 4izp + h2o --> forglu + h Confidence Level: 4 genes: none proteins: none citations: AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# GENE ASSOCIATION:
Abbreviation: R_BUTt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: but[e] + h[e] <==> but[c] + h[c] Confidence Level: 4 JLR genes: LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1#ABBREVIATION:Slc16a1.1# citations: AUTHORS:Halestrap AP, Price NT#TITLE:The proton-linked monocarboxylate transporter (MCT) family: structure, function and regulation#JOURNAL:Biochem J#YEAR:1999#PUBMED:10510291# AUTHORS:Garcia CK, Li X, Luna J, Francke U#TITLE:cDNA cloning of the human monocarboxylate transporter 1 and chromosomal localization of the SLC16A1 locus to 1p13.2-p12#JOURNAL:Genomics#YEAR:1994#PUBMED:7835905# AUTHORS:Poole RC, Halestrap AP#TITLE:Transport of lactate and other monocarboxylates across mammalian plasma membranes#JOURNAL:Am J Physiol#YEAR:1993#PUBMED:8476015# AUTHORS:Ritzhaupt A, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:The characterization of butyrate transport across pig and human colonic luminal membrane#JOURNAL:J Physiol#YEAR:1998#PUBMED:9508842# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification of a monocarboxylate transporter isoform type 1 (MCT1) on the luminal membrane of human and pig colon#JOURNAL:Biochem Soc Trans#YEAR:1998#PUBMED:9649795# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification and characterization of a monocarboxylate transporter (MCT1) in pig and human colon: its potential to transport L-lactate as well as butyrate#JOURNAL:J Physiol#YEAR:1998#PUBMED:9824713# AUTHORS:Halestrap AP, Meredith D#TITLE:The SLC16 gene family-from monocarboxylate transporters (MCTs) to aromatic amino acid transporters and beyond#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739169# AUTHORS:Kim do K, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H#TITLE:The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location#JOURNAL:Genomics#YEAR:2002#PUBMED:11827462# GENE ASSOCIATION: (6566.1)
Butyrate transport via proton symport, reversible
Abbreviation: R_ACGAGBSIDEtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: acgagbside_hs[c] <==> acgagbside_hs[l] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# GENE ASSOCIATION:
Abbreviation: R_ARTFR206 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: arachdcoa[c] + (2) fadh2[m] + (2) h[m] + (2) nadph[m] --> (1.25) R2coa_hs[c] + (2) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ARGLYSex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: arg-L[e] + lys-L[c] --> arg-L[c] + lys-L[e] Confidence Level: 4 SAB see PMID 14770310 genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_9057#TRANSCRIPT:1#ABBREVIATION:SLC7A6# proteins: NAME:solute carrier family 3 member 2/family 7 member 6#ABBREVIATION:Slc3a2/7a6# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (9057.1 and 6520.1)
Abbreviation: R_DMHPTCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: dmhptcrn[c] <==> dmhptcrn[m] Confidence Level: 1 dmhptcoa is 'special' because its biochemical fat isn't known NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UGALNACter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: udpacgal[c] + ump[r] <==> udpacgal[r] + ump[c] Confidence Level: 4 NCD genes: LOCUS:_23169#TRANSCRIPT:1#ABBREVIATION:SLC35D1# proteins: NAME:solute carrier family 35 (UDP-glucuronic acid/UDP-N-acetylgalactosamine dual transporter), member D1#ABBREVIATION:Slc35d1.1-r# citations: AUTHORS:Muraoka M, Kawakita M, Ishida N#TITLE:Molecular characterization of human UDP-glucuronic acid/UDP-N-acetylgalactosamine transporter, a novel nucleotide sugar transporter with dual substrate specificity#JOURNAL:FEBS Lett.#YEAR:2001#PUBMED:11322953# GENE ASSOCIATION: (23169.1)
UDP-GalNAc endoplasmic reticulum transport via CMP antiport
Dehydrodolichol, human uterine homolog_C1025H1660O10
Dehydrodolichol phosphate, human uterine homolog_C1025H1650O40P10
Abbreviation: R_DEDOLP2_U Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dedolp_U + h2o --> (0.1) dedol_U + pi Confidence Level: 0 EC-3.6.1.- NCD genes: none proteins: none citations: AUTHORS:Schenk B, Fernandez F, Waechter CJ.#TITLE:The ins(ide) and out(side) of dolichyl phosphate biosynthesis and recycling in the endoplasmic reticulum.#JOURNAL:Glycobiology#YEAR:2001#PUBMED:11425794# GENE ASSOCIATION:
dehydrodolichol phosphate phosphatase (uterus)
Abbreviation: R_GLNt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gln-L[e] + na1[e] --> gln-L[c] + na1[c] Confidence Level: 4 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# GENE ASSOCIATION: (81539.1) or (11254.1) or (54407.1) or (55089.1)
L-glutamine reversible transport via sodium symport
Abbreviation: R_CRTSLtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: crtsl[c] <==> crtsl[m] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FATP4t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: arach[c] + na1[c] <==> arach[e] + na1[e] Confidence Level: 4 electroneutral transport NJ genes: LOCUS:_10998#TRANSCRIPT:1#ABBREVIATION:SLC27A5# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 5#ABBREVIATION:Slc27a5.1# citations: AUTHORS:Steinberg SJ, Wang SJ, McGuinness MC, Watkins PA.#TITLE:Human liver-specific very-long-chain acyl-coenzyme A synthetase: cDNA cloning and characterization of a second enzymatically active protein.#JOURNAL:Mol Genet Metab.#YEAR:1999#PUBMED:10479480# AUTHORS:Stahl A.#TITLE:A current review of fatty acid transport proteins (SLC27).#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12856180# AUTHORS:Pohl J, Ring A, Hermann T, Stremmel W.#TITLE:Role of FATP in parenchymal cell fatty acid uptake.#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15522816# GENE ASSOCIATION: (10998.1)
Abbreviation: R_C204CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : arachdcoa + crn --> arachdcrn + coa Confidence Level: 4 TDV genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (126129.1) or (1375.3) or (1374.1) or (1375.2) or (1375.1) or (1375.4)
Abbreviation: R_NDP10ex Synonyms: _0 EC Number: 3.6.1.6 SUBSYSTEM: Nucleotides Equation: [e] : h2o + idp --> h + imp + pi Confidence Level: 4 IT genes: LOCUS:_124583#TRANSCRIPT:1#ABBREVIATION:CANT1# proteins: NAME:calcium activated nucleotidase 1 #ABBREVIATION:Cant1.1-e# citations: AUTHORS:Smith TM, Hicks-Berger CA, Kim S, Kirley TL.#TITLE:Cloning, expression, and characterization of a soluble calcium-activated nucleotidase, a human enzyme belonging to a new family of extracellular nucleotidases.#JOURNAL:Arch Biochem Biophys. #YEAR:2002#PUBMED:12234496# AUTHORS:Murphy DM, Ivanenkov VV, Kirley TL. #TITLE:Bacterial expression and characterization of a novel, soluble, calcium-binding, and calcium-activated human nucleotidase. #JOURNAL:Biochemistry#YEAR:2003#PUBMED:12600208# GENE ASSOCIATION: (124583.1)
nucleoside-diphosphatase (IDP), extracellular
H5 mannosyltransferase, endoplasmic reticulum
Abbreviation: R_H5MTer_U Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_U + emgacpail_hs --> (0.1) dolp_U + h + memgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_55650#TRANSCRIPT:1#ABBREVIATION:PIGV# proteins: NAME:phosphatidylinositol glycan, class V, endoplasmic reticulum#ABBREVIATION:Pigv.1-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Kang JY, Hong Y, Ashida H, Shishioh N, Murakami Y, Morita YS, Maeda Y, Kinoshita T#TITLE:IG-V involved in transferring the second mannose in glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15623507# GENE ASSOCIATION: (55650.1)
_4-[(1R)-1-Hydroxy-2-(methylamino)ethyl]-1,2-benzenediol:oxygen oxidoreductase(deaminating)(flavin-containing)
Abbreviation: R_41R1H2MAE12BOOX Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Tyrosine metabolism Equation: [c] : adrnl + h2o + o2 --> 34dhmald + h2o2 + mma Confidence Level: 4 SAB genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# proteins: NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# GENE ASSOCIATION: (4128.1) or (4129.1)
Abbreviation: R_EX_ocdca_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ocdca <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6T9g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre19 + paps --> h + ksi_pre20 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_AMY1e Synonyms: _0 EC Number: 3.2.1.1 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [e] : (8) h2o + strch1 --> (8) glc-D + strch2 Confidence Level: 0 NCD genes: LOCUS:_276#TRANSCRIPT:1#ABBREVIATION:AMY1A#ECNUMBERS:3.2.1.1# LOCUS:_280#TRANSCRIPT:1#ABBREVIATION:AMY2B#ECNUMBERS:3.2.1.1# LOCUS:_279#TRANSCRIPT:1#ABBREVIATION:AMY2A#ECNUMBERS:3.2.1.1# LOCUS:_278#TRANSCRIPT:1#ABBREVIATION:AMY1C#ECNUMBERS:3.2.1.1# LOCUS:_277#TRANSCRIPT:1#ABBREVIATION:AMY1B#ECNUMBERS:3.2.1.1# LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_6519#TRANSCRIPT:1#ABBREVIATION:SLC3A1# LOCUS:_11136#TRANSCRIPT:1#ABBREVIATION:SLC7A9# LOCUS:_276#TRANSCRIPT:2#ABBREVIATION:AMY1A#ECNUMBERS:3.2.1.1# proteins: NAME:amylase, alpha 1A; salivary, extracellular (saliva)#ABBREVIATION:Amy1a.1-e# NAME:amylase, alpha 1A; salivary, extracellular (saliva)#ABBREVIATION:Amy1a.2-e# NAME:amylase, alpha 1c; salivary, extracellular (saliva)#ABBREVIATION:Amy1b.1-e# NAME:amylase, alpha 1c; salivary, extracellular (saliva)#ABBREVIATION:Amy1c.1-e# NAME:amylase, alpha 2A; pancreatic, extracellular (sm intestine)#ABBREVIATION:Amy2a.1-e# NAME:amylase, alpha 2B; pancreatic, extracellular (sm intestine)#ABBREVIATION:Amy2b.1-e# NAME:solute carrier family (amino acid transporter), extracellular#ABBREVIATION:Slc3a1/7a9-e# NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# citations: none GENE ASSOCIATION: (6520.1) or (280.1) or (278.1) or (276.1) or (277.1) or (6519.1 and 11136.1) or (276.2) or (279.1)
alpha-amylase, extracellular (strch1 -> strch2)
Abbreviation: R_DCT Synonyms: _8 EC Number: 5.3.3.12 SUBSYSTEM: Tyrosine metabolism Equation: [c] : L-dpchrm <==> 56dihindlcrbxlt Confidence Level: 4 ISOMERASE SAB added EC and adjusted compounds for balance genes: LOCUS:_1638#TRANSCRIPT:1#ABBREVIATION:DCT#ECNUMBERS:5.3.3.12# proteins: NAME:dopachrome tautomerase#ABBREVIATION:Dct.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# AUTHORS:Yokoyama K, Suzuki H, Yasumoto K, Tomita Y, Shibahara S#TITLE:Molecular cloning and functional analysis of a cDNA coding for human DOPAchrome tautomerase/tyrosinase-related protein-2#JOURNAL:Biochim Biophys Acta#YEAR:1994#PUBMED:8148378# GENE ASSOCIATION: (1638.1)
Abbreviation: R_EX_whddca_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : whddca <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
omega hydroxy dodecanoate (n-C12:0) exchange
Abbreviation: R_PDE4 Synonyms: _0 EC Number: 3.1.4.17 SUBSYSTEM: Nucleotides Equation: [c] : 35cgmp + h2o --> gmp + h Confidence Level: 4 NCD genes: LOCUS:_5139#TRANSCRIPT:1#ABBREVIATION:PDE3A#ECNUMBERS:3.1.4.17# LOCUS:_5149#TRANSCRIPT:1#ABBREVIATION:PDE6H# LOCUS:_5145#TRANSCRIPT:1#ABBREVIATION:PDE6A# LOCUS:_8654#TRANSCRIPT:1#ABBREVIATION:PDE5A#ECNUMBERS:3.1.4.17# LOCUS:_5138#TRANSCRIPT:1#ABBREVIATION:PDE2A# LOCUS:_5148#TRANSCRIPT:1#ABBREVIATION:PDE6G# LOCUS:_5153#TRANSCRIPT:1#ABBREVIATION:PDE1B#ECNUMBERS:3.1.4.17# LOCUS:_5137#TRANSCRIPT:1#ABBREVIATION:PDE1C# LOCUS:_5136#TRANSCRIPT:1#ABBREVIATION:PDE1A#ECNUMBERS:3.1.4.17# LOCUS:_10846#TRANSCRIPT:1#ABBREVIATION:PDE10A# LOCUS:_5147#TRANSCRIPT:1#ABBREVIATION:PDE6D#ECNUMBERS:3.1.4.17# LOCUS:_5146#TRANSCRIPT:1#ABBREVIATION:PDE6C# LOCUS:_5140#TRANSCRIPT:1#ABBREVIATION:PDE3B# LOCUS:_5158#TRANSCRIPT:1#ABBREVIATION:PDE6B# LOCUS:_50940#TRANSCRIPT:1#ABBREVIATION:PDE11A# LOCUS:_5152#TRANSCRIPT:15#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:5#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:16#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:2#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:17#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5140#TRANSCRIPT:2#ABBREVIATION:PDE3B# LOCUS:_5152#TRANSCRIPT:18#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:6#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:10#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:4#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:7#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:19#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:11#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:12#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:20#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:13#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:3#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5152#TRANSCRIPT:14#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# LOCUS:_5136#TRANSCRIPT:2#ABBREVIATION:PDE1A# LOCUS:_8654#TRANSCRIPT:2#ABBREVIATION:PDE5A# LOCUS:_8654#TRANSCRIPT:3#ABBREVIATION:PDE5A# proteins: NAME:phosphodiesterase 6, cGMP-specific, cone, gamma #ABBREVIATION:Pde6_2# NAME:phosphodiesterase 6, cGMP-specific, rod (congenital stationary night blindness 3, autosomal dominant)#ABBREVIATION:Pde6_1# NAME:phosphodiesterase 6C, cGMP-specific, cone, alpha prime #ABBREVIATION:Pde6_3.1# NAME:phosphodiesterase 3A, cGMP-inhibited #ABBREVIATION:Pde3A.1# NAME:phosphodiesterase 3B, cGMP-inhibited #ABBREVIATION:Pde3B.1# NAME:phosphodiesterase 3B, cGMP-inhibited#ABBREVIATION:Pde3B.2# NAME:phosphodiesterase 2A, cGMP-stimulated #ABBREVIATION:Pde2A.1# NAME:phosphodiesterase 1A, calmodulin-dependent #ABBREVIATION:Pde1A.1# NAME:phosphodiesterase 1A, calmodulin-dependent #ABBREVIATION:Pde1A.2# NAME:phosphodiesterase 1B, calmodulin-dependent #ABBREVIATION:Pde1B.1# NAME:phosphodiesterase 1C, calmodulin-dependent 70kDa #ABBREVIATION:Pde1C.1# NAME:phosphodiesterase 10A #ABBREVIATION:Pde10A# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.2# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.3# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.4# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.5# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.6# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.7# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.11# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.20# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.19# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.18# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.17# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.16# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.15# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.14# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.13# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.10# NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.12# NAME:phosphodiesterase 11A #ABBREVIATION:Pde11A.1# NAME:phosphodiesterase 5A, cGMP-specific #ABBREVIATION:Pde5A.1# NAME:phosphodiesterase 5A, cGMP-specific #ABBREVIATION:Pde5A.2# NAME:phosphodiesterase 5A, cGMP-specific #ABBREVIATION:Pde5A.3# citations: AUTHORS:Shimizu-Matsumoto A, Itoh K, Inazawa J, Nishida K, Matsumoto Y, Kinoshita S, Matsubara K, Okubo K.#TITLE:Isolation and chromosomal localization of the human cone cGMP phosphodiesterase gamma cDNA (PDE6H).#JOURNAL:Genomics#YEAR:1996#PUBMED:8786098# AUTHORS:White JB, Thompson WJ, Pittler SJ. #TITLE:Characterization of 3',5' cyclic nucleotide phosphodiesterase activity in Y79 retinoblastoma cells: absence of functional PDE6.#JOURNAL:Mol Vis.#YEAR:2004#PUBMED:15480303# AUTHORS:Lefievre L, de Lamirande E, Gagnon C.#TITLE:Presence of cyclic nucleotide phosphodiesterases PDE1A, existing as a stable complex with calmodulin, and PDE3A in human spermatozoa.#JOURNAL:Biol Reprod. #YEAR:2002#PUBMED:12135876# AUTHORS:Wechsler J, Choi YH, Krall J, Ahmad F, Manganiello VC, Movsesian MA.#TITLE:Isoforms of cyclic nucleotide phosphodiesterase PDE3A in cardiac myocytes.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12154085# AUTHORS:Shakur Y, Takeda K, Kenan Y, Yu ZX, Rena G, Brandt D, Houslay MD, Degerman E, Ferrans VJ, Manganiello VC. #TITLE:Membrane localization of cyclic nucleotide phosphodiesterase 3 (PDE3). Two N-terminal domains are required for the efficient targeting to, and association of, PDE3 with endoplasmic reticulum.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10952971# AUTHORS:Scapin G, Patel SB, Chung C, Varnerin JP, Edmondson SD, Mastracchio A, Parmee ER, Singh SB, Becker JW, Van der Ploeg LH, Tota MR.#TITLE:Crystal structure of human phosphodiesterase 3B: atomic basis for substrate and inhibitor specificity. #JOURNAL:Biochemistry. #YEAR:2004#PUBMED:15147193# AUTHORS:Miki T, Taira M, Hockman S, Shimada F, Lieman J, Napolitano M, Ward D, Taira M, Makino H, Manganiello VC.#TITLE:Characterization of the cDNA and gene encoding human PDE3B, the cGIP1 isoform of the human cyclic GMP-inhibited cyclic nucleotide phosphodiesterase family.#JOURNAL:Genomics#YEAR:1996#PUBMED:8884271# AUTHORS:Rosman GJ, Martins TJ, Sonnenburg WK, Beavo JA, Ferguson K, Loughney K. #TITLE:Isolation and characterization of human cDNAs encoding a cGMP-stimulated 3',5'-cyclic nucleotide phosphodiesterase.#JOURNAL:Gene#YEAR:1997#PUBMED:9210593# AUTHORS:Manns JM, Brenna KJ, Colman RW, Sheth SB. #TITLE:Differential regulation of human platelet responses by cGMP inhibited and stimulated cAMP phosphodiesterases. #JOURNAL:Thromb Haemost.#YEAR:2002#PUBMED:12038792# AUTHORS:Yu J, Wolda SL, Frazier AL, Florio VA, Martins TJ, Snyder PB, Harris EA, McCaw KN, Farrell CA, Steiner B, Bentley JK, Beavo JA, Ferguson K, Gelinas R.#TITLE:Identification and characterisation of a human calmodulin-stimulated phosphodiesterase PDE1B1.#JOURNAL:Cell Signal#YEAR:1997#PUBMED:9419816# AUTHORS:Goraya TA, Masada N, Ciruela A, Cooper DM.#TITLE:Sustained entry of Ca2+ is required to activate Ca2+-calmodulin-dependent phosphodiesterase 1A.#JOURNAL: J Biol Chem. #YEAR:2004#PUBMED:15272012# AUTHORS:Kotera J, Sasaki T, Kobayashi T, Fujishige K, Yamashita Y, Omori K.#TITLE:Subcellular localization of cyclic nucleotide phosphodiesterase type 10A variants, and alteration of the localization by cAMP-dependent protein kinase-dependent phosphorylation. #JOURNAL:J Biol Chem.#YEAR:2004#PUBMED:14604994# AUTHORS:Wang P, Wu P, Egan RW, Billah MM.#TITLE:Identification and characterization of a new human type 9 cGMP-specific phosphodiesterase splice variant (PDE9A5). Differential tissue distribution and subcellular localization of PDE9A variants.#JOURNAL:Gene#YEAR:2003#PUBMED:14527714# AUTHORS:Identification and distribution of different mRNA variants produced by differential splicing in the human phosphodiesterase 9A gene. #TITLE:Identification and distribution of different mRNA variants produced by differential splicing in the human phosphodiesterase 9A gene. #JOURNAL:Biochem Biophys Res Commun.#YEAR:2003#PUBMED:12565835# AUTHORS:Fisher DA, Smith JF, Pillar JS, St Denis SH, Cheng JB.#TITLE:Isolation and characterization of PDE9A, a novel human cGMP-specific phosphodiesterase.#JOURNAL:J Biol Chem.#YEAR:1998#PUBMED:9624146# AUTHORS:Yanaka N, Kotera J, Ohtsuka A, Akatsuka H, Imai Y, Michibata H, Fujishige K, Kawai E, Takebayashi S, Okumura K, Omori K.#TITLE:Expression, structure and chromosomal localization of the human cGMP-binding cGMP-specific phosphodiesterase PDE5A gene.#JOURNAL: Eur J Biochem. #YEAR:1998#PUBMED:9716380# GENE ASSOCIATION: (5139.1) or (5146.1) or (5152.3) or (5152.14) or (5153.1) or (50940.1) or (5152.18) or (5140.1) or (5152.13) or (10846.1) or (5152.17) or (5145.1 and 5158.1 and 5149.1) or (5152.12) or (5152.19) or (5152.5) or (8654.3) or (8654.2) or (5152.10) or (5136.2) or (8654.1) or (5140.2) or (5152.2) or (5152.4) or (5152.15) or (5152.6) or (5152.7) or (5137.1) or (5138.1) or (5152.11) or (5152.16) or (5152.20) or (5145.1 and 5146.1 and 5147.1 and 5158.1 and 5148.1) or (5136.1)
_3',5'-cyclic-nucleotide phosphodiesterase
phosphorylase-limit dextrin (glycogenin-1,6{4[1,4-Glc], 4[1,4-Glc]})_C48H81O41X
malonate-semialdehyde dehydrogenase (acetylating), mitochondrial
Abbreviation: R_MMTSADm Synonyms: _1 EC Number: 1.2.1.18 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 2mop + coa + nad --> h + mmcoa-R + nadh Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION:
Abbreviation: R_DHCRD2 Synonyms: _1 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : dhcrm_hs + fad --> crm_hs + fadh2 Confidence Level: 4 NJ genes: LOCUS:_8560#TRANSCRIPT:1#ABBREVIATION:DEGS1# LOCUS:_8560#TRANSCRIPT:2#ABBREVIATION:DEGS1# proteins: NAME:degenerative spermatocyte homolog 1, lipid desaturase#ABBREVIATION:Degs1.1# NAME:degenerative spermatocyte homolog 1, lipid desaturase#ABBREVIATION:Degs1.2# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Cadena DL, Kurten RC, Gill GN.#TITLE:The product of the MLD gene is a member of the membrane fatty acid desaturase family: overexpression of MLD inhibits EGF receptor biosynthesis.#JOURNAL:Biochemistry#YEAR:1997#PUBMED:9188692# GENE ASSOCIATION: (8560.1) or (8560.2)
Abbreviation: R_Htr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: h[c] <==> h[r] Confidence Level: 1 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PTRCOX1 Synonyms: _0 EC Number: 1.4.3.6 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : h2o + o2 + ptrc --> 4abutn + h2o2 + nh4 Confidence Level: 2 SAB genes: LOCUS:_314#TRANSCRIPT:1#ABBREVIATION:AOC2#ECNUMBERS:1.4.3.6# LOCUS:_8639#TRANSCRIPT:1#ABBREVIATION:AOC3#ECNUMBERS:1.4.3.6# LOCUS:_26#TRANSCRIPT:1#ABBREVIATION:ABP1#ECNUMBERS:1.4.3.6# LOCUS:_314#TRANSCRIPT:2#ABBREVIATION:AOC2# proteins: NAME:amine oxidase, copper containing 3#ABBREVIATION:Aoc3.1# NAME:amiloride binding protein 1 (amine oxidase (copper-containing))#ABBREVIATION:Abp1.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.2# citations: AUTHORS:Imamura Y, Kubota R, Wang Y, Asakawa S, Kudoh J, Mashima Y, Oguchi Y, Shimizu N#TITLE:Human retina-specific amine oxidase (RAO): cDNA cloning, tissue expression, and chromosomal mapping#JOURNAL:Genomics#YEAR:1997#PUBMED:9119395# GENE ASSOCIATION: (26.1) or (314.1) or (8639.1) or (314.2)
Putrescine:oxygen oxidoreductase (deaminating)
Abbreviation: R_FUT93g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fuc12gal14acglcgalgluside_hs + gdpfuc --> fucfuc12gal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
Abbreviation: R_NDPK2n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + udp <==> adp + utp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:UDP), nuclear
Abbreviation: R_LNLNCAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lnlnca[e] <==> lnlnca[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_NP1 Synonyms: _0 EC Number: 2.4.2.1 SUBSYSTEM: NAD Metabolism Equation: [c] : h + nac + r1p --> nicrns + pi Confidence Level: 2 NCD genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (4860.1)
Abbreviation: R_HCO3Em Synonyms: _0 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : co2 + h2o <==> h + hco3 Confidence Level: 2 IF genes: none proteins: none citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION:
Type IIIH glycolipid_C70H122N3O40FULLRCO
Abbreviation: R_FAEL204 Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : arachdcoa + (5) h + malcoa + (4) nadph + o2 --> adrncoa + co2 + coa + (3) h2o + (4) nadp Confidence Level: 4 fatty acid elongation step NJ genes: LOCUS:_6785#TRANSCRIPT:1#ABBREVIATION:ELOVL4# proteins: NAME:elongation of very long chain fatty acids - like 4#ABBREVIATION:Elovl4.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Zhang K, Kniazeva M, Han M, Li W, Yu Z, Yang Z, Li Y, Metzker ML, Allikmets R, Zack DJ, Kakuk LE, Lagali PS, Wong PW, MacDonald IM, Sieving PA, Figueroa DJ, Austin CP, Gould RJ, Ayyagari R, Petrukhin K.#TITLE:A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy.#JOURNAL:Nat Genet#YEAR:2001#PUBMED:11138005# AUTHORS:Nakamura MT, Nara TY.#TITLE:Structure, function, and dietary regulation of delta6, delta5, and delta9 desaturases.#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189125# GENE ASSOCIATION: (6785.1)
Abbreviation: R_S2L2FN2M2MASNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: s2l2fn2m2masn[g] --> s2l2fn2m2masn[e] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
s2l2fn2m2masn transport, Golgi to extracellular
Abbreviation: R_EX_mthgxl_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : mthgxl <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_XYLTD_Dr Synonyms: _0 EC Number: 1.1.1.9 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : nad + xylt <==> h + nadh + xylu-D Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Wang YM, van Eys J#TITLE:Nutritional significance of fructose and sugar alcohols#JOURNAL:Annu Rev Nutr.#YEAR:1981#PUBMED:6821187# GENE ASSOCIATION:
xylitol dehydrogenase (D-xyulose-forming)
fatty acyl-CoA desaturase (n-C24:5CoA -> n-C24:6CoA)
Abbreviation: R_DESAT24_1 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + nadh + o2 + tetpent3coa --> (2) h2o + nad + tethex3coa Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_9415#TRANSCRIPT:1#ABBREVIATION:FADS2#ECNUMBERS:1.14.99.25# proteins: NAME:fatty acid desaturase 2#ABBREVIATION:Fads2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION: (9415.1)
Abbreviation: R_EX_thymd_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : thymd <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SPHINGStr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: sphings[c] <==> sphings[r] Confidence Level: 1 unknown mechanism, however sphingosine manages to get in different compartments of cells. NOT presumed to be flip-flop. NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RTOTAL3CRNCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : Rtotal3coa + crn --> Rtotal3crn + coa Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (1375.1) or (1375.4) or (1375.1) or (1374.1) or (126129.1) or (1375.2)
Abbreviation: R_UROLACer Synonyms: _1 EC Number: 3.1.1.19 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [r] : glcur + h <==> glac + h2o Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Winkelman J, Lehninger AL#TITLE:Aldono- and uronolactonases of animal tissues.#JOURNAL:J Biol Chem#YEAR:1958#PUBMED:13587494# GENE ASSOCIATION:
Abbreviation: R_EX_fe3_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fe3 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FUC14GALACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fuc14galacglcgalgluside_hs[g] <==> fuc14galacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_AMACR2p Synonyms: _0 EC Number: 5.1.99.4 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : dhcholestancoa + (0.5) o2 --> dhcholoylcoa + h2o Confidence Level: 2 should be analogous to AMACRp as per KEGG - in this case: reductase not a racemase NJ genes: LOCUS:_23600#TRANSCRIPT:1#ABBREVIATION:AMACR# LOCUS:_23600#TRANSCRIPT:2#ABBREVIATION:AMACR# proteins: NAME:alpha-methylacyl-CoA racemase#ABBREVIATION:Amacr.1# NAME:alpha-methylacyl-CoA racemase#ABBREVIATION:Amacr.2# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (23600.1) or (23600.2)
alpha-methylacyl-CoA racemase (reductase)
Abbreviation: R_GQ1BALPHAtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gq1balpha_hs[c] <==> gq1balpha_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_EX_glygn5_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : glygn5 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
glycogen, structure 5 (glycogenin-2[1,4-Glc]) exchange
Abbreviation: R_MLTG1ly Synonyms: _0 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [l] : h2o + malttr --> glc-D + malt Confidence Level: 4 JLR genes: LOCUS:_2548#TRANSCRIPT:1#ABBREVIATION:GAA#ECNUMBERS:3.2.1.20# proteins: NAME:alpha glucosidase; acid (Pompe disease, glycogen storage disease type II), lysosome#ABBREVIATION:Gaa.1-l# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Hoefsloot LH, Hoogeveen-Westerveld M, Kroos MA, van Beeumen J, Reuser AJ, Oostra BA#TITLE:Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex#JOURNAL:EMBO J#YEAR:1988#PUBMED:3049072# GENE ASSOCIATION: (2548.1)
Maltodextrin glucosidase (maltotriose), lysosome
Abbreviation: R_ME2m Synonyms: _0 EC Number: 1.1.1.40 SUBSYSTEM: Pyruvate Metabolism Equation: [m] : mal-L + nadp --> co2 + nadph + pyr Confidence Level: 0 IF genes: LOCUS:_4200#TRANSCRIPT:1#ABBREVIATION:ME2#ECNUMBERS:1.1.1.40# LOCUS:_10873#TRANSCRIPT:1#ABBREVIATION:ME3#ECNUMBERS:1.1.1.40# proteins: NAME:malic enzyme 2, NAD(+)-dependent, mitochondrial#ABBREVIATION:Me2.1-m# NAME:malic enzyme 3, NADP(+)-dependent, mitochondrial#ABBREVIATION:Me3.1-m# citations: AUTHORS:Loeber G, Infante AA, Maurer-Fogy I, Krystek E, Dworkin MB.#TITLE:Human NAD(+)-dependent mitochondrial malic enzyme. cDNA cloning, primary structure, and expression in Escherichia coli.#JOURNAL:J Biol Chem.#YEAR:1991#PUBMED:1993674# GENE ASSOCIATION: (4200.1) or (10873.1)
Abbreviation: R_EX_tmndnc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tmndnc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DOLMANP_Lter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: (0.1) dolmanp_L[c] --> (0.1) dolmanp_L[r] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION:
dolichol-phosphate mannose flippase (liver)
Abbreviation: R_PI3P5K Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + pail3p_hs --> adp + h + pail35p_hs Confidence Level: 2 NCD FULLR corresponds to acyl/R groups in texts. NJ changed EC from 2.7.1.149 (pail5p) to 2.7.1.68 (pail4p) - NCD genes: LOCUS:_8394#TRANSCRIPT:1#ABBREVIATION:PIP5K1A#ECNUMBERS:2.7.1.68# LOCUS:_23396#TRANSCRIPT:1#ABBREVIATION:PIP5K1C# LOCUS:_8395#TRANSCRIPT:1#ABBREVIATION:PIP5K1B# proteins: NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, gamma #ABBREVIATION:Pip5k1c.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, beta#ABBREVIATION:Pip5k1b.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, alpha#ABBREVIATION:Pip5k1a.1# citations: AUTHORS:Tolias KF, Cantley LC#TITLE:Pathways for phosphoinositide synthesis#JOURNAL:Chem Phys Lipids#YEAR:1999#PUBMED:10358929# GENE ASSOCIATION: (8394.1) or (23396.1) or (8395.1)
phosphatidylinositol 3-phosphate 5-kinase
Abbreviation: R_S6T10g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre22 + paps --> h + ksi_pre23 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_ADNt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: adn[e] + na1[e] --> adn[c] + na1[c] Confidence Level: 4 MM genes: LOCUS:_9154#TRANSCRIPT:1#ABBREVIATION:SLC28A1# LOCUS:_9153#TRANSCRIPT:1#ABBREVIATION:SLC28A2# LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# LOCUS:_9154#TRANSCRIPT:2#ABBREVIATION:SLC28A1# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 1, extracellular #ABBREVIATION:Slc28a1.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 2, extracellular #ABBREVIATION:Slc28a2.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 1 #ABBREVIATION:Slc28a1.2-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (9154.2) or (9154.1) or (64078.1) or (9153.1)
adenosine transport (Na/Adn cotransport)
Abbreviation: R_EX_pnto_DASH_R_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pnto-R <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_leu_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : leu-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLCt1r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glc-D[e] <==> glc-D[c] Confidence Level: 4 genes: LOCUS:_6515#TRANSCRIPT:1#ABBREVIATION:SLC2A3# LOCUS:_155184#TRANSCRIPT:1#ABBREVIATION:SLC2A7# LOCUS:_66035#TRANSCRIPT:1#ABBREVIATION:SLC2A11# LOCUS:_56606#TRANSCRIPT:1#ABBREVIATION:SLC2A9# LOCUS:_6514#TRANSCRIPT:1#ABBREVIATION:SLC2A2# LOCUS:_11182#TRANSCRIPT:1#ABBREVIATION:SLC2A6# LOCUS:_144195#TRANSCRIPT:1#ABBREVIATION:SLC2A14# LOCUS:_6513#TRANSCRIPT:1#ABBREVIATION:SLC2A1# LOCUS:_81031#TRANSCRIPT:1#ABBREVIATION:SLC2A10# LOCUS:_6517#TRANSCRIPT:1#ABBREVIATION:SLC2A4# LOCUS:_56606#TRANSCRIPT:2#ABBREVIATION:SLC2A9# LOCUS:_29988#TRANSCRIPT:1#ABBREVIATION:SLC2A8# LOCUS:_154091#TRANSCRIPT:1#ABBREVIATION:SLC2A12# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 1, plasma membrane#ABBREVIATION:Slc2a1.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 2, extracellular#ABBREVIATION:Slc2a2.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 3, extracellular#ABBREVIATION:Slc2a3.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 4, extracellular#ABBREVIATION:Slc2a4.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 6, extracellular#ABBREVIATION:Slc2a6.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 7, extracellular#ABBREVIATION:Slc2a7.1-e# NAME:solute carrier family 2, (facilitated glucose transporter) member 8, extracellular#ABBREVIATION:Slc2a8.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 9, extracellular#ABBREVIATION:Slc2a9.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 9, extracellular#ABBREVIATION:Slc2a9.2-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 10, extracellular#ABBREVIATION:Slc2a10.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 11, extracellular#ABBREVIATION:Slc2a11.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 12, extracellular#ABBREVIATION:Slc2a12.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 14, extracellular#ABBREVIATION:Slc2a14.1-e# citations: AUTHORS:Hosokawa M, Thorens B#TITLE:Glucose release from GLUT2-null hepatocytes: characterization of a major and a minor pathway#JOURNAL:Am J Physiol Endocrinol Metab#YEAR:2002#PUBMED:11882499# AUTHORS:Guillam MT, Burcelin R, Thorens B#TITLE:Normal hepatic glucose production in the absence of GLUT2 reveals an alternative pathway for glucose release from hepatocytes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9770484# AUTHORS:Uldry M, Thorens B#TITLE:The SLC2 family of facilitated hexose and polyol transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750891# AUTHORS:Flier JS, Mueckler MM, Usher P, Lodish HF#TITLE:Elevated levels of glucose transport and transporter messenger RNA are induced by ras or src oncogenes#JOURNAL:Science#YEAR:1987#PUBMED:3103217# AUTHORS:Maher F, Vannucci SJ, Simpson IA#TITLE:Glucose transporter proteins in brain#JOURNAL:FASEB J#YEAR:1994#PUBMED:7926364# AUTHORS:Mueckler M, Caruso C, Baldwin SA, Panico M, Blench I, Morris HR, Allard WJ, Lienhard GE, Lodish HF#TITLE:Sequence and structure of a human glucose transporter#JOURNAL:Science#YEAR:1985#PUBMED:3839598# AUTHORS:Uldry M, Ibberson M, Hosokawa M, Thorens B#TITLE:GLUT2 is a high affinity glucosamine transporter#JOURNAL:FEBS Lett#YEAR:2002#PUBMED:12135767# AUTHORS:Fukumoto H, Seino S, Imura H, Seino Y, Eddy RL, Fukushima Y, Byers MG, Shows TB, Bell GI#TITLE:Sequence, tissue distribution, and chromosomal localization of mRNA encoding a human glucose transporter-like protein#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1988#PUBMED:3399500# AUTHORS:Johnson JH, Newgard CB, Milburn JL, Lodish HF, Thorens B#TITLE:The high Km glucose transporter of islets of Langerhans is functionally similar to the low affinity transporter of liver and has an identical primary sequence#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2182619# AUTHORS:Thorens B, Cheng ZQ, Brown D, Lodish HF#TITLE:Liver glucose transporter: a basolateral protein in hepatocytes and intestine and kidney cells#JOURNAL:Am J Physiol#YEAR:1990#PUBMED:1701966# AUTHORS:Haber RS, Weinstein SP, O'Boyle E, Morgello S#TITLE:Tissue distribution of the human GLUT3 glucose transporte#JOURNAL:Endocrinology#YEAR:1993#PUBMED:8504756# AUTHORS:Heijnen HF, Oorschot V, Sixma JJ, Slot JW, James DE#TITLE:Thrombin stimulates glucose transport in human platelets via the translocation of the glucose transporter GLUT-3 from alpha-granules to the cell surface#JOURNAL:J Cell Biol#YEAR:1997#PUBMED:9230074# AUTHORS:Kayano T, Fukumoto H, Eddy RL, Fan YS, Byers MG, Shows TB, Bell GI#TITLE:Evidence for a family of human glucose transporter-like proteins. Sequence and gene localization of a protein expressed in fetal skeletal muscle and other tissues#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3170580# AUTHORS:Stuart CA, Wen G, Jiang J#TITLE:GLUT3 protein and mRNA in autopsy muscle specimens#JOURNAL:Metabolism#YEAR:1999#PUBMED:10421229# AUTHORS:Fukumoto H, Kayano T, Buse JB, Edwards Y, Pilch PF, Bell GI, Seino S#TITLE:Cloning and characterization of the major insulin-responsive glucose transporter expressed in human skeletal muscle and other insulin-responsive tissues#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2656669# AUTHORS:Doege H, Bocianski A, Joost HG, Schurmann A#TITLE:Activity and genomic organization of human glucose transporter 9 (GLUT9), a novel member of the family of sugar-transport facilitators predominantly expressed in brain and leucocytes#JOURNAL:Biochem J#YEAR:2000#PUBMED:10970791# AUTHORS:Li Q, Manolescu A, Ritzel M, Yao S, Slugoski M, Young JD, Chen XZ, Cheeseman CI#TITLE:Cloning and functional characterization of the human GLUT7 isoform SLC2A7 from the small intestine#JOURNAL:Am J Physiol Gastrointest Liver Physiol#YEAR:2004#PUBMED:15033637# AUTHORS:Joost HG, Thorens B#TITLE:The extended GLUT-family of sugar/polyol transport facilitators: nomenclature, sequence characteristics, and potential function of its novel members (review)#JOURNAL:Mol Membr Biol#YEAR:2001#PUBMED:11780753# AUTHORS:Doege H, Schurmann A, Bahrenberg G, Brauers A, Joost HG#TITLE:GLUT8, a novel member of the sugar transport facilitator family with glucose transport activity#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10821868# AUTHORS:Carayannopoulos MO, Chi MM, Cui Y, Pingsterhaus JM, McKnight RA, Mueckler M, Devaskar SU, Moley KH#TITLE:GLUT8 is a glucose transporter responsible for insulin-stimulated glucose uptake in the blastocyst#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10860996# AUTHORS:Augustin R, Carayannopoulos MO, Dowd LO, Phay JE, Moley JF, Moley KH#TITLE:Identification and characterization of human glucose transporter-like protein-9 (GLUT9): alternative splicing alters trafficking#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14739288# AUTHORS:Phay JE, Hussain HB, Moley JF#TITLE:Cloning and expression analysis of a novel member of the facilitative glucose transporter family, SLC2A9 (GLUT9)#JOURNAL:Genomics#YEAR:2000#PUBMED:10860667# AUTHORS:Dawson PA, Mychaleckyj JC, Fossey SC, Mihic SJ, Craddock AL, Bowden DW#TITLE:Sequence and functional analysis of GLUT10: a glucose transporter in the Type 2 diabetes-linked region of chromosome 20q12-13.1#JOURNAL:Mol Genet Metab#YEAR:2001#PUBMED:11592815# AUTHORS:McVie-Wylie AJ, Lamson DR, Chen YT#TITLE:Molecular cloning of a novel member of the GLUT family of transporters, SLC2a10 (GLUT10), localized on chromosome 20q13.1: a candidate gene for NIDDM susceptibility#JOURNAL:Genomics#YEAR:2001#PUBMED:11247674# AUTHORS:Doege H, Bocianski A, Scheepers A, Axer H, Eckel J, Joost HG, Schurmann A#TITLE:Characterization of human glucose transporter (GLUT) 11 (encoded by SLC2A11), a novel sugar-transport facilitator specifically expressed in heart and skeletal muscle#JOURNAL:Biochem J#YEAR:2001#PUBMED:11583593# AUTHORS:Rogers S, Macheda ML, Docherty SE, Carty MD, Henderson MA, Soeller WC, Gibbs EM, James DE, Best JD#TITLE:Identification of a novel glucose transporter-like protein-GLUT-12#JOURNAL:Am J Physiol Endocrinol Metab#YEAR:2002#PUBMED:11832379# AUTHORS:Macheda ML, Williams ED, Best JD, Wlodek ME, Rogers S#TITLE:Expression and localisation of GLUT1 and GLUT12 glucose transporters in the pregnant and lactating rat mammary gland#JOURNAL:Cell Tissue Res#YEAR:2003#PUBMED:12483288# AUTHORS:Rogers S, Chandler JD, Clarke AL, Petrou S, Best JD#TITLE:Glucose transporter GLUT12-functional characterization in Xenopus laevis oocytes#JOURNAL:Biochem Biophys Res Commun#YEAR:2003#PUBMED:12914765# AUTHORS:Wu X, Freeze HH#TITLE:GLUT14, a duplicon of GLUT3, is specifically expressed in testis as alternative splice forms#JOURNAL:Genomics#YEAR:2002#PUBMED:12504846# GENE ASSOCIATION: (66035.1) or (6517.1) or (56606.1) or (154091.1) or (6513.1) or (11182.1) or (29988.1) or (6514.1) or (155184.1) or (56606.2) or (144195.1) or (6515.1) or (81031.1)
Abbreviation: R_PGISr Synonyms: _2 EC Number: 5.3.99.4 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : prostgh2 <==> prostgi2 Confidence Level: 4 NJ genes: LOCUS:_5740#TRANSCRIPT:1#ABBREVIATION:PTGIS#ECNUMBERS:5.3.99.4# proteins: NAME:prostaglandin I2 (prostacyclin) synthase#ABBREVIATION:Ptgis.1# citations: TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# GENE ASSOCIATION: (5740.1)
Abbreviation: R_M4ATAer Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : m(em)3gacpail_hs + pre_prot --> gpi_sig + m(em)3gacpail_prot_hs Confidence Level: 4 EC 2.6.-.- NCD genes: LOCUS:_10026#TRANSCRIPT:1#ABBREVIATION:PIGK# LOCUS:_8733#TRANSCRIPT:1#ABBREVIATION:GPAA1# LOCUS:_94005#TRANSCRIPT:1#ABBREVIATION:PIGS# LOCUS:_128869#TRANSCRIPT:1#ABBREVIATION:CDC91L1# proteins: NAME:GPAA1P anchor attachment protein 1 homolog (yeast), endoplasmic reticulum#ABBREVIATION:Gpaa1.1-r# NAME:phosphatidylinositol glycan, class K, endoplasmic reticulum#ABBREVIATION:Pigk.1-r# NAME:phosphatidylinositol glycan, class S, endoplasmic reticulum#ABBREVIATION:Pigs.1-r# NAME:CDC91 cell division cycle 91-like 1 (S. cerevisiae), endoplasmic reticulum#ABBREVIATION:Cdc91l1.1-r# citations: AUTHORS:Hiroi Y, Komuro I, Chen R, Hosoda T, Mizuno T, Kudoh S, Georgescu SP, Medof ME, Yazaki Y#TITLE:Molecular cloning of human homolog of yeast GAA1 which is required for attachment of glycosylphosphatidylinositols to proteins#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9468317# AUTHORS:Yu J, Nagarajan S, Knez JJ, Udenfriend S, Chen R, Medof ME#TITLE:The affected gene underlying the class K glycosylphosphatidylinositol (GPI) surface protein defect codes for the GPI transamidase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9356492# AUTHORS:Meyer U, Benghezal M, Imhof I, Conzelmann A#TITLE:Active site determination of Gpi8p, a caspase-related enzyme required for glycosylphosphatidylinositol anchor addition to proteins#JOURNAL:Biochemistry#YEAR:2000#PUBMED:10727241# AUTHORS:Ohishi K, Inoue N, Kinoshita T#TITLE:PIG-S and PIG-T, essential for GPI anchor attachment to proteins, form a complex with GAA1 and GPI8#JOURNAL:EMBO J#YEAR:2001#PUBMED:11483512# AUTHORS:Hong Y, Ohishi K, Kang JY, Tanaka S, Inoue N, Nishimura J, Maeda Y, Kinoshita T#TITLE:Human PIG-U and yeast Cdc91p are the fifth subunit of GPI transamidase that attaches GPI-anchors to proteins#JOURNAL:Mol Biol Cell#YEAR:2003#PUBMED:12802054# AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (10026.1) and (8733.1) and (128869.1) and (94005.1)
Abbreviation: R_PPOR Synonyms: _0 EC Number: 1.13.11.27 SUBSYSTEM: Phenylalanine metabolism Equation: [c] : o2 + phpyr --> 2hyoxplac + co2 Confidence Level: 1 SAB genes: LOCUS:_3242#TRANSCRIPT:1#ABBREVIATION:HPD#ECNUMBERS:1.13.11.27# proteins: NAME:_4-hydroxyphenylpyruvate dioxygenase#ABBREVIATION:Hpd.1# citations: none GENE ASSOCIATION: (3242.1)
Phenylpyruvate:oxygen oxidoreductase (hydroxylating,decarboxylating)
Abbreviation: R_CHSTEROLt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + chsterol[c] + h2o[c] --> adp[c] + chsterol[e] + h[c] + pi[c] Confidence Level: 4 IT genes: LOCUS:_19#TRANSCRIPT:1#ABBREVIATION:ABCA1# proteins: NAME:ATP-binding cassette, sub-family A (ABC1), member 1 #ABBREVIATION:Abca1.1-e# citations: AUTHORS:Wollmer MA, Streffer JR, Lutjohann D, Tsolaki M, Iakovidou V, Hegi T, Pasch T, Jung HH, Bergmann K, Nitsch RM, Hock C, Papassotiropoulos A. #TITLE:ABCA1 modulates CSF cholesterol levels and influences the age at onset of Alzheimer's disease.#JOURNAL:Neurobiol Aging. #YEAR:2003#PUBMED:12600718# AUTHORS:Orso E, Broccardo C, Kaminski WE, Bottcher A, Liebisch G, Drobnik W, Gotz A, Chambenoit O, Diederich W, Langmann T, Spruss T, Luciani MF, Rothe G, Lackner KJ, Chimini G, Schmitz G. #TITLE:Transport of lipids from golgi to plasma membrane is defective in tangier disease patients and Abc1-deficient mice.#JOURNAL:Nat Genet. #YEAR:2000#PUBMED:10655069# GENE ASSOCIATION: (19.1)
Abbreviation: R_ALCD22_D Synonyms: _0 EC Number: 1.1.1.78 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : lald-D + nad --> h + mthgxl + nadh Confidence Level: 2 NCD genes: LOCUS:_130#TRANSCRIPT:1#ABBREVIATION:ADH6#ECNUMBERS:1.1.1.1# LOCUS:_126#TRANSCRIPT:1#ABBREVIATION:ADH1C#ECNUMBERS:1.1.1.1# LOCUS:_125#TRANSCRIPT:1#ABBREVIATION:ADH1B#ECNUMBERS:1.1.1.1# LOCUS:_128#TRANSCRIPT:1#ABBREVIATION:ADH5#ECNUMBERS:1.1.1.1# LOCUS:_127#TRANSCRIPT:1#ABBREVIATION:ADH4#ECNUMBERS:1.1.1.1# LOCUS:_124#TRANSCRIPT:1#ABBREVIATION:ADH1A#ECNUMBERS:1.1.1.1# LOCUS:_131#TRANSCRIPT:1#ABBREVIATION:ADH7#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:1#ABBREVIATION:ZADH2# LOCUS:_137872#TRANSCRIPT:1#ABBREVIATION:ADHFE1#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:2#ABBREVIATION:ZADH2# proteins: NAME:alcohol dehydrogenase IV #ABBREVIATION:Adh4# NAME:alcohol dehydrogenase V#ABBREVIATION:Adh5# NAME:alcohol dehydrogenase 1A (class I), alpha polypeptide#ABBREVIATION:Adh1a.1# NAME:alcohol dehydrogenase IB (class I), beta polypeptide#ABBREVIATION:Adh1b.1# NAME:alcohol dehydrogenase 1C (class I), gamma polypeptide#ABBREVIATION:Adh1c.1# NAME:alcohol dehydrogenase 6 (class V)#ABBREVIATION:Adh6.1# NAME:alcohol dehydrogenase 7 (class IV), mu or sigma polypeptide#ABBREVIATION:Adh7.1# NAME:alcohol dehydrogenase, iron containing, 1 (liver)#ABBREVIATION:Adhfe1.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ab.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ac# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1bc# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.1# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.2# citations: AUTHORS:Ewaschuk JB, Naylor JM, Zello GA#TITLE:D-lactate in human and ruminant metabolism#JOURNAL:J Nutr#YEAR:2005#PUBMED:15987839# AUTHORS:Christopher MM, Eckfeldt JH, Eaton JW.#TITLE:Propylene glycol ingestion causes D-lactic acidosis#JOURNAL:Lab Invest.#YEAR:1990#PUBMED:2296157# GENE ASSOCIATION: (126.1) or (137872.1) or (126.1 and 124.1) or (127.1) or (130.1) or (284273.1) or (125.1 and 124.1) or (128.1) or (124.1) or (125.1) or (284273.2) or (131.1) or (125.1 and 126.1)
Abbreviation: R_3NTD7l Synonyms: _0 SUBSYSTEM: Nucleic acid degradation Equation: [l] : 3amp + h2o --> adn + pi Confidence Level: 4 MKA AMF IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:R.L. Pisoni#TITLE:Lysosomal Nucleic Acid and Phosphate Metabolism and Related Metabolic Reactions, Chapter 9#YEAR:1996# GENE ASSOCIATION: (53.1)
Abbreviation: R_BHBtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: bhb[c] + h[c] <==> bhb[m] + h[m] Confidence Level: 4 IT, TDV genes: none proteins: none citations: AUTHORS:Latruffe N.#TITLE:Transport of D-beta-hydroxybutyrate across rat liver mitochondrial membranes. #JOURNAL:Comp Biochem Physiol B#YEAR:1987#PUBMED:3427918# GENE ASSOCIATION:
(R)-3-Hydroxybutanoate mitochondrial transport via H+ symport
fucosyl galactosylgloboside (homo sapiens)_C56H99N2O31FULLRCO
Abbreviation: R_EX_hdca_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hdca <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
chenodeoxycholoyl coenzyme a_C45H70N7O19P3S
Abbreviation: R_BAAT4x Synonyms: _1 EC Number: 2.3.1.65 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : dcholcoa + gly --> coa + gdchola Confidence Level: 2 fourth version analog added in similarity to BAAT1x, BAAT2x NJ genes: LOCUS:_570#TRANSCRIPT:1#ABBREVIATION:BAAT#ECNUMBERS:2.3.1.65# proteins: NAME:bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase)#ABBREVIATION:Baat.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Falany CN, Johnson MR, Barnes S, Diasio RB#TITLE:Glycine and taurine conugation of bile acids by a single enzyme#JOURNAL:Journal of Biological Chemistry#YEAR:1994# GENE ASSOCIATION: (570.1)
Abbreviation: R_EX_cholate_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cholate <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAOXC226205x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : c226coa + coa + h2o + nad --> accoa + h + nadh + tmndnccoa Confidence Level: 4 _1 round of beta-oxidation, loses degree of desat, so no fad - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) peroxisomal (very long chain FA) c22:6,n-3 -> c20:5, n-3 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1) or (51.1 and 30.1 and 1962.1)
chondroitin sulfate D (GlcNAc6S-GlcA2S) precursor 2_C31H46NO34S3X
Abbreviation: R_MM6ag Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m6masnA --> m5masnC + man Confidence Level: 0 NCD genes: LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# GENE ASSOCIATION: (57134.1)
Abbreviation: R_DHPM1 Synonyms: _0 EC Number: 3.5.2.2 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : 56dura + h2o --> cala + h Confidence Level: 4 genes: LOCUS:_1807#TRANSCRIPT:1#ABBREVIATION:DPYS# LOCUS:_1808#TRANSCRIPT:1#ABBREVIATION:DPYSL2# LOCUS:_1809#TRANSCRIPT:1#ABBREVIATION:DPYSL3# proteins: NAME:dihydropyrimidinase#ABBREVIATION:Dpys.1# NAME:dihydropyrimidinase-like 2#ABBREVIATION:Dpysl2.1# NAME:dihydropyrimidinase-like 3#ABBREVIATION:Dpysl3.1# citations: AUTHORS:Hamajima N, Matsuda K, Sakata S, Tamaki N, Sasaki M, Nonaka M. #TITLE:A novel gene family defined by human dihydropyrimidinase and three related proteins with differential tissue distribution. #JOURNAL:Gene#YEAR:1996#PUBMED:8973361# GENE ASSOCIATION: (1807.1) or (1809.1) or (1808.1)
Abbreviation: R_ACGAM2E Synonyms: _0 EC Number: 5.1.3.8 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acgam <==> acmana Confidence Level: 4 NCD genes: LOCUS:_5973#TRANSCRIPT:1#ABBREVIATION:RENBP# proteins: NAME:renin binding protein#ABBREVIATION:Renbp.1# citations: AUTHORS:Luchansky SJ, Yarema KJ, Takahashi S, Bertozzi CR#TITLE:GlcNAc 2-epimerase can serve a catabolic role in sialic acid metabolism#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12499362# GENE ASSOCIATION: (5973.1)
Abbreviation: R_S2T1g Synonyms: _0 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_c,d,e_pre1 + paps --> cs_d_pre2 + h + pap Confidence Level: 4 EC 2.8.2- NCD genes: LOCUS:_10090#TRANSCRIPT:1#ABBREVIATION:UST# proteins: NAME:uronyl-2-sulfotransferase, Golgi apparatus#ABBREVIATION:Ust.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Kobayashi M, Sugumaran G, Liu J, Shworak NW, Silbert JE, Rosenberg RD#TITLE:Molecular cloning and characterization of a human uronyl 2-sulfotransferase that sulfates iduronyl and glucuronyl residues in dermatan/chondroitin sulfate#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10187838# GENE ASSOCIATION: (10090.1)
Abbreviation: R_GALt2_2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gal[e] + (2) h[e] <==> gal[c] + (2) h[c] Confidence Level: 4 genes: LOCUS:_6523#TRANSCRIPT:1#ABBREVIATION:SLC5A1# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 1, extracellular#ABBREVIATION:Slc5a1.1-e# citations: AUTHORS:Hirayama BA, Loo DD, Wright EM.#TITLE:Protons drive sugar transport through the Na+/glucose cotransporter (SGLT1)#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8063771# AUTHORS:Hediger MA, Turk E, Wright EM#TITLE:Homology of the human intestinal Na+/glucose and Escherichia coli Na+/proline cotransporters#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1989#PUBMED:2490366# AUTHORS:Quick M, Loo DD, Wright EM#TITLE:Neutralization of a conserved amino acid residue in the human Na+/glucose transporter (hSGLT1) generates a glucose-gated H+ channel#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11024018# AUTHORS:Wright EM, Hirayama BA, Loo DDF, Turk E, Hager K#TITLE:Intestinal sugar transport#YEAR:1994# AUTHORS:Leung DW, Loo DD, Hirayama BA, Zeuthen T, Wright EM#TITLE:Urea transport by cotransporters#JOURNAL:J Physiol#YEAR:2000#PUBMED:11034615# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# GENE ASSOCIATION: (6523.1)
D-galactose transport via proton symport
Abbreviation: R_H7ET2er Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : em2emgacpail_hs + pe_hs --> dag_hs + gpi_hs Confidence Level: 4 NCD genes: LOCUS:_5281#TRANSCRIPT:1#ABBREVIATION:PIGF# LOCUS:_5281#TRANSCRIPT:2#ABBREVIATION:PIGF# LOCUS:_54872#TRANSCRIPT:1#ABBREVIATION:GPI7# proteins: NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.1-r# NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.2-r# NAME:GPI protein, endoplasmic reticulum#ABBREVIATION:Gpi7.1-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shishioh N, Hong Y, Ohishi K, Ashida H, Maeda Y, Kinoshita T#TITLE:GPI7 is the second partner of PIG-F and involved in modification of glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15632136# GENE ASSOCIATION: (5281.1) and (54872.1) or (5281.2) and (54872.1)
H7 phosphoethanolaminyl transferase, endoplasmic reticulum
Abbreviation: R_PNTOt5 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + (2) na1[e] + pnto-R[e] --> adp[c] + h[c] + (2) na1[c] + pi[c] + pnto-R[c] Confidence Level: 4 _01-25-05 based on Wang et al., JBC, 1999,1 274(21), 14875-14888. IT 03-07-05:replaced IT: now based on Balamurugan et al, 2005, Am J Physiol Renal Physiol, 288, F823-831,2005 genes: LOCUS:_8884#TRANSCRIPT:1#ABBREVIATION:SMVT#ECNUMBERS:TC,2.A.21.1.1# proteins: NAME:sodium-dependent vitamin transporter (biotin, pantothenate,lipoate) #ABBREVIATION:SmvT.1# citations: AUTHORS:Prasad PD, Wang H, Kekuda R, Fujita T, Fei YJ, Devoe LD, Leibach FH, Ganapathy V.#TITLE:Cloning and functional expression of a cDNA encoding a mammalian sodium-dependent vitamin transporter mediating the uptake of pantothenate, biotin, and lipoate.#JOURNAL: J Biol Chem.#YEAR:1998#PUBMED:9516450# AUTHORS:Balamurugan K, Ortiz A, Said HM.#TITLE:Biotin uptake by human intestinal and liver epithelial cells: role of the SMVT system.#JOURNAL:Am J Physiol Gastrointest Liver Physiol. #YEAR:2003#PUBMED:12646417# AUTHORS:Prasad PD, Wang H, Huang W, Fei YJ, Leibach FH, Devoe LD, Ganapathy V.#TITLE:Molecular and functional characterization of the intestinal Na+-dependent multivitamin transporter. #JOURNAL:Arch Biochem Biophys.#YEAR:1999#PUBMED:10334869# AUTHORS:Wang H, Huang W, Fei YJ, Xia H, Yang-Feng TL, Leibach FH, Devoe LD, Ganapathy V, Prasad PD.#TITLE:Human placental Na+-dependent multivitamin transporter. Cloning, functional expression, gene structure, and chromosomal localization.#JOURNAL: J Biol Chem. #YEAR:1999#PUBMED:10329687# AUTHORS:Balamurugan K, Vaziri ND, Said HM.#TITLE:Biotin uptake by human proximal tubular epithelial cells: Cellular and molecular aspects. #JOURNAL:Am J Physiol Renal Physiol. #YEAR:2005#PUBMED:15561972# GENE ASSOCIATION: (8884.1)
Abbreviation: R_P4502D6 Synonyms: _3 SUBSYSTEM: CYP Metabolism Equation: [c] : debrisoquine + h + nadph + o2 --> 4hdebrisoquine + h2o + nadp Confidence Level: 2 debrisoquine 4-hydroxylation assumed to take place on outer membrane NJ genes: LOCUS:_1565#TRANSCRIPT:1#ABBREVIATION:CYP2D6# proteins: NAME:cytochrome P450, family 2, subfamily D, polypeptide 6#ABBREVIATION:Cyp2d6.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# GENE ASSOCIATION: (1565.1)
Abbreviation: R_DNDPt57m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + dgtp[c] --> atp[c] + dgtp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_but_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : but <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ARTFR208 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (3) fadh2[m] + (2) h[m] + (2) nadph[m] + tmndnccoa[c] --> (1.25) R2coa_hs[c] + (3) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DGCHOLtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: dgchol[x] <==> dgchol[c] Confidence Level: 2 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DOLPH_Uer Synonyms: _0 EC Number: 3.1.3.51 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolp_U + h2o --> (0.1) dolichol_U + pi Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Schenk B, Fernandez F, Waechter CJ.#TITLE:The ins(ide) and out(side) of dolichyl phosphate biosynthesis and recycling in the endoplasmic reticulum.#JOURNAL:Glycobiology#YEAR:2001#PUBMED:11425794# GENE ASSOCIATION:
Dolichol-phosphate phosphohydrolase, human (uterus)
L-serine/L-threonine Na-dependent exchange (Ser-L in)
Abbreviation: R_SERTHRNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + ser-L[e] + thr-L[c] --> na1[c] + ser-L[c] + thr-L[e] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
Abbreviation: R_TYRTA Synonyms: _13 EC Number: 2.6.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : akg + tyr-L <==> 34hpp + glu-L Confidence Level: 4 genes: LOCUS:_2805#TRANSCRIPT:1#ABBREVIATION:GOT1#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase #ABBREVIATION:Got1.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (2805.1)
Abbreviation: R_FACOAL244_1 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + tettet6 <==> amp + ppi + tettet6coa Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
tetracosatetraenoyl coenzyme A_C45H70N7O17P3S
keratan sulfate I biosynthesis, precursor 14_C143H230N10O114S4X
Abbreviation: R_GLACO Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : glac + (2) h2o + nad --> glcr + (3) h + nadh Confidence Level: 2 NCD genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Marsh CA#TITLE:Biosynthesis of D-glucaric acid in mammals: a free-radical mechanism?#JOURNAL:Carbohydr Res#YEAR:1986#PUBMED:3779687# GENE ASSOCIATION: (223.1) or (220.1) or (224.1) or (8854.2) or (218.1) or (8854.1) or (216.1) or (221.1) or (8854.3) or (222.1)
Abbreviation: R_EX_5adtststerones_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 5adtststerones <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_5alpha-Dihydrotestosterone sulfate exchange
Abbreviation: R_FAS140COA Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : ddcacoa + (3) h + malcoa + (2) nadph --> co2 + coa + h2o + (2) nadp + tdcoa Confidence Level: 4 NCD genes: LOCUS:_2194#TRANSCRIPT:1#ABBREVIATION:FASN#ECNUMBERS:2.3.1.85# proteins: NAME:fatty acid synthase#ABBREVIATION:Fasn.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Chakravarty B, Gu Z, Chirala SS, Wakil SJ, Quiocho FA.#TITLE:Human fatty acid synthase: structure and substrate selectivity of the thioesterase domain.#YEAR:2004#PUBMED:15507492# AUTHORS:Brink J, Ludtke SJ, Yang CY, Gu ZW, Wakil SJ, Chiu W.#TITLE:Quaternary structure of human fatty acid synthase by electron cryomicroscopy.#JOURNAL:Proc Natl Acad Sci#YEAR:2002#PUBMED:11756679# AUTHORS:Wakil SJ.#TITLE:Fatty acid synthase, a proficient multifunctional enzyme.#JOURNAL:Biochemistry#YEAR:1989#PUBMED:2669958# GENE ASSOCIATION: (2194.1)
Dodecanoyl-CoA (n-C12:0CoA)_C33H54N7O17P3S
Abbreviation: R_AG13T13g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre27 + uacgam --> h + ksi_pre28 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
keratan sulfate I biosynthesis, precursor 28_C213H341N15O176S8X
Abbreviation: R_DCSPTN1COAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: dcsptn1coa[c] <==> dcsptn1coa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: AUTHORS:Sprecher H#TITLE:An update on the pathways of polyunsaturated fatty acid metabolism#JOURNAL:Curr Opn in Clinical Nutrition and Metabolic Care#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_MLTG1e Synonyms: _0 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [e] : h2o + malttr --> glc-D + malt Confidence Level: 4 JLR genes: LOCUS:_8972#TRANSCRIPT:1#ABBREVIATION:MGAM# proteins: NAME:maltase-glucoamylase (alpha-glucosidase), extracellular (intestine)#ABBREVIATION:Mgam.1-e# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Naim HY, Sterchi EE, Lentze MJ#TITLE:Structure, biosynthesis, and glycosylation of human small intestinal maltase-glucoamylase#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3143729# GENE ASSOCIATION: (8972.1)
Maltodextrin glucosidase (maltotriose), extracellular
Abbreviation: R_TETPENT6CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + tetpent6crn --> crn + tetpent6coa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
keratan sulfate I, degradation product 31_C90H148N6O72S2
Abbreviation: R_S6TASE23ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core2_deg3 <==> h + ksii_core2_deg4 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_PI345P5P Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail345p_hs --> pail34p_hs + pi Confidence Level: 4 NCD genes: LOCUS:_8867#TRANSCRIPT:1#ABBREVIATION:SYNJ1#ECNUMBERS:3.1.3.56# LOCUS:_3632#TRANSCRIPT:1#ABBREVIATION:INPP5A#ECNUMBERS:3.1.3.56# LOCUS:_3633#TRANSCRIPT:1#ABBREVIATION:INPP5B#ECNUMBERS:3.1.3.56# LOCUS:_3636#TRANSCRIPT:1#ABBREVIATION:INPPL1# LOCUS:_27124#TRANSCRIPT:1#ABBREVIATION:PIB5PA# LOCUS:_3635#TRANSCRIPT:1#ABBREVIATION:INPP5D# LOCUS:_56623#TRANSCRIPT:1#ABBREVIATION:INPP5E# LOCUS:_8871#TRANSCRIPT:1#ABBREVIATION:SYNJ2# LOCUS:_27124#TRANSCRIPT:2#ABBREVIATION:PIB5PA# LOCUS:_8867#TRANSCRIPT:2#ABBREVIATION:SYNJ1# proteins: NAME:synaptojanin 2#ABBREVIATION:Synj2.1# NAME:synaptojanin 1#ABBREVIATION:Synj1.1# NAME:inositol polyphosphate-5-phosphatase, 75kDa#ABBREVIATION:Inpp5b.1# NAME:inositol polyphosphate-5-phosphatase, 72 kDa#ABBREVIATION:Inpp5e.1# NAME:synaptojanin 1#ABBREVIATION:Synj1.2# NAME:inositol polyphosphate-5-phosphatase, 40kDa#ABBREVIATION:Inpp5a.1# NAME:inositol polyphosphate-5-phosphatase, 145kDa#ABBREVIATION:Inpp5d.1# NAME:inositol polyphosphate phosphatase-like 1#ABBREVIATION:Inppl1.1# NAME:phosphatidylinositol (4,5) bisphosphate 5-phosphatase, A#ABBREVIATION:Pib5pa.1# NAME:phosphatidylinositol (4,5) bisphosphate 5-phosphatase, A#ABBREVIATION:Pib5pa.2# citations: AUTHORS:Tolias KF, Cantley LC#TITLE:Pathways for phosphoinositide synthesis#JOURNAL:Chem Phys Lipids#YEAR:1999#PUBMED:10358929# GENE ASSOCIATION: (3633.1) or (27124.1) or (3635.1) or (3636.1) or (8871.1) or (56623.1) or (8867.2) or (27124.2) or (8867.1) or (3632.1)
phosphatidylinositol-3,4,5-trisphosphate 5-phosphatase
Abbreviation: R_NTD8l Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleic acid degradation Equation: [l] : dgmp + h2o --> dgsn + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:R.L. Pisoni#TITLE:Lysosomal Nucleic Acid and Phosphate Metabolism and Related Metabolic Reactions, Chapter 9#YEAR:1996# GENE ASSOCIATION: (53.1)
Abbreviation: R_GLUDC Synonyms: _0 EC Number: 4.1.1.15 SUBSYSTEM: Glutamate metabolism Equation: [c] : glu-L + h --> 4abut + co2 Confidence Level: 4 JLR- added h to balance genes: LOCUS:_2572#TRANSCRIPT:1#ABBREVIATION:GAD2#ECNUMBERS:4.1.1.15# LOCUS:_2571#TRANSCRIPT:1#ABBREVIATION:GAD1#ECNUMBERS:4.1.1.15# LOCUS:_2571#TRANSCRIPT:2#ABBREVIATION:GAD1#ECNUMBERS:4.1.1.15# proteins: NAME:glutamate decarboxylase 1 (brain, 67kDa)#ABBREVIATION:Gad1.1# NAME:glutamate decarboxylase 1 (brain, 67kDa)#ABBREVIATION:Gad1.2# NAME:glutamate decarboxylase 2 (pancreatic islets and brain, 65kDa)#ABBREVIATION:Gad2.1# citations: AUTHORS:Bu DF, Tobin AJ. #TITLE:The exon-intron organization of the genes (GAD1 and GAD2) encoding two human glutamate decarboxylases (GAD67 and GAD65) suggests that they derive from a common ancestral GAD.#YEAR:1994#PUBMED:8088791# AUTHORS:Karlsen AE, Hagopian WA, Grubin CE, Dube S, Disteche CM, Adler DA, Barmeier H, Mathewes S, Grant FJ, Foster D, et al.#TITLE:Cloning and primary structure of a human islet isoform of glutamic acid decarboxylase from chromosome 10.#YEAR:1991#PUBMED:1924293# GENE ASSOCIATION: (2571.1) or (2572.1) or (2571.2)
Abbreviation: R_34DHPLACOX Synonyms: _0 EC Number: 1.2.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 34dhpac + h2o + nad --> 34dhpha + (2) h + nadh Confidence Level: 4 SAB genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Ambroziak W, Pietruszko R#TITLE:Human aldehyde dehydrogenase. Activity with aldehyde metabolites of monoamines, diamines, and polyamines#JOURNAL:J Biol Chem#YEAR:1991#PUBMED:2071588# AUTHORS:Keung WM#TITLE:Biogenic aldehyde(s) derived from the action of monoamine oxidase may mediate the antidipsotropic effect of daidzin#JOURNAL:Chem Biol Interact#YEAR:2001#PUBMED:11306106# GENE ASSOCIATION: (218.1) or (222.1) or (220.1) or (221.1)
_3,4-Dihydroxyphenylacetaldehyde:NAD+ oxidoreductase
heparan sulfate, degradation product 23_C31H49NO29S
Abbreviation: R_CDPDAGtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: cdpdag_hs[c] <==> cdpdag_hs[m] Confidence Level: 1 unknown mechanism, presumed diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MI14PP Synonyms: _1 EC Number: 3.1.3.57 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi14p --> mi4p-D + pi Confidence Level: 4 NCD genes: LOCUS:_3628#TRANSCRIPT:1#ABBREVIATION:INPP1#ECNUMBERS:3.1.3.61# proteins: NAME:inositol polyphosphate-1-phosphatase#ABBREVIATION:Inpp1.1# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:York JD, Veile RA, Donis-Keller H, Majerus PW#TITLE:Cloning, heterologous expression, and chromosomal localization of human inositol polyphosphate 1-phosphatase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1993#PUBMED:8390685# GENE ASSOCIATION: (3628.1)
Abbreviation: R_ENMAN5g Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : g1m7masnB + h2o --> glc1man + m6masnB2 Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Endomannosidase (m6masnB2-producing), Golgi apparatus
Abbreviation: R_IDHPOXOXb Synonyms: _0 EC Number: 1.11.1.8 SUBSYSTEM: Tyrosine metabolism Equation: [c] : iodine + tyr-L --> 3ityr-L + h + i Confidence Level: 4 SAB different because now HI = H + I due to pKa of -10 genes: LOCUS:_7173#TRANSCRIPT:1#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:2#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:5#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:4#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:3#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# proteins: NAME:thyroid peroxidase#ABBREVIATION:Tpo.1# NAME:thyroid peroxidase#ABBREVIATION:Tpo.2# NAME:thyroid peroxidase#ABBREVIATION:Tpo.3# NAME:thyroid peroxidase#ABBREVIATION:Tpo.4# NAME:thyroid peroxidase#ABBREVIATION:Tpo.5# citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# AUTHORS:Nakamura M, Yamazaki I, Kotani T, Ohtaki S#TITLE:Thyroid peroxidase selects the mechanism of either 1- or 2-electron oxidation of phenols, depending on their substituents#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2997169# AUTHORS:Kimura S, Kotani T, McBride OW, Umeki K, Hirai K, Nakayama T, Ohtaki S#TITLE:Human thyroid peroxidase: complete cDNA and protein sequence, chromosome mapping, and identification of two alternately spliced mRNAs#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1987#PUBMED:3475693# AUTHORS:Magnusson RP, Chazenbalk GD, Gestautas J, Seto P, Filetti S, DeGroot LJ, Rapoport B. #TITLE:Molecular cloning of the complementary deoxyribonucleic acid for human thyroid peroxidase.#JOURNAL:Mol Endocrinol#YEAR:1987#PUBMED:3153466# GENE ASSOCIATION: (7173.4) or (7173.1) or (7173.3) or (7173.2) or (7173.5)
Abbreviation: R_SR5ARr Synonyms: _4 EC Number: 1.3.99.5 SUBSYSTEM: Steroid Metabolism Equation: [r] : h + nadph + tststerone --> 5adtststerone + nadp Confidence Level: 4 there are actually two types, but the difference between them lies in optimal pH function - grouped as one reaction at his point to eliminate redundancy. NADP known to be cofactor NJ genes: LOCUS:_6716#TRANSCRIPT:1#ABBREVIATION:SRD5A2#ECNUMBERS:1.3.99.5# LOCUS:_6715#TRANSCRIPT:1#ABBREVIATION:SRD5A1#ECNUMBERS:1.3.99.5# proteins: NAME:steroid-5-alpha-reductase, alpha polypeptide 1#ABBREVIATION:Srd5a1.1# NAME:steroid-5-alpha-reductase, alpha polypeptide 2#ABBREVIATION:Srd5a2.1# citations: AUTHORS:Andersson S, Berman DM, Jenkins EP, Russel DW#TITLE:Deletion of steroid 5-alpha-reductase 2 gene in male pseudohermaphroditism#JOURNAL:Nature#YEAR:1991# AUTHORS:Wigley WC, Prihoda JS, Mowaszowicz I, Mendonca BB, New MI, Wilson JD, Russell DW#TITLE:Natural mutagenesis study of the human steroid 5-alpha-reductase 2 isozyme#JOURNAL:Biochemistry#YEAR:1994# GENE ASSOCIATION: (6716.1) or (6715.1)
Abbreviation: R_ALR3 Synonyms: _0 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : acetol + h + nadph --> 12ppd-S + nadp Confidence Level: 4 NCD genes: LOCUS:_10327#TRANSCRIPT:1#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# LOCUS:_231#TRANSCRIPT:1#ABBREVIATION:AKR1B1#ECNUMBERS:1.1.1.21# LOCUS:_8574#TRANSCRIPT:1#ABBREVIATION:AKR7A2# LOCUS:_10327#TRANSCRIPT:2#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# proteins: NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.1# NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.2# NAME:aldo-keto reductase family 1, member B1 (aldose reductase)#ABBREVIATION:Akr1b1.1# NAME:aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)#ABBREVIATION:Akr7a2.1# citations: AUTHORS:Vander Jagt DL, Robinson B, Taylor KK, Hunsaker LA.#TITLE:Reduction of trioses by NADPH-dependent aldo-keto reductases. Aldose reductase, methylglyoxal, and diabetic complications#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1537826# AUTHORS:Kelly VP, Sherratt PJ, Crouch DH, Hayes JD#TITLE:Novel homodimeric and heterodimeric rat gamma-hydroxybutyrate synthases that associate with the Golgi apparatus define a distinct subclass of aldo-keto reductase 7 family proteins.#JOURNAL:Biochem J#YEAR:2002#PUBMED:12071861# AUTHORS:Praml C, Savelyeva L, Perri P, Schwab M.#TITLE:Cloning of the human aflatoxin B1-aldehyde reductase gene at 1p35-1p36.1 in a region frequently altered in human tumor cells.#JOURNAL:Cancer Res#YEAR:1998#PUBMED:9823300# GENE ASSOCIATION: (10327.2) or (8574.1) or (10327.1) or (231.1)
glycogen, structure 2 (glycogenin-1,6-{7[1,4-Glc], 4[1,4-Glc]})_C66H111O56X
Abbreviation: R_AMY2e Synonyms: _0 EC Number: 3.2.1.1 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [e] : glygn2 + (8) h2o --> (8) glc-D + glygn4 Confidence Level: 0 NCD genes: LOCUS:_276#TRANSCRIPT:1#ABBREVIATION:AMY1A#ECNUMBERS:3.2.1.1# LOCUS:_280#TRANSCRIPT:1#ABBREVIATION:AMY2B#ECNUMBERS:3.2.1.1# LOCUS:_279#TRANSCRIPT:1#ABBREVIATION:AMY2A#ECNUMBERS:3.2.1.1# LOCUS:_278#TRANSCRIPT:1#ABBREVIATION:AMY1C#ECNUMBERS:3.2.1.1# LOCUS:_277#TRANSCRIPT:1#ABBREVIATION:AMY1B#ECNUMBERS:3.2.1.1# LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_6519#TRANSCRIPT:1#ABBREVIATION:SLC3A1# LOCUS:_11136#TRANSCRIPT:1#ABBREVIATION:SLC7A9# LOCUS:_276#TRANSCRIPT:2#ABBREVIATION:AMY1A#ECNUMBERS:3.2.1.1# proteins: NAME:amylase, alpha 1A; salivary, extracellular (saliva)#ABBREVIATION:Amy1a.1-e# NAME:amylase, alpha 1A; salivary, extracellular (saliva)#ABBREVIATION:Amy1a.2-e# NAME:amylase, alpha 1c; salivary, extracellular (saliva)#ABBREVIATION:Amy1b.1-e# NAME:amylase, alpha 1c; salivary, extracellular (saliva)#ABBREVIATION:Amy1c.1-e# NAME:amylase, alpha 2A; pancreatic, extracellular (sm intestine)#ABBREVIATION:Amy2a.1-e# NAME:amylase, alpha 2B; pancreatic, extracellular (sm intestine)#ABBREVIATION:Amy2b.1-e# NAME:solute carrier family (amino acid transporter), extracellular#ABBREVIATION:Slc3a1/7a9-e# NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# citations: none GENE ASSOCIATION: (6520.1) or (280.1) or (278.1) or (276.1) or (277.1) or (6519.1 and 11136.1) or (276.2) or (279.1)
alpha-amylase, extracellular (glygn2 -> glygn4)
Abbreviation: R_EX_thmtp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : thmtp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GARFT Synonyms: _1 EC Number: 2.1.2.2 SUBSYSTEM: IMP Biosynthesis Equation: [c] : 10fthf + gar <==> fgam + h + thf Confidence Level: 4 genes: LOCUS:_2618#TRANSCRIPT:1#ABBREVIATION:GART#ECNUMBERS:2.1.2.2,,6.3.1.# LOCUS:_2618#TRANSCRIPT:2#ABBREVIATION:GART#ECNUMBERS:2.1.2.2,,6.3.1.# proteins: NAME:phosphoribosylglycinamide formyltransferase, phosphoribosylglycinamide synthetase, phosphoribosylaminoimidazole synthetase #ABBREVIATION:GarT.1# NAME:phosphoribosylglycinamide formyltransferase, phosphoribosylglycinamide synthetase, phosphoribosylaminoimidazole synthetase #ABBREVIATION:GarT.2# citations: AUTHORS:Brodsky G, Barnes T, Bleskan J, Becker L, Cox M, Patterson D.#TITLE:The human GARS-AIRS-GART gene encodes two proteins which are differentially expressed during human brain development and temporally overexpressed in cerebellum of individuals with Down syndrome. #JOURNAL:Hum Mol Genet. #YEAR:1997#PUBMED:9328467# AUTHORS:Zhang Y, Desharnais J, Greasley SE, Beardsley GP, Boger DL, Wilson IA.#TITLE:Crystal structures of human GAR Tfase at low and high pH and with substrate beta-GAR.#JOURNAL:Biochemistry.#YEAR:2002#PUBMED:12450384# AUTHORS:Schild D, Brake AJ, Kiefer MC, Young D, Barr PJ.#TITLE:Cloning of three human multifunctional de novo purine biosynthetic genes by functional complementation of yeast mutations. #JOURNAL:Proc Natl Acad Sci U S A. #YEAR:1990#PUBMED:2183217# AUTHORS:Poch MT, Qin W, Caperelli CA. #TITLE:The human trifunctional enzyme of de novo purine biosynthesis: heterologous expression, purification, and preliminary characterization.#JOURNAL:Protein Expr Purif.#YEAR:1998#PUBMED:9473452# GENE ASSOCIATION: (2618.1) or (2618.2)
phosphoribosylglycinamide formyltransferase
chondroitin 4-sulfotransferase, Golgi apparatus
chondroitin sulfate B (IdoA2S-GalNAc4s), precursor 5_C45H66N2O45S3X
Abbreviation: R_S4T6g Synonyms: _1 EC Number: 2.8.2.5 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_b_pre4 + paps --> cs_b_pre5 + h + pap Confidence Level: 4 NCD genes: LOCUS:_50515#TRANSCRIPT:1#ABBREVIATION:CHST11# LOCUS:_55501#TRANSCRIPT:1#ABBREVIATION:CHST12# LOCUS:_166012#TRANSCRIPT:1#ABBREVIATION:CHST13# LOCUS:_113189#TRANSCRIPT:1#ABBREVIATION:D4ST1# proteins: NAME:carbohydrate (chondroitin 4) sulfotransferase 11, Golgi#ABBREVIATION:Chst11.1-g# NAME:carbohydrate (chondroitin 4) sulfotransferase 12, Golgi apparatus#ABBREVIATION:Chst12.1-g# NAME:carbohydrate (chondroitin 4) sulfotransferase 13, Golgi apparatus#ABBREVIATION:Chst13.1-g# NAME:dermatan 4 sulfotransferase 1, Golgi apparatus#ABBREVIATION:D4st.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Yamauchi S, Mita S, Matsubara T, Fukuta M, Habuchi H, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of chondroitin 4-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10722746# AUTHORS:Okuda T, Mita S, Yamauchi S, Matsubara T, Yagi F, Yamamori D, Fukuta M, Kuroiwa A, Matsuda Y, Habuchi O#TITLE:Molecular cloning, expression, and chromosomal mapping of human chondroitin 4-sulfotransferase, whose expression pattern in human tissues is different from that of chondroitin 6-sulfotransferase#JOURNAL:J Biochem (Tokyo)#YEAR:2000#PUBMED:11056388# AUTHORS:Hiraoka N, Nakagawa H, Ong E, Akama TO, Fukuda MN, Fukuda M#TITLE:Molecular cloning and expression of two distinct human chondroitin 4-O-sulfotransferases that belong to the HNK-1 sulfotransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781601# AUTHORS:Kang HG, Evers MR, Xia G, Baenziger JU, Schachner M#TITLE:Molecular cloning and characterization of chondroitin-4-O-sulfotransferase-3. A novel member of the HNK-1 family of sulfotransferases#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12080076# AUTHORS:Evers MR, Xia G, Kang HG, Schachner M, Baenziger JU#TITLE:Molecular cloning and characterization of a dermatan-specific N-acetylgalactosamine 4-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:1147079# AUTHORS:Mikami T, Mizumoto S, Kago N, Kitagawa H, Sugahara K.#TITLE:Specificities of three distinct human chondroitin/dermatan N-acetylgalactosamine 4-O-sulfotransferases demonstrated using partially desulfated dermatan sulfate as an acceptor: implication of differential roles in dermatan sulfate biosynthesis.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12847091# GENE ASSOCIATION: (166012.1) or (113189.1) or (50515.1) or (55501.1)
chondroitin sulfate B (IdoA2S-GalNAc4S), precursor 4_C45H67N2O42S2X
Abbreviation: R_LNLNCACPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + lnlncacrn --> crn + lnlncacoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_MM7Ag Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m7masnA --> m6masnA + man Confidence Level: 0 NCD genes: LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# GENE ASSOCIATION: (57134.1)
Abbreviation: R_ETHP Synonyms: _0 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : ethamp + h2o --> etha + pi Confidence Level: 0 no high energy phosphate compounds found associated w/ rxn NJ genes: LOCUS:_162466#TRANSCRIPT:1#ABBREVIATION:PHOSPHO1# proteins: NAME:phosphatase, orphan 1#ABBREVIATION:Phospho1.1# citations: AUTHORS:Roberts SJ, Stewart AJ, Sadler PJ, Farquharson C.#TITLE:Human PHOSPHO1 exhibits high specific phosphoethanolamine and phosphocholine phosphatase activities.#JOURNAL:Biochem J#YEAR:2004#PUBMED:15175005# GENE ASSOCIATION: (162466.1)
chondroitin sulfate C (GalNAc6S-GlcA), free chain_C45H67N2O46S3
Abbreviation: R_NACHEXA3ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_c + (2) h2o --> acgal + cs_c_deg2 + h + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_CYTK11 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : dcmp + dgtp <==> dcdp + dgdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_NNDPR Synonyms: _0 EC Number: 2.4.2.19 SUBSYSTEM: NAD Metabolism Equation: [c] : (2) h + prpp + quln --> co2 + nicrnt + ppi Confidence Level: 2 genes: LOCUS:_23475#TRANSCRIPT:1#ABBREVIATION:QPRT#ECNUMBERS:2.4.2.19# proteins: NAME:quinolinate phosphoribosyltransferase (nicotinate-nucleotide pyrophosphorylase (carboxylating)) #ABBREVIATION:Qprt.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Fukuoka SI, Nyaruhucha CM, Shibata K.#TITLE:Characterization and functional expression of the cDNA encoding human brain quinolinate phosphoribosyltransferase.#JOURNAL:Biochim Biophys Acta. #YEAR:1998#PUBMED:9473669# AUTHORS:Okuno E, White RJ, Schwarcz R.#TITLE:Quinolinic acid phosphoribosyltransferase: purification and partial characterization from human liver and brain. #JOURNAL:J Biochem (Tokyo). #YEAR:1988#PUBMED:3139649# GENE ASSOCIATION: (23475.1)
nicotinate-nucleotide diphosphorylase (carboxylating)
Abbreviation: R_CORE8GTg Synonyms: _2 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : Tn_antigen + udpgal --> core8 + h + udp Confidence Level: 0 EC 2.4.1.- NCD genes: none proteins: none citations: none GENE ASSOCIATION:
N-acetylgalactosamine 3-alpha-galactosyltransferase, Golgi apparatus
Abbreviation: R_SELMETAT Synonyms: _0 EC Number: 2.5.1.6 SUBSYSTEM: Selenoamino acid metabolism Equation: [c] : atp + h2o + selmeth --> pi + ppi + seasmet Confidence Level: 1 SAB genes: LOCUS:_4144#TRANSCRIPT:1#ABBREVIATION:MAT2A# LOCUS:_4143#TRANSCRIPT:1#ABBREVIATION:MAT1A# LOCUS:_27430#TRANSCRIPT:1#ABBREVIATION:MAT2B# LOCUS:_27430#TRANSCRIPT:2#ABBREVIATION:MAT2B# proteins: NAME:methionine adenosyltransferase I, alpha #ABBREVIATION:Mat1a.1# NAME:methionine adenosyltransferase II 1#ABBREVIATION:Mat2ab1# NAME:methionine adenosyltransferase II 2#ABBREVIATION:Mat2ab2# citations: AUTHORS:Katsuhiko Nakamuro, Tomofumi Okuno, and Tatsuya Hasegawa#TITLE:Metabolism of Selenoamino Acids and Contribution of Selenium Methylation to Their Toxicity#JOURNAL:Journal of Health Science#YEAR:2000#PUBMED:?# GENE ASSOCIATION: (4143.1) or (27430.2 and 4144.1) or (27430.1 and 4144.1)
Abbreviation: R_ALDD20x Synonyms: _0 SUBSYSTEM: Tryptophan metabolism Equation: [c] : h2o + id3acald + nad --> (2) h + ind3ac + nadh Confidence Level: 1 NCD genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# citations: none GENE ASSOCIATION: (8854.3) or (8854.1) or (223.1) or (224.1) or (216.1) or (501.1) or (8854.2)
aldehyde dehydrogenase (indole-3-acetaldehyde, NAD)
Abbreviation: R_C180CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: stcrn[c] --> stcrn[m] Confidence Level: 4 TV genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (788.1)
Abbreviation: R_NADtpu Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: nadh[x] --> nadh[c] Confidence Level: 0 diffusion - but unidirectional uptake into peroxisome (to avoid loops w/ NAD/NADP) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PSFLIP Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + ps_hs[e] --> adp[c] + h[c] + pi[c] + ps_hs[c] Confidence Level: 4 See PMID: 10856717 - flippase activity NJ genes: LOCUS:_10396#TRANSCRIPT:1#ABBREVIATION:ATP8A1#ECNUMBERS:3.6.3.1# LOCUS:_57194#TRANSCRIPT:1#ABBREVIATION:ATP10A#ECNUMBERS:3.6.3.1# proteins: NAME:ATPase, aminophospholipid transporter (APLT), Class I, type 8A, member 1#ABBREVIATION:Atp8a1.1# NAME:ATPase, Class V, type 10A#ABBREVIATION:Atp10a.1# citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# AUTHORS:Herzing LB, Kim SJ, Cook EH Jr, Ledbetter DH.#TITLE:The human aminophospholipid-transporting ATPase gene ATP10C maps adjacent to UBE3A and exhibits similar imprinted expression.#JOURNAL:Am J Hum Genet#YEAR:2001#PUBMED:11353404# AUTHORS:Mouro I, Halleck MS, Schlegel RA, Mattei MG, Williamson P, Zachowski A, Devaux P, Cartron JP, Colin Y.#TITLE:Cloning, expression, and chromosomal mapping of a human ATPase II gene, member of the third subfamily of P-type ATPases and orthologous to the presumed bovine and murine aminophospholipid translocase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10198212# GENE ASSOCIATION: (10396.1) or (57194.1)
(alpha-D-Mannosyl)4-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1485H2440N20O420P20
Abbreviation: R_M4MPDOL_Uter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: m4mpdol_U[c] --> m4mpdol_U[r] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
UDPGal-chondroitin acetylgalactosaminyltransferase I, Golgi
Abbreviation: R_GALNACT1g Synonyms: _4 EC Number: 2.4.1.174 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs,hs_linkage + udpacgal --> cs_pre + h + udp Confidence Level: 4 NCD genes: LOCUS:_55454#TRANSCRIPT:1#ABBREVIATION:GALNACT-2# LOCUS:_55790#TRANSCRIPT:1#ABBREVIATION:ChGn# proteins: NAME:chondroitin sulfate GalNAcT-2, Golgi apparatus#ABBREVIATION:Galnact-2.1-g# NAME:chondroitin beta1,4 N-acetylgalactosaminyltransferase, Golgi apparatus#ABBREVIATION:Chgn.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Sato T, Gotoh M, Kiyohara K, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Togayachi A, Kudo T, Asada M, Watanabe H, Imamura T, Kimata K, Narimatsu H#TITLE:Differential roles of two N-acetylgalactosaminyltransferases, CSGalNAcT-1, and a novel enzyme, CSGalNAcT-2. Initiation and elongation in synthesis of chondroitin sulfate#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12446672# AUTHORS:Uyama T, Kitagawa H, Tamura Ji J, Sugahara K#TITLE:Molecular cloning and expression of human chondroitin N-acetylgalactosaminyltransferase: the key enzyme for chain initiation and elongation of chondroitin/dermatan sulfate on the protein linkage region tetrasaccharide shared by heparin/heparan sulfate#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11788602# AUTHORS:Gotoh M, Sato T, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Zhang Y, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Nishihara S, Watanabe H, Kimata K, Narimatsu H#TITLE:Enzymatic synthesis of chondroitin with a novel chondroitin sulfate N-acetylgalactosaminyltransferase that transfers N-acetylgalactosamine to glucuronic acid in initiation and elongation of chondroitin sulfate synthesis#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12163485# GENE ASSOCIATION: (55790.1) or (55454.1)
Abbreviation: R_TYRt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tyr-L[e] <==> tyr-L[c] Confidence Level: 4 genes: LOCUS:_117247#TRANSCRIPT:1#ABBREVIATION:SLC16A10# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 10#ABBREVIATION:Slc16a10.1-e# citations: AUTHORS:Kim do K, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H#TITLE:The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location#JOURNAL:Genomics#YEAR:2002#PUBMED:11827462# GENE ASSOCIATION: (117247.1)
Abbreviation: R_EX_estrones_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : estrones <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACODA Synonyms: _0 EC Number: 3.5.1.16 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : acorn + h2o --> ac + orn Confidence Level: 1 genes: LOCUS:_95#TRANSCRIPT:1#ABBREVIATION:ACY1#ECNUMBERS:3.5.1.14# proteins: NAME:aminoacylase 1#ABBREVIATION:Acy1.1# citations: AUTHORS:Jones WM, Scaloni A, Bossa F, Popowicz AM, Schneewind O, Manning JM#TITLE:Genetic relationship between acylpeptide hydrolase and acylase, two hydrolytic enzymes with similar binding but different catalytic specificities#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:2006156# GENE ASSOCIATION: (95.1)
Abbreviation: R_AGLPH Synonyms: _0 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : ak2gp_hs + h2o --> ak2g_hs + pi Confidence Level: 0 NJ genes: none proteins: none citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Brites P, Waterham HR, Wander RJA#TITLE:Functions and biosynthesis of plasmalogens in health and disease#JOURNAL:Biochimica et Biophysica Acta#YEAR:2004# AUTHORS:Nagan N, Zoeller RA#TITLE:Plasmalogens: biosynthesis and functions#JOURNAL:Progress in Lipid Research#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_PYDXDH Synonyms: _0 EC Number: 1.2.3.1 SUBSYSTEM: Vitamin B6 Metabolism Equation: [c] : h2o + o2 + pydx --> 4pyrdx + h2o2 Confidence Level: 4 IT genes: LOCUS:_316#TRANSCRIPT:1#ABBREVIATION:AOX1# proteins: NAME:aldehyde oxidase 1#ABBREVIATION:Aox1.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# GENE ASSOCIATION: (316.1)
Abbreviation: R_NDP6 Synonyms: _0 EC Number: 3.6.1.6 SUBSYSTEM: Nucleotides Equation: [c] : dcdp + h2o --> dcmp + h + pi Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_COQ5m Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 2dp6mobq + amet --> 2dp6mobq_me + ahcys + h Confidence Level: 2 _2.1.1.- IT genes: none proteins: none citations: AUTHORS:Kang DC, Takeshige K, Minakami S.#TITLE:An intermediate of ubiquinone biosynthesis exists in the microsomal fraction of HepG2 cells. #JOURNAL:J Biochem (Tokyo).#YEAR:1990#PUBMED:1965190# AUTHORS:Nambudiri AM, Ranganathan S, Rudney H#TITLE:The role of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in the regulation of ubiquinone synthesis in human fibroblasts. #JOURNAL:J Biol Chem.#YEAR:1980#PUBMED:7380842# AUTHORS:Jonassen T, Clarke CF.#TITLE:Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis. #JOURNAL:J Biol Chem.#YEAR:2000#PUBMED:10777520# GENE ASSOCIATION:
Ubiquinone biosynthesis methyltransferase COQ5
keratan sulfate II (core 4-linked), degradation product 4_C66H108N6O48SX
Abbreviation: R_NACHEX26ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksii_core4_deg4 --> (2) acgam + ksii_core2_deg5 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3073.1) or (3074.1)
Abbreviation: R_34DHXMANDACOX_LPAREN_NADP_RPAREN_ Synonyms: _0 EC Number: 1.2.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 34dhmald + h2o + nadp <==> 34dhoxmand + (2) h + nadph Confidence Level: 4 SAB genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Sladek NE#TITLE:Human aldehyde dehydrogenases: potential pathological, pharmacological, and toxicological impact#JOURNAL:J Biochem Mol Toxicol#YEAR:2003#PUBMED:12616643# AUTHORS:Kawamura M, Eisenhofer G, Kopin IJ, Kador PF, Lee YS, Tsai JY, Fujisawa S, Lizak MJ, Sinz A, Sato S#TITLE:Aldose reductase, a key enzyme in the oxidative deamination of norepinephrine in rats#JOURNAL:Biochem Pharmacol#YEAR:1999#PUBMED:10424772# GENE ASSOCIATION: (221.1) or (222.1)
_3,4-Dihydroxymandelaldehyde:NADP+ oxidoreductase
Abbreviation: R_ACTNMO Synonyms: _0 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : acetone + h + nadph + o2 --> acetol + h2o + nadp Confidence Level: 4 stoichiometry described in Thornalley PJ. Gen Pharmacol. 1996 Jun;27(4):565-73. PMID: 8853285 NCD genes: LOCUS:_1571#TRANSCRIPT:1#ABBREVIATION:CYP2E1# proteins: NAME:cytochrome P450, family 2, subfamily E, polypeptide 1#ABBREVIATION:Cyp2e1.1# citations: AUTHORS:Vander Jagt DL, Robinson B, Taylor KK, Hunsaker LA.#TITLE:Reduction of trioses by NADPH-dependent aldo-keto reductases. Aldose reductase, methylglyoxal, and diabetic complications#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1537826# AUTHORS:Thornalley PJ#TITLE:Pharmacology of methylglyoxal: formation, modification of proteins and nucleic acids, and enzymatic detoxification--a role in pathogenesis and antiproliferative chemotherapy.#JOURNAL:Gen Pharmacol#YEAR:1996#PUBMED:8853285# AUTHORS:Beisswenger BG, Delucia EM, Lapoint N, Sanford RJ, Beisswenger PJ.#TITLE:Ketosis leads to increased methylglyoxal production on the atkins diet#JOURNAL:Ann N Y Acad Sci#YEAR:2005#PUBMED:16037240# GENE ASSOCIATION: (1571.1)
Abbreviation: R_EX_xolest_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : xolest_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_P45027A16m Synonyms: _4 EC Number: 1.14.13.15 SUBSYSTEM: Bile Acid Biosynthesis Equation: [m] : nadph + o2 + xol7ah2al --> dhcholestanate + h2o + nadp Confidence Level: 4 from uniprot NJ genes: LOCUS:_1593#TRANSCRIPT:1#ABBREVIATION:CYP27A1# proteins: NAME:cytochrome P450, family 27, subfamily A, polypeptide 1#ABBREVIATION:Cyp27a1.1-m# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1593.1)
Abbreviation: R_EX_orn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : orn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLCter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: glc-D[c] <==> glc-D[r] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:van Schaftingen E, Gerin I#TITLE:The glucose-6-phopsphatase system#JOURNAL:Biochem J#YEAR:2002#PUBMED:11879177# GENE ASSOCIATION:
glucose transport, endoplasmic reticulum
alpha-D-mannosyl-beta-D-mannosyl-diacylchitobiosyldiphosphodolichol, human uterine homolog_C1305H2140N20O270P20
Abbreviation: R_DHEASt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dheas[e] + hco3[c] <==> dheas[c] + hco3[e] Confidence Level: 4 NJ genes: LOCUS:_11309#TRANSCRIPT:1#ABBREVIATION:SLCO2B1# LOCUS:_28234#TRANSCRIPT:1#ABBREVIATION:SLCO1B3# LOCUS:_6579#TRANSCRIPT:1#ABBREVIATION:SLCO1A2# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1A2#ABBREVIATION:Slco1a2.1# NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# NAME:solute carrier organic anion transporter family, member 1B3#ABBREVIATION:Slco1b3.1# NAME:solute carrier organic anion transporter family, member 2B1#ABBREVIATION:Slco2b1.1# citations: AUTHORS:Kullak-Ublick GA, Beuers U, Meier PJ, Domdey H, Paumgartner G.#TITLE:Assignment of the human organic anion transporting polypeptide (OATP) gene to chromosome 12p12 by fluorescence in situ hybridization.#JOURNAL:J Hepatol.#YEAR:1996#PUBMED:9007731# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (11309.1) or (6579.1) or (28234.1) or (10599.1)
dehydroepiandrosterone sulfate transport via bicarbonate countertransport
Abbreviation: R_NADHtpu Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: nadh[c] --> nadh[x] Confidence Level: 0 diffusion - but unidirectional uptake into ER (to avoid loops w/ NAD/NADP) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
UDP-D-galactose:D-xylose galactosyltransferase, Golgi apparatus
Abbreviation: R_GALTg Synonyms: _4 EC Number: 2.4.1.133 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : udpgal + xser --> h + lxser + udp Confidence Level: 4 NCD genes: LOCUS:_11285#TRANSCRIPT:1#ABBREVIATION:B4GALT7#ECNUMBERS:2.4.1.133# proteins: NAME:xylosylprotein beta 1,4-galactosyltransferase, polypeptide 7 (galactosyltransferase I), Golgi apparatus#ABBREVIATION:B4galt7.1-g# citations: AUTHORS:Okajima T, Yoshida K, Kondo T, Furukawa K#TITLE:Human homolog of Caenorhabditis elegans sqv-3 gene is galactosyltransferase I involved in the biosynthesis of the glycosaminoglycan-protein linkage region of proteoglycans#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10438455# AUTHORS:Almeida R, Levery SB, Mandel U, Kresse H, Schwientek T, Bennett EP, Clausen H#TITLE:Cloning and expression of a proteoglycan UDP-galactose:beta-xylose beta1,4-galactosyltransferase I. A seventh member of the human beta4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:1073568# AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# GENE ASSOCIATION: (11285.1)
Abbreviation: R_BCDO Synonyms: _0 EC Number: 1.14.99.36 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : caro + o2 --> (2) retinal Confidence Level: 4 IT genes: LOCUS:_53630#TRANSCRIPT:1#ABBREVIATION:BCDO1#ECNUMBERS:1.13.11.21# proteins: NAME:beta-carotene 15, 15'-dioxygenase #ABBREVIATION:BcdO1.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Yan W, Jang GF, Haeseleer F, Esumi N, Chang J, Kerrigan M, Campochiaro M, Campochiaro P, Palczewski K, Zack DJ.#TITLE:Cloning and characterization of a human beta,beta-carotene-15,15'-dioxygenase that is highly expressed in the retinal pigment epithelium.#JOURNAL:Genomics#YEAR:2001#PUBMED:11401432# AUTHORS:Wyss A, Wirtz G, Woggon W, Brugger R, Wyss M, Friedlein A, Bachmann H, Hunziker W.#TITLE:Cloning and expression of beta,beta-carotene 15,15'-dioxygenase. #JOURNAL:Biochem Biophys Res Commun.#YEAR:2000#PUBMED:10799297# GENE ASSOCIATION: (53630.1)
phosphatidylinositol-5-phosphate 4-kinase
Abbreviation: R_PI5P4K Synonyms: _0 EC Number: 2.7.1.149 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + pail5p_hs --> adp + h + pail45p_hs Confidence Level: 4 NCD genes: LOCUS:_5297#TRANSCRIPT:1#ABBREVIATION:PIK4CA#ECNUMBERS:2.7.1.67,2.7.1.68# LOCUS:_5298#TRANSCRIPT:1#ABBREVIATION:PIK4CB# LOCUS:_55361#TRANSCRIPT:1#ABBREVIATION:PI4KII# proteins: NAME:phosphatidylinositol 4-kinase, catalytic, alpha polypeptide#ABBREVIATION:Pik4ca.1# NAME:phosphatidylinositol 4-kinase, catalytic, beta polypeptide#ABBREVIATION:Pik4cb.1# NAME:phosphtaidylinositol 4-kinase, type II#ABBREVIATION:Pi4kii.1# citations: AUTHORS:Tolias KF, Cantley LC#TITLE:Pathways for phosphoinositide synthesis#JOURNAL:Chem Phys Lipids#YEAR:1999#PUBMED:10358929# AUTHORS:Zhang X, Loijens JC, Boronenkov IV, Parker GJ, Norris FA, Chen J, Thum O, Prestwich GD, Majerus PW, Anderson RA#TITLE:Phosphatidylinositol-4-phosphate 5-kinase isozymes catalyze the synthesis of 3-phosphate-containing phosphatidylinositol signaling molecules#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9211928# AUTHORS:Rameh LE, Tolias KF, Duckworth BC, Cantley LC#TITLE:A new pathway for synthesis of phosphatidylinositol-4,5-bisphosphate#JOURNAL:Nature#YEAR:1997#PUBMED:9367159# GENE ASSOCIATION: (5297.1) or (55361.1) or (5298.1)
Abbreviation: R_FAOXC241181x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : (3) coa + (3) h2o + (3) nad + nrvnccoa + (3) o2 --> (3) accoa + (3) h + (3) h2o2 + (3) nadh + odecoa Confidence Level: 4 peroxisomal (very long chain FA) - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) c24:1 -> c18:1 - 3 rounds beta-ox 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (51.1 and 30.1 and 1962.1) or (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1)
Beta-1,4 N-acetylgalactosaminyltransferase
Abbreviation: R_GALGT4 Synonyms: _3 EC Number: 2.4.1.92 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gt3_hs + udpacgal --> gt2_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_2583#TRANSCRIPT:1#ABBREVIATION:GALGT#ECNUMBERS:2.4.1.92# proteins: NAME:UDP-N-acetyl-alpha-D-galactosamine:(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase (GalNAc-T)#ABBREVIATION:Galgt.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Nagata Y, Yamashiro S, Yodoi J, Lloyd KO, Shiku H, Furukawa K.#TITLE:Expression cloning of beta 1,4 N-acetylgalactosaminyltransferase cDNAs that determine the expression of GM2 and GD2 gangliosides.#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1601877# GENE ASSOCIATION: (2583.1)
Abbreviation: R_XOLTRIOLtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: xoltriol[c] <==> xoltriol[m] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_DM_dctp_LPAREN_m_RPAREN_ Synonyms: _0 Equation: [m] : dctp --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NDPK10n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + didp <==> adp + ditp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:dIDP), nuclear
Abbreviation: R_ICDHyp Synonyms: _0 EC Number: 1.1.1.42 SUBSYSTEM: Citric Acid Cycle Equation: [x] : icit + nadp --> akg + co2 + nadph Confidence Level: 4 Strathern indicates this reaction as irreversible. It may be reversible. Location is the peroxisomal matrix (MIPS). IF genes: LOCUS:_3417#TRANSCRIPT:1#ABBREVIATION:IDH1#ECNUMBERS:1.1.1.42# proteins: NAME:isocitrate dehydrogenase 1 (NADP+), soluble, peroxisome#ABBREVIATION:Idh1.1-x# citations: AUTHORS:Geisbrecht BV, Gould SJ#TITLE:The human PICD gene encodes a cytoplasmic and peroxisomal NADP(+)-dependent isocitrate dehydrogenase#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10521434# GENE ASSOCIATION: (3417.1)
Abbreviation: R_FUMAC Synonyms: _2 EC Number: 3.7.1.2 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 4fumacac + h2o --> acac + fum + h Confidence Level: 4 NCD genes: LOCUS:_2184#TRANSCRIPT:1#ABBREVIATION:FAH#ECNUMBERS:3.7.1.2# proteins: NAME:fumarylacetoacetate hydrolase (fumarylacetoacetase)#ABBREVIATION:Fah.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (2184.1)
Abbreviation: R_RAtn3 Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: _13-cis-retn[c] <==> 13-cis-retn[n] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
_13-cis-retinoic acid transport, nuclear
chondroitin sulfate C (GalNAc6S-GlcA), degradation product 1_C45H68N2O43S2
Abbreviation: R_B3GNT310g Synonyms: _9 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acglcgalgluside_hs + udpgal --> gal14acglcgalgluside_hs + h + udp Confidence Level: 2 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3#ABBREVIATION:B3gnt3.1# citations: AUTHORS:Yokoyama-Kobayashi M, Yamaguchi T, Sekine S, Kato S.#TITLE:Selection of cDNAs encoding putative type II membrane proteins on the cell surface from a human full-length cDNA bank.#JOURNAL:Gene#YEAR:1999#PUBMED:10072769# GENE ASSOCIATION: (10331.1)
(Gal)1 (Glc)1 (GlcNAc)1 (Cer)1_C38H69N2O17FULLRCO
Abbreviation: R_NACHEX4ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_c_deg1 + h2o --> acgal + cs_c_deg2 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1) or (3074.1)
Abbreviation: R_ACOAD10m Synonyms: _0 EC Number: 1.3.99.12 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 2mbcoa + fad --> 2mb2coa + fadh2 Confidence Level: 4 MM genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_36#TRANSCRIPT:1#ABBREVIATION:ACADSB#ECNUMBERS:1.3.99.-# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# NAME:acyl-Coenzyme A dehydrogenase, short/branched chain#ABBREVIATION:Acadsb.1-m# citations: AUTHORS:Andresen,B.S., Christensen,E., Corydon,T.J., Bross,P., etc.#TITLE:Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency....#YEAR:2000#PUBMED:11013134# GENE ASSOCIATION: (36.1) or (34.1)
acyl-CoA dehydrogenase (2-methylbutanoyl-CoA), mitochondrial
Abbreviation: R_CYSt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[e] + na1[e] --> cys-L[c] + na1[c] Confidence Level: 2 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# proteins: NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# AUTHORS:Van Winkle LJ#TITLE:Amino acid transport regulation and early embryo development#JOURNAL:Biol Reprod#YEAR:2001#PUBMED:11133652# GENE ASSOCIATION: (11254.1)
L-cysteine reversible transport via sodium symport
Abbreviation: R_EX_5thf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 5thf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAOXC200180m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : arachcoa + coa + fad + h2o + nad --> accoa + fadh2 + h + nadh + stcoa Confidence Level: 4 c20:0 -> c18:0 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_FAOXC18480m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (5) coa + fad + (5) h2o + (5) nad + strdnccoa --> (5) accoa + fadh2 + (5) h + (5) nadh + occoa Confidence Level: 4 _5 rounds of beta ox, 4 degrees loss of saturation -> only 1 fad oxidized to fadh2 peroxisomal (very long chain FA) c18:4,n-6 -> c8:0, 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_S6T18g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core4_pre10 + paps --> h + ksii_core4 + pap Confidence Level: 4 NCD genes: LOCUS:_8534#TRANSCRIPT:1#ABBREVIATION:CHST1# LOCUS:_9469#TRANSCRIPT:1#ABBREVIATION:CHST3# proteins: NAME:carbohydrate (keratan sulfate Gal-6) sulfotransferase 1, Golgi#ABBREVIATION:Chst1.1-g# NAME:carbohydrate (chondroitin 6) sulfotransferase 3, Golgi#ABBREVIATION:Chst3.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Habuchi O, Hirahara Y, Uchimura K, Fukuta M#TITLE:Enzymatic sulfation of galactose residue of keratan sulfate by chondroitin 6-sulfotransferase#JOURNAL:Glycobiology#YEAR:1996#PUBMED:8991509# AUTHORS:Fukuta M, Kobayashi Y, Uchimura K, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of human chondroitin 6-sulfotransferase#JOURNAL:Biochim Biophys Acta#YEAR:1998#PUBMED:9714738# AUTHORS:Fukuta M, Inazawa J, Torii T, Tsuzuki K, Shimada E, Habuchi O#TITLE:Molecular cloning and characterization of human keratan sulfate Gal-6-sulfotransferase#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405439# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (8534.1) or (9469.1)
keratan sulfate II biosynthesis, precursor 10_C89H143N7O69S2X
Abbreviation: R_AMETt2m Synonyms: _3 SUBSYSTEM: Transport, Mitochondrial Equation: ahcys[m] + amet[c] <==> ahcys[c] + amet[m] Confidence Level: 4 Added by RS/TV (6/17/05) genes: LOCUS:_115286#TRANSCRIPT:1#ABBREVIATION:SLC25A26# LOCUS:_115286#TRANSCRIPT:2#ABBREVIATION:SLC25A26# LOCUS:_115286#TRANSCRIPT:3#ABBREVIATION:SLC25A26# proteins: NAME:Solute carrier family 25, member 26 (mitochondrial)#ABBREVIATION:Slc25a26.1-m# NAME:Solute carrier family 25, member 26 (mitochondrial) #ABBREVIATION:Slc25a26.2-m# NAME:Solute carrier family 25, member 26 (mitochondrial)#ABBREVIATION:Slc25a26.3-m# citations: AUTHORS:Agrimi G, Di Noia MA, Marobbio CM, Fiermonte G, Lasorsa FM, Palmieri F#TITLE:Identification of the human mitochondrial S-adenosylmethionine transporter: bacterial expression, reconstitution, functional characterization and tissue distribution#JOURNAL:Biochem J#YEAR:2004#PUBMED:14674884# GENE ASSOCIATION: (115286.3) or (115286.2) or (115286.1)
S-Adenosyl-L-methionine reversible transport, mitochondrial
Abbreviation: R_FUT98g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacglcgal14acglcgalgluside_hs + gdpfuc --> fuc13galacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
Abbreviation: R_N2M2NMASNtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: n2m2nmasn[e] --> n2m2nmasn[l] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
n2m2nmasn transport, extracellular to lysosome
Abbreviation: R_GAMt1r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gam[e] <==> gam[c] Confidence Level: 4 genes: LOCUS:_6514#TRANSCRIPT:1#ABBREVIATION:SLC2A2# LOCUS:_6513#TRANSCRIPT:1#ABBREVIATION:SLC2A1# LOCUS:_6517#TRANSCRIPT:1#ABBREVIATION:SLC2A4# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 1, plasma membrane#ABBREVIATION:Slc2a1.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 2, extracellular#ABBREVIATION:Slc2a2.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 4, extracellular#ABBREVIATION:Slc2a4.1-e# citations: AUTHORS:Hosokawa M, Thorens B#TITLE:Glucose release from GLUT2-null hepatocytes: characterization of a major and a minor pathway#JOURNAL:Am J Physiol Endocrinol Metab#YEAR:2002#PUBMED:11882499# AUTHORS:Guillam MT, Burcelin R, Thorens B#TITLE:Normal hepatic glucose production in the absence of GLUT2 reveals an alternative pathway for glucose release from hepatocytes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9770484# AUTHORS:Uldry M, Thorens B#TITLE:The SLC2 family of facilitated hexose and polyol transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750891# AUTHORS:Flier JS, Mueckler MM, Usher P, Lodish HF#TITLE:Elevated levels of glucose transport and transporter messenger RNA are induced by ras or src oncogenes#JOURNAL:Science#YEAR:1987#PUBMED:3103217# AUTHORS:Maher F, Vannucci SJ, Simpson IA#TITLE:Glucose transporter proteins in brain#JOURNAL:FASEB J#YEAR:1994#PUBMED:7926364# AUTHORS:Mueckler M, Caruso C, Baldwin SA, Panico M, Blench I, Morris HR, Allard WJ, Lienhard GE, Lodish HF#TITLE:Sequence and structure of a human glucose transporter#JOURNAL:Science#YEAR:1985#PUBMED:3839598# AUTHORS:Uldry M, Ibberson M, Hosokawa M, Thorens B#TITLE:GLUT2 is a high affinity glucosamine transporter#JOURNAL:FEBS Lett#YEAR:2002#PUBMED:12135767# AUTHORS:Fukumoto H, Seino S, Imura H, Seino Y, Eddy RL, Fukushima Y, Byers MG, Shows TB, Bell GI#TITLE:Sequence, tissue distribution, and chromosomal localization of mRNA encoding a human glucose transporter-like protein#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1988#PUBMED:3399500# AUTHORS:Johnson JH, Newgard CB, Milburn JL, Lodish HF, Thorens B#TITLE:The high Km glucose transporter of islets of Langerhans is functionally similar to the low affinity transporter of liver and has an identical primary sequence#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2182619# AUTHORS:Thorens B, Cheng ZQ, Brown D, Lodish HF#TITLE:Liver glucose transporter: a basolateral protein in hepatocytes and intestine and kidney cells#JOURNAL:Am J Physiol#YEAR:1990#PUBMED:1701966# AUTHORS:Fukumoto H, Kayano T, Buse JB, Edwards Y, Pilch PF, Bell GI, Seino S#TITLE:Cloning and characterization of the major insulin-responsive glucose transporter expressed in human skeletal muscle and other insulin-responsive tissues#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2656669# GENE ASSOCIATION: (6513.1) or (6517.1) or (6514.1)
_1D-myo-Inositol 3,4,5,6-tetrakisphosphate_C6H8O18P4
Abbreviation: R_RTOT2 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : R2coa_hs --> Rtotalcoa Confidence Level: 0 User defined, tissue specific fluxes that can be customized w/ various ratios of R1 through R5 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GAO1g Synonyms: _1 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : accoa + gd3_hs --> coa + oagd3_hs Confidence Level: 4 for humans - seq predicted SLC transporters have been found to have O-acetylation activity on gangliosides NJ genes: LOCUS:_9197#TRANSCRIPT:1#ABBREVIATION:SLC33A1# proteins: NAME:acetyl-CoA transporter/O-acetylator#ABBREVIATION:Slc33a1.1# citations: AUTHORS:Kanamori A, Nakayama J, Fukuda MN, Stallcup WB, Sasaki K, Fukuda M, Hirabayashi Y#TITLE:Expression cloning and characterization of a cDNA encoding a novel membrane protein required for the formation of O-acetylated ganglioside: A putative acetyl-CoA-transporter#JOURNAL:Proc Natl Acad Sci#YEAR:1997# GENE ASSOCIATION: (9197.1)
Abbreviation: R_GALGALFUCFUCGALACGLCGALACGLCGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: galgalfucfucgalacglcgalacglcgal14acglcgalgluside_hs[e] <==> galgalfucfucgalacglcgalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_VITD2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: vitd2[c] --> vitd2[e] Confidence Level: 2 I am not sure how vitamin D3 is released from Skin cells based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_FAOXC226 Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : c226coa + (10) coa + (4) fad + (10) h2o + (10) nad --> (11) accoa + (4) fadh2 + (10) h + (10) nadh Confidence Level: 4 TV Other enzymes: 1.3.3.6, 4.2.1.74, 4.2.1.17, 1.1.1.35, 1.1.1.211 genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Kelly DP, Kim JJ, Billadello JJ, Hainline BE, Chu TW, Strauss AW#TITLE:Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3035565# AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# AUTHORS:Weiping Le, Azfar S. Abbas, Howard Sprecher, Jerry Vockley and Horst Schulz#TITLE:Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial [beta]-oxidation of unsaturated fatty acids#JOURNAL:Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids#YEAR:2000# GENE ASSOCIATION: (35.1) or (34.1)
Hexacosanoyl-CoA (n-C26:0CoA)_C47H82N7O17P3S
Abbreviation: R_HEXCCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + hexccrn --> crn + hexccoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_ARTPLM3 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : Rtotal3coa <==> pmtcoa Confidence Level: 0 cytosolic conversion of pooled FA into palmitate (so subsequent transport into mit w/ FA oxidation can occur). Since Carbon stoichiometry normalized wrt C16 ratio between Rgroupcoa and pmtcoa is 1:1 HR fatty acid metabolism - R groups = acyl groups, Rtotal, Rtotal2, Rtotal3 correspond to three possible acyl groups added to glycerol R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ARTFR33 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + vacccoa[c] --> (1.125) R3coa_hs[c] + fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UGT1A4r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Steroid Metabolism Equation: [r] : tststerone + udpglcur --> tststeroneglc + udp Confidence Level: 4 NJ genes: LOCUS:_54575#TRANSCRIPT:1#ABBREVIATION:UGT1A10# LOCUS:_54490#TRANSCRIPT:1#ABBREVIATION:UGT2B28# LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A10#ABBREVIATION:Ugt1a10.1# NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# NAME:UDP glycosyltransferase 2 family, polypeptide B28#ABBREVIATION:Ugt2b28.1# citations: AUTHORS:Basu NK, Kubota S, Meselhy MR, Ciotti M, Chowdhury B, Hartori M, Owens IS.#TITLE:Free Full Text Gastrointestinally distributed UDP-glucuronosyltransferase 1A10, which metabolizes estrogens and nonsteroidal anti-inflammatory drugs, depends upon phosphorylation.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117964# AUTHORS:Mackenzie PI, Owens IS, Burchell B, Bock KW, Bairoch A, Belanger A, Fournel-Gigleux S, Green M, Hum DW, Iyanagi T, Lancet D, Louisot P, Magdalou J, Chowdhury JR, Ritter JK, Schachter H, Tephly TR, Tipton KF, Nebert DW.#TITLE:The UDP glycosyltransferase gene superfamily: recommended nomenclature update based on evolutionary divergence.#JOURNAL:Pharmacogenetics#YEAR:1997#PUBMED:9295054# AUTHORS:Levesque E, Turgeon D, Carrier JS, Montminy V, Beaulieu M, Belanger A.#TITLE:Isolation and characterization of the UGT2B28 cDNA encoding a novel human steroid conjugating UDP-glucuronosyltransferase.#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11300766# GENE ASSOCIATION: (54575.1) or (54490.1) or (54576.1)
Abbreviation: R_EX_andrstrn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : andrstrn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PPPG9tm Synonyms: _0 SUBSYSTEM: Heme Biosynthesis Equation: pppg9[c] <==> pppg9[m] Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
protoporphyrinogen IX mitochondrial transport
Abbreviation: R_EX_acn23acngalgbside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acn23acngalgbside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
sialyl (2,3) sialyl (2,6) galactosylgloboside (homo sapiens) exchange
Abbreviation: R_DNDPt32m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + dgdp[c] --> atp[c] + dgdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_ARGSS Synonyms: _0 EC Number: 6.3.4.5 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [c] : asp-L + atp + citr-L --> amp + argsuc + h + ppi Confidence Level: 4 genes: LOCUS:_445#TRANSCRIPT:1#ABBREVIATION:ASS#ECNUMBERS:6.3.4.5# LOCUS:_445#TRANSCRIPT:2#ABBREVIATION:ASS# proteins: NAME:argininosuccinate synthetase#ABBREVIATION:Ass.1# NAME:argininosuccinate synthetase#ABBREVIATION:Ass.2# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (445.1) or (445.2)
Abbreviation: R_DNDPt33m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgdp[c] + dudp[m] --> dgdp[m] + dudp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_UREAtm Synonyms: _0 SUBSYSTEM: Urea cycle/amino group metabolism Equation: urea[c] <==> urea[m] Confidence Level: 2 JLR SAB m genes: LOCUS:_366#TRANSCRIPT:1#ABBREVIATION:AQP9# proteins: NAME:aquaporin 9#ABBREVIATION:Aqp9.1# citations: AUTHORS:Amiry-Moghaddam M, Lindland H, Zelenin S, Roberg BA, Gundersen BB, Petersen P, Rinvik E, Torgner IA, Ottersen OP#TITLE:Brain mitochondria contain aquaporin water channels: evidence for the expression of a short AQP9 isoform in the inner mitochondrial membrane#JOURNAL:FASEB J#YEAR:2005#PUBMED:16126913# AUTHORS:Tsukaguchi H, Shayakul C, Berger UV, Mackenzie B, Devidas S, Guggino WB, van Hoek AN, Hediger MA#TITLE:Molecular characterization of a broad selectivity neutral solute channel#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9733774# AUTHORS:Agre P, King LS, Yasui M, Guggino WB, Ottersen OP, Fujiyoshi Y, Engel A, Nielsen S#TITLE:Aquaporin water channels--from atomic structure to clinical medicine#JOURNAL:J Physiol#YEAR:2002#PUBMED:12096044# GENE ASSOCIATION: (366.1)
Abbreviation: R_STS1 Synonyms: _4 EC Number: 3.1.6.2 SUBSYSTEM: Steroid Metabolism Equation: [c] : dheas + h2o --> dhea + h + so4 Confidence Level: 4 NJ genes: LOCUS:_412#TRANSCRIPT:1#ABBREVIATION:STS#ECNUMBERS:3.1.6.2# proteins: NAME:steroid sulfatase (microsomal), arylsulfatase C#ABBREVIATION:Sts.1# citations: AUTHORS:Stein C, Hille A, Seidel J, Rijnbout S, Waheed A, Schmidt B, Geuze H, von Figura K.#TITLE:Cloning and expression of human steroid-sulfatase. Membrane topology, glycosylation, and subcellular distribution in BHK-21 cells.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2668275# AUTHORS:Migeon BR, Shapiro LJ, Norum RA, Mohandas T, Axelman J, Dabora RL.#TITLE:Abstract Differential expression of steroid sulphatase locus on active and inactive human X chromosome.#JOURNAL:Nature#YEAR:1982#PUBMED:6957717# GENE ASSOCIATION: (412.1)
Abbreviation: R_ST8SIA53g Synonyms: _13 EC Number: 2.4.99.8 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gd1a_hs --> cmp + gt1a_hs + h Confidence Level: 4 NJ genes: LOCUS:_29906#TRANSCRIPT:1#ABBREVIATION:SIAT8E# proteins: NAME:ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 5 #ABBREVIATION:St8sia5.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Harduin-Lepers A, Vallejo-Ruiz V, Krzewinski-Recchi MA, Smyn-Petit B, Julien S, Delannoy P#TITLE:The human sialyltransferase family#JOURNAL:Biochimie#YEAR:2001# AUTHORS:Kim YJ, Kim KS, Do S, Kim CH, Kim SK, Lee YC.#TITLE:Molecular cloning and expression of human alpha2,8-sialyltransferase (hST8Sia V).#JOURNAL:Biochem Biophys Res Commun.#YEAR:1997#PUBMED:9199191# GENE ASSOCIATION: (29906.1)
Abbreviation: R_FBP26 Synonyms: _0 EC Number: 3.1.3.46 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : f26bp + h2o --> f6p + pi Confidence Level: 0 IF genes: LOCUS:_8789#TRANSCRIPT:1#ABBREVIATION:FBP2#ECNUMBERS:2.7.1.11# LOCUS:_5207#TRANSCRIPT:1#ABBREVIATION:PFKFB1#ECNUMBERS:2.7.1.105,,3.1.# LOCUS:_5210#TRANSCRIPT:1#ABBREVIATION:PFKFB4#ECNUMBERS:2.7.1.105,3.1.3.46# LOCUS:_2203#TRANSCRIPT:1#ABBREVIATION:FBP1#ECNUMBERS:3.1.3.11# LOCUS:_5209#TRANSCRIPT:1#ABBREVIATION:PFKFB3#ECNUMBERS:2.7.1.105,3.1.3.46# LOCUS:_5208#TRANSCRIPT:1#ABBREVIATION:PFKFB2#ECNUMBERS:2.7.1.105,,3.1.# proteins: NAME:fructose-1,6-bisphosphatase 1 (liver)#ABBREVIATION:Fbp1.1# NAME:fructose-1,6-bisphosphatase 2 (muscle)#ABBREVIATION:Fbp2.1# NAME:_6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1#ABBREVIATION:Pfkfb1.1# NAME:_6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 2#ABBREVIATION:Pfkfb2.1# NAME:_6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 3#ABBREVIATION:Pfkfb3.1# NAME:_6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 4#ABBREVIATION:Pfkfb4.1# citations: none GENE ASSOCIATION: (2203.1) or (5210.1) or (5207.1) or (5209.1) or (5208.1) or (8789.1)
keratan sulfate I, degradation product 41_C34H58N2O26
Abbreviation: R_EX_fucfucfucgalacglcgal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucfucfucgalacglcgal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(Gal)3 (Glc)1 (GlcNAc)2 (LFuc)3 (Cer)1 exchange
Abbreviation: R_EX_CLPND_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : clpnd <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
clupanodonic acid (docosapentaenoic (n-3)) exchange
Abbreviation: R_EX_gam_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gam <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_H2CO3Dm Synonyms: _0 EC Number: 4.2.1.1 SUBSYSTEM: Miscellaneous Equation: [m] : co2 + h2o <==> h2co3 Confidence Level: 4 tv genes: LOCUS:_11238#TRANSCRIPT:1#ABBREVIATION:CA5B# LOCUS:_763#TRANSCRIPT:1#ABBREVIATION:CA5A#ECNUMBERS:4.2.1.1# proteins: NAME:Carbonic anhydrase VA(liver), mitochondrial #ABBREVIATION:Ca5a.1-m# NAME:Carbonic anhydrase VB, mitochondrial #ABBREVIATION:Ca5b.1-m# citations: AUTHORS:Shah GN, Hewett-Emmett D, Grubb JH, Migas MC, Fleming RE, Waheed A, Sly WS.#TITLE:Mitochondrial carbonic anhydrase CA VB: differences in tissue distribution and pattern of evolution from those of CA VA suggest distinct physiological roles.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2000#PUBMED:10677517# GENE ASSOCIATION: (763.1) or (11238.1)
Abbreviation: R_Uritm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: uri[c] <==> uri[m] Confidence Level: 4 IT genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-m# citations: AUTHORS:Lai Y, Tse CM, Unadkat JD.#TITLE:Mitochondrial expression of the human equilibrative nucleoside transporter 1 (hENT1) results in enhanced mitochondrial toxicity of antiviral drugs.#JOURNAL:J Biol Chem. #YEAR:2004#PUBMED:14607828# AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (2030.1)
uridine facilated transport in mitochondria
Abbreviation: R_DEDOLP1_L Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dedoldp_L + h2o --> (0.1) dedolp_L + h + pi Confidence Level: 0 EC-3.6.1.- NCD genes: none proteins: none citations: AUTHORS:Schenk B, Fernandez F, Waechter CJ.#TITLE:The ins(ide) and out(side) of dolichyl phosphate biosynthesis and recycling in the endoplasmic reticulum.#JOURNAL:Glycobiology#YEAR:2001#PUBMED:11425794# GENE ASSOCIATION:
dehydrodolichol diphosphate phosphatase (liver)
Dehydrodolichol phosphate, human liver homolog_C1080H1738O40P10
Abbreviation: R_EGMESTr Synonyms: _0 SUBSYSTEM: Alkaloid biosynthesis II Equation: [r] : egme + h2o --> ecgon + h + meoh Confidence Level: 2 SAB EC 3.1.1.- genes: LOCUS:_13#TRANSCRIPT:1#ABBREVIATION:AADAC# proteins: NAME:arylacetamide deacetylase (esterase)#ABBREVIATION:Aadac.1-r# citations: AUTHORS:Probst MR, Beer M, Beer D, Jeno P, Meyer UA, Gasser R#TITLE:Human liver arylacetamide deacetylase. Molecular cloning of a novel esterase involved in the metabolic activation of arylamine carcinogens with high sequence similarity to hormone-sensitive lipase#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8063807# GENE ASSOCIATION: (13.1)
glucose 6-phosphate dehydrogenase, endoplasmic reticulum
Abbreviation: R_G6PDH2rer Synonyms: _0 SUBSYSTEM: Pentose Phosphate Pathway Equation: [r] : g6p + nadp <==> 6pgl + h + nadph Confidence Level: 4 NCD genes: LOCUS:_9563#TRANSCRIPT:1#ABBREVIATION:H6PD#ECNUMBERS:1.1.1.47# proteins: NAME:hexose-6-phosphate dehydrogenase, endoplasmic reticulum#ABBREVIATION:H6pd.1-r# citations: AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Mason PJ, Stevens D, Diez A, Knight SW, Scopes DA, Vulliamy TJ.#TITLE:Human hexose-6-phosphate dehydrogenase (glucose 1-dehydrogenase) encoded at 1p36: coding sequence and expression.#JOURNAL: Blood Cells Mol Dis#YEAR:1999#PUBMED:10349511# AUTHORS:Clarke JL, Mason PJ#TITLE:Murine hexose-6-phosphate dehydrogenase: a bifunctional enzyme with broad substrate specificity and 6-phosphogluconolactonase activity.#JOURNAL:Arch Biochem Biophys#YEAR:2003#PUBMED:12831846# AUTHORS:Barash V, Erlich T, Bashan N.#TITLE:Microsomal hexose-6-phosphate and 6-phosphogluconate dehydrogenases in extrahepatic tissues: human placenta and pig kidney cortex.#JOURNAL:Biochem Int.#YEAR:1990#PUBMED:2156506# GENE ASSOCIATION: (9563.1)
Abbreviation: R_GLCMter Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glc-D[r] --> glc-D[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Hosokawa M, Thorens B#TITLE:Glucose release from GLUT2-null hepatocytes: characterization of a major and a minor pathway#JOURNAL:Am J Physiol Endocrinol Metab#YEAR:2002#PUBMED:11882499# GENE ASSOCIATION:
Abbreviation: R_PSERT Synonyms: _0 EC Number: 2.6.1.52 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : 3php + glu-L --> akg + pser-L Confidence Level: 4 genes: LOCUS:_29968#TRANSCRIPT:1#ABBREVIATION:PSAT1#ECNUMBERS:2.6.1.52# LOCUS:_29968#TRANSCRIPT:2#ABBREVIATION:PSAT1#ECNUMBERS:2.6.1.52# proteins: NAME:phosphoserine aminotransferase 1#ABBREVIATION:Psat1.1# NAME:phosphoserine aminotransferase 1#ABBREVIATION:Psat1.2# citations: AUTHORS:Baek,J.Y. , Jun,d. o Y , Taub,D. , Kim,Y.H. #TITLE: Characterization of human phosphoserine aminotransferase involved in the phosphorylated pathway of L-serine biosynthesis #YEAR:2003#PUBMED:12633500# GENE ASSOCIATION: (29968.1) or (29968.2)
Abbreviation: R_EX_retinol_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : retinol <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TDCHOLAte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tdchola[e] <==> tdchola[c] Confidence Level: 2 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLAl Synonyms: _5 EC Number: 3.2.1.22 SUBSYSTEM: Sphingolipid Metabolism Equation: [l] : digalside_hs + h2o --> gal + galside_hs Confidence Level: 4 NJ genes: LOCUS:_2717#TRANSCRIPT:1#ABBREVIATION:GLA#ECNUMBERS:3.2.1.22# proteins: NAME:alpha galactosidase, lysosomal#ABBREVIATION:Gla.1-l# citations: AUTHORS:Ishii S, Nakao S, Minamikawa-Tachino R, Desnick RJ, Fan JQ.#TITLE:Alternative splicing in the alpha-galactosidase A gene: increased exon inclusion results in the Fabry cardiac phenotype.#JOURNAL:Am J Hum Genet#YEAR:2002#PUBMED:11828341# AUTHORS:Lam CW, Mak YT, Lo YM, Tong SF, To KF, Lai FM.#TITLE:Molecular genetic analysis of a Chinese patient with Fabry disease.#JOURNAL:Chin Med J (Engl)#YEAR:2000#PUBMED:11775551# AUTHORS:Garman SC, Garboczi DN.#TITLE:The molecular defect leading to Fabry disease: structure of human alpha-galactosidase.#JOURNAL:J Mol Biol#YEAR:2004#PUBMED:15003450# AUTHORS:Bishop DF, Kornreich R, Desnick RJ#TITLE:Structural organization of the human alpha-galactosidase A gene: further evidence for the absence of a 3' untranslated region#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1988#PUBMED:2836863# GENE ASSOCIATION: (2717.1)
Abbreviation: R_H2Otp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: h2o[c] <==> h2o[x] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DOLGPP_Ler Synonyms: _3 EC Number: 3.1.4.48 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolglcp_L + h2o --> (0.1) dolp_L + glc-D + h Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION:
Dolichyl-beta-D-glucosyl-phosphate dolichylphosphohydrolase (liver)
Abbreviation: R_MAOLNOR Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Tyrosine metabolism Equation: [c] : h2o + normete-L + o2 --> 3m4hpga + h2o2 + nh4 Confidence Level: 4 SAB genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# proteins: NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (4128.1) or (4129.1)
Abbreviation: R_EX_ksi_deg1_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ksi_deg1 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I, degradation product 1 exchange
keratan sulfate I biosynthesis, precursor 35_C241H384N17O205S11X
Abbreviation: R_AG13T9g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre15 + uacgam --> h + ksi_pre16 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
keratan sulfate I biosynthesis, precursor 15_C149H240N10O119S4X
Abbreviation: R_ALAR Synonyms: _0 EC Number: 5.1.1.1 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [c] : ala-L <==> ala-D Confidence Level: 1 genes: LOCUS:_11212#TRANSCRIPT:1#ABBREVIATION:PROSC# proteins: NAME:alanine racemase#ABBREVIATION:Prosc.1# citations: none GENE ASSOCIATION: (11212.1)
Abbreviation: R_SCP2x Synonyms: _2 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : coa + dhocholoylcoa --> dgcholcoa + ppcoa Confidence Level: 4 SCP has two transcripts, second isoform specific to peroxisomes, first is specific for cyt and mit need to double check stoichiometry - reaction cofactors (additional coa + release of prop coa) from BM (Roche) Biochemical Pathways version 2 analog added as per KEGG NJ genes: LOCUS:_6342#TRANSCRIPT:1#ABBREVIATION:SCP2# proteins: NAME:sterol carrier protein 2#ABBREVIATION:Scp2x.1# citations: AUTHORS:Yamamoto R, Kallen CB, Babalola GO, Rennert H, Billheimer JT, Strauss JF 3rd.#TITLE:Cloning and expression of a cDNA encoding human sterol carrier protein 2.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1703300# GENE ASSOCIATION: (6342.1)
Abbreviation: R_FACOAL2042 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + eicostet <==> amp + eicostetcoa + ppi Confidence Level: 4 rev version so additional rxn doesn't need to be added to "unload" AA alternate to arachidonic acid (n-3 rather than n-6) NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_DNDPt62m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgdp[m] + dgtp[c] --> dgdp[c] + dgtp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_PI3PP Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail3p_hs --> pail_hs + pi Confidence Level: 4 EC 3.1.3.- NCD genes: LOCUS:_8897#TRANSCRIPT:1#ABBREVIATION:MTMR3# LOCUS:_8897#TRANSCRIPT:2#ABBREVIATION:MTMR3# LOCUS:_8897#TRANSCRIPT:3#ABBREVIATION:MTMR3# proteins: NAME:myotubularin related protein 3#ABBREVIATION:Mtmr3.1# NAME:myotubularin related protein 3#ABBREVIATION:Mtmr3.2# NAME:myotubularin related protein 3#ABBREVIATION:Mtmr3.3# citations: AUTHORS:Walker DM, Urbe S, Dove SK, Tenza D, Raposo G, Clague MJ#TITLE:Characterization of MTMR3. an inositol lipid 3-phosphatase with novel substrate specificity#JOURNAL:Curr Biol#YEAR:2001#PUBMED:11676921# GENE ASSOCIATION: (8897.2) or (8897.3) or (8897.1)
phosphatidylinositol-3-phosphate 3-phosphatase
Abbreviation: R_EX_gd1c_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gd1c_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_C204CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: arachdcrn[c] --> arachdcrn[m] Confidence Level: 4 TDV C20:4 transport genes: none proteins: none citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION:
arachidonic acid transport into the mitochondria
Abbreviation: R_CSCPASEly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cspg_c + h2o --> Ser-Gly/Ala-X-Gly + cs_c Confidence Level: 1 Note that this reaction occurs in the endosome during endocytosis, but has been compartmentalized as lysosomal. NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
chondroitin sulfate C proteoglycan protease, lysosome (endosome)
Abbreviation: R_EX_onpthl_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : onpthl <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
heparan sulfate, degradation product 11_C55H81N3O67S7
Abbreviation: R_S6TASE3ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg11 <==> h + hs_deg12 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_MI1PP Synonyms: _6 EC Number: 3.1.3.25 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi1p-D --> inost + pi Confidence Level: 4 JLR genes: LOCUS:_3613#TRANSCRIPT:1#ABBREVIATION:IMPA2# LOCUS:_3612#TRANSCRIPT:1#ABBREVIATION:IMPA1#ECNUMBERS:3.1.3.25# proteins: NAME:inositol(myo)-1(or 4)-monophosphatase 1#ABBREVIATION:Impa1.1# NAME:inositol(myo)-1(or 4)-monophosphatase 2#ABBREVIATION:Impa2.1# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:McAllister G, Whiting P, Hammond EA, Knowles MR, Atack JR, Bailey FJ, Maigetter R, Ragan CI.#TITLE:cDNA cloning of human and rat brain myo-inositol monophosphatase. Expression and characterization of the human recombinant enzyme#JOURNAL:Biochem J#YEAR:1992#PUBMED:1377913# AUTHORS:Yoshikawa T, Turner G, Esterling LE, Sanders AR, Detera-Wadleigh SD#TITLE:A novel human myo-inositol monophosphatase gene, IMP.18p, maps to a susceptibility region for bipolar disorder#JOURNAL:Mol Psychiatry#YEAR:1997#PUBMED:9322233# GENE ASSOCIATION: (3612.1) or (3613.1)
Abbreviation: R_COQ6m Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 2dp6mep + o2 --> 2dp6mobq + h2o Confidence Level: 4 _1.13.12.- IT genes: LOCUS:_51004#TRANSCRIPT:1#ABBREVIATION:COQ6# LOCUS:_51004#TRANSCRIPT:2#ABBREVIATION:COQ6# proteins: NAME:coenzyme Q6 homolog (yeast) #ABBREVIATION:Coq6.1-m# NAME:coenzyme Q6 homolog (yeast) #ABBREVIATION:Coq6.2-m# citations: AUTHORS:Kang DC, Takeshige K, Minakami S.#TITLE:An intermediate of ubiquinone biosynthesis exists in the microsomal fraction of HepG2 cells. #JOURNAL:J Biochem (Tokyo).#YEAR:1990#PUBMED:1965190# AUTHORS:Nambudiri AM, Ranganathan S, Rudney H#TITLE:The role of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in the regulation of ubiquinone synthesis in human fibroblasts. #JOURNAL:J Biol Chem.#YEAR:1980#PUBMED:7380842# AUTHORS:Szkopinska A.#TITLE:Ubiquinone. Biosynthesis of quinone ring and its isoprenoid side chain. Intracellular localization.#JOURNAL:Acta Biochim Pol. #YEAR:2000#PUBMED:11051212# GENE ASSOCIATION: (51004.1) or (51004.2)
Ubiquinone biosynthesis monooxgenase COQ6
Abbreviation: R_ACACT1x Synonyms: _0 EC Number: 2.3.1.9 SUBSYSTEM: Cholesterol Metabolism Equation: [x] : (2) accoa --> aacoa + coa Confidence Level: 4 NCD NJ - added peroxisomal version genes: LOCUS:_39#TRANSCRIPT:1#ABBREVIATION:ACAT2#ECNUMBERS:2.3.1.9# proteins: NAME:acetyl-Coenzyme A acetyltransferase 2 (acetoacetyl Coenzyme A thiolase)#ABBREVIATION:Acat2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (39.1)
acetyl-CoA C-acetyltransferase, mitochondrial
Abbreviation: R_PTE2x Synonyms: _1 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : h2o + pmtcoa --> coa + h + hdca Confidence Level: 4 NJ genes: LOCUS:_10005#TRANSCRIPT:1#ABBREVIATION:PTE1#ECNUMBERS:3.1.2.2# LOCUS:_10005#TRANSCRIPT:2#ABBREVIATION:PTE1#ECNUMBERS:3.1.2.2# LOCUS:_10005#TRANSCRIPT:3#ABBREVIATION:PTE1#ECNUMBERS:3.1.2.2# LOCUS:_10965#TRANSCRIPT:1#ABBREVIATION:ZAP128# proteins: NAME:peroxisomal acyl-CoA thioesterase#ABBREVIATION:Pte1.1# NAME:peroxisomal acyl-CoA thioesterase#ABBREVIATION:Pte1.2# NAME:peroxisomal acyl-CoA thioesterase#ABBREVIATION:Pte1.3# NAME:peroxisomal long-chain acyl-CoA thioesterase#ABBREVIATION:Zap128.1# citations: AUTHORS:Jones JM, Nau K, Geraghty MT, Erdmann R, Gould SJ.#TITLE:Identification of peroxisomal acyl-CoA thioesterases in yeast and humans.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10092594# GENE ASSOCIATION: (10965.1) or (10005.1) or (10005.3) or (10005.2)
Abbreviation: R_DARGOp Synonyms: _0 EC Number: 1.4.3.3 SUBSYSTEM: D-arg and D-orn metabolism Equation: [x] : arg-D + h2o + o2 --> 5g2oxpt + h2o2 + nh4 Confidence Level: 4 SAB genes: LOCUS:_1610#TRANSCRIPT:1#ABBREVIATION:DAO#ECNUMBERS:1.4.3.3# proteins: NAME:D-amino-acid oxidase#ABBREVIATION:Dao.1-x# citations: AUTHORS:Vanoni MA, Cosma A, Mazzeo D, Mattevi A, Todone F, Curti B#TITLE:Limited proteolysis and X-ray crystallography reveal the origin of substrate specificity and of the rate-limiting product release during oxidation of D-amino acids catalyzed by mammalian D-amino acid oxidase#JOURNAL:Biochemistry#YEAR:1997#PUBMED:9153402# AUTHORS:D'Aniello A, Vetere A, Petrucelli L#TITLE:Further study on the specificity of D-amino acid oxidase and D-aspartate oxidase and time course for complete oxidation of D-amino acids#JOURNAL:Comp Biochem Physiol B#YEAR:1993#PUBMED:8103425# GENE ASSOCIATION: (1610.1)
Abbreviation: R_LGNCCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: lgnccrn[c] --> lgnccrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_EX_ade_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ade <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLUtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: glu-L[c] <==> glu-L[r] Confidence Level: 1 unknown mechanism, needed to transport glu-L out of ER in conversion of leuktrC4-> leuktrD4 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_BAAT5x Synonyms: _1 EC Number: 2.3.1.65 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : dcholcoa + taur --> coa + tdchola Confidence Level: 2 fifth version added in analogy to BAAT1x and BAAT2x NJ genes: LOCUS:_570#TRANSCRIPT:1#ABBREVIATION:BAAT#ECNUMBERS:2.3.1.65# proteins: NAME:bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase)#ABBREVIATION:Baat.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Falany CN, Johnson MR, Barnes S, Diasio RB#TITLE:Glycine and taurine conugation of bile acids by a single enzyme#JOURNAL:Journal of Biological Chemistry#YEAR:1994# GENE ASSOCIATION: (570.1)
Abbreviation: R_NT5C Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: NAD Metabolism Equation: [c] : h2o + nicrnt --> nicrns + pi Confidence Level: 4 IT genes: LOCUS:_22978#TRANSCRIPT:1#ABBREVIATION:NT5C2# proteins: NAME:_5'-nucleotidase, cytosolic II #ABBREVIATION:Nt5C2.1# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (22978.1)
Abbreviation: R_LGNCCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: lgnccoa[c] <==> lgnccoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
glucose-1,2-(2)[glucose-1,3]-mannose oligosaccharide_C24H42O21
Abbreviation: R_ENMAN3g Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : g3m8masn + h2o --> glc3man + m7masnA Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# AUTHORS:Roth J, Ziak M, Zuber C.#TITLE:The role of glucosidase II and endomannosidase in glucose trimming of asparagine-linked oligosaccharides.#JOURNAL:Biochimie#YEAR:2003#PUBMED:12770767# GENE ASSOCIATION:
Endomannosidase (glc3man-producing), Golgi apparatus
Abbreviation: R_DNDPt10m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[c] + dcdp[m] --> dadp[m] + dcdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_FT Synonyms: _1 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : frdp + ipdp --> ppi + ttc-ggdp Confidence Level: 0 EC 2.5.1.- NCD genes: none proteins: none citations: none GENE ASSOCIATION:
farnesyltranstransferase (trans,trans,cis-geranylgeranyl diphosphate-generating)
Abbreviation: R_EX_acnacngal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acnacngal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_5-Hydroxyindoleacetaldehyde:NAD+ oxidoreductase (mito)
Abbreviation: R_5HOXINDACTOXm Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Tryptophan metabolism Equation: [m] : 5hoxindact + h2o + nad --> 5hoxindoa + (2) h + nadh Confidence Level: 2 SAB genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: AUTHORS:Keung WM#TITLE:Biogenic aldehyde(s) derived from the action of monoamine oxidase may mediate the antidipsotropic effect of daidzin#JOURNAL:Chem Biol Interact#YEAR:2001#PUBMED:11306106# GENE ASSOCIATION: (8659.2) or (219.1) or (8659.1) or (217.1)
Abbreviation: R_C4STMO1r Synonyms: _0 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : 44mzym + (3) h + (3) nadph + (3) o2 --> 4mzym_int1 + (4) h2o + (3) nadp Confidence Level: 4 EC 1.-.-.- NCD ER version NJ genes: LOCUS:_6307#TRANSCRIPT:1#ABBREVIATION:SC4MOL# proteins: NAME:sterol-C4-methyl oxidase#ABBREVIATION:Sc4mol.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (6307.1)
C-4 sterol methyl oxidase (4,4-dimethylzymosterol)
_4-Methylzymosterol intermediate 1_C29H46O3
Abbreviation: R_ASCBt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ascb-L[e] + na1[e] <==> ascb-L[c] + na1[c] Confidence Level: 4 NCD genes: LOCUS:_9963#TRANSCRIPT:1#ABBREVIATION:SLC23A1# LOCUS:_9963#TRANSCRIPT:2#ABBREVIATION:SLC23A1# LOCUS:_9962#TRANSCRIPT:1#ABBREVIATION:SLC23A2# LOCUS:_9962#TRANSCRIPT:2#ABBREVIATION:SLC23A2# proteins: NAME:solute carrier family 23 (nucleobase transporters), member 1#ABBREVIATION:Slc23a1.1-e# NAME:solute carrier family 23 (nucleobase transporters), member 1#ABBREVIATION:Slc23a1.2-e# NAME:solute carrier family 23 (nucleobase transporters), member 2#ABBREVIATION:Slc23a2.1-e# NAME:solute carrier family 23 (nucleobase transporters), member 2#ABBREVIATION:Slc23a2.2-e# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Daruwala R, Song J, Koh WS, Rumsey SC, Levine M#TITLE:Cloning and functional characterization of the human sodium-dependent vitamin C transporters hSVCT1 and hSVCT2#JOURNAL:FEBS Lett#YEAR:1999#PUBMED:10556521# AUTHORS:Wang Y, Mackenzie B, Tsukaguchi H, Weremowicz S, Morton CC, Hediger MA#TITLE:Human vitamin C (L-ascorbic acid) transporter SVCT1#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10631088# AUTHORS:Rajan DP, Huang W, Dutta B, Devoe LD, Leibach FH, Ganapathy V, Prasad PD#TITLE:Human placental sodium-dependent vitamin C transporter (SVCT2): molecular cloning and transport function#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10471399# AUTHORS:Wang H, Dutta B, Huang W, Devoe LD, Leibach FH, Ganapathy V, Prasad PD#TITLE:Human Na(+)-dependent vitamin C transporter 1 (hSVCT1): primary structure, functional characteristics and evidence for a non-functional splice variant.#JOURNAL:Biochim Biophys Acta#YEAR:1999#PUBMED:10556483# AUTHORS:Wilson JX#TITLE:Regulation of Vitamin C Transport#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15705056# GENE ASSOCIATION: (9962.2) or (9963.1) or (9962.1) or (9963.2)
L-ascorbate transport via proton symport
keratan sulfate I biosynthesis, precursor 12_C135H218N9O106S3X
Abbreviation: R_XYLUR Synonyms: _0 EC Number: 1.1.1.10 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : h + nadph + xylu-L <==> nadp + xylt Confidence Level: 4 NCD genes: LOCUS:_51181#TRANSCRIPT:1#ABBREVIATION:DCXR#ECNUMBERS:1.1.1.10# proteins: NAME:dicarbonyl/L-xylulose reductase#ABBREVIATION:Dcxr.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Nakagawa J, Ishikura S, Asami J, Isaji T, Usami N, Hara A, Sakurai T, Tsuritani K, Oda K, Takahashi M, Yoshimoto M, Otsuka N, Kitamura K#TITLE:Molecular characterization of mammalian dicarbonyl/L-xylulose reductase and its localization in kidney#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11882650# AUTHORS:Wang YM, van Eys J#TITLE:Nutritional significance of fructose and sugar alcohols#JOURNAL:Annu Rev Nutr.#YEAR:1981#PUBMED:6821187# GENE ASSOCIATION: (51181.1)
Abbreviation: R_DOPACHRMISO Synonyms: _0 SUBSYSTEM: Tyrosine metabolism Equation: [c] : (2) 2c23dh56dhoxin + o2 --> (2) L-dpchrm + (2) h2o Confidence Level: 4 SAB genes: LOCUS:_7299#TRANSCRIPT:1#ABBREVIATION:TYR#ECNUMBERS:1.14.18.1# proteins: NAME:tyrosinase#ABBREVIATION:Tyr.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# AUTHORS:Aaron Bunsen Lerner, Thomas B. Fitzpatrick, Evan Calkins, and William H. Summerson#TITLE:MAMMALIAN TYROSINASE: PREPARATION AND PROPERTIES#JOURNAL:J. Biol. Chem#YEAR:1949# AUTHORS:Pomerantz SH#TITLE:The tyrosine hydroxylase activity of mammalian tyrosinase#JOURNAL:J Biol Chem#YEAR:1968#PUBMED:5294951# GENE ASSOCIATION: (7299.1)
(R)-3-Hydroxybutanoate:NAD+ oxidoreductase
Abbreviation: R_BDHm Synonyms: _0 EC Number: 1.1.1.30 SUBSYSTEM: Butanoate Metabolism Equation: [m] : bhb + nad <==> acac + h + nadh Confidence Level: 4 IT genes: LOCUS:_622#TRANSCRIPT:1#ABBREVIATION:BDH1#ECNUMBERS:1.1.1.30# LOCUS:_622#TRANSCRIPT:3#ABBREVIATION:BDH1#ECNUMBERS:1.1.1.30# LOCUS:_622#TRANSCRIPT:2#ABBREVIATION:BDH1#ECNUMBERS:1.1.1.30# proteins: NAME:_3-hydroxybutyrate dehydrogenase (heart, mitochondrial)#ABBREVIATION:Bdh1.1-m# NAME:_3-hydroxybutyrate dehydrogenase (heart, mitochondrial)#ABBREVIATION:Bdh1.2-m# NAME:_3-hydroxybutyrate dehydrogenase (heart, mitochondrial)#ABBREVIATION:Bdh1.3-m# citations: AUTHORS:Marks AR, McIntyre JO, Duncan TM, Erdjument-Bromage H, Tempst P, Fleischer S#TITLE:Molecular cloning and characterization of (R)-3-hydroxybutyrate dehydrogenase from human heart#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1639787# AUTHORS:Green D, Marks AR, Fleischer S, McIntyre JO#TITLE:Wild type and mutant human heart (R)-3-hydroxybutyrate dehydrogenase expressed in insect cells#JOURNAL:Biochemistry#YEAR:1996#PUBMED:8679568# GENE ASSOCIATION: (622.2) or (622.3) or (622.1)
Abbreviation: R_GSNtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: gsn[c] <==> gsn[m] Confidence Level: 4 IT genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-m# citations: AUTHORS:Lai Y, Tse CM, Unadkat JD.#TITLE:Mitochondrial expression of the human equilibrative nucleoside transporter 1 (hENT1) results in enhanced mitochondrial toxicity of antiviral drugs.#JOURNAL:J Biol Chem. #YEAR:2004#PUBMED:14607828# AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (2030.1)
guanosine facilated transport in mitochondria
Abbreviation: R_BTNt4i Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: btn[e] + h[c] --> btn[c] + h[e] Confidence Level: 4 IT genes: LOCUS:_80704#TRANSCRIPT:1#ABBREVIATION:SLC19A3# proteins: NAME:solute carrier family 19, member 3 #ABBREVIATION:Slc19a3.1# citations: AUTHORS:Vlasova TI, Stratton SL, Wells AM, Mock NI, Mock DM.#TITLE:Biotin deficiency reduces expression of SLC19A3, a potential biotin transporter, in leukocytes from human blood.#JOURNAL:J Nutr.#YEAR:2005#PUBMED:15623830# AUTHORS:Ganapathy V, Smith SB, Prasad PD.#TITLE:SLC19: the folate/thiamine transporter family.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:14770311# GENE ASSOCIATION: (80704.1)
Abbreviation: R_MEVK1x Synonyms: _0 EC Number: 2.7.1.36 SUBSYSTEM: Cholesterol Metabolism Equation: [x] : atp + mev-R --> 5pmev + adp + h Confidence Level: 4 peroxisomal version NJ genes: LOCUS:_4598#TRANSCRIPT:1#ABBREVIATION:MVK#ECNUMBERS:2.7.1.36# proteins: NAME:mevalonate kinase#ABBREVIATION:Mvk.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (4598.1)
Abbreviation: R_CERT1gt Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: crm_hs[c] <==> crm_hs[g] Confidence Level: 2 non-vesicular intracellular transport of ceramide genes: LOCUS:_10087#TRANSCRIPT:1#ABBREVIATION:COL4A3BP# proteins: NAME:collagen, type IV, alpha 3 (Goodpasture antigen) binding protein#ABBREVIATION:Col4a3bp.1# citations: AUTHORS:Kumagai K, Yasuda S, Okemoto K, Nishijima M, Kobayashi S, Hanada K.#TITLE:CERT mediates intermembrane transfer of various molecular species of ceramides.#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15596449# AUTHORS:Hanada K, Kumagai K, Yasuda S, Miura Y, Kawano M, Fukasawa M, Nishijima M.#TITLE:Molecular machinery for non-vesicular trafficking of ceramide.#JOURNAL:Nature#YEAR:2003#PUBMED:14685229# AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# AUTHORS:van Meer G, Wolthoorn J, Degroote S.#TITLE:The fate and function of glycosphingolipid glucosylceramide.#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:2003#PUBMED:12803919# GENE ASSOCIATION: (10087.1)
Abbreviation: R_XOL7AONEtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: xol7aone[c] <==> xol7aone[r] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
_3,4-Dihydroxyphenylacetate:amet O-methyltransferase
Abbreviation: R_34DHPHAMT Synonyms: _0 EC Number: 2.1.1.6 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 34dhpha + amet --> ahcys + h + homoval Confidence Level: 1 SAB genes: LOCUS:_1312#TRANSCRIPT:1#ABBREVIATION:COMT#ECNUMBERS:2.1.1.6# LOCUS:_1312#TRANSCRIPT:2#ABBREVIATION:COMT#ECNUMBERS:2.1.1.6# proteins: NAME:catechol-O-methyltransferase#ABBREVIATION:Comt.1# NAME:catechol-O-methyltransferase#ABBREVIATION:Comt.2# citations: AUTHORS:Bertocci B, Miggiano V, Da Prada M, Dembic Z, Lahm HW, Malherbe P#TITLE:Human catechol-O-methyltransferase: cloning and expression of the membrane-associated form#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1847521# GENE ASSOCIATION: (1312.1) or (1312.2)
Abbreviation: R_LYSMTF2n Synonyms: _7 EC Number: 2.1.1.43 SUBSYSTEM: Lysine Metabolism Equation: [n] : Nmelys + amet --> Ndmelys + ahcys Confidence Level: 4 MM genes: LOCUS:_79723#TRANSCRIPT:1#ABBREVIATION:SUV39H2# LOCUS:_10919#TRANSCRIPT:1#ABBREVIATION:BAT8# LOCUS:_9869#TRANSCRIPT:1#ABBREVIATION:SETDB1# LOCUS:_6839#TRANSCRIPT:1#ABBREVIATION:SUV39H1# LOCUS:_83852#TRANSCRIPT:1#ABBREVIATION:SETDB2# LOCUS:_58508#TRANSCRIPT:1#ABBREVIATION:MLL3# LOCUS:_58508#TRANSCRIPT:2#ABBREVIATION:MLL3# LOCUS:_10919#TRANSCRIPT:2#ABBREVIATION:BAT8# LOCUS:_84444#TRANSCRIPT:1#ABBREVIATION:DOT1L# LOCUS:_80854#TRANSCRIPT:1#ABBREVIATION:SET7# LOCUS:_79813#TRANSCRIPT:1#ABBREVIATION:EHMT1# LOCUS:_387893#TRANSCRIPT:1#ABBREVIATION:SET8# proteins: NAME:suppressor of variegation 3-9 homolog 1 (Drosophila), nuclear #ABBREVIATION:Suv39h1.1-n# NAME:SET domain, bifurcated 1, nuclear #ABBREVIATION:Setdb1.1-n# NAME:myeloid/lymphoid or mixed-lineage leukemia 3, nuclear#ABBREVIATION:Mll3.1-n# NAME:myeloid/lymphoid or mixed-lineage leukemia 3, nuclear#ABBREVIATION:Mll3.2-n# NAME:DOT1-like, histone H3 methyltransferase (S. cerevisiae), nuclear #ABBREVIATION:Dot1l.1-n# NAME:SET domain-containing protein 7, nuclear #ABBREVIATION:Set7.1-n# NAME:HLA-B associated transcript 8, nuclear#ABBREVIATION:Bat8.1-n# NAME:HLA-B associated transcript 8, nuclear#ABBREVIATION:Bat8.2-n# NAME:SET domain, bifurcated 2, nuclear #ABBREVIATION:Setdb2.1-n# NAME:suppressor of variegation 3-9 homolog 2 (Drosophila), nuclear #ABBREVIATION:Suv39h2.1-n# NAME:euchromatic histone methyltransferase 1, nuclear #ABBREVIATION:Ehmt1.1-n# NAME:PR/SET domain containing protein 8, nuclear #ABBREVIATION:Set8.1-n# citations: none GENE ASSOCIATION: (387893.1) or (58508.2) or (83852.1) or (80854.1) or (9869.1) or (84444.1) or (10919.2) or (6839.1) or (10919.1) or (79723.1) or (58508.1) or (79813.1)
Abbreviation: R_FK Synonyms: _0 EC Number: 2.7.1.52 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : atp + fuc-L --> adp + fuc1p-L + h Confidence Level: 0 kh 1/6/03 NCD genes: LOCUS:_197258#TRANSCRIPT:1#ABBREVIATION:FUK# proteins: NAME:fucokinase#ABBREVIATION:Fuk.1# citations: none GENE ASSOCIATION: (197258.1)
(phosphoethanolaminyl-mannosyl),(phosphoethanolaminyl)-dimannosyl-glucosaminyl-acylphosphatidylinositol (H7')_C53H100N3O35P3FULLRCO2FULLR2CO2
Abbreviation: R_H7_APOS_ETer Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : emem2gacpail_hs + pe_hs --> dag_hs + gpi_hs Confidence Level: 2 NCD genes: LOCUS:_23556#TRANSCRIPT:1#ABBREVIATION:PIGN# LOCUS:_23556#TRANSCRIPT:2#ABBREVIATION:PIGN# proteins: NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.1-r# NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.2-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Hong Y, Maeda Y, Watanabe R, Ohishi K, Mishkind M, Riezman H, Kinoshita T#TITLE:Pig-n, a mammalian homologue of yeast Mcd4p, is involved in transferring phosphoethanolamine to the first mannose of the glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10574991# GENE ASSOCIATION: (23556.1) or (23556.2)
H7' phosphoethanolaminyl transferase, endoplasmic reticulum
alcohol dehydrogenase (ethanol, NADP), forward reaction
Abbreviation: R_ALCD2yf Synonyms: _0 EC Number: 1.1.1.2 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : etoh + nadp --> acald + h + nadph Confidence Level: 0 NCD genes: LOCUS:_10327#TRANSCRIPT:1#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# LOCUS:_10327#TRANSCRIPT:2#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# proteins: NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.1# NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.2# citations: none GENE ASSOCIATION: (10327.1) or (10327.2)
Abbreviation: R_PIter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: pi[r] <==> pi[c] Confidence Level: 1 NCD genes: LOCUS:_2544#TRANSCRIPT:1#ABBREVIATION:G6PT2# proteins: NAME:glucose-6-phosphatase, transport (phosphate/pyrophosphate) protein 2#ABBREVIATION:G6pt2# citations: AUTHORS:van Schaftingen E, Gerin I#TITLE:The glucose-6-phopsphatase system#JOURNAL:Biochem J#YEAR:2002#PUBMED:11879177# GENE ASSOCIATION: (2544.1)
phosphate transport, endoplasmic reticulum
keratan sulfate I, degradation product 4_C222H355N15O197S12
keratan sulfate I, degradation product 3_C233H371N16O205S12
Abbreviation: R_SIAASE2ly Synonyms: _4 EC Number: 3.2.1.18 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg3 --> acnam + ksi_deg4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_XANtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: xan[c] --> xan[x] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_34dhoxpeg_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 34dhoxpeg <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_3,4-Dihydroxyphenylethyleneglycol exchange
Abbreviation: R_THMMPt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: oh1[c] + thmmp[e] <==> oh1[e] + thmmp[c] Confidence Level: 4 IT genes: LOCUS:_6573#TRANSCRIPT:1#ABBREVIATION:SLC19A1# LOCUS:_6573#TRANSCRIPT:2#ABBREVIATION:SLC19A1# proteins: NAME:solute carrier family 19 (folate transporter), member 1 #ABBREVIATION:Slc19a1.1# NAME:solute carrier family 19 (folate transporter), member 1#ABBREVIATION:Slc19a1.2# citations: AUTHORS:Dutta B, Huang W, Molero M, Kekuda R, Leibach FH, Devoe LD, Ganapathy V, Prasad PD. #TITLE:Cloning of the human thiamine transporter, a member of the folate transporter family.#JOURNAL:J Biol Chem. #YEAR:1999#PUBMED:10542220# AUTHORS:Ganapathy V, Smith SB, Prasad PD.#TITLE:SLC19: the folate/thiamine transporter family.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:14770311# GENE ASSOCIATION: (6573.1) or (6573.2)
Thiamine monophosphate transport in via anion antiport
Abbreviation: R_HISTAtu Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hista[e] <==> hista[c] Confidence Level: 4 SAB electrogenic, Na indepdendent & rev NJ genes: LOCUS:_6582#TRANSCRIPT:1#ABBREVIATION:SLC22A2# LOCUS:_6582#TRANSCRIPT:2#ABBREVIATION:SLC22A2# LOCUS:_6581#TRANSCRIPT:1#ABBREVIATION:SLC22A3# proteins: NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.2# NAME:solute carrier family 22 (extraneuronal monoamine transporter), member 3#ABBREVIATION:Slc22a3.1# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# GENE ASSOCIATION: (6582.1) or (6581.1) or (6582.2)
phosphogluconate dehydrogenase, endoplasmic reticulum
Abbreviation: R_GNDer Synonyms: _0 EC Number: 1.1.1.44 SUBSYSTEM: Pentose Phosphate Pathway Equation: [r] : 6pgc + nadp --> co2 + nadph + ru5p-D Confidence Level: 4 SMP Note no proton generation by the the reaction NCD genes: none proteins: none citations: AUTHORS:Barash V, Erlich T, Bashan N.#TITLE:Microsomal hexose-6-phosphate and 6-phosphogluconate dehydrogenases in extrahepatic tissues: human placenta and pig kidney cortex.#JOURNAL:Biochem Int.#YEAR:1990#PUBMED:2156506# GENE ASSOCIATION:
Abbreviation: R_NACHEX6ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_d_deg1 + h2o --> acgal + cs_d_deg2 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3074.1) or (3073.1)
Abbreviation: R_EX_pro_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pro-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FUT14g Synonyms: _4 EC Number: 2.4.1.69 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : gal14acglcgalgluside_hs + gdpfuc --> fuc12gal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2523#TRANSCRIPT:1#ABBREVIATION:FUT1#ECNUMBERS:2.4.1.69# proteins: NAME:fucosyltransferase 1#ABBREVIATION:Fut1.1# citations: AUTHORS:Larsen RD, Ernst LK, Nair RP, Lowe JB.#TITLE:Molecular cloning, sequence, and expression of a human GDP-L-fucose:beta-D-galactoside 2-alpha-L-fucosyltransferase cDNA that can form the H blood group antigen.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990#PUBMED:2118655# GENE ASSOCIATION: (2523.1)
Abbreviation: R_THD1m Synonyms: _0 EC Number: 1.6.1.1 SUBSYSTEM: NAD Metabolism Equation: h[c] + nadh[m] + nadp[m] <==> h[m] + nad[m] + nadph[m] Confidence Level: 4 TDV (2-6-2003) genes: LOCUS:_23530#TRANSCRIPT:1#ABBREVIATION:NNT#ECNUMBERS:1.6.1.2# LOCUS:_23530#TRANSCRIPT:2#ABBREVIATION:NNT#ECNUMBERS:1.6.1.2# proteins: NAME:nicotinamide nucleotide transhydrogenase #ABBREVIATION:Nnt.1# NAME:nicotinamide nucleotide transhydrogenase #ABBREVIATION:Nnt.2# citations: AUTHORS:Hatefi Y, Yamaguchi M.#TITLE:Nicotinamide nucleotide transhydrogenase: a model for utilization of substrate binding energy for proton translocation.#JOURNAL:FASEB #YEAR:1996#PUBMED:8647343# AUTHORS:Zieger B, Ware J. #TITLE:Cloning and deduced amino acid sequence of human nicotinamide nucleotide transhydrogenase. #JOURNAL:DNA Seq#YEAR:1997#PUBMED:9524818# AUTHORS:Arkblad EL, Egorov M, Shakhparonov M, Romanova L, Polzikov M, Rydstrom J.#TITLE:Expression of proton-pumping nicotinamide nucleotide transhydrogenase in mouse, human brain and C elegans.#JOURNAL:Comp Biochem Physiol B Biochem Mol Biol#YEAR:2002#PUBMED:12223207# GENE ASSOCIATION: (23530.2) or (23530.1)
Abbreviation: R_ACALDtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: acald[m] <==> acald[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Lieber CS#TITLE:Ethanol metabolism, cirrhosis and alcoholism#JOURNAL:Clin Chim Acta#YEAR:1997#PUBMED:9028626# GENE ASSOCIATION:
Abbreviation: R_PCHOL_HSter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: pchol_hs[c] <==> pchol_hs[r] Confidence Level: 2 for assumed reversible, non-energy dep scramblase mechanism (PMID: 10856717). NJ genes: none proteins: none citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# GENE ASSOCIATION:
Abbreviation: R_XOLDIOLONEt Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: xoldiolone[c] <==> xoldiolone[r] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_FAOXC260240x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : coa + h2o + hexccoa + nad + o2 --> accoa + h + h2o2 + lgnccoa + nadh Confidence Level: 4 peroxisomal (very long chain FA) - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) c26:0 -> c24:0 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 -> into pmtcoa NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (51.1 and 30.1 and 1962.1) or (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1)
Abbreviation: R_TMLYSter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: tmlys[r] --> tmlys[c] Confidence Level: 1 MM genes: none proteins: none citations: none GENE ASSOCIATION:
trimethyl-L-lysine transport (ER to cytosol)
Abbreviation: R_MM8Cg Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m8masn --> m7masnC + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
Abbreviation: R_AGMTm Synonyms: _0 EC Number: 3.5.3.11 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : agm + h2o --> ptrc + urea Confidence Level: 4 SAB m genes: LOCUS:_79814#TRANSCRIPT:1#ABBREVIATION:AGMAT# proteins: NAME:agmatine ureohydrolase (agmatinase)#ABBREVIATION:Agmat.1-m# citations: AUTHORS:Mistry SK, Burwell TJ, Chambers RM, Rudolph-Owen L, Spaltmann F, Cook WJ, Morris SM Jr.#TITLE:Cloning of human agmatinase. An alternate path for polyamine synthesis induced in liver by hepatitis B virus.#JOURNAL:Am J Physiol Gastrointest Liver Physiol#YEAR:2002#PUBMED:11804860# AUTHORS:Iyer RK, Kim HK, Tsoa RW, Grody WW, Cederbaum SD.#TITLE:Cloning and characterization of human agmatinase.#JOURNAL:Mol Genet Metab#YEAR:2002#PUBMED:11914032# AUTHORS:Morris SM Jr.#TITLE:Vertebrate agmatinases: what role do they play in agmatine catabolism?#JOURNAL:Ann N Y Acad Sci#YEAR:2003#PUBMED:15028567# AUTHORS:Morris SM Jr#TITLE:Enzymes of arginine metabolism#JOURNAL:J Nutr#YEAR:2004#PUBMED:15465778# GENE ASSOCIATION: (79814.1)
Abbreviation: R_DNDPt47m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dtdp[m] + dttp[c] --> dtdp[c] + dttp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_HAS1 Synonyms: _0 EC Number: 2.4.1.212 SUBSYSTEM: Hyaluronan Metabolism Equation: uacgam[c] + udpglcur[c] --> (2) h[c] + ha_pre1[e] + (2) udp[c] Confidence Level: 4 NCD genes: LOCUS:_3036#TRANSCRIPT:1#ABBREVIATION:HAS1# LOCUS:_3037#TRANSCRIPT:1#ABBREVIATION:HAS2# LOCUS:_3038#TRANSCRIPT:1#ABBREVIATION:HAS3#ECNUMBERS:2.4.1.-# LOCUS:_3038#TRANSCRIPT:2#ABBREVIATION:HAS3# proteins: NAME:hyaluronan synthase 1#ABBREVIATION:Has1.1# NAME:hyaluronan synthase 2#ABBREVIATION:Has2.1# NAME:hyaluronan synthase 3#ABBREVIATION:Has3.1# NAME:hyaluronan synthase 3#ABBREVIATION:Has3.2# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Itano N, Kimata K.#TITLE:Molecular cloning of human hyaluronan synthase#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8651928# AUTHORS:Shyjan AM, Heldin P, Butcher EC, Yoshino T, Briskin MJ#TITLE:Functional cloning of the cDNA for a human hyaluronan synthase#JOURNAL:J Biol Chem.#YEAR:1996#PUBMED:8798544# AUTHORS:Watanabe K, Yamaguchi Y#TITLE:Molecular identification of a putative human hyaluronan synthase#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8798477# AUTHORS:Spicer AP, Olson JS, McDonald JA#TITLE:Molecular cloning and characterization of a cDNA encoding the third putative mammalian hyaluronan synthase#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9083017# AUTHORS:Itano N, Kimata K#TITLE:Mammalian hyaluronan synthases#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512858# GENE ASSOCIATION: (3038.2) or (3036.1) or (3038.1) or (3037.1)
Abbreviation: R_UDPG4E Synonyms: _0 EC Number: 5.1.3.2 SUBSYSTEM: Galactose metabolism Equation: [c] : udpg <==> udpgal Confidence Level: 0 genes: LOCUS:_2582#TRANSCRIPT:1#ABBREVIATION:GALE#ECNUMBERS:5.1.3.2# LOCUS:_2582#TRANSCRIPT:2#ABBREVIATION:GALE#ECNUMBERS:5.1.3.2# proteins: NAME:UDP-galactose-4-epimerase #ABBREVIATION:Gale.1# NAME:UDP-galactose-4-epimerase #ABBREVIATION:Gale.2# citations: none GENE ASSOCIATION: (2582.1) or (2582.2)
Abbreviation: R_STS4 Synonyms: _0 EC Number: 3.1.6.2 SUBSYSTEM: Steroid Metabolism Equation: [c] : h2o + prgnlones --> h + prgnlone + so4 Confidence Level: 4 NJ genes: LOCUS:_412#TRANSCRIPT:1#ABBREVIATION:STS#ECNUMBERS:3.1.6.2# proteins: NAME:steroid sulfatase (microsomal), arylsulfatase C#ABBREVIATION:Sts.1# citations: AUTHORS:Stein C, Hille A, Seidel J, Rijnbout S, Waheed A, Schmidt B, Geuze H, von Figura K.#TITLE:Cloning and expression of human steroid-sulfatase. Membrane topology, glycosylation, and subcellular distribution in BHK-21 cells.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2668275# AUTHORS:Migeon BR, Shapiro LJ, Norum RA, Mohandas T, Axelman J, Dabora RL.#TITLE:Abstract Differential expression of steroid sulphatase locus on active and inactive human X chromosome.#JOURNAL:Nature#YEAR:1982#PUBMED:6957717# GENE ASSOCIATION: (412.1)
Abbreviation: R_DNDPt16m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[m] + dudp[c] --> dadp[c] + dudp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_DM_datp_LPAREN_m_RPAREN_ Synonyms: _0 Equation: [m] : datp --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
heparan sulfate 6-sulfotransferase, Golgi apparatus
heparan sulfate, precursor 13_C79H116N5O92S9X
Abbreviation: R_S6T25g Synonyms: _0 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre12 + (3) paps --> (3) h + hs_pre13 + (3) pap Confidence Level: 4 EC 2.8.2.- NCD genes: LOCUS:_9394#TRANSCRIPT:1#ABBREVIATION:HS6ST1# LOCUS:_90161#TRANSCRIPT:1#ABBREVIATION:HS6ST2# LOCUS:_266722#TRANSCRIPT:1#ABBREVIATION:HS6ST3# LOCUS:_90161#TRANSCRIPT:2#ABBREVIATION:HS6ST2# proteins: NAME:heparan sulfate 6-O-sulfotransferase 1, Golgi apparatus#ABBREVIATION:Hs6st1.1-g# NAME:heparan sulfate 6-O-sulfotransferase 2, Golgi apparatus#ABBREVIATION:Hs6st2.2-g# NAME:heparan sulfate 6-O-sulfotransferase 2, Golgi apparatus#ABBREVIATION:Hs6st2.1-g# NAME:heparan sulfate 6-O-sulfotransferase 3, Golgi apparatus#ABBREVIATION:Hs6st3.1-g# citations: AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Habuchi H, Kobayashi M, Kimata K#TITLE:Molecular characterization and expression of heparan-sulfate 6-sulfotransferase. Complete cDNA cloning in human and partial cloning in Chinese hamster ovary cells#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9535912# AUTHORS:Habuchi H, Miyake G, Nogami K, Kuroiwa A, Matsuda Y, Kusche-Gullberg M, Habuchi O, Tanaka M, Kimata K#TITLE:Biosynthesis of heparan sulphate with diverse structures and functions: two alternatively spliced forms of human heparan sulphate 6-O-sulphotransferase-2 having different expression patterns and properties#JOURNAL:Biochem J#YEAR:2003#PUBMED:12492399# AUTHORS:Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, et al#TITLE:Complete sequencing and characterization of 21,243 full-length human cDNAs.#JOURNAL:Nat Genet#YEAR:2004#PUBMED:14702039# GENE ASSOCIATION: (266722.1) or (9394.1) or (90161.2) or (90161.1)
heparan sulfate, precursor 12_C79H119N5O83S6X
Abbreviation: R_XYLtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: xyl-D[l] --> xyl-D[c] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Pisoni RL, Thoene JG#TITLE:The transport systems of mammalian lysosomes#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1751541# AUTHORS:Lloyd JB#TITLE:Metabolite efflux and influx across the lysosome membrane#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993166# GENE ASSOCIATION:
Abbreviation: R_PPAtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: ppa[c] --> ppa[m] Confidence Level: 4 TDV Short-chain fatty acid can diffuse through mitochondrial membrane genes: none proteins: none citations: AUTHORS:Tassani V, Cattapan F, Magnanimi L, Peschechera A.#TITLE:Anaplerotic effect of propionyl carnitine in rat heart mitochondria.#JOURNAL:Biochem Biophys Res Commun#YEAR:1994#PUBMED:8135845# GENE ASSOCIATION:
Abbreviation: R_GALFUCGALACGLCGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: galfucgalacglcgal14acglcgalgluside_hs[e] <==> galfucgalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_P5CRxm Synonyms: _0 EC Number: 1.5.1.2 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : 1pyr5c + (2) h + nadh --> nad + pro-L Confidence Level: 2 PRO1x is irreversible in the other direction genes: LOCUS:_5831#TRANSCRIPT:1#ABBREVIATION:PYCR1#ECNUMBERS:1.5.1.2# LOCUS:_5831#TRANSCRIPT:2#ABBREVIATION:PYCR1#ECNUMBERS:1.5.1.2# proteins: NAME:pyrroline-5-carboxylate reductase 1#ABBREVIATION:Pycr1.1-m# NAME:pyrroline-5-carboxylate reductase 1#ABBREVIATION:Pycr1.2-m# citations: none GENE ASSOCIATION: (5831.1) or (5831.2)
Abbreviation: R_RAtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: retn[c] <==> retn[n] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_BILGLCURtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: bilglcur[c] <==> bilglcur[r] Confidence Level: 2 unknown mechanism, however glucuronidated metabolites need to get out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HDCAtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hdca[e] <==> hdca[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_PI35P3P Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail35p_hs --> pail5p_hs + pi Confidence Level: 4 EC 3.1.3.- NCD genes: LOCUS:_8897#TRANSCRIPT:1#ABBREVIATION:MTMR3# LOCUS:_8897#TRANSCRIPT:2#ABBREVIATION:MTMR3# LOCUS:_8897#TRANSCRIPT:3#ABBREVIATION:MTMR3# proteins: NAME:myotubularin related protein 3#ABBREVIATION:Mtmr3.1# NAME:myotubularin related protein 3#ABBREVIATION:Mtmr3.2# NAME:myotubularin related protein 3#ABBREVIATION:Mtmr3.3# citations: AUTHORS:Walker DM, Urbe S, Dove SK, Tenza D, Raposo G, Clague MJ#TITLE:Characterization of MTMR3. an inositol lipid 3-phosphatase with novel substrate specificity#JOURNAL:Curr Biol#YEAR:2001#PUBMED:11676921# GENE ASSOCIATION: (8897.1) or (8897.3) or (8897.2)
phosphatidylinositol-3,5-bisphosphate 3-phosphatase
Abbreviation: R_SUCOASm Synonyms: _0 EC Number: 6.2.1.5 SUBSYSTEM: Citric Acid Cycle Equation: [m] : atp + coa + succ <==> adp + pi + succoa Confidence Level: 0 MIPS: cofactor ATP IF changed EC from 6.2.1.4 to 6.2.1.5 - NCD genes: LOCUS:_8802#TRANSCRIPT:1#ABBREVIATION:SUCLG1#ECNUMBERS:6.2.1.4# LOCUS:_8803#TRANSCRIPT:1#ABBREVIATION:SUCLA2#ECNUMBERS:6.2.1.5# proteins: NAME:succinate--CoA ligase, ADP forming, mitochondrial#ABBREVIATION:Sucla-m# citations: AUTHORS:Johnson JD, Mehus JG, Tews K, Milavetz BI, Lambeth DO#TITLE:Genetic evidence for the expression of ATP- and GTP-specific succinyl-CoA synthetases in multicellular eucaryotes#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9765291# GENE ASSOCIATION: (8802.1 and 8803.1)
Abbreviation: R_ONPTHLte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: onpthl[e] <==> onpthl[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GALt4_2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gal[e] + (2) na1[e] <==> gal[c] + (2) na1[c] Confidence Level: 4 NCD genes: LOCUS:_6523#TRANSCRIPT:1#ABBREVIATION:SLC5A1# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 1, extracellular#ABBREVIATION:Slc5a1.1-e# citations: AUTHORS:Hirayama BA, Loo DD, Wright EM.#TITLE:Protons drive sugar transport through the Na+/glucose cotransporter (SGLT1)#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8063771# AUTHORS:Hediger MA, Turk E, Wright EM#TITLE:Homology of the human intestinal Na+/glucose and Escherichia coli Na+/proline cotransporters#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1989#PUBMED:2490366# AUTHORS:Quick M, Loo DD, Wright EM#TITLE:Neutralization of a conserved amino acid residue in the human Na+/glucose transporter (hSGLT1) generates a glucose-gated H+ channel#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11024018# AUTHORS:Wright EM, Hirayama BA, Loo DDF, Turk E, Hager K#TITLE:Intestinal sugar transport#YEAR:1994# AUTHORS:Leung DW, Loo DD, Hirayama BA, Zeuthen T, Wright EM#TITLE:Urea transport by cotransporters#JOURNAL:J Physiol#YEAR:2000#PUBMED:11034615# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# GENE ASSOCIATION: (6523.1)
Abbreviation: R_FACOAL226 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + crvnc <==> amp + c226coa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_GGH_DASH_10FTHF6GLUl Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [l] : 10fthf6glu + h2o --> 10fthf5glu + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-l# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (10FTHF6GLU), lysosomal
Abbreviation: R_EX_akg_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : akg <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAOXC16080x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : (4) coa + (4) h2o + (4) nad + (4) o2 + pmtcoa --> (4) accoa + (4) h + (4) h2o2 + (4) nadh + occoa Confidence Level: 4 _4 rounds of beta ox - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) c16:0 -> c8:0, 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (51.1 and 3295.1 and 30.1) or (51.1 and 30.1 and 1962.1) or (30.1 and 51.2 and 1962.1) or (3295.1 and 30.1 and 51.2)
Abbreviation: R_HGNTOR Synonyms: _0 EC Number: 1.13.11.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : hgentis + o2 --> 4mlacac + h Confidence Level: 4 NCD genes: LOCUS:_3081#TRANSCRIPT:1#ABBREVIATION:HGD#ECNUMBERS:1.13.11.5# proteins: NAME:homogentisate 1,2-dioxygenase#ABBREVIATION:Hgd.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (3081.1)
Homogentisate:oxygen 1,2-oxidoreductase (decyclizing)
Abbreviation: R_PPItr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: ppi[c] <==> ppi[r] Confidence Level: 1 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Diphosphate transporter, endoplasmic reticulum
Abbreviation: R_SCP22x Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: dmnoncoa[c] <==> dmnoncoa[x] Confidence Level: 4 intracellular transport, mechanism details not presently known NJ genes: LOCUS:_6342#TRANSCRIPT:1#ABBREVIATION:SCP2# proteins: NAME:sterol carrier protein 2#ABBREVIATION:Scp2.1# citations: AUTHORS:Yamamoto R, Kallen CB, Babalola GO, Rennert H, Billheimer JT, Strauss JF 3rd.#TITLE:Cloning and expression of a cDNA encoding human sterol carrier protein 2.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1703300# GENE ASSOCIATION: (6342.1)
Electron transfer flavoprotein reduced_RH2
heparan sulfate, degradation product 25_C23H37O21
Abbreviation: R_FUC13GALACGLCGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fuc13galacglcgal14acglcgalgluside_hs[g] <==> fuc13galacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SCP21x Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: phytcoa[c] <==> phytcoa[x] Confidence Level: 4 intracellular transport, mechanism details not presently known NJ genes: LOCUS:_6342#TRANSCRIPT:1#ABBREVIATION:SCP2# proteins: NAME:sterol carrier protein 2#ABBREVIATION:Scp2.1# citations: AUTHORS:Yamamoto R, Kallen CB, Babalola GO, Rennert H, Billheimer JT, Strauss JF 3rd.#TITLE:Cloning and expression of a cDNA encoding human sterol carrier protein 2.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1703300# GENE ASSOCIATION: (6342.1)
Abbreviation: R_DNDPt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + datp[c] --> atp[c] + datp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_MI1P_DASH_Dtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: mi1p-D[c] <==> mi1p-D[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
inositol 1-phosphate nuclear transport (diffusion)
Abbreviation: R_HISt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: his-L[e] + na1[e] --> his-L[c] + na1[c] Confidence Level: 4 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# proteins: NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# GENE ASSOCIATION: (11254.1) or (54407.1)
L-histidine transport in via sodium symport
fatty acyl-CoA desaturase (n-C20:3CoA -> n-C20:4CoA)
Abbreviation: R_DESAT20_1 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : dlnlcgcoa + h + nadh + o2 --> arachdcoa + (2) h2o + nad Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_3992#TRANSCRIPT:1#ABBREVIATION:FADS1#ECNUMBERS:1.14.99.25# proteins: NAME:fatty acid desaturase 1#ABBREVIATION:Fads1.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Cho HP, Nakamura M, Clarke SD#TITLE:Cloning, expression, and fatty acid regulation of the human delta-5 desaturase#JOURNAL:The Journal of Biological Chemistry#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION: (3992.1)
Abbreviation: R_EX_thmmp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : thmmp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
GDPmannose:chitobiosyldiphosphodolichol beta-D-mannosyltransferase (uterus)
Abbreviation: R_BDMT_U Synonyms: _0 EC Number: 2.4.1.142 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) chito2pdol_U + gdpmann --> gdp + h + (0.1) mpdol_U Confidence Level: 0 NCD genes: LOCUS:_56052#TRANSCRIPT:1#ABBREVIATION:ALG1# proteins: NAME:beta-1,4-mannosyltransferase#ABBREVIATION:Alg1.1# citations: none GENE ASSOCIATION: (56052.1)
Abbreviation: R_ACNACNGALGBSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acnacngalgbside_hs[c] <==> acnacngalgbside_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
acnacngalgbside_hs intracellular transport
keratan sulfate II (core 2-linked), degradation product 7_C44H73N4O30X
Abbreviation: R_NACHEX24ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core2_deg7 --> acgam + ksii_core2_deg8 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3074.1) or (3073.1) or (3073.1 and 3074.1)
chondroitin sulfate D (GlcNAc6S-GlcA2S), degradation product 5_C31H48NO32S2
Abbreviation: R_BHMT Synonyms: _3 EC Number: 2.1.1.5 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : glyb + hcys-L --> dmgly + met-L Confidence Level: 4 genes: LOCUS:_635#TRANSCRIPT:1#ABBREVIATION:BHMT#ECNUMBERS:2.1.1.5# LOCUS:_23743#TRANSCRIPT:1#ABBREVIATION:BHMT2# proteins: NAME:betaine-homocysteine methyltransferase #ABBREVIATION:Bhmt.1# NAME:betaine-homocysteine methyltransferase 2#ABBREVIATION:Bhmt2.1# citations: AUTHORS:Sunden SL, Renduchintala MS, Park EI, Miklasz SD, Garrow TA.#TITLE:Betaine-homocysteine methyltransferase expression in porcine and human tissues and chromosomal localization of the human gene.#YEAR:1997#PUBMED:9281325# GENE ASSOCIATION: (23743.1) or (635.1)
betaine-homocysteine S-methyltransferase
Abbreviation: R_C180CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + stcoa --> coa + stcrn Confidence Level: 4 TV (see C160CRN) genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (1375.4) or (1375.2) or (126129.1) or (1375.1) or (1374.1) or (1375.3)
Abbreviation: R_TCHOLAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: tchola[x] <==> tchola[c] Confidence Level: 2 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NDPK2 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + udp <==> adp + utp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.2) or (10201.1) or (4831.1 and 4830.1) or (4832.1) or (4831.1 and 4830.2) or (29922.1)
heparan sulfate, degradation product 6_C67H98N4O83S9
Abbreviation: R_S6TASE2ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg5 <==> h + hs_deg6 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
heparan sulfate, degradation product 5_C67H97N4O86S10
Abbreviation: R_GTMLTe Synonyms: _0 EC Number: 2.3.2.2 SUBSYSTEM: Glutathione Metabolism Equation: ala-L[c] + gthrd[c] --> cgly[e] + gluala[e] Confidence Level: 4 NCD SAB e see Disussion section of PMID 15469854 genes: LOCUS:_2678#TRANSCRIPT:1#ABBREVIATION:GGT1#ECNUMBERS:2.3.2.2# LOCUS:_2679#TRANSCRIPT:1#ABBREVIATION:GGT2#ECNUMBERS:2.3.2.2# LOCUS:_2678#TRANSCRIPT:3#ABBREVIATION:GGT1#ECNUMBERS:2.3.2.2# LOCUS:_2678#TRANSCRIPT:2#ABBREVIATION:GGT1#ECNUMBERS:2.3.2.2# proteins: NAME:gamma-glutamyltransferase 1#ABBREVIATION:Ggt1.1-e# NAME:gamma-glutamyltransferase 1#ABBREVIATION:Ggt1.2-e# NAME:gamma-glutamyltransferase 1#ABBREVIATION:Ggt1.3-e# NAME:gamma-glutamyltransferase 2#ABBREVIATION:Ggt2.1-e# citations: AUTHORS:Hultberg B, Hultberg M#TITLE:High glutathione turnover in human cell lines revealed by acivicin inhibition of gamma-glutamyltranspeptidase and the effects of thiol-reactive metals during acivicin inhibition#JOURNAL:Clin Chim Acta#YEAR:2004#PUBMED:15469854# GENE ASSOCIATION: (2679.1) or (2678.1) or (2678.3) or (2678.2)
Abbreviation: R_S4TASE2ly Synonyms: _5 EC Number: 3.1.6.12 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_a_deg3 + h2o <==> cs_a_deg4 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_411#TRANSCRIPT:1#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# LOCUS:_411#TRANSCRIPT:2#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# proteins: NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.1-l# NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.2-l# citations: AUTHORS:Schuchman EH, Jackson CE, Desnick RJ#TITLE:Human arylsulfatase B: MOPAC cloning, nucleotide sequence of a full-length cDNA, and regions of amino acid identity with arylsulfatases A and C#JOURNAL:Genomics#YEAR:1990#PUBMED:1968043# AUTHORS:Peters C, Schmidt B, Rommerskirch W, Rupp K, Zuhlsdorf M, Vingron M, Meyer HE, Pohlmann R, von Figura K#TITLE:Phylogenetic conservation of arylsulfatases. cDNA cloning and expression of human arylsulfatase B.#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2303452# GENE ASSOCIATION: (411.2) or (411.1)
chondroitin sulfate A (GalNAc4S-GlcA), degradation product 1_C45H68N2O43S2
Abbreviation: R_2DR1PP Synonyms: _0 EC Number: 3.1.3.10 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : 2dr1p + h2o --> drib + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Brown NS, Bicknell R.#TITLE:Thymidine phosphorylase, 2-deoxy-D-ribose and angiogenesis#JOURNAL:Biochem J#YEAR:1998#PUBMED:9693094# GENE ASSOCIATION:
_2-deoxy-D-ribose 1-phosphate phosphorylase
Abbreviation: R_ENMAN1g Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : g1m8masn + h2o --> glc1man + m7masnA Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# AUTHORS:Roth J, Ziak M, Zuber C.#TITLE:The role of glucosidase II and endomannosidase in glucose trimming of asparagine-linked oligosaccharides.#JOURNAL:Biochimie#YEAR:2003#PUBMED:12770767# GENE ASSOCIATION:
Endomannosidase (glc1man-producing), Golgi apparatus
glycogen, structure 3 (glycogenin-7[1,4-Glc])_C42H71O36X
Abbreviation: R_GLDBRAN Synonyms: _1 EC Number: 3.2.1.33 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : dxtrn + h2o --> glc-D + glygn3 Confidence Level: 4 lumped reaction, combination of 4-alpha-D-glucanotransferase (2.4.1.25) and amylo-alpha-1,6-glucosidase (3.2.1.33) NCD genes: LOCUS:_178#TRANSCRIPT:1#ABBREVIATION:AGL#ECNUMBERS:2.4.1.25,,3.2.1.33# LOCUS:_178#TRANSCRIPT:6#ABBREVIATION:AGL#ECNUMBERS:2.4.1.25,,3.2.1.33# LOCUS:_178#TRANSCRIPT:5#ABBREVIATION:AGL#ECNUMBERS:2.4.1.25,,3.2.1.33# LOCUS:_178#TRANSCRIPT:4#ABBREVIATION:AGL#ECNUMBERS:2.4.1.25,,3.2.1.33# LOCUS:_178#TRANSCRIPT:3#ABBREVIATION:AGL#ECNUMBERS:2.4.1.25,,3.2.1.33# LOCUS:_178#TRANSCRIPT:2#ABBREVIATION:AGL#ECNUMBERS:2.4.1.25,,3.2.1.33# proteins: NAME:amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III) (liver, muscle)#ABBREVIATION:Agl.1# NAME:amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III) (muscle)#ABBREVIATION:Agl.2# NAME:amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III) (muscle)#ABBREVIATION:Agl.3# NAME:amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III) (muscle)#ABBREVIATION:Agl.4# NAME:amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III) (liver, muscle)#ABBREVIATION:Agl.5# NAME:amylo-1, 6-glucosidase, 4-alpha-glucanotransferase (glycogen debranching enzyme, glycogen storage disease type III) (liver, muscle)#ABBREVIATION:Agl.6# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Yang BZ, Ding JH, Enghild JJ, Bao Y, Chen YT#TITLE:Molecular cloning and nucleotide sequence of cDNA encoding human muscle glycogen debranching enzyme#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1374391# GENE ASSOCIATION: (178.4) or (178.5) or (178.1) or (178.3) or (178.2) or (178.6)
Abbreviation: R_DEDOLR_L Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dedol_L + h + nadph --> (0.1) dolichol_L + nadp Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Schenk B, Fernandez F, Waechter CJ.#TITLE:The ins(ide) and out(side) of dolichyl phosphate biosynthesis and recycling in the endoplasmic reticulum.#JOURNAL:Glycobiology#YEAR:2001#PUBMED:11425794# GENE ASSOCIATION:
Dehydrodolichol, human liver homolog_C1080H1748O10
Abbreviation: R_PCHOLP_hs Synonyms: _0 EC Number: 3.1.4.4 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : h2o + pchol_hs --> chol + h + pa_hs Confidence Level: 4 NJ genes: LOCUS:_5338#TRANSCRIPT:1#ABBREVIATION:PLD2#ECNUMBERS:3.1.4.4# proteins: NAME:phospholipase D2#ABBREVIATION:Pld2.1# citations: AUTHORS:Colley WC, Sung TC, Roll R, Jenco J, Hammond SM, Altshuller Y, Bar-Sagi D, Morris AJ, Frohman MA.#TITLE:Phospholipase D2, a distinct phospholipase D isoform with novel regulatory properties that provokes cytoskeletal reorganization.#JOURNAL:Curr Biol#YEAR:1997#PUBMED:9395408# AUTHORS:Lopez I, Arnold RS, Lambeth JD.#TITLE:Cloning and initial characterization of a human phospholipase D2 (hPLD2). ADP-ribosylation factor regulates hPLD2.#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9582313# GENE ASSOCIATION: (5338.1)
Abbreviation: R_NTD3 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : dcmp + h2o --> dcyt + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_30833#TRANSCRIPT:1#ABBREVIATION:NT5C#ECNUMBERS:3.1.3.5# LOCUS:_93034#TRANSCRIPT:1#ABBREVIATION:NT5C1B#ECNUMBERS:3.1.3.5# LOCUS:_93034#TRANSCRIPT:2#ABBREVIATION:NT5C1B#ECNUMBERS:3.1.3.5# proteins: NAME:_5', 3'-nucleotidase, cytosolic #ABBREVIATION:Nt5C.1# NAME:_5'-nucleotidase, cytosolic IB #ABBREVIATION:Nt5c1B.1# NAME:_5'-nucleotidase, cytosolic IB #ABBREVIATION:Nt5c1B.2# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# AUTHORS:Hunsucker SA, Spychala J, Mitchell BS.#TITLE:Human cytosolic 5'-nucleotidase I: characterization and role in nucleoside analog resistance. #JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11133996# GENE ASSOCIATION: (93034.1) or (30833.1) or (93034.2)
Abbreviation: R_MTHFD2m Synonyms: _0 EC Number: 1.5.1.5 SUBSYSTEM: Folate Metabolism Equation: [m] : mlthf + nad <==> methf + nadh Confidence Level: 2 NCD genes: LOCUS:_10797#TRANSCRIPT:1#ABBREVIATION:MTHFD2#ECNUMBERS:1.5.1.15,3.5.4.9# proteins: NAME:methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 2, methenyltetrahydrofolate cyclohydrolase, mitochondrial#ABBREVIATION:Mthfd2.1-m# citations: AUTHORS:Yang XM, MacKenzie RE#TITLE:NAD-dependent methylenetetrahydrofolate dehydrogenase-methenyltetrahydrofolate cyclohydrolase is the mammalian homolog of the mitochondrial enzyme encoded by the yeast MIS1 gene#JOURNAL:Biochemistry#YEAR:1993#PUBMED:8218174# GENE ASSOCIATION: (10797.1)
methylenetetrahydrofolate dehydrogenase (NAD), mitochondrial
Abbreviation: R_EX_cca_d3_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cca_d3 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
succinate-semialdehyde dehydrogenase (NAD) reversible (mitochondrial)
Abbreviation: R_SSALxm Synonyms: _0 EC Number: 1.2.1.24 SUBSYSTEM: Glutamate metabolism Equation: [m] : h2o + nad + sucsal --> (2) h + nadh + succ Confidence Level: 4 MM genes: LOCUS:_7915#TRANSCRIPT:1#ABBREVIATION:ALDH5A1#ECNUMBERS:1.2.1.24# LOCUS:_7915#TRANSCRIPT:2#ABBREVIATION:ALDH5A1# proteins: NAME:aldehyde dehydrogenase 5 family, member A1 (succinate-semialdehyde dehydrogenase), mitochondrial#ABBREVIATION:Aldh5a1.1-m# NAME:aldehyde dehydrogenase 5 family, member A1 (succinate-semialdehyde dehydrogenase), mitochondrial#ABBREVIATION:Aldh5a1.2-m# citations: AUTHORS:Chambliss KL, Caudle DL, Hinson DD, Moomaw CR, Slaughter CA, Jakobs C, Gibson KM.#TITLE:Molecular cloning of the mature NAD(+)-dependent succinic semialdehyde dehydrogenase from rat and human. cDNA isolation, evolutionary homology, and tissue expression.#YEAR:1995#PUBMED:7814412# AUTHORS:Chambliss KL, Hinson DD, Trettel F, Malaspina P, Novelletto A, Jakobs C, Gibson KM.#TITLE:Two exon-skipping mutations as the molecular basis of succinic semialdehyde dehydrogenase deficiency (4-hydroxybutyric aciduria).#YEAR:1998#PUBMED:9683595# GENE ASSOCIATION: (7915.1) or (7915.2)
Abbreviation: R_MALSO4tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: mal-L[c] + so4[m] <==> mal-L[m] + so4[c] Confidence Level: 4 MM genes: LOCUS:_1468#TRANSCRIPT:1#ABBREVIATION:SLC25A10# proteins: NAME:Solute carrier family 25 (dicarboxylate transporter) member 10, mitochondrial #ABBREVIATION:Slc25a10.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# AUTHORS:Crompton M, Palmieri F, Capano M, Quagliariello E.#TITLE:The transport of sulphate and sulphite in rat liver mitochondria. #YEAR:1974#PUBMED:4441366# GENE ASSOCIATION: (1468.1)
Abbreviation: R_CYTK9n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : cmp + dctp <==> cdp + dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_FAOXC160 Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (7) coa + (7) fad + (7) h2o + (7) nad + pmtcoa --> (8) accoa + (7) fadh2 + (7) h + (7) nadh Confidence Level: 4 TV Other enzymes: 1.3.3.6, 4.2.1.74, 4.2.1.17, 1.1.1.35, 1.1.1.211 genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Kelly DP, Kim JJ, Billadello JJ, Hainline BE, Chu TW, Strauss AW#TITLE:Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3035565# AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_C161CPT22 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + hdd2crn --> crn + hdd2coa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_NACHEXA14ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg21 --> acgam + h + ksi_deg23 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_EX_perillyl_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : perillyl <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FPGS4 Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [c] : (4) atp + dhf + (4) glu-L --> 5dhf + (4) adp + (4) h + (4) pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_CMPSASn Synonyms: _0 EC Number: 2.7.7.43 SUBSYSTEM: Aminosugar Metabolism Equation: [n] : acnam + ctp --> cmpacna + ppi Confidence Level: 2 NCD genes: LOCUS:_55907#TRANSCRIPT:1#ABBREVIATION:CMAS#ECNUMBERS:2.7.7.43# proteins: NAME:cytidine monophosphate N-acetylneuraminic acid synthetase, nucleus#ABBREVIATION:Cmas.1-n# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Munster AK, Eckhardt M, Potvin B, Muhlenhoff M, Stanley P, Gerardy-Schahn R#TITLE:Mammalian cytidine 5'-monophosphate N-acetylneuraminic acid synthetase: a nuclear protein with evolutionarily conserved structural motifs#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9689047# GENE ASSOCIATION: (55907.1)
aldehyde dehydrogenase (phenylacetaldehyde, NADP)
Abbreviation: R_ALDD19x_LPAREN_P_RPAREN_ Synonyms: _0 EC Number: 1.2.1.39 SUBSYSTEM: Phenylalanine metabolism Equation: [c] : h2o + nadp + pacald --> (2) h + nadph + pac Confidence Level: 2 JLR SAB genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: none GENE ASSOCIATION: (222.1) or (218.1) or (221.1) or (220.1)
Abbreviation: R_GGH_DASH_5DHFl Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [l] : 5dhf + (4) h2o --> dhf + (4) glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) - last step from 5-glutamyl to monoglutamyl is in one step IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-l# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (5DHF), lysosomal
Abbreviation: R_UMPK7n Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : dgtp + ump <==> dgdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_25HVITD3tin Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _25hvitd3[e] --> 25hvitd3[c] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_25-hydroxyvitamin D3 transport in cytoplasm
Abbreviation: R_ALDD21 Synonyms: _0 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + nad + pristanal --> (2) h + nadh + prist Confidence Level: 2 NJ genes: LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# proteins: NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# citations: AUTHORS:Verhoeven NM, Jakobs C.#TITLE:Human metabolism of phytanic acid and pristanic acid.#JOURNAL:Prog Lipid Res#YEAR:2001#PUBMED:11591435# AUTHORS:De Laurenzi V, Rogers GR, Hamrock DJ, Marekov LN, Steinert PM, Compton JG, Markova N, Rizzo WB.#TITLE:Sjogren-Larsson syndrome is caused by mutations in the fatty aldehyde dehydrogenase gene.#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8528251# GENE ASSOCIATION: (224.1)
keratan sulfate I, degradation product 27_C104H169N7O88S4
Abbreviation: R_SOAT11r Synonyms: _9 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : R1coa_hs + chsterol --> coa + xolest_hs Confidence Level: 4 SOAT11 and SOAT12 are templates, customized rxns can be formed particular ester moieties. NJ genes: LOCUS:_6646#TRANSCRIPT:1#ABBREVIATION:SOAT1#ECNUMBERS:2.3.1.26# proteins: NAME:sterol O-acetyltransferase (acyl-Coenzyme A: cholesterol acyltransferase) 1#ABBREVIATION:Soat1.1# citations: AUTHORS:Guo ZY, Chang CC, Lu X, Chen J, Li BL, Chang TY.#TITLE:The disulfide linkage and the free sulfhydryl accessibility of acyl-coenzyme A:cholesterol acyltransferase 1 as studied by using mPEG5000-maleimide.#JOURNAL:Biochemistry#YEAR:2005#PUBMED:15850387# AUTHORS:Chang CC, Huh HY, Cadigan KM, Chang TY.#TITLE:Molecular cloning and functional expression of human acyl-coenzyme A:cholesterol acyltransferase cDNA in mutant Chinese hamster ovary cells.#JOURNAL:J Biol Chem.#YEAR:1993#PUBMED:8407899# GENE ASSOCIATION: (6646.1)
Abbreviation: R_EX_ebastine_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ebastine <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LNLNCGt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lnlncg[e] <==> lnlncg[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_FTCD Synonyms: _0 EC Number: 4.3.1.4 SUBSYSTEM: Folate Metabolism Equation: [c] : 5forthf + (2) h --> methf + nh4 Confidence Level: 4 IT genes: LOCUS:_10841#TRANSCRIPT:1#ABBREVIATION:FTCD#ECNUMBERS:2.1.2.5,4.3.1.4# LOCUS:_10841#TRANSCRIPT:2#ABBREVIATION:FTCD# proteins: NAME:formiminotransferase cyclodeaminase#ABBREVIATION:Ftcd.1# NAME:formiminotransferase cyclodeaminase#ABBREVIATION:Ftcd.2# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (10841.1) or (10841.2)
Abbreviation: R_FACOAL246_1 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + tethex3 <==> amp + ppi + tethex3coa Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_SRTNtu Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: srtn[e] <==> srtn[c] Confidence Level: 4 SAB electrogenic, Na indepdendent & rev NJ genes: LOCUS:_6580#TRANSCRIPT:1#ABBREVIATION:SLC22A1# LOCUS:_6582#TRANSCRIPT:1#ABBREVIATION:SLC22A2# LOCUS:_6582#TRANSCRIPT:2#ABBREVIATION:SLC22A2# proteins: NAME:solute carrier family 22 (organic cation transporter), member 1#ABBREVIATION:Slc22a1.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.2# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# GENE ASSOCIATION: (6580.1) or (6582.2) or (6582.1)
nicotinamide-nucleotide adenylyltransferase
Abbreviation: R_NMNATr Synonyms: _0 EC Number: 2.7.7.1 SUBSYSTEM: NAD Metabolism Equation: [c] : atp + h + nmn <==> nad + ppi Confidence Level: 4 genes: LOCUS:_349565#TRANSCRIPT:1#ABBREVIATION:NMNAT3# LOCUS:_23057#TRANSCRIPT:1#ABBREVIATION:NMNAT2#ECNUMBERS:2.7.7.1# LOCUS:_23057#TRANSCRIPT:2#ABBREVIATION:NMNAT2#ECNUMBERS:2.7.7.1# proteins: NAME:Nicotinamide nucleotide adenylyltransferase 2 #ABBREVIATION:Nmnat2.1# NAME:Nicotinamide nucleotide adenylyltransferase 2 #ABBREVIATION:Nmnat2.2# NAME:nicotinamide nucleotide adenylyltransferase 3 #ABBREVIATION:Nmnat3.1# citations: AUTHORS:Zhang X, Kurnasov OV, Karthikeyan S, Grishin NV, Osterman AL, Zhang H. #TITLE:Structural characterization of a human cytosolic NMN/NaMN adenylyltransferase and implication in human NAD biosynthesis.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:12574164# AUTHORS: Emanuelli M, Carnevali F, Saccucci F, Pierella F, Amici A, Raffaelli N, Magni G. #TITLE:Molecular cloning, chromosomal localization, tissue mRNA levels, bacterial expression, and enzymatic properties of human NMN adenylyltransferase.#JOURNAL:J Biol Chem.#YEAR:2001#PUBMED:11027696# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Raffaelli N, Sorci L, Amici A, Emanuelli M, Mazzola F, Magni G.#TITLE:Identification of a novel human nicotinamide mononucleotide adenylyltransferase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12359228# GENE ASSOCIATION: (349565.1) or (23057.1) or (23057.2)
Abbreviation: R_ABO8g Synonyms: _11 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucgalacglcgal14acglcgalgluside_hs + udpgal --> galfucgalacglcgal14acglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_28#TRANSCRIPT:1#ABBREVIATION:ABO# proteins: NAME:ABO blood group#ABBREVIATION:Abo.1# citations: AUTHORS:Lloyd KO.#TITLE:The chemistry and immunochemistry of blood group A, B, H, and Lewis antigens: past, present and future.#JOURNAL:Glycoconj J#YEAR:2000#PUBMED:11421346# AUTHORS:Yamamoto F.-I., Hakomori S.-I.;#TITLE:Sugar-nucleotide donor specificity of histo-blood group A and B transferases is based on amino acid substitutions#JOURNAL:J. Biol. Chem#YEAR:1990#PUBMED:2121736# AUTHORS:Patenaude S.I., Seto N.O.L., Borisova S.N., Szpacenko A., Marcus S.L., Palcic M.M., Evans S.V.;#TITLE:The structural basis for specificity in human ABO(H) blood group biosynthesis#JOURNAL:Nat. Struct. Biol#YEAR:2002#PUBMED:12198488# GENE ASSOCIATION: (28.1)
Abbreviation: R_NTD4l Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleic acid degradation Equation: [l] : cmp + h2o --> cytd + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:R.L. Pisoni#TITLE:Lysosomal Nucleic Acid and Phosphate Metabolism and Related Metabolic Reactions, Chapter 9#YEAR:1996# GENE ASSOCIATION: (53.1)
inositol-1,3,4,6-tetrakisphosphate 5-kinase, nucleus
Abbreviation: R_MI1346PKn Synonyms: _1 EC Number: 2.7.1.140 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + mi1346p --> adp + h + mi13456p Confidence Level: 4 NCD genes: LOCUS:_253430#TRANSCRIPT:1#ABBREVIATION:IPMK# proteins: NAME:inositol polyphosphate multikinase, nucleus#ABBREVIATION:Ipmk.1-n# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:Chang SC, Miller AL, Feng Y, Wente SR, Majerus PW. #TITLE:The human homolog of the rat inositol phosphate multikinase is an inositol 1,3,4,6-tetrakisphosphate 5-kinase#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12223481# AUTHORS:Nalaskowski MM, Deschermeier C, Fanick W, Mayr GW#TITLE:The human homologue of yeast ArgRIII protein is an inositol phosphate multikinase with predominantly nuclear localization#JOURNAL:Biochem J#YEAR:2002#PUBMED:12027805# GENE ASSOCIATION: (253430.1)
Abbreviation: R_FUT15g Synonyms: _4 EC Number: 2.4.1.69 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacgalfuc12gal14acglcgalgluside_hs + gdpfuc --> fucgalacgalfuc12gal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2523#TRANSCRIPT:1#ABBREVIATION:FUT1#ECNUMBERS:2.4.1.69# proteins: NAME:fucosyltransferase 1#ABBREVIATION:Fut1.1# citations: AUTHORS:Larsen RD, Ernst LK, Nair RP, Lowe JB.#TITLE:Molecular cloning, sequence, and expression of a human GDP-L-fucose:beta-D-galactoside 2-alpha-L-fucosyltransferase cDNA that can form the H blood group antigen.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990#PUBMED:2118655# GENE ASSOCIATION: (2523.1)
Abbreviation: R_GD1B2tg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gd1b2_hs[c] <==> gd1b2_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_HSD17B3r Synonyms: _1 EC Number: 1.1.1.62 SUBSYSTEM: Steroid Metabolism Equation: [r] : andrstndn + h + nadph --> nadp + tststerone Confidence Level: 4 microsomal NADP as cofactor HSD type 3 recheck/confirm comparmentalization - type 1: cytosol, types 2,3: microsomes NJ NJ genes: LOCUS:_3293#TRANSCRIPT:1#ABBREVIATION:HSD17B3# proteins: NAME:hydroxysteroid (17-beta) dehydrogenase 3#ABBREVIATION:Hsd17b3.1# citations: AUTHORS:Leenders F, Dolez V, Begue A, Moller G, Gloeckner JC, deLaunoit Y, Adamski J#TITLE:Structure of the gene for the human 17-beta-hydroxysteroid dehydrogenase type IV#JOURNAL:Mammalian Genome#YEAR:1998# AUTHORS:Adamski J, Leenders F Carstensen JF, Kaumann M, Markus MM, Husen B, Tesdorpf JG, Seedor U, deLaunoit Y, Jakob F#TITLE:Steroids, fatty acyl-CoA, and sterols are substrates of 80-kDa multifunctional protein#JOURNAL:Steroids#YEAR:1997# AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# AUTHORS:Andersson S, Moghrabi N#TITLE:Physiology and molecular genetics of 17beta-hydroxysteroid dehydrogenases#JOURNAL:Steroids#YEAR:1997# GENE ASSOCIATION: (3293.1)
phosphatidylinositol synthase (Homo sapiens)
Abbreviation: R_CDIPTr Synonyms: _3 EC Number: 2.7.8.11 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : cdpdag_hs + inost <==> cmp + h + pail_hs Confidence Level: 4 NJ - reversibility of enzyme found in lit by NCD genes: LOCUS:_10423#TRANSCRIPT:1#ABBREVIATION:CDIPT#ECNUMBERS:2.7.8.11# LOCUS:_10423#TRANSCRIPT:2#ABBREVIATION:CDIPT#ECNUMBERS:2.7.8.11# proteins: NAME:phosphatidylinositol synthase#ABBREVIATION:Cdipt.1# NAME:phosphatidylinositol synthase#ABBREVIATION:Cdipt.2# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Kent C#TITLE:Eukaryotic phospholipid biosynthesis#JOURNAL:Annu Rev Biochem#YEAR:1995# AUTHORS:Lykidis A, Jackson PD, Rock CO, Jackowski S#TITLE:The role of CDP-diacylglycerol synthetase and phosphatidylinositol synthase activity levels in the regulation of cellular phosphatidylinositol content#JOURNAL:Journal of Biological Chemistry#YEAR:1997# AUTHORS:Hajra AK#TITLE:Glycerolipid biosynthesis in peroxisomes#JOURNAL:Prog Lipid Res#YEAR:1995# AUTHORS:Nagan N, Zoeller RA#TITLE:Plasmalogens: biosynthesis and functions#JOURNAL:Progress in Lipid Research#YEAR:2001# AUTHORS:Holub BJ#TITLE:Metabolism and function of myo-inositol and inositol phospholipids#JOURNAL:Annu Rev Nutr#YEAR:1986#PUBMED:2425833# GENE ASSOCIATION: (10423.2) or (10423.1)
Abbreviation: R_CHSTEROLt1 Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: chsterol[l] <==> chsterol[m] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: LOCUS:_10948#TRANSCRIPT:1#ABBREVIATION:STARD3# proteins: NAME:START domain containing 3#ABBREVIATION:Stard3.1# citations: AUTHORS:Zhang M, Liu P, Dwyer NK, Christenson LK, Fujimoto T, Martinez F, Comly M, Hanover JA, Blanchette-Mackie EJ, Strauss JF 3rd.#TITLE:MLN64 mediates mobilization of lysosomal cholesterol to steroidogenic mitochondria.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12070139# AUTHORS:Jefcoate C.#TITLE:High-flux mitochondrial cholesterol trafficking, a specialized function of the adrenal cortex.#JOURNAL:J Clin Invest#YEAR:2002#PUBMED:12370263# AUTHORS:Strauss JF 3rd, Kishida T, Christenson LK, Fujimoto T, Hiroi H.#TITLE:START domain proteins and the intracellular trafficking of cholesterol in steroidogenic cells.#JOURNAL:Mol Cell Endocrinol#YEAR:2003#PUBMED:12770731# GENE ASSOCIATION: (10948.1)
Abbreviation: R_COAtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: coa[c] <==> coa[n] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_co_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : co <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(trimannosyl),(phosphoethanolaminyl)-mannosyl-glucosaminyl-acylphosphatidylinositol (M4C)_C57H104N2O37P2FULLRCO2FULLR2CO2
Abbreviation: R_M4CET3er Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : m3emgacpail_hs + pe_hs --> dag_hs + mem2emgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_5281#TRANSCRIPT:1#ABBREVIATION:PIGF# LOCUS:_84720#TRANSCRIPT:1#ABBREVIATION:PIGO# LOCUS:_84720#TRANSCRIPT:2#ABBREVIATION:PIGO# LOCUS:_5281#TRANSCRIPT:2#ABBREVIATION:PIGF# proteins: NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.1-r# NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.2-r# NAME:phosphatidylinositol glycan, class O, endoplasmic reticulum#ABBREVIATION:Pigo.1-r# NAME:phosphatidylinositol glycan, class O, endoplasmic reticulum#ABBREVIATION:Pigo.2-r# citations: AUTHORS:Shishioh N, Hong Y, Ohishi K, Ashida H, Maeda Y, Kinoshita T#TITLE:GPI7 is the second partner of PIG-F and involved in modification of glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15632136# AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (84720.2) and (5281.2) or (5281.1) and (84720.1) or (84720.1) and (5281.2) or (84720.2) and (5281.1)
M4C phosphoethanolaminyl transferase, endoplasmic reticulum
Abbreviation: R_ABTti Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: abt[c] --> abt[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Huck JH, Roos B, Jakobs C, van der Knaap MS, Verhoeven NM#TITLE:Evaluation of pentitol metabolism in mammalian tissues provides new insight into disorders of human sugar metabolism#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15234337# GENE ASSOCIATION:
L-arabinitol transport via passive diffusion
Abbreviation: R_ARTFR205 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: dlnlcgcoa[c] + (2) fadh2[m] + h[m] + nadph[m] --> (1.25) R2coa_hs[c] + (2) fad[m] + nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PPAer Synonyms: _0 EC Number: 3.6.1.1 SUBSYSTEM: Miscellaneous Equation: [r] : h2o + ppi --> h + (2) pi Confidence Level: 2 IF genes: LOCUS:_2538#TRANSCRIPT:1#ABBREVIATION:G6PC#ECNUMBERS:3.1.3.9# LOCUS:_92579#TRANSCRIPT:1#ABBREVIATION:G6PC3# LOCUS:_57818#TRANSCRIPT:1#ABBREVIATION:G6PC2# proteins: NAME:glucose-6-phosphatase, catalytic (glycogen storage disease type I, von Gierke disease), endoplasmic reticulum (liver, kidney, intestine)#ABBREVIATION:G6pc.1-r# NAME:glucose-6-phosphatase, catalytic, 2, endoplasmic reticulum (pancreatic B islets)#ABBREVIATION:G6pc2.1-r# NAME:glucose 6 phosphatase, catalytic, 3, endoplasmic reticulum (ubiquitous)#ABBREVIATION:G6pc3.1-r# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (2538.1) or (92579.1) or (57818.1)
inorganic diphosphatase, endoplasmic reticulum
Abbreviation: R_FAS100COA Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : (3) h + malcoa + (2) nadph + occoa --> co2 + coa + dcacoa + h2o + (2) nadp Confidence Level: 4 NCD genes: LOCUS:_2194#TRANSCRIPT:1#ABBREVIATION:FASN#ECNUMBERS:2.3.1.85# proteins: NAME:fatty acid synthase#ABBREVIATION:Fasn.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Chakravarty B, Gu Z, Chirala SS, Wakil SJ, Quiocho FA.#TITLE:Human fatty acid synthase: structure and substrate selectivity of the thioesterase domain.#YEAR:2004#PUBMED:15507492# AUTHORS:Brink J, Ludtke SJ, Yang CY, Gu ZW, Wakil SJ, Chiu W.#TITLE:Quaternary structure of human fatty acid synthase by electron cryomicroscopy.#JOURNAL:Proc Natl Acad Sci#YEAR:2002#PUBMED:11756679# AUTHORS:Wakil SJ.#TITLE:Fatty acid synthase, a proficient multifunctional enzyme.#JOURNAL:Biochemistry#YEAR:1989#PUBMED:2669958# GENE ASSOCIATION: (2194.1)
Abbreviation: R_EX_tststeroneglc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tststeroneglc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_XYLTt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: xylt[e] <==> xylt[c] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Wang YM, van Eys J#TITLE:Nutritional significance of fructose and sugar alcohols#JOURNAL:Annu Rev Nutr.#YEAR:1981#PUBMED:6821187# GENE ASSOCIATION:
Abbreviation: R_GLYCLTDym Synonyms: _1 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [m] : glx + h + nadph --> glyclt + nadp Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP.#TITLE:Glycolate and glyoxylate metabolism in HepG2 cells.#YEAR:2004#PUBMED:15240345# AUTHORS:Taylor SW, Fahy E, Zhang B, Glenn GM, Warnock DE, Wiley S, Murphy AN, Gaucher SP, Capaldi RA, Gibson BW, Ghosh SS#TITLE:Characterization of the human heart mitochondrial proteome#JOURNAL:Nat Biotechnol#YEAR:2003#PUBMED:1252411# AUTHORS:Knight J, Holmes RP#TITLE:Mitochondrial hydroxyproline metabolism: implications for primary hyperoxaluria#JOURNAL:Am J Nephrol#YEAR:2005#PUBMED:15849464# GENE ASSOCIATION:
Glycolate dehydrogenase (NADP), mitochondrial
heparan sulfate, degradation product 19_C43H67N2O42S2
heparan sulfate, degradation product 20_C45H68N2O43S2
Abbreviation: R_HSAT4ly Synonyms: _1 EC Number: 2.3.1.78 SUBSYSTEM: Heparan sulfate degradation Equation: accoa[c] + hs_deg19[l] --> coa[c] + h[l] + hs_deg20[l] Confidence Level: 4 The proton is located in the lysosome since it comes from oxidation of the NH3+ group in glucosamine. This reaction assumes that the other proton liberated from glucosamine reduces coa in the cytosol. NCD genes: none proteins: none citations: AUTHORS:Ausseil J, Loredo-Osti JC, Verner A, Darmond-Zwaig C, Maire I, Poorthuis B, van Diggelen OP, Hudson TJ, Fujiwara TM, Morgan K, Pshezhetsky AV#TITLE:Localisation of a gene for mucopolysaccharidosis IIIC to the pericentromeric region of chromosome 8#JOURNAL:J Med Genet#YEAR:2004#PUBMED:15591281# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
(Gal)3 (GalNAc)1 (Glc)1 (GlcNAc)2 (LFuc)1 (Cer)1_C72H125N4O41FULLRCO
Abbreviation: R_ABO6g Synonyms: _10 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucgalacglcgal14acglcgalgluside_hs + udpacgal --> acgalfucgalacglcgal14acglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_28#TRANSCRIPT:1#ABBREVIATION:ABO# proteins: NAME:ABO blood group#ABBREVIATION:Abo.1# citations: AUTHORS:Lloyd KO.#TITLE:The chemistry and immunochemistry of blood group A, B, H, and Lewis antigens: past, present and future.#JOURNAL:Glycoconj J#YEAR:2000#PUBMED:11421346# AUTHORS:Yamamoto F.-I., Hakomori S.-I.;#TITLE:Sugar-nucleotide donor specificity of histo-blood group A and B transferases is based on amino acid substitutions#JOURNAL:J. Biol. Chem#YEAR:1990#PUBMED:2121736# AUTHORS:Patenaude S.I., Seto N.O.L., Borisova S.N., Szpacenko A., Marcus S.L., Palcic M.M., Evans S.V.;#TITLE:The structural basis for specificity in human ABO(H) blood group biosynthesis#JOURNAL:Nat. Struct. Biol#YEAR:2002#PUBMED:12198488# GENE ASSOCIATION: (28.1)
Abbreviation: R_FUCASEly Synonyms: _1 EC Number: 3.2.1.51 SUBSYSTEM: N-Glycan Degradation Equation: [l] : h2o + s2l2fn2m2masn --> fuc-L + s2l2n2m2masn Confidence Level: 0 NCD genes: LOCUS:_2517#TRANSCRIPT:1#ABBREVIATION:FUCA1#ECNUMBERS:3.2.1.51# proteins: NAME:alpha-fucosidase 1, lysosomal (tissue)#ABBREVIATION:Fuca1.1-l# citations: none GENE ASSOCIATION: (2517.1)
Abbreviation: R_LNSTLSr Synonyms: _3 EC Number: 5.4.99.7 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : Ssq23epx --> lanost Confidence Level: 2 NCD ER version - NJ genes: LOCUS:_4047#TRANSCRIPT:1#ABBREVIATION:LSS#ECNUMBERS:5.4.99.7# proteins: NAME:lanosterol synthase (2,3-oxidosqualene-lanosterol cyclase)#ABBREVIATION:Lss.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (4047.1)
Abbreviation: R_CO2tg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: co2[c] <==> co2[g] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UMPK3n Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : ump + utp <==> (2) udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_G1M6MASNB1terg Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: g1m6masnB1[r] --> g1m6masnB1[g] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# GENE ASSOCIATION:
g1m6masnB1 endoplasmic reticulum to Golgi transport
Abbreviation: R_CRTSTRNtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: crtstrn[c] <==> crtstrn[r] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
L-cysteine/L-threonine Na-dependent exchange (Thr-L in)
Abbreviation: R_THRCYSNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[c] + na1[e] + thr-L[e] --> cys-L[e] + na1[c] + thr-L[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
Abbreviation: R_MAN1_6B1er Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : g1m7masnC + h2o --> g1m6masnB1 + man Confidence Level: 0 NCD genes: LOCUS:_11253#TRANSCRIPT:1#ABBREVIATION:MAN1B1#ECNUMBERS:3.2.1.113# proteins: NAME:mannosidase, alpha, class 1B, member 1, endoplasmic reticulum (ubiquitous)#ABBREVIATION:Man1b1.1-er# citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# AUTHORS:Roth J, Zuber C, Guhl B, Fan JY, Ziak M.#TITLE:The importance of trimming reactions on asparagine-linked oligosaccharides for protein quality control.#JOURNAL:Histochem Cell Biol#YEAR:2002#PUBMED:11935292# AUTHORS:Gonzalez DS, Karaveg K, Vandersall-Nairn AS, Lal A, Moremen KW.#TITLE:Identification, expression, and characterization of a cDNA encoding human endoplasmic reticulum mannosidase I, the enzyme that catalyzes the first mannose trimming step in mammalian Asn-linked oligosaccharide biosynthesis.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10409699# GENE ASSOCIATION: (11253.1)
mannosidase I, endoplasmic reticulum (g1m6masnB1-producing)
Abbreviation: R_UMPKm Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [m] : atp + ump <==> adp + udp Confidence Level: 1 IT genes: none proteins: none citations: none GENE ASSOCIATION:
UDP-GlcNAc:N-acetyl-D-glucosaminyl diphosphodolichol N-acetyl-D-glucosaminyltransferase (uterus)
N-Acetyl-D-glucosaminyldiphosphodolichol, human uterine homolog_C1105H1810N10O120P20
Abbreviation: R_GLCNACT_U Synonyms: _1 EC Number: 2.4.1.141 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) naglc2p_U + uacgam --> (0.1) chito2pdol_U + h + udp Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
aldehyde dehydrogenase (acetaldehyde, NAD)
Abbreviation: R_ALDD2x Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : acald + h2o + nad --> ac + (2) h + nadh Confidence Level: 0 Only acetaldehyde considered for now. See also 1.2.1.4. Wide specificity, including oxidation of D-glucuronolactone to D-glucarate. Formerly EC 1.1.1.70 genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Sladek NE#TITLE:Human aldehyde dehydrogenases: potential pathological, pharmacological, and toxicological impact#JOURNAL:J Biochem Mol Toxicol#YEAR:2003#PUBMED:12616643# GENE ASSOCIATION: (218.1) or (501.1) or (8854.2) or (224.1) or (222.1) or (8854.1) or (221.1) or (216.1) or (8854.3) or (223.1) or (220.1)
keratan sulfate I, degradation product 34_C76H126N5O59S1
Abbreviation: R_S6TASE20ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg33 <==> h + ksi_deg34 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
peroxisomal lumped long chain fatty acid oxidation
Abbreviation: R_PEROXx Synonyms: _0 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : (3) coa + (3) h2o + (3) nad + (2) o2 + pristcoa --> accoa + dmnoncoa + (3) h + (2) h2o2 + (3) nadh + (2) ppcoa Confidence Level: 4 lumped peroxisomal alpha and beta oxidation pristanoyl-CoA -> 4,8 diemthylnonanoyl-CoA (result of compound not known) stoichiometry for O2 is questionable at present no fad in peroxisome - 1st step of beta ox is acyl-co oxidase (not reductase) NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_8310#TRANSCRIPT:1#ABBREVIATION:ACOX3#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal alpha and beta oxidation grouped#ABBREVIATION:Perox.1-x# citations: AUTHORS:Hoefler G, Forstner M, McGuinness MC, Hulla W, Hiden M, Krisper P, Kenner L, Ried T, Lengauer C, Zechner R, et al.#TITLE:DNA cloning of the human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase bifunctional enzyme and localization to chromosome 3q26.3-3q28: a free left Alu Arm is inserted in the 3' noncoding region.#JOURNAL:Genomics#YEAR:1994#PUBMED:8188243# AUTHORS:Verhoeven NM, Jakobs C.#TITLE:Human metabolism of phytanic acid and pristanic acid.#JOURNAL:Prog Lipid Res#YEAR:2001#PUBMED:11591435# AUTHORS:Mukherji M, Schofield CJ, Wierzbicki AS, Jansen GA, Wanders RJ, Lloyd MD.#TITLE:The chemical biology of branched-chain lipid metabolism.#JOURNAL:Prog Lipid Res.#YEAR:2003#PUBMED:12814641# AUTHORS:Zha S, Ferdinandusse S, Hicks JL, Denis S, Dunn TA, Wanders RJ, Luo J, De Marzo AM, Isaacs WB.#TITLE:Peroxisomal branched chain fatty acid beta-oxidation pathway is upregulated in prostate cancer.#JOURNAL:Prostate#YEAR:2005#PUBMED:15599942# AUTHORS:Bout A, Franse MM, Collins J, Blonden L, Tager JM, Benne R.#TITLE:Characterization of the gene encoding human peroxisomal 3-oxoacyl-CoA thiolase (ACAA). No large DNA rearrangement in a thiolase-deficient patient.#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1679347# AUTHORS:Adamski J, Normand T, Leenders F, Monte D, Begue A, Stehelin D, Jungblut PW, de Launoit Y.#TITLE:Molecular cloning of a novel widely expressed human 80 kDa 17 beta-hydroxysteroid dehydrogenase IV.#JOURNAL:Biochem J#YEAR:1995#PUBMED:7487879# GENE ASSOCIATION: (3295.1 and 8310.1 and 30.1 and 1962.1)
Abbreviation: R_BTND1n Synonyms: _0 EC Number: 3.5.1.12 SUBSYSTEM: Biotin Metabolism Equation: [n] : biocyt + h2o --> btn + lys-L Confidence Level: 4 IT genes: LOCUS:_686#TRANSCRIPT:1#ABBREVIATION:BTD#ECNUMBERS:3.5.1.12# proteins: NAME:biotinidase #ABBREVIATION:Btd.4-n# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Pispa J.#TITLE:Animal biotinidase.#JOURNAL:Ann Med Exp Biol Fenn. #YEAR:1965#PUBMED:5867120# AUTHORS:Zempleni J., Mock D.M.#TITLE:Biotin homeostasis during the cell cycle #JOURNAL:Nutrition Research Reviews#YEAR:2001# AUTHORS:Hymes J, Fleischhauer K, Wolf B.#TITLE:Biotinylation of biotinidase following incubation with biocytin.#JOURNAL:Clin Chim Acta#YEAR:1995#PUBMED:7758201# AUTHORS:Hymes J, Fleischhauer K, Wolf B.#TITLE:Biotinylation of histones by human serum biotinidase: assessment of biotinyl-transferase activity in sera from normal individuals and children with biotinidase deficiency.#JOURNAL:Biochem Mol Med.#YEAR:1995#PUBMED:8593541# AUTHORS:Zempleni J.#TITLE:Uptake, localization, and noncarboxylase roles of biotin.#JOURNAL:Annu Rev Nutr#YEAR:2005#PUBMED:16011464# GENE ASSOCIATION: (686.1)
Abbreviation: R_UDPGNP Synonyms: _0 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : h2o + udpglcur --> glcur + h + udp Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Banhegyi G, Braun L, Csala M, Puskas F, Mandl J#TITLE:Ascorbate metabolism and its regulation in animals#JOURNAL:Free Radic Biol Med#YEAR:1997#PUBMED:9296457# GENE ASSOCIATION:
UDPglucuronate uridine-diphosphohydrolase
Abbreviation: R_HDD2COAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: hdd2coa[c] <==> hdd2coa[x] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DHCR72r Synonyms: _3 EC Number: 1.3.1.21 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : 7dhchsterol + h + nadph --> chsterol + nadp Confidence Level: 4 lanosterol -> cholesterol pathway see paper refs Moebius et al PNAS 95:1899-1902 - check/re-check lit for more specific details (NADPH known to be cofactor - also NADH?, possibly rev?) NJ genes: LOCUS:_1717#TRANSCRIPT:1#ABBREVIATION:DHCR7#ECNUMBERS:1.3.1.21# proteins: NAME:_7-dehydrocholesterol reductase#ABBREVIATION:Dhcr7.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# AUTHORS:Moebius FF, Fitzky BU, Lee, JN, Paik YK, Glossmann H#TITLE:Molecular cloning and expression of the human delta-7-sterol reductase#JOURNAL:PNAS#YEAR:1998# AUTHORS:Correa-Cerro LS, Porter FD.#TITLE:_3beta-hydroxysterol Delta7-reductase and the Smith-Lemli-Opitz syndrome.#JOURNAL:Mol Genet Metab#YEAR:2005#PUBMED:15670717# GENE ASSOCIATION: (1717.1)
Abbreviation: R_DRBK Synonyms: _0 EC Number: 2.7.1.15 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : atp + drib --> 2dr5p + adp + h Confidence Level: 2 NCD genes: LOCUS:_64080#TRANSCRIPT:1#ABBREVIATION:RBKS#ECNUMBERS:2.7.1.15# proteins: NAME:ribokinase#ABBREVIATION:Rbks.1# citations: AUTHORS:Brown NS, Bicknell R.#TITLE:Thymidine phosphorylase, 2-deoxy-D-ribose and angiogenesis#JOURNAL:Biochem J#YEAR:1998#PUBMED:9693094# GENE ASSOCIATION: (64080.1)
Abbreviation: R_FA141ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + tdeACP <==> ACP + h + ttdcea Confidence Level: 4 NCD genes: LOCUS:_55301#TRANSCRIPT:1#ABBREVIATION:THEDC1# proteins: NAME:s-acyl fatty acid synthase thioesterase#ABBREVIATION:Thedc1.1# citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION: (55301.1)
N-Acetyl-L-glutamate 5-semialdehyde_C7H10NO4
Abbreviation: R_ASPDt6 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asp-D[e] + h[e] + k[c] + (3) na1[e] --> asp-D[c] + h[c] + k[e] + (3) na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2003 genes: LOCUS:_6511#TRANSCRIPT:1#ABBREVIATION:SLC1A6# LOCUS:_6507#TRANSCRIPT:1#ABBREVIATION:SLC1A3# LOCUS:_6506#TRANSCRIPT:1#ABBREVIATION:SLC1A2# LOCUS:_6505#TRANSCRIPT:1#ABBREVIATION:SLC1A1# LOCUS:_6512#TRANSCRIPT:1#ABBREVIATION:SLC1A7# proteins: NAME:solute carrier family 1 (neuronal/epithelial high affinity glutamate transporter, system Xag), member 1#ABBREVIATION:Slc1a1.1# NAME:solute carrier family 1 (glial high affinity glutamate transporter), member 2#ABBREVIATION:Slc1a2.1# NAME:solute carrier family 1 (glial high affinity glutamate transporter), member 3#ABBREVIATION:Slc1a3.1# NAME:solute carrier family 1 (high affinity aspartate/glutamate transporter), member 6#ABBREVIATION:Slc1a6.1# NAME:solute carrier family 1 (glutamate transporter), member 7#ABBREVIATION:Slc1a7.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6511.1) or (6505.1) or (6507.1) or (6506.1) or (6512.1)
D-aspartate transport via Na, H symport and K antiport
keratan sulfate I biosynthesis, precursor 4_C101H165N7O72X
Abbreviation: R_S6T4g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre4 + paps --> h + ksi_pre5 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_11DOCRTSLtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _11docrtsl[c] <==> 11docrtsl[m] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_INStl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: ins[l] <==> ins[c] Confidence Level: 4 IT genes: LOCUS:_55315#TRANSCRIPT:1#ABBREVIATION:SLC29A3# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 3 #ABBREVIATION:Slc29a3.1-l# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Pisoni RL, Thoene JG.#TITLE:Detection and characterization of a nucleoside transport system in human fibroblast lysosomes.#JOURNAL:J Biol Chem.#YEAR:1989#PUBMED:2925670# AUTHORS:Baldwin SA, Yao SY, Hyde RJ, Ng AM, Foppolo S, Barnes K, Ritzel MW, Cass CE, Young JD.#TITLE:Functional characterization of novel human and mouse equilibrative nucleoside transporters (hENT3 and mENT3) located in intracellular membranes.#JOURNAL:J Biol Chem. #YEAR:2005#PUBMED:15701636# GENE ASSOCIATION: (55315.1)
inosine facilated transport from lysosome
Abbreviation: R_EX_5adtststerone_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 5adtststerone <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_5adtststeroneglc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 5adtststeroneglc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_5alpha-Dihydrotestosterone glucuronide exchange
Abbreviation: R_DADNt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dad-2[e] <==> dad-2[c] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION:
Abbreviation: R_FACOAL204i Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : arachd + atp + coa --> amp + arachdcoa + ppi Confidence Level: 4 TV catalyzed by acyl-CoA synthetases genes: LOCUS:_2182#TRANSCRIPT:1#ABBREVIATION:ACSL4# LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# LOCUS:_2181#TRANSCRIPT:1#ABBREVIATION:ACSL3# LOCUS:_2182#TRANSCRIPT:2#ABBREVIATION:ACSL4# LOCUS:_2181#TRANSCRIPT:2#ABBREVIATION:ACSL3# LOCUS:_22305#TRANSCRIPT:1#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# LOCUS:_22305#TRANSCRIPT:2#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.1# NAME:acyl-CoA synthetase long-chain family member 4#ABBREVIATION:Acsl4.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.2# NAME:acyl-CoA synthetase long-chain family member 4#ABBREVIATION:Acsl4.2# NAME:acyl-CoA synthetase long-chain family member 6#ABBREVIATION:Acsl6.1# NAME:acyl-CoA synthetase long-chain family member 6 #ABBREVIATION:Acsl6.2# citations: none GENE ASSOCIATION: (22305.1) or (2181.2) or (2181.1) or (2180.1) or (22305.2) or (2182.1) or (2182.2)
Abbreviation: R_EX_malt_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : malt <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_Rtotaltp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: Rtotal[c] <==> Rtotal[x] Confidence Level: 1 unknown mechanism for transport - carnitine transporter for malonyl CoA found in peroxisome, however transport of other, longer chain FA not yet discovered (PMID: 15590999) genes: none proteins: none citations: none GENE ASSOCIATION:
(alpha-D-Glucosyl)2-(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1845H3040N20O720P20
S-Adenosyl-L-methionine:N-acetylserotonin O-methyltransferase
Abbreviation: R_ACSOMT Synonyms: _0 EC Number: 2.1.1.4 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 5hoxindoa + amet --> 5moxact + ahcys + h Confidence Level: 1 SAB genes: LOCUS:_438#TRANSCRIPT:1#ABBREVIATION:ASMT#ECNUMBERS:2.1.1.4# proteins: NAME:acetylserotonin O-methyltransferase #ABBREVIATION:Asmt.1# citations: none GENE ASSOCIATION: (438.1)
Abbreviation: R_RNDR4 Synonyms: _0 EC Number: 1.17.4.1 SUBSYSTEM: Nucleotides Equation: [c] : trdrd + udp --> dudp + h2o + trdox Confidence Level: 2 genes: LOCUS:_6241#TRANSCRIPT:1#ABBREVIATION:RRM2#ECNUMBERS:1.17.4.1# LOCUS:_6240#TRANSCRIPT:1#ABBREVIATION:RRM1#ECNUMBERS:1.7.14.1# proteins: NAME:ribonucleotide reductase #ABBREVIATION:Rrm12.1# citations: AUTHORS:Shao J, Zhou B, Zhu L, Qiu W, Yuan YC, Xi B, Yen Y.#TITLE: In vitro characterization of enzymatic properties and inhibition of the p53R2 subunit of human ribonucleotide reductase. #JOURNAL:Cancer Res. #YEAR:2004#PUBMED:14729598# GENE ASSOCIATION: (6240.1 and 6241.1)
ribonucleoside-diphosphate reductase (UDP)
Abbreviation: R_LALDD Synonyms: _0 EC Number: 1.1.1.79 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : lald-D + nadp <==> h + mthgxl + nadph Confidence Level: 0 NCD genes: LOCUS:_9380#TRANSCRIPT:1#ABBREVIATION:GRHPR#ECNUMBERS:1.1.1.79# proteins: NAME:glyoxylate reductase/hydroxypyruvate reductase#ABBREVIATION:Grhpr.1# citations: none GENE ASSOCIATION: (9380.1)
Abbreviation: R_PYDAMK Synonyms: _0 EC Number: 2.7.1.35 SUBSYSTEM: Vitamin B6 Metabolism Equation: [c] : atp + pydam --> adp + h + pyam5p Confidence Level: 4 NCD genes: LOCUS:_8566#TRANSCRIPT:1#ABBREVIATION:PDXK#ECNUMBERS:2.7.1.35# proteins: NAME:pyridoxal (pyridoxine, vitamin B6) kinase #ABBREVIATION:PdxK.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# GENE ASSOCIATION: (8566.1)
Abbreviation: R_EX_gly_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gly <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_P4502C93 Synonyms: _5 SUBSYSTEM: Limonene and pinene degradation Equation: [c] : h + limnen + nadph + o2 --> h2o + nadp + perillyl Confidence Level: 4 assumed to take place on outer membrane NJ genes: LOCUS:_1557#TRANSCRIPT:1#ABBREVIATION:CYP2C19#ECNUMBERS:1.14.14.1# LOCUS:_1559#TRANSCRIPT:1#ABBREVIATION:CYP2C9#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 2, subfamily C, polypeptide 19#ABBREVIATION:Cyp2c19.1# NAME:cytochrome P450, family 2, subfamily C, polypeptide 9#ABBREVIATION:Cyp2c9.1# citations: AUTHORS:Rendic S.#TITLE:Summary of information on human CYP enzymes: human P450 metabolism data.#JOURNAL:Drug Metab Rev#YEAR:2002#PUBMED:11996015# AUTHORS:Miyazawa M, Shindo M, Shimada T.#TITLE:Metabolism of (+)- and (-)-limonenes to respective carveols and perillyl alcohols by CYP2C9 and CYP2C19 in human liver microsomes.#JOURNAL:Drug Metab Dispos#YEAR:2002#PUBMED:11950794# GENE ASSOCIATION: (1559.1) or (1557.1)
Abbreviation: R_DIGALSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: digalside_hs[c] <==> digalside_hs[g] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FUCFUCGALACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucfucgalacglcgalgluside_hs[g] <==> fucfucgalacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HEX1 Synonyms: _1 EC Number: 2.7.1.1 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : atp + glc-D --> adp + g6p + h Confidence Level: 2 D-Glucose, D-mannose, D-fructose, sorbitol and D-glucosamine can act as acceptors; ITP and dATP can act as donors. The liver isoenzyme has sometimes been called glucokinase. Reaction also associated with EC 2.7.1.2 genes: LOCUS:_3098#TRANSCRIPT:1#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3101#TRANSCRIPT:1#ABBREVIATION:HK3#ECNUMBERS:2.7.1.1# LOCUS:_3099#TRANSCRIPT:1#ABBREVIATION:HK2#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:1#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# LOCUS:_80201#TRANSCRIPT:1#ABBREVIATION:FLJ22761# LOCUS:_3098#TRANSCRIPT:4#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:3#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:5#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:2#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:2#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:3#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# proteins: NAME:glucokinase (hexokinase 4) (pancreatic beta islet)#ABBREVIATION:Gck.1# NAME:glucokinase (hexokinase 4) (major liver isoform)#ABBREVIATION:Gck.2# NAME:glucokinase (hexokinase 4) (minor liver isoform)#ABBREVIATION:Gck.3# NAME:hexokinase 1 (ubiquitous)#ABBREVIATION:Hk1.1# NAME:hexokinase 1 (erythrocyte)#ABBREVIATION:Hk1.2# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.3# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.4# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.5# NAME:hexokinase 2 (sk muscle)#ABBREVIATION:Hk2.1# NAME:hexokinase 3 (liver, lung, placenta)#ABBREVIATION:Hk3.1# NAME:hypothetical protein FLJ22761#ABBREVIATION:Flj22761.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (3098.3) or (80201.1) or (2645.3) or (3098.4) or (3099.1) or (2645.2) or (3098.5) or (2645.1) or (3098.1) or (3101.1) or (3098.2)
Abbreviation: R_NACHEXA16ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg27 --> acgam + h + ksi_deg29 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_AMACRr Synonyms: _0 EC Number: 5.1.99.4 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : cholcoar --> cholcoas Confidence Level: 4 see Russell bile acid biosynthesis review uniprot NJ genes: LOCUS:_23600#TRANSCRIPT:1#ABBREVIATION:AMACR# LOCUS:_23600#TRANSCRIPT:2#ABBREVIATION:AMACR# proteins: NAME:alpha-methylacyl-CoA racemase#ABBREVIATION:Amacr.1# NAME:alpha-methylacyl-CoA racemase#ABBREVIATION:Amacr.2# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:VanVeldhoven PP, Croes K, Casteels M, Mannaerts GP#TITLE:_2-methylacyl racemase: a couple assay based on the use of pristanoyl-CoA oxidase/peroxidase and reinvestigation of its subcellular distribution in rat and human liver#JOURNAL:Biochimica et Biophysica Acta#YEAR:1997# GENE ASSOCIATION: (23600.1) or (23600.2)
Abbreviation: R_25HVITD3tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _25hvitd3[m] --> 25hvitd3[c] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_25-hydroxyvitamin D3 transport from mitochondria
Abbreviation: R_M7MASNBterg Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: m7masnB[r] --> m7masnB[g] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
m7masnB transport from endoplasmic reticulum to Golgi apparatus
Abbreviation: R_CHLtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: chol[c] <==> chol[m] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:de Ridder JJ.#TITLE:The uptake of choline by rat liver mitochondria.#YEAR:1976#PUBMED:10984# GENE ASSOCIATION:
choline transport via diffusion (cytosol to mitochondria)
Abbreviation: R_PYAM5Ptm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: pyam5p[c] <==> pyam5p[m] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Lui A, Lumeng L, Li TK.#TITLE:Metabolism of vitamin B6 in rat liver mitochondria.#JOURNAL:J Biol Chem.#YEAR:1981#PUBMED:6263901# AUTHORS:Lui A, Lumeng L, Li TK.#TITLE:Transport of pyridoxine and pyridoxal 5'-phosphate in isolated rat liver mitochondria.#JOURNAL:J Biol Chem.#YEAR:1982#PUBMED:7174673# GENE ASSOCIATION:
Pyridoxamine 5'-phosphate transport via diffusion, mitochondrial
Abbreviation: R_TETPENT6CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + tetpent6coa --> coa + tetpent6crn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_GLB1 Synonyms: _2 EC Number: 3.2.1.23 SUBSYSTEM: Sphingolipid Metabolism Equation: [l] : galgluside_hs + h2o --> gal + gluside_hs Confidence Level: 4 NJ genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
chondroitin sulfate E (GalNAc4,6diS-GlcA), degradation product 7_C23H35O27S2
Abbreviation: R_NACHEXA8ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e_deg4 + (3) h2o --> acgal + cs_e_deg7 + (2) h + (2) so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_NACHEXA12ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg15 --> acgam + h + ksi_deg17 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_ACCOAL Synonyms: _1 EC Number: 6.2.1.13 SUBSYSTEM: Propanoate Metabolism Equation: [c] : atp + coa + ppa --> adp + pi + ppcoa Confidence Level: 4 JLR- also acts on propanoate genes: LOCUS:_55902#TRANSCRIPT:1#ABBREVIATION:ACAS2# LOCUS:_55902#TRANSCRIPT:2#ABBREVIATION:ACAS2# proteins: NAME:acetly-CoA synthetase 2 (ADP forming)#ABBREVIATION:Acas2.1# NAME:acetyl-CoA synthetase 2 (ADP forming)#ABBREVIATION:Acas2.2# citations: AUTHORS:Luong A, Hannah VC, Brown MS, Goldstein JL#TITLE:Molecular characterization of human acetyl-CoA synthetase, an enzyme regulated by sterol regulatory element-binding proteins#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10843999# GENE ASSOCIATION: (55902.1) or (55902.2)
Abbreviation: R_LPS2 Synonyms: _1 EC Number: 3.1.1.3 SUBSYSTEM: Triacylglycerol Synthesis Equation: [c] : dag_hs + h2o --> Rtotal + h + mag_hs Confidence Level: 4 FULLR corresponds to acyl/R groups in texts. NJ genes: LOCUS:_4023#TRANSCRIPT:1#ABBREVIATION:LPL#ECNUMBERS:3.1.1.34# proteins: NAME:lipoprotein lipase#ABBREVIATION:Lpl.1# citations: AUTHORS:Emi M, Wilson DE, Iverius PH, Wu L, Hata A, Hegele R, Williams RR, Lalouel JM.#TITLE:Missense mutation (Gly----Glu188) of human lipoprotein lipase imparting functional deficiency.#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:1969408# GENE ASSOCIATION: (4023.1)
Abbreviation: R_NDP7ex Synonyms: _0 EC Number: 3.6.1.6 SUBSYSTEM: Nucleotides Equation: [e] : h2o + udp --> h + pi + ump Confidence Level: 4 genes: LOCUS:_124583#TRANSCRIPT:1#ABBREVIATION:CANT1# proteins: NAME:calcium activated nucleotidase 1 #ABBREVIATION:Cant1.1-e# citations: AUTHORS:Murphy DM, Ivanenkov VV, Kirley TL. #TITLE:Bacterial expression and characterization of a novel, soluble, calcium-binding, and calcium-activated human nucleotidase. #JOURNAL:Biochemistry#YEAR:2003#PUBMED:12600208# GENE ASSOCIATION: (124583.1)
nucleoside-diphosphatase (UDP), extracellular
Abbreviation: R_ARGNm Synonyms: _0 EC Number: 3.5.3.1 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : arg-L + h2o --> orn + urea Confidence Level: 4 SAB m genes: LOCUS:_384#TRANSCRIPT:1#ABBREVIATION:ARG2# proteins: NAME:arginase, type II#ABBREVIATION:Arg2.1-m# citations: AUTHORS:Gotoh T, Araki M, Mori M#TITLE:Chromosomal localization of the human arginase II gene and tissue distribution of its mRNA#JOURNAL:Biochem Biophys Res Commun#YEAR:1997#PUBMED:9144563# GENE ASSOCIATION: (384.1)
Abbreviation: R_EX_nac_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : nac <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LAPCOAl Synonyms: _0 SUBSYSTEM: CoA Catabolism Equation: [l] : coa + h2o --> dpcoa + pi Confidence Level: 2 IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# GENE ASSOCIATION: (53.1)
Abbreviation: R_CSPG_Ct Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cspg_c[g] --> cspg_c[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate C transport, golgi to extracellular
Abbreviation: R_OXAHCOtex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) hco3[c] + oxa[e] --> (2) hco3[e] + oxa[c] Confidence Level: 2 SAB genes: LOCUS:_10861#TRANSCRIPT:1#ABBREVIATION:SLC26A1# LOCUS:_1836#TRANSCRIPT:1#ABBREVIATION:SLC26A2# LOCUS:_10861#TRANSCRIPT:3#ABBREVIATION:SLC26A1# LOCUS:_10861#TRANSCRIPT:2#ABBREVIATION:SLC26A1# LOCUS:_1811#TRANSCRIPT:1#ABBREVIATION:SLC26A3# proteins: NAME:solute carrier family 26 (sulfate transporter), member 1#ABBREVIATION:Slc26a1.1# NAME:solute carrier family 26 (sulfate transporter), member 1#ABBREVIATION:Slc26a1.2# NAME:solute carrier family 26 (sulfate transporter), member 1#ABBREVIATION:Slc26a1.3# NAME:solute carrier family 26 (sulfate transporter), member 2#ABBREVIATION:Slc26a2.1# NAME:solute carrier family 26, member 3#ABBREVIATION:Slc26a3.1# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (1836.1) or (10861.1) or (10861.3) or (10861.2) or (1811.1)
oxalate transport via bicarbonate countertransport
Abbreviation: R_ATPH1e Synonyms: _5 EC Number: 3.6.1.5 SUBSYSTEM: Nucleotides Equation: [e] : atp + (2) h2o --> amp + (2) h + (2) pi Confidence Level: 4 IT genes: LOCUS:_953#TRANSCRIPT:1#ABBREVIATION:ENTPD1# LOCUS:_956#TRANSCRIPT:1#ABBREVIATION:ENTPD3# LOCUS:_955#TRANSCRIPT:1#ABBREVIATION:ENTPD6# LOCUS:_954#TRANSCRIPT:1#ABBREVIATION:ENTPD2# LOCUS:_954#TRANSCRIPT:2#ABBREVIATION:ENTPD2# LOCUS:_957#TRANSCRIPT:1#ABBREVIATION:ENTPD5# proteins: NAME:ectonucleoside triphosphate diphosphohydrolase 1 #ABBREVIATION:Entpd1.1# NAME:ectonucleoside triphosphate diphosphohydrolase 6#ABBREVIATION:Entpd6.1# NAME:ectonucleoside triphosphate diphosphohydrolase 3 #ABBREVIATION:Entpd3.1# NAME:ectonucleoside triphosphate diphosphohydrolase 2 #ABBREVIATION:Entpd2.1# NAME:ectonucleoside triphosphate diphosphohydrolase 2 #ABBREVIATION:Entpd2.2# NAME:ectonucleoside triphosphate diphosphohydrolase 5 #ABBREVIATION:Entpd5.1# citations: AUTHORS:Kaczmarek E, Koziak K, Sevigny J, Siegel JB, Anrather J, Beaudoin AR, Bach FH, Robson SC.#TITLE:Identification and characterization of CD39/vascular ATP diphosphohydrolase.#JOURNAL:J Biol Chem.#YEAR:1996#PUBMED:8955160# AUTHORS:Chadwick BP, Frischauf AM.#TITLE:The CD39-like gene family: identification of three new human members (CD39L2, CD39L3, and CD39L4), their murine homologues, and a member of the gene family from Drosophila melanogaster. #JOURNAL:Genomics#YEAR:1998#PUBMED:9676430# GENE ASSOCIATION: (954.1) or (954.2) or (953.1) or (956.1) or (955.1) or (957.1)
keratan sulfate II biosynthesis, precursor 6_C69H111N6O51SX
Abbreviation: R_S6T16g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core4_pre5 + paps --> h + ksii_core4_pre6 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_DAGKn_hs Synonyms: _0 EC Number: 2.7.1.107 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [n] : atp + dag_hs <==> adp + h + pa_hs Confidence Level: 4 NJ genes: LOCUS:_9162#TRANSCRIPT:1#ABBREVIATION:DGKI# LOCUS:_8525#TRANSCRIPT:1#ABBREVIATION:DGKZ# proteins: NAME:diacylglycerol kinase, zeta#ABBREVIATION:Dgkz.1# NAME:diacylglycerol kinase, iota#ABBREVIATION:Dgki.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Tang W, Bunting M, Zimmerman GA, McIntyre TM, Prescott SM#TITLE:Molecular cloning of a novel human diacylglycerol kinase highly selective for arachidonate-containing substrates#JOURNAL:Journal of Biological Chemistry#YEAR:1996# GENE ASSOCIATION: (8525.1) or (9162.1)
Abbreviation: R_EX_ser_DASH_D_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ser-D <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLXO2p Synonyms: _0 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [x] : glx + h2o + o2 --> h + h2o2 + oxa Confidence Level: 4 NCD genes: LOCUS:_54363#TRANSCRIPT:1#ABBREVIATION:HAO1#ECNUMBERS:1.1.3.15# LOCUS:_51179#TRANSCRIPT:1#ABBREVIATION:HAO2# LOCUS:_51179#TRANSCRIPT:2#ABBREVIATION:HAO2# proteins: NAME:hydroxyacid oxidase 2 (long chain), peroxisomal (liver, kidney)#ABBREVIATION:Hao2.1-x# NAME:hydroxyacid oxidase 2 (long chain), peroxisomal (liver, kidney)#ABBREVIATION:Hao2.2-x# NAME:hydroxyacid oxidase (glycolate oxidase) 1, peroxisome#ABBREVIATION:Hao1.1-x# citations: AUTHORS:Jones JM, Morrell JC, Gould SJ#TITLE:Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10777549# AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# AUTHORS:Ushijima Y#TITLE:Identity of aliphatic L- -hydroxyacid oxidase and glycolate oxidase from rat livers#JOURNAL:Arch Biochem Biophys#YEAR:1973#PUBMED:4705431# GENE ASSOCIATION: (54363.1) or (51179.2) or (51179.1)
Dolichol diphosphate, human liver homolog_C1080H1758O70P20
Abbreviation: R_CLI2tex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[e] + (2) i[c] --> cl[c] + (2) i[e] Confidence Level: 2 SAB genes: LOCUS:_5172#TRANSCRIPT:1#ABBREVIATION:SLC26A4# proteins: NAME:solute carrier family 26, member 4#ABBREVIATION:Slc26a4.1# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (5172.1)
chloride transport via iodide countertransport
Abbreviation: R_FORtrn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: for[c] <==> for[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HSPGtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: hspg[e] --> hspg[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
heparan sulfate transport, extracellular to lysosome
Abbreviation: R_ALCD22_L Synonyms: _0 EC Number: 1.2.1.49 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : lald-L + nad --> h + mthgxl + nadh Confidence Level: 2 NCD genes: LOCUS:_130#TRANSCRIPT:1#ABBREVIATION:ADH6#ECNUMBERS:1.1.1.1# LOCUS:_126#TRANSCRIPT:1#ABBREVIATION:ADH1C#ECNUMBERS:1.1.1.1# LOCUS:_125#TRANSCRIPT:1#ABBREVIATION:ADH1B#ECNUMBERS:1.1.1.1# LOCUS:_128#TRANSCRIPT:1#ABBREVIATION:ADH5#ECNUMBERS:1.1.1.1# LOCUS:_127#TRANSCRIPT:1#ABBREVIATION:ADH4#ECNUMBERS:1.1.1.1# LOCUS:_124#TRANSCRIPT:1#ABBREVIATION:ADH1A#ECNUMBERS:1.1.1.1# LOCUS:_131#TRANSCRIPT:1#ABBREVIATION:ADH7#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:1#ABBREVIATION:ZADH2# LOCUS:_137872#TRANSCRIPT:1#ABBREVIATION:ADHFE1#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:2#ABBREVIATION:ZADH2# proteins: NAME:alcohol dehydrogenase IV #ABBREVIATION:Adh4# NAME:alcohol dehydrogenase V#ABBREVIATION:Adh5# NAME:alcohol dehydrogenase 1A (class I), alpha polypeptide#ABBREVIATION:Adh1a.1# NAME:alcohol dehydrogenase IB (class I), beta polypeptide#ABBREVIATION:Adh1b.1# NAME:alcohol dehydrogenase 1C (class I), gamma polypeptide#ABBREVIATION:Adh1c.1# NAME:alcohol dehydrogenase 6 (class V)#ABBREVIATION:Adh6.1# NAME:alcohol dehydrogenase 7 (class IV), mu or sigma polypeptide#ABBREVIATION:Adh7.1# NAME:alcohol dehydrogenase, iron containing, 1 (liver)#ABBREVIATION:Adhfe1.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ab.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ac# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1bc# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.1# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.2# citations: AUTHORS:Ewaschuk JB, Naylor JM, Zello GA#TITLE:D-lactate in human and ruminant metabolism#JOURNAL:J Nutr#YEAR:2005#PUBMED:15987839# AUTHORS:Christopher MM, Eckfeldt JH, Eaton JW.#TITLE:Propylene glycol ingestion causes D-lactic acidosis#JOURNAL:Lab Invest.#YEAR:1990#PUBMED:2296157# GENE ASSOCIATION: (126.1) or (137872.1) or (126.1 and 124.1) or (127.1) or (130.1) or (284273.1) or (125.1 and 124.1) or (128.1) or (124.1) or (125.1) or (284273.2) or (131.1) or (125.1 and 126.1)
Abbreviation: R_UDPGALtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: udpgal[c] + ump[g] <==> udpgal[g] + ump[c] Confidence Level: 4 NCD genes: LOCUS:_7355#TRANSCRIPT:1#ABBREVIATION:SLC35A2# proteins: NAME:solute carrier family 35 (UDP-galactose transporter), member A2#ABBREVIATION:Slc35a2.1-g# citations: AUTHORS:Ishida N, Miura N, Yoshioka S, Kawakita M#TITLE:Molecular cloning and characterization of a novel isoform of the human UDP-galactose transporter, and of related complementary DNAs belonging to the nucleotide-sugar transporter gene family#JOURNAL:J Biochem (Tokyo)#YEAR:1996#PUBMED:9010752# AUTHORS:Miura N, Ishida N, Hoshino M, Yamauchi M, Hara T, Ayusawa D, Kawakita M#TITLE:Human UDP-galactose translocator: molecular cloning of a complementary DNA that complements the genetic defect of a mutant cell line deficient in UDP-galactose translocator#JOURNAL:J Biochem (Tokyo)#YEAR:1996#PUBMED:8889805# AUTHORS:Yoshioka S, Sun-Wada GH, Ishida N, Kawakita M#TITLE:Expression of the human UDP-galactose transporter in the Golgi membranes of murine Had-1 cells that lack the endogenous transporter#JOURNAL:J Biochem (Tokyo)#YEAR:1997#PUBMED:9399569# AUTHORS:Segawa H, Kawakita M, Ishida N#TITLE:Human and Drosophila UDP-galactose transporters transport UDP-N-acetylgalactosamine in addition to UDP-galactose#JOURNAL:Eur J Biochem.#YEAR:2002#PUBMED:11784306# AUTHORS:Sun-Wada GH, Yoshioka S, Ishida N, Kawakita M#TITLE:Functional expression of the human UDP-galactose transporters in the yeast Saccharomyces cerevisiae#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9562625# GENE ASSOCIATION: (7355.1)
UDP-Gal Golgi transport via CMP antiport
Abbreviation: R_EX_2hb_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 2hb <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_AG13T2g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre4 + uacgam --> h + ksii_core2_pre5 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (79369.1) or (374907.1) or (11041.1) or (284208.1) or (374907.2) or (93010.1) or (10331.1) or (10678.1) or (10678.2)
keratan sulfate II biosynthesis, precursor 5_C61H99N5O43X
Abbreviation: R_ACN23ACNGALGBSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acn23acngalgbside_hs[c] <==> acn23acngalgbside_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
L-Proline,2-oxoglutarate:oxygen oxidoreductase (4-hydroxylating) (ER)
Abbreviation: R_PROAKGOX1r Synonyms: _0 EC Number: 1.14.11.2 SUBSYSTEM: Arginine and Proline Metabolism Equation: [r] : akg + o2 + pro-L --> 4hpro-LT + co2 + succ Confidence Level: 4 SAB genes: LOCUS:_5033#TRANSCRIPT:1#ABBREVIATION:P4HA1#ECNUMBERS:1.14.11.2# LOCUS:_5034#TRANSCRIPT:1#ABBREVIATION:P4HB#ECNUMBERS:1.14.11.2,,5.3.# LOCUS:_8974#TRANSCRIPT:1#ABBREVIATION:P4HA2# proteins: NAME:procollagen-proline, 2-oxoglutarate 4-dioxygenase (proline 4-hydroxylase), alpha polypeptide I#ABBREVIATION:P4ha1.1-r# NAME:procollagen-proline, 2-oxoglutarate 4-dioxygenase (proline 4-hydroxylase), beta polypeptide (protein disulfide isomerase; thyroid hormone binding protein p55)#ABBREVIATION:P4hb.1-r# NAME:procollagen-proline, 2-oxoglutarate 4-dioxygenase (proline 4-hydroxylase), alpha polypeptide II#ABBREVIATION:P4ha2.1-r# citations: AUTHORS:Helaakoski T, Vuori K, Myllyla R, Kivirikko KI, Pihlajaniemi T.#TITLE:Molecular cloning of the alpha-subunit of human prolyl 4-hydroxylase: the complete cDNA-derived amino acid sequence and evidence for alternative splicing of RNA transcripts.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1989#PUBMED:2543975# AUTHORS:Pihlajaniemi T, Helaakoski T, Tasanen K, Myllyla R, Huhtala ML, Koivu J, Kivirikko KI.#TITLE:Molecular cloning of the beta-subunit of human prolyl 4-hydroxylase. This subunit and protein disulphide isomerase are products of the same gene.#JOURNAL:EMBO J#YEAR:1987#PUBMED:3034602# AUTHORS:Myllyharju J#TITLE:Prolyl 4-hydroxylases, the key enzymes of collagen biosynthesis#JOURNAL:Matrix Biol.#YEAR:2003#PUBMED:12714038# GENE ASSOCIATION: (5033.1) and (5034.1) or (8974.1) and (5034.1)
Abbreviation: R_EX_6dhf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 6dhf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S2L2N2M2MASNtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: s2l2n2m2masn[e] --> s2l2n2m2masn[l] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
s2l2n2m2masn transport, extracellular to lysosome
Abbreviation: R_G1M7MASNBterg Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: g1m7masnB[r] --> g1m7masnB[g] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# GENE ASSOCIATION:
g1m7masnC endoplasmic reticulum to Golgi transport
Abbreviation: R_THFtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: thf[c] <==> thf[l] Confidence Level: 2 NCD,IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_5,6,7,8-Tetrahydrofolate transport, diffusion, lysosomal
chondroitin sulfate E (GalNAc4,6diS-GlcA), degradation product 3_C37H53NO44S4
Abbreviation: R_GLCAASE7ly Synonyms: _3 EC Number: 3.2.1.31 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e_deg3 + h2o --> cs_e_deg4 + glcur Confidence Level: 4 NCD genes: LOCUS:_2990#TRANSCRIPT:1#ABBREVIATION:GUSB#ECNUMBERS:3.2.1.31# proteins: NAME:glucuronidase, beta, lysosomal#ABBREVIATION:Gusb.1-l# citations: AUTHORS:Guise KS, Korneluk RG, Waye J, Lamhonwah AM, Quan F, Palmer R, Ganschow RE, Sly WS, Gravel RA#TITLE:Isolation and expression in Escherichia coli of a cDNA clone encoding human beta-glucuronidase#JOURNAL:Gene#YEAR:1985#PUBMED:3924735# AUTHORS:Ho YC, Ho LH, Ho KJ#TITLE:Human hepatic beta-glucuronidase: an enzyme kinetic study#JOURNAL:Enzyme#YEAR:1985#PUBMED:3987656# AUTHORS:Oshima A, Kyle JW, Miller RD, Hoffmann JW, Powell PP, Grubb JH, Sly WS, Tropak M, Guise KS, Gravel RA#TITLE:Cloning, sequencing, and expression of cDNA for human beta-glucuronidase#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3468507# GENE ASSOCIATION: (2990.1)
Abbreviation: R_MALT Synonyms: _0 EC Number: 3.2.1.20 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : h2o + malt --> (2) glc-D Confidence Level: 4 IF genes: LOCUS:_2595#TRANSCRIPT:1#ABBREVIATION:GANC#ECNUMBERS:3.2.1.20# proteins: NAME:alpha glucosidase; neutral C#ABBREVIATION:Ganc.1# citations: AUTHORS:Hirschhorn R, Huie ML, Kasper JS#TITLE:Computer assisted cloning of human neutral alpha-glucosidase C (GANC): a new paralog in the glycosyl hydrolase gene family 31#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2002#PUBMED:12370436# AUTHORS:Martiniuk F, Hirschhorn R#TITLE:Characterization of neutral isozymes of human alpha-glucosidase: differences in substrate specificity, molecular weight and electrophoretic mobility#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7018580# GENE ASSOCIATION: (2595.1)
Abbreviation: R_5DHFtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _5dhf[c] --> 5dhf[l] Confidence Level: 2 carrier-mediated transport - but which and how is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
Abbreviation: R_EX_tsul_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tsul <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: na1[c] <==> na1[x] Confidence Level: 1 SAB genes: none proteins: none citations: none GENE ASSOCIATION:
sodium transport via diffusion (perioxisome)
Abbreviation: R_SCP3x Synonyms: _2 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : coa + dhcholestancoa + o2 --> dcholcoa + h2o + ppcoa Confidence Level: 4 SCP has two transcripts, second isoform specific to peroxisomes, first is specific for cyt and mit need to double check stoichiometry - reaction cofactors (additional coa + release of prop coa) from BM (Roche) Biochemical Pathways version 3 analog added as per KEGG NJ genes: LOCUS:_6342#TRANSCRIPT:1#ABBREVIATION:SCP2# proteins: NAME:sterol carrier protein 2#ABBREVIATION:Scp2x.1# citations: AUTHORS:Yamamoto R, Kallen CB, Babalola GO, Rennert H, Billheimer JT, Strauss JF 3rd.#TITLE:Cloning and expression of a cDNA encoding human sterol carrier protein 2.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1703300# GENE ASSOCIATION: (6342.1)
keratan sulfate I, degradation product 32_C82H135N5O67S2
Abbreviation: R_NACHEX18ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg31 --> acgam + ksi_deg32 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1) or (3074.1)
Abbreviation: R_DOPASULT Synonyms: _3 EC Number: 2.8.2.1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : dopa + paps --> dopasf + h + pap Confidence Level: 4 RS/TV (6/14/2005) genes: LOCUS:_6818#TRANSCRIPT:1#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# LOCUS:_6799#TRANSCRIPT:1#ABBREVIATION:SULT1A2#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:1#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:2#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:3#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:4#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:5#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6799#TRANSCRIPT:2#ABBREVIATION:SULT1A2#ECNUMBERS:2.8.2.1# LOCUS:_6818#TRANSCRIPT:2#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# LOCUS:_6818#TRANSCRIPT:3#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# proteins: NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.2# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.3# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.4# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.5# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 2 #ABBREVIATION:Sult1a2.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 2#ABBREVIATION:Sult1a2.2# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.2# NAME:sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.3# citations: AUTHORS:Chapman E, Best MD, Hanson SR, Wong CH.#TITLE:Sulfotransferases: structure, mechanism, biological activity, inhibition, and synthetic utility.#JOURNAL:Angew Chem Int Ed Engl#YEAR:2004#PUBMED:15293241# AUTHORS:Glatt H, Boeing H, Engelke CE, Ma L, Kuhlow A, Pabel U, Pomplun D, Teubner W, Meinl W.#TITLE:Human cytosolic sulphotransferases: genetics, characteristics, toxicological aspects.#JOURNAL:Mutat Res#YEAR:2001#PUBMED:11535246# GENE ASSOCIATION: (6799.1) or (6817.3) or (6799.2) or (6818.2) or (6817.2) or (6818.1) or (6817.1) or (6817.4) or (6818.3) or (6817.5)
Abbreviation: R_BTNDm Synonyms: _0 EC Number: 3.5.1.12 SUBSYSTEM: Biotin Metabolism Equation: [m] : biocyt + h2o --> btn + lys-L Confidence Level: 4 Also acts on biotin esters IT genes: LOCUS:_686#TRANSCRIPT:1#ABBREVIATION:BTD#ECNUMBERS:3.5.1.12# proteins: NAME:Biotinidase#ABBREVIATION:Btn.3-m# citations: AUTHORS:Stanley CM, Hymes J, Wolf B.#TITLE:Identification of alternatively spliced human biotinidase mRNAs and putative localization of endogenous biotinidase.#JOURNAL:Mol Genet Metab.#YEAR:2004#PUBMED:15059618# AUTHORS:Pacheco-Alvarez D, Solorzano-Vargas RS, Del Rio AL.#TITLE:Biotin in metabolism and its relationship to human disease.#JOURNAL:Arch Med Res. #YEAR:2002#PUBMED:12459313# GENE ASSOCIATION: (686.1)
Abbreviation: R_CLPNDCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: clpndcoa[c] <==> clpndcoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: AUTHORS:Sprecher H#TITLE:An update on the pathways of polyunsaturated fatty acid metabolism#JOURNAL:Curr Opn in Clinical Nutrition and Metabolic Care#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_PPAP Synonyms: _2 EC Number: 3.1.3.4 SUBSYSTEM: Triacylglycerol Synthesis Equation: [c] : h2o + pa_hs --> dag_hs + pi Confidence Level: 4 carbonyl and extra H balanced in R group NJ genes: LOCUS:_8613#TRANSCRIPT:1#ABBREVIATION:PPAP2B#ECNUMBERS:3.1.3.4# LOCUS:_8612#TRANSCRIPT:1#ABBREVIATION:PPAP2C#ECNUMBERS:3.1.3.4# LOCUS:_8611#TRANSCRIPT:1#ABBREVIATION:PPAP2A#ECNUMBERS:3.1.3.4# LOCUS:_8611#TRANSCRIPT:2#ABBREVIATION:PPAP2A#ECNUMBERS:3.1.3.4# proteins: NAME:phosphatidic acid phosphatase#ABBREVIATION:Ppap2a.1# NAME:phosphatidic acid phosphatase#ABBREVIATION:Ppap2a.2# NAME:phosphatidic acid phosphatase#ABBREVIATION:Ppap2b.1# NAME:phosphatidic acid phosphatase#ABBREVIATION:Ppap2c.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Coleman RA, Lee DP#TITLE:Enzymes of triacylglycerol synthesis and their regulation#JOURNAL:Prog in Lipid Research#YEAR:2004# AUTHORS:Roberts R, Sciorra VA, Morris AJ#TITLE:Human type 2 phosphatidic acid phosphohydrolases#JOURNAL:The Journal of Biological Chemistry#YEAR:1998# GENE ASSOCIATION: (8612.1) or (8611.1) or (8613.1) or (8611.2)
Abbreviation: R_DOLPH_Ler Synonyms: _0 EC Number: 3.1.3.51 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolp_L + h2o --> (0.1) dolichol_L + pi Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Schenk B, Fernandez F, Waechter CJ.#TITLE:The ins(ide) and out(side) of dolichyl phosphate biosynthesis and recycling in the endoplasmic reticulum.#JOURNAL:Glycobiology#YEAR:2001#PUBMED:11425794# GENE ASSOCIATION:
Dolichol-phosphate phosphohydrolase, human (liver)
Abbreviation: R_MALTt1r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: malt[e] <==> malt[c] Confidence Level: 4 NCD genes: LOCUS:_6515#TRANSCRIPT:1#ABBREVIATION:SLC2A3# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 3, extracellular#ABBREVIATION:Slc2a3.1-e# citations: AUTHORS:Uldry M, Thorens B#TITLE:The SLC2 family of facilitated hexose and polyol transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750891# AUTHORS:Haber RS, Weinstein SP, O'Boyle E, Morgello S#TITLE:Tissue distribution of the human GLUT3 glucose transporte#JOURNAL:Endocrinology#YEAR:1993#PUBMED:8504756# AUTHORS:Heijnen HF, Oorschot V, Sixma JJ, Slot JW, James DE#TITLE:Thrombin stimulates glucose transport in human platelets via the translocation of the glucose transporter GLUT-3 from alpha-granules to the cell surface#JOURNAL:J Cell Biol#YEAR:1997#PUBMED:9230074# AUTHORS:Kayano T, Fukumoto H, Eddy RL, Fan YS, Byers MG, Shows TB, Bell GI#TITLE:Evidence for a family of human glucose transporter-like proteins. Sequence and gene localization of a protein expressed in fetal skeletal muscle and other tissues#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3170580# AUTHORS:Stuart CA, Wen G, Jiang J#TITLE:GLUT3 protein and mRNA in autopsy muscle specimens#JOURNAL:Metabolism#YEAR:1999#PUBMED:10421229# GENE ASSOCIATION: (6515.1)
Abbreviation: R_GT1Ate Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gt1a_hs[c] <==> gt1a_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_TAXOLte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: taxol[e] <==> taxol[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
N-Acetylneuraminate 9-phosphate_C11H17NO12P
Abbreviation: R_DOLPGT1_Ler Synonyms: _2 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolglcp_L + (0.1) m8mpdol_L --> (0.1) dolp_L + (0.1) g1m8mpdol_L + h Confidence Level: 0 EC 2.4.1.- NCD genes: LOCUS:_29929#TRANSCRIPT:1#ABBREVIATION:ALG6# proteins: NAME:alpha-1,3-glucosyltransferase, endoplasmic reticulum (ubiquitous)#ABBREVIATION:Alg6.1-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (29929.1)
chondroitin sulfate A (GalNAc4S-GlcA), B (IdoA2S-GalNAc4S), and E (GalNAc4,6diS-GlcA), precursor 1_C31H47NO31S2X
Abbreviation: R_STS2 Synonyms: _4 EC Number: 3.1.6.2 SUBSYSTEM: Steroid Metabolism Equation: [c] : estrones + h2o --> estrone + h + so4 Confidence Level: 4 NJ genes: LOCUS:_412#TRANSCRIPT:1#ABBREVIATION:STS#ECNUMBERS:3.1.6.2# proteins: NAME:steroid sulfatase (microsomal), arylsulfatase C#ABBREVIATION:Sts.1# citations: AUTHORS:Stein C, Hille A, Seidel J, Rijnbout S, Waheed A, Schmidt B, Geuze H, von Figura K.#TITLE:Cloning and expression of human steroid-sulfatase. Membrane topology, glycosylation, and subcellular distribution in BHK-21 cells.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2668275# AUTHORS:Migeon BR, Shapiro LJ, Norum RA, Mohandas T, Axelman J, Dabora RL.#TITLE:Abstract Differential expression of steroid sulphatase locus on active and inactive human X chromosome.#JOURNAL:Nature#YEAR:1982#PUBMED:6957717# GENE ASSOCIATION: (412.1)
Abbreviation: R_DOPASFt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dopasf[c] --> dopasf[e] Confidence Level: 1 SAB excretion of dopamine sulfate genes: none proteins: none citations: none GENE ASSOCIATION:
Dopamine 3-0-sulfate transport (diffusion)
Abbreviation: R_ORETNF2 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : (2) 13-cis-retn + o2 --> (2) 13-cis-oretn Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
_13-cis-4-oxo-retinoic acid formation (oxidation)
Abbreviation: R_CK Synonyms: _0 EC Number: 2.7.3.2 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : atp + creat <==> adp + pcreat Confidence Level: 4 kegg C02305 MJC genes: LOCUS:_1160#TRANSCRIPT:1#ABBREVIATION:CKMT2#ECNUMBERS:2.7.3.2# LOCUS:_1159#TRANSCRIPT:1#ABBREVIATION:CKMT1B#ECNUMBERS:2.7.3.2# proteins: NAME:Creatine kinase, mitochondrial 1B (ubiquitous)#ABBREVIATION:Ckmt1b.1-m# NAME:creatine kinase, mitochondrial 2 (sarcomeric)#ABBREVIATION:Ckmt2.1-m# citations: AUTHORS:Haas RC, Korenfeld C, Zhang ZF, Perryman B, Roman D, Strauss AW.#TITLE:Isolation and characterization of the gene and cDNA encoding human mitochondrial creatine kinase.#JOURNAL:J Biol Chem. #YEAR:1989#PUBMED:2914937# AUTHORS:Klein SC, Haas RC, Perryman MB, Billadello JJ, Strauss AW.#TITLE:Regulatory element analysis and structural characterization of the human sarcomeric mitochondrial creatine kinase gene.#JOURNAL:J Biol Chem#YEAR:1991#PUBMED:1917943# GENE ASSOCIATION: (1159.1) or (1160.1)
Abbreviation: R_TAGAT_DASH_Dt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tagat-D[e] --> tagat-D[c] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate II biosynthesis, precursor 1_C36H60N3O25X
Abbreviation: R_PNTK Synonyms: _0 EC Number: 2.7.1.33 SUBSYSTEM: CoA Biosynthesis Equation: [c] : atp + pnto-R --> 4ppan + adp + h Confidence Level: 4 genes: LOCUS:_55229#TRANSCRIPT:1#ABBREVIATION:PANK4#ECNUMBERS:2.7.1.33# LOCUS:_53354#TRANSCRIPT:1#ABBREVIATION:PANK1# LOCUS:_79646#TRANSCRIPT:1#ABBREVIATION:PANK3# LOCUS:_53354#TRANSCRIPT:2#ABBREVIATION:PANK1# LOCUS:_53354#TRANSCRIPT:3#ABBREVIATION:PANK1# LOCUS:_80025#TRANSCRIPT:1#ABBREVIATION:PANK2#ECNUMBERS:2.7.1.33# LOCUS:_80025#TRANSCRIPT:2#ABBREVIATION:PANK2#ECNUMBERS:2.7.1.33# LOCUS:_80025#TRANSCRIPT:3#ABBREVIATION:PANK2#ECNUMBERS:2.7.1.33# LOCUS:_80025#TRANSCRIPT:4#ABBREVIATION:PANK2#ECNUMBERS:2.7.1.33# proteins: NAME:Pantothenate kinase 2 #ABBREVIATION:PanK2.2# NAME:Pantothenate kinase 2 #ABBREVIATION:PanK2.1# NAME:Pantothenate kinase 2 #ABBREVIATION:PanK2.3# NAME:Pantothenate kinase 2 #ABBREVIATION:PanK2.4# NAME:pantothenate kinase 4 #ABBREVIATION:PanK4.1# NAME:pantothenate kinase 3 #ABBREVIATION:PanK3.1# NAME:pantothenate kinase 1 #ABBREVIATION:PanK1.1# NAME:pantothenate kinase 1 #ABBREVIATION:PanK1.2# NAME:pantothenate kinase 1 #ABBREVIATION:PanK1.3# citations: AUTHORS:Daugherty M, Polanuyer B, Farrell M, Scholle M, Lykidis A, de Crecy-Lagard V, Osterman A. #TITLE:Complete reconstitution of the human coenzyme A biosynthetic pathway via comparative genomics.#JOURNAL:J Biol Chem.#YEAR:2002#PUBMED:11923312# AUTHORS:Zhou B, Westaway SK, Levinson B, Johnson MA, Gitschier J, Hayflick SJ. #TITLE:A novel pantothenate kinase gene (PANK2) is defective in Hallervorden-Spatz syndrome.#JOURNAL:Nat Genet. #YEAR:2001#PUBMED:11479594# AUTHORS:Ching KH, Westaway SK, Gitschier J, Higgins JJ, Hayflick SJ.#TITLE:HARP syndrome is allelic with pantothenate kinase-associated neurodegeneration.#JOURNAL:Neurology#YEAR:2002#PUBMED:Neurology# GENE ASSOCIATION: (79646.1) or (80025.2) or (53354.1) or (53354.3) or (80025.1) or (53354.2) or (80025.3) or (80025.4) or (55229.1)
Abbreviation: R_LACZly Synonyms: _0 EC Number: 3.2.1.23 SUBSYSTEM: Galactose metabolism Equation: [l] : h2o + lcts --> gal + glc-D Confidence Level: 4 Some enzymes in this group hydrolyse a-L-arabinosides; some animal enzymes also hydrolyse b-D-fucosides and b-D-glucosides; cf. EC 3.2.1.108 lactase genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_GLACter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: glac[c] <==> glac[r] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Marsh CA#TITLE:Biosynthesis of D-glucaric acid in mammals: a free-radical mechanism?#JOURNAL:Carbohydr Res#YEAR:1986#PUBMED:3779687# GENE ASSOCIATION:
D-glucurono-6,3-lactone transport, endoplasmic reticulum
Abbreviation: R_5ADTSTSTERONEtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: _5adtststerone[r] <==> 5adtststerone[c] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: none GENE ASSOCIATION:
_5 alpha dihydrotesterone intracellular transport
_3-Methylimidazole acetaldehyde:NAD+ oxidoreductase
Abbreviation: R_MACOXO Synonyms: _0 EC Number: 1.2.1.5 SUBSYSTEM: Histidine Metabolism Equation: [c] : 3mldz + h2o + nad --> 3mlda + (2) h + nadh Confidence Level: 4 SAB added 2 h to balance--not certain genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Zimatkin SM, Anichtchik OV#TITLE:Alcohol-histamine interactions#JOURNAL:Alcohol Alcohol#YEAR:1999#PUBMED:10344773# GENE ASSOCIATION: (221.1) or (4129.1) or (220.1) or (218.1) or (222.1)
Abbreviation: R_EX_xoltri24_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : xoltri24 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_7-alpha,24(S)-Dihydroxycholesterol exchange
Abbreviation: R_ADCim Synonyms: _0 EC Number: 4.1.1.4 SUBSYSTEM: Propanoate Metabolism Equation: [m] : acac + h --> acetone + co2 Confidence Level: 2 TDV (Voet & Voet pg 581) Non-enzymatic decarboxylation; spontaneous reaction added "m" suffix - NCD genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION:
Acetoacetate decarboxylation (irreversible), mitochondrial
Abbreviation: R_PUNP4 Synonyms: _0 EC Number: 2.4.2.1 SUBSYSTEM: Purine Catabolism Equation: [c] : dgsn + pi <==> 2dr1p + gua Confidence Level: 4 Specificity not completely determined. Can also catalyse ribosyltransferase reactions of the type catalysed by EC 2.4.2.5, nucleoside ribosyltransferase genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# citations: AUTHORS:Erion MD, Stoeckler JD, Guida WC, Walter RL, Ealick SE.#TITLE:Purine nucleoside phosphorylase. 2. Catalytic mechanism. #JOURNAL:Biochemistry#YEAR:1997#PUBMED:9305963# AUTHORS:Williams SR, Goddard JM, Martin DW Jr. #TITLE:Human purine nucleoside phosphorylase cDNA sequence and genomic clone characterization.#JOURNAL:Nucleic Acids Res.#YEAR:1984#PUBMED:6087295# GENE ASSOCIATION: (4860.1)
purine-nucleoside phosphorylase (Deoxyguanosine)
Abbreviation: R_5HOXINOXDA Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Tryptophan metabolism Equation: [c] : h2o + o2 + srtn --> 5hoxindact + h2o2 + nh4 Confidence Level: 4 SAB genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# proteins: NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# AUTHORS:Geha RM, Rebrin I, Chen K, Shih JC#TITLE:Substrate and inhibitor specificities for human monoamine oxidase A and B are influenced by a single amino acid#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11134050# GENE ASSOCIATION: (4128.1) or (4129.1)
_5-Hydroxytryptamine:oxygen oxidoreductase(deaminating)(flavin-containing)
Abbreviation: R_HPYRDCm Synonyms: _0 EC Number: 4.1.1.40 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [m] : h + hpyr --> co2 + gcald Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Rofe AM, James HM, Bais R, Conyers RA#TITLE:Hepatic oxalate production: the role of hydroxypyruvate#JOURNAL:Biochem Med Metab Biol#YEAR:1986#PUBMED:3778681# GENE ASSOCIATION:
hydroxypyruvate decarboxylase, mitochondria
Abbreviation: R_NH4tn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: nh4[c] <==> nh4[n] Confidence Level: 1 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_retpalm_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : retpalm [deleted 10/09/2005 06:18:49 PM] <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_YVITEt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: yvite[e] --> yvite[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_G14T11g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre17 + udpgal --> h + ksi_pre18 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (8704.2) or (8704.1) or (2683.1) or (9334.1)
Abbreviation: R_LIPOti Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + lipoate[e] + (2) na1[e] --> adp[c] + h[c] + lipoate[c] + (2) na1[c] + pi[c] Confidence Level: 4 _01-25-05 based on Wang et al., JBC, 1999,1 274(21), 14875-14888. IT 03-07-05:replaced IT: now based on Balamurugan et al, 2005, Am J Physiol Renal Physiol, 288, F823-831,2005 genes: LOCUS:_8884#TRANSCRIPT:1#ABBREVIATION:SMVT#ECNUMBERS:TC,2.A.21.1.1# proteins: NAME:sodium-dependent vitamin transporter (biotin, pantothenate,lipoate) #ABBREVIATION:SmvT.1# citations: AUTHORS:Prasad PD, Wang H, Kekuda R, Fujita T, Fei YJ, Devoe LD, Leibach FH, Ganapathy V.#TITLE:Cloning and functional expression of a cDNA encoding a mammalian sodium-dependent vitamin transporter mediating the uptake of pantothenate, biotin, and lipoate.#JOURNAL: J Biol Chem.#YEAR:1998#PUBMED:9516450# AUTHORS:Balamurugan K, Ortiz A, Said HM.#TITLE:Biotin uptake by human intestinal and liver epithelial cells: role of the SMVT system.#JOURNAL:Am J Physiol Gastrointest Liver Physiol. #YEAR:2003#PUBMED:12646417# AUTHORS:Prasad PD, Wang H, Huang W, Fei YJ, Leibach FH, Devoe LD, Ganapathy V.#TITLE:Molecular and functional characterization of the intestinal Na+-dependent multivitamin transporter. #JOURNAL:Arch Biochem Biophys.#YEAR:1999#PUBMED:10334869# AUTHORS:Wang H, Huang W, Fei YJ, Xia H, Yang-Feng TL, Leibach FH, Devoe LD, Ganapathy V, Prasad PD.#TITLE:Human placental Na+-dependent multivitamin transporter. Cloning, functional expression, gene structure, and chromosomal localization.#JOURNAL: J Biol Chem. #YEAR:1999#PUBMED:10329687# AUTHORS:Balamurugan K, Vaziri ND, Said HM.#TITLE:Biotin uptake by human proximal tubular epithelial cells: Cellular and molecular aspects. #JOURNAL:Am J Physiol Renal Physiol. #YEAR:2005#PUBMED:15561972# GENE ASSOCIATION: (8884.1)
Abbreviation: R_DGK2m Synonyms: _0 EC Number: 2.7.4.8 SUBSYSTEM: Nucleotides Equation: [m] : datp + dgmp <==> dadp + dgdp Confidence Level: 2 Added EC- JLR IT genes: none proteins: none citations: none GENE ASSOCIATION:
deoxyguanylate kinase (dGMP:dATP) (mitochondrial)
Abbreviation: R_EX_asn_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : asn-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_L_DASH_LACtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h[c] + lac-L[c] <==> h[m] + lac-L[m] Confidence Level: 0 NCD genes: LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1 #ABBREVIATION:Slc16a1.1-m# citations: none GENE ASSOCIATION: (6566.1)
dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase (uterus)
Abbreviation: R_DOLPGT1_Uer Synonyms: _2 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolglcp_U + (0.1) m8mpdol_U --> (0.1) dolp_U + (0.1) g1m8mpdol_U + h Confidence Level: 0 EC 2.4.1.- NCD genes: LOCUS:_29929#TRANSCRIPT:1#ABBREVIATION:ALG6# proteins: NAME:alpha-1,3-glucosyltransferase, endoplasmic reticulum (ubiquitous)#ABBREVIATION:Alg6.1-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Imbach T, Burda P, Kuhnert P, Wevers RA, Aebi M, Berger EG, Hennet T.#TITLE:A mutation in the human ortholog of the Saccharomyces cerevisiae ALG6 gene causes carbohydrate-deficient glycoprotein syndrome type-Ic.#JOURNAL:Proc Natl Acad Sci USA#YEAR:1999#PUBMED:10359825# GENE ASSOCIATION: (29929.1)
(alpha-D-Mannosyl)8-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1725H2840N20O620P20
Abbreviation: R_LEUt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: leu-L[e] + na1[e] --> leu-L[c] + na1[c] Confidence Level: 4 SAB genes: LOCUS:_84889#TRANSCRIPT:1#ABBREVIATION:SLC7A3# LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_6542#TRANSCRIPT:1#ABBREVIATION:SLC7A2# LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_6541#TRANSCRIPT:1#ABBREVIATION:SLC7A1# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 1#ABBREVIATION:Slc7a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 2#ABBREVIATION:Slc7a2.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 3#ABBREVIATION:Slc7a3.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# AUTHORS:Albritton LM, Bowcock AM, Eddy RL, Morton CC, Tseng L, Farrer LA, Cavalli-Sforza LL, Shows TB, Cunningham JM#TITLE:The human cationic amino acid transporter (ATRC1): physical and genetic mapping to 13q12-q14#JOURNAL:Genomics#YEAR:1992#PUBMED:1348489# AUTHORS:Cariappa R, Heath-Monnig E, Furesz TC, Kamath SG, Smith CH#TITLE:Stable polarized expression of hCAT-1 in an epithelial cell line#JOURNAL:J Membr Biol#YEAR:2002#PUBMED:11891586# AUTHORS:Hoshide R, Ikeda Y, Karashima S, Matsuura T, Komaki S, Kishino T, Niikawa N, Endo F, Matsuda I#TITLE:Molecular cloning, tissue distribution, and chromosomal localization of human cationic amino acid transporter 2 (HCAT2)#JOURNAL:Genomics#YEAR:1996#PUBMED:8954799# AUTHORS:Furesz TC, Moe AJ, Smith CH#TITLE:Lysine uptake by human placental microvillous membrane: comparison of system y+ with basal membrane#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7534987# AUTHORS:Wolf S, Janzen A, Vekony N, Martine U, Strand D, Closs EI#TITLE:Expression of solute carrier 7A4 (SLC7A4) in the plasma membrane is not sufficient to mediate amino acid transport activity#JOURNAL:Biochem J#YEAR:2002#PUBMED:12049641# AUTHORS:Vekony N, Wolf S, Boissel JP, Gnauert K, Closs EI#TITLE:Human cationic amino acid transporter hCAT-3 is preferentially expressed in peripheral tissues#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11591158# AUTHORS:Pineda M, Fernandez E, Torrents D, Estevez R, Lopez C, Camps M, Lloberas J, Zorzano A, Palacin M#TITLE:Identification of a membrane protein, LAT-2, that Co-expresses with 4F2 heavy chain, an L-type amino acid transport activity with broad specificity for small and large zwitterionic amino acids#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10391915# AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Bertran J, Magagnin S, Werner A, Markovich D, Biber J, Testar X, Zorzano A, Kuhn LC, Palacin M, Murer H#TITLE:Stimulation of system y(+)-like amino acid transport by the heavy chain of human 4F2 surface antigen in Xenopus laevis oocytes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1992#PUBMED:1376926# GENE ASSOCIATION: (6542.1) or (81539.1) or (11254.1) or (55089.1) or (84889.1) or (6520.1) or (6541.1)
L-leucine transport in via sodium symport
Abbreviation: R_RETNGLCt2 Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: _13-cis-retnglc[c] --> 13-cis-retnglc[e] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Barua AB, Sidell N.#TITLE:Retinoyl beta-glucuronide: a biologically active interesting retinoid.#JOURNAL:J Nutr.#YEAR:2004#PUBMED:14704335# AUTHORS:Tang GW, Russell RM.#TITLE:_13-cis-retinoic acid is an endogenous compound in human serum.#JOURNAL:J Lipid Res.#YEAR:1990#PUBMED:2324641# GENE ASSOCIATION:
L-hydroxyproline dehydrogenase (NAD), mitochondrial
Abbreviation: R_HPROxm Synonyms: _0 EC Number: 1.5.1.12 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : 4hpro-LT + nad --> 1p3h5c + (2) h + nadh Confidence Level: 2 NCD genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: AUTHORS:Takayama T, Fujita K, Suzuki K, Sakaguchi M, Fujie M, Nagai E, Watanabe S, Ichiyama A, Ogawa Y#TITLE:Control of oxalate formation from L-hydroxyproline in liver mitochondria#JOURNAL:J Am Soc Nephrol#YEAR:2003#PUBMED:12660328# AUTHORS:Knight J, Holmes RP#TITLE:Mitochondrial hydroxyproline metabolism: implications for primary hyperoxaluria#JOURNAL:Am J Nephrol#YEAR:2005#PUBMED:15849464# GENE ASSOCIATION: (8659.1) or (8659.2)
Abbreviation: R_CHOLtu Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: chol[e] <==> chol[c] Confidence Level: 4 SAB electrogenic, Na indepdendent & rev genes: LOCUS:_6582#TRANSCRIPT:1#ABBREVIATION:SLC22A2# LOCUS:_6582#TRANSCRIPT:2#ABBREVIATION:SLC22A2# LOCUS:_6584#TRANSCRIPT:1#ABBREVIATION:SLC22A5# proteins: NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.2# NAME:solute carrier family 22 (organic cation transporter), member 5#ABBREVIATION:Slc22a5.1# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# GENE ASSOCIATION: (6582.1) or (6584.1) or (6582.2)
aspartate-glutamate mitochondrial shuttle
Abbreviation: R_ASPGLUm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: asp-L[m] + glu-L[c] + h[c] <==> asp-L[c] + glu-L[m] + h[m] Confidence Level: 4 TDV genes: LOCUS:_8604#TRANSCRIPT:1#ABBREVIATION:SLC25A12# LOCUS:_10165#TRANSCRIPT:1#ABBREVIATION:SLC25A13# proteins: NAME:Solute carrier family 25 member 12, mitochondrial #ABBREVIATION:Slc25a12.1-m# NAME: solute carrier family 25 member 13, mitochondrial #ABBREVIATION:Slc25a13.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (8604.1) or (10165.1)
Abbreviation: R_BHBt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: bhb[e] + h[e] <==> bhb[c] + h[c] Confidence Level: 2 IT, TDV genes: none proteins: none citations: AUTHORS:Alonso de la Torre SR, Serrano MA, Medina JM.#TITLE:Carrier-mediated beta-D-hydroxybutyrate transport in brush-border membrane vesicles from rat placenta.#JOURNAL:Pediatr Res#YEAR:1992#PUBMED:1408469# AUTHORS:Guo K, Lukacik P, Papagrigoriou E, Meier M, Lee WH, Adamski J, Oppermann U.#TITLE:Characterization of Human DHRS6, an Orphan Short Chain Dehydrogenase/Reductase Enzyme: A NOVEL, CYTOSOLIC TYPE 2 R-beta-HYDROXYBUTYRATE DEHYDROGENASE.#JOURNAL:J Biol Chem#YEAR:2006#PUBMED:16380372# GENE ASSOCIATION:
(R)-3-Hydroxybutanoate transport via H+ symport
Abbreviation: R_EX_ttdca_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ttdca <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PI34P5Kn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + pail34p_hs --> adp + h + pail345p_hs Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
phosphatidylinositol 3,4-bisphosphate 5-kinase, nuclear
Abbreviation: R_ACACT1 Synonyms: _0 EC Number: 2.3.1.9 SUBSYSTEM: Cholesterol Metabolism Equation: [c] : (2) accoa --> aacoa + coa Confidence Level: 4 genes: LOCUS:_39#TRANSCRIPT:1#ABBREVIATION:ACAT2#ECNUMBERS:2.3.1.9# proteins: NAME:acetyl-Coenzyme A acetyltransferase 2 (acetoacetyl Coenzyme A thiolase)#ABBREVIATION:Acat2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (39.1)
Abbreviation: R_FUCACNGALACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucacngalacglcgalgluside_hs[g] <==> fucacngalacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GGH_DASH_6DHFl Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [l] : 6dhf + h2o --> 5dhf + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-l# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (6DHF), lysosomal
Abbreviation: R_3MOBt2im Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _3mob[c] + h[c] --> 3mob[m] + h[m] Confidence Level: 4 MM genes: none proteins: none citations: AUTHORS:Hutson SM, Hall TR.#TITLE:Identification of the mitochondrial branched chain aminotransferase as a branched chain alpha-keto acid transport protein.#YEAR:1993#PUBMED:8428987# GENE ASSOCIATION:
_3-methyl-2-oxobutanoate mitochondrial transport via proton symport
Abbreviation: R_ACGALFUCGALACGALFUC12GAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acgalfucgalacgalfuc12gal14acglcgalgluside_hs[g] <==> acgalfucgalacgalfuc12gal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GMPR Synonyms: _0 EC Number: 1.7.1.7 SUBSYSTEM: Nucleotides Equation: [c] : gmp + (2) h + nadph --> imp + nadp + nh4 Confidence Level: 4 EC- changed from 1.7.1.7 genes: LOCUS:_51292#TRANSCRIPT:1#ABBREVIATION:GMPR2# LOCUS:_2766#TRANSCRIPT:1#ABBREVIATION:GMPR#ECNUMBERS:1.6.6.8# LOCUS:_51292#TRANSCRIPT:3#ABBREVIATION:GMPR2# LOCUS:_51292#TRANSCRIPT:2#ABBREVIATION:GMPR2# proteins: NAME:guanosine monophosphate reductase 2 #ABBREVIATION:GmpR2.1# NAME:guanosine monophosphate reductase 2 #ABBREVIATION:GmpR2.2# NAME:guanosine monophosphate reductase 2 #ABBREVIATION:GmpR2.3# NAME:guanosine monophosphate reductase #ABBREVIATION:GmpR.1# citations: AUTHORS:Deng Y, Wang Z, Ying K, Gu S, Ji C, Huang Y, Gu X, Wang Y, Xu Y, Li Y, Xie Y, Mao Y.#TITLE:NADPH-dependent GMP reductase isoenzyme of human (GMPR2). Expression, purification, and kinetic properties.#JOURNAL:Int J Biochem Cell Biol.#YEAR:2002#PUBMED:12009299# AUTHORS:Murano I, Tsukahara M, Kajii T, Yoshida A.#TITLE:Mapping of the human guanosine monophosphate reductase gene (GMPR) to chromosome 6p23 by fluorescence in situ hybridization.#JOURNAL:Genomics#YEAR:1994#PUBMED:8188226# GENE ASSOCIATION: (2766.1) or (51292.1) or (51292.3) or (51292.2)
Abbreviation: R_FACOAL206 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + phyt <==> amp + phytcoa + ppi Confidence Level: 4 NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 2#ABBREVIATION:Slc27a2.1# citations: AUTHORS:Steinberg SJ, Wang SJ, Kim DG, Mihalik J, Watkins PA#TITLE:Human very-long-chain-acyl-CoA synthetase#JOURNAL:Biochemical and biophysical research communications#YEAR:1999# GENE ASSOCIATION: (11001.1)
Abbreviation: R_PDE4g Synonyms: _0 EC Number: 3.1.4.17 SUBSYSTEM: Nucleotides Equation: [g] : 35cgmp + h2o --> gmp + h Confidence Level: 4 NCD. IT genes: LOCUS:_10846#TRANSCRIPT:1#ABBREVIATION:PDE10A# proteins: NAME:phosphodiesterase 10A #ABBREVIATION:Pde10A# citations: AUTHORS:Kotera J, Sasaki T, Kobayashi T, Fujishige K, Yamashita Y, Omori K.#TITLE:Subcellular localization of cyclic nucleotide phosphodiesterase type 10A variants, and alteration of the localization by cAMP-dependent protein kinase-dependent phosphorylation. #JOURNAL:J Biol Chem.#YEAR:2004#PUBMED:14604994# GENE ASSOCIATION: (10846.1)
_3',5'-cyclic-nucleotide phosphodiesterase, Golgi
Abbreviation: R_LNLCCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + lnlccrn --> crn + lnlccoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_PHCDm Synonyms: _0 EC Number: 1.5.1.12 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : 1p3h5c + (2) h2o + nad --> e4hglu + h + nadh Confidence Level: 2 NCD genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: AUTHORS:Takayama T, Fujita K, Suzuki K, Sakaguchi M, Fujie M, Nagai E, Watanabe S, Ichiyama A, Ogawa Y#TITLE:Control of oxalate formation from L-hydroxyproline in liver mitochondria#JOURNAL:J Am Soc Nephrol#YEAR:2003#PUBMED:12660328# AUTHORS:Valle D, Goodman SI, Harris SC, Phang JM.#TITLE:Genetic evidence for a common enzyme catalyzing the second step in the degradation of proline and hydroxyproline.#JOURNAL: J Clin Invest#YEAR:1979#PUBMED:500817# AUTHORS:Knight J, Holmes RP#TITLE:Mitochondrial hydroxyproline metabolism: implications for primary hyperoxaluria#JOURNAL:Am J Nephrol#YEAR:2005#PUBMED:15849464# GENE ASSOCIATION: (8659.1) or (8659.2)
L-1-pyrroline-3-hydroxy-5-carboxylate dehydrogenase
phosphatidylinositol 3,4-bisphosphate 5-kinase
Abbreviation: R_PI34P5K Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + pail34p_hs --> adp + h + pail345p_hs Confidence Level: 4 NCD genes: LOCUS:_8394#TRANSCRIPT:1#ABBREVIATION:PIP5K1A#ECNUMBERS:2.7.1.68# LOCUS:_23396#TRANSCRIPT:1#ABBREVIATION:PIP5K1C# LOCUS:_8395#TRANSCRIPT:1#ABBREVIATION:PIP5K1B# proteins: NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, gamma #ABBREVIATION:Pip5k1c.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, beta#ABBREVIATION:Pip5k1b.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, alpha#ABBREVIATION:Pip5k1a.1# citations: AUTHORS:Tolias KF, Cantley LC#TITLE:Pathways for phosphoinositide synthesis#JOURNAL:Chem Phys Lipids#YEAR:1999#PUBMED:10358929# AUTHORS:Stephens LR, Hughes KT, Irvine RF#TITLE:Pathway of phosphatidylinositol(3,4,5)-trisphosphate synthesis in activated neutrophils#JOURNAL:Nature#YEAR:1991#PUBMED:1851250# AUTHORS:Cunningham TW, Lips DL, Bansal VS, Caldwell KK, Mitchell CA, Majerus PW#TITLE:Pathway for the formation of D-3 phosphate containing inositol phospholipids in intact human platelets#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2174884# AUTHORS:Cunningham TW, Majerus PW#TITLE:Pathway for the formation of D-3 phosphate containing inositol phospholipids in PDGF stimulated NIH 3T3 fibroblasts#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1850246# AUTHORS:Carter AN, Huang R, Sorisky A, Downes CP, Rittenhouse SE#TITLE:Phosphatidylinositol 3,4,5-trisphosphate is formed from phosphatidylinositol 4,5-bisphosphate in thrombin-stimulated platelets#JOURNAL:Biochem J#YEAR:1994#PUBMED:8042983# GENE ASSOCIATION: (8394.1) or (23396.1) or (8395.1)
heparan sulfate, degradation product 24_C31H50NO26
Abbreviation: R_ETOHMO Synonyms: _0 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : etoh + h + nadph + o2 --> acald + (2) h2o + nadp Confidence Level: 2 NCD genes: LOCUS:_1571#TRANSCRIPT:1#ABBREVIATION:CYP2E1# proteins: NAME:cytochrome P450, family 2, subfamily E, polypeptide 1#ABBREVIATION:Cyp2e1.1# citations: AUTHORS:Colleen Smith, Allan Marks, Michael Lieberman#TITLE:Marks' Basic Medical Biochemistry#YEAR:2004# GENE ASSOCIATION: (1571.1)
Abbreviation: R_ABO1g Synonyms: _10 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucgalacglcgalgluside_hs + udpacgal --> acgalfucgalacglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_28#TRANSCRIPT:1#ABBREVIATION:ABO# proteins: NAME:ABO blood group#ABBREVIATION:Abo.1# citations: AUTHORS:Lloyd KO.#TITLE:The chemistry and immunochemistry of blood group A, B, H, and Lewis antigens: past, present and future.#JOURNAL:Glycoconj J#YEAR:2000#PUBMED:11421346# AUTHORS:Yamamoto F.-I., Hakomori S.-I.;#TITLE:Sugar-nucleotide donor specificity of histo-blood group A and B transferases is based on amino acid substitutions#JOURNAL:J. Biol. Chem#YEAR:1990#PUBMED:2121736# AUTHORS:Patenaude S.I., Seto N.O.L., Borisova S.N., Szpacenko A., Marcus S.L., Palcic M.M., Evans S.V.;#TITLE:The structural basis for specificity in human ABO(H) blood group biosynthesis#JOURNAL:Nat. Struct. Biol#YEAR:2002#PUBMED:12198488# GENE ASSOCIATION: (28.1)
Abbreviation: R_MM6B1bg Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m6masnB2 --> m5masnB1 + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (10905.1) or (57134.1)
Abbreviation: R_CHOLATEt3 Synonyms: _10 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + cholate[c] + h2o[c] --> adp[c] + cholate[e] + h[c] + pi[c] Confidence Level: 4 for bile acid secretion into bile NJ genes: LOCUS:_8647#TRANSCRIPT:1#ABBREVIATION:ABCB11# LOCUS:_8714#TRANSCRIPT:1#ABBREVIATION:ABCC3# LOCUS:_8714#TRANSCRIPT:2#ABBREVIATION:ABCC3# LOCUS:_8714#TRANSCRIPT:3#ABBREVIATION:ABCC3# proteins: NAME:ABC, sub-family B (MDR/TAP), member 11#ABBREVIATION:Abcb11.1# NAME:ABC, sub-family C (CFTR/MRP), member 3#ABBREVIATION:Abcc3.1# NAME:ABC, sub-family C, (CFTR/MRP), member 3#ABBREVIATION:Abcc3.2# NAME:ABC, sub-family C, (CFTR/MRP), member 3#ABBREVIATION:Abcc3.3# citations: AUTHORS:Strautnieks SS, Bull LN, Knisely AS, Kocoshis SA, Dahl N, Arnell H, Sokal E, Dahan K, Childs S, Ling V, Tanner MS, Kagalwalla AF, Nemeth A, Pawlowska J, Baker A, Mieli-Vergani G, Freimer NB, Gardiner RM, Thompson RJ.#TITLE:A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis.#JOURNAL:Nat Genet.#YEAR:1998#PUBMED:9806540# AUTHORS:Kiuchi Y, Suzuki H, Hirohashi T, Tyson CA, Sugiyama Y.#TITLE:cDNA cloning and inducible expression of human multidrug resistance associated protein 3 (MRP3).#JOURNAL:FEBS Lett.#YEAR:1998#PUBMED:9738950# AUTHORS:Uchiumi T, Hinoshita E, Haga S, Nakamura T, Tanaka T, Toh S, Furukawa M, Kawabe T, Wada M, Kagotani K, Okumura K, Kohno K, Akiyama S, Kuwano M.#TITLE:Isolation of a novel human canalicular multispecific organic anion transporter, cMOAT2/MRP3, and its expression in cisplatin-resistant cancer cells with decreased ATP-dependent drug transport.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1998#PUBMED:9813153# GENE ASSOCIATION: (8714.3) or (8647.1) or (8714.2) or (8714.1)
all trans Decaprenyl diphosphate_C50H81O7P2
Abbreviation: R_DECDPtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: decdp[c] <==> decdp[m] Confidence Level: 2 ubiquinone precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_HPDCACRNCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + hpdcacoa --> coa + hpdcacrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (1375.2) or (1375.3) or (1374.1) or (126129.1) or (1375.1)
Abbreviation: R_EX_ala_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ala-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TETPENT6COAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: tetpent6coa[c] <==> tetpent6coa[x] Confidence Level: 0 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PRISTtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: prist[x] <==> prist[c] Confidence Level: 0 Mechanism not known, see PMID 11591435 for justification. NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_P45019A1r Synonyms: _4 EC Number: 1.14.14.1 SUBSYSTEM: Steroid Metabolism Equation: [r] : andrstndn + nadph + (2) o2 --> estrone + for + (2) h2o + nadp Confidence Level: 4 uniprot - states "membrane bound" - non-specific formate production inferred from cyp P450 review paper Korzekwa J Steroid Biochemistry (1993) 44:367-73-RETURN FOLLOWING DEEPER LIT SEARCH H2O added for balance purposes - in analogy to other P450 reactions NJ genes: LOCUS:_1588#TRANSCRIPT:1#ABBREVIATION:CYP19A1# LOCUS:_1588#TRANSCRIPT:2#ABBREVIATION:CYP19A1# proteins: NAME:cytochrome P450, family 19, subfamily A, polypeptide 1#ABBREVIATION:Cyp19a1.1# NAME:cytochrome P450, family 19, subfamily A, polypeptide 1#ABBREVIATION:Cyp19a1.2# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# AUTHORS:Korzekwa KR, Trager WF, Mancewicz J, Osawa Y#TITLE:Studies on the mechanism of aromatase and other cytochrome P450 mediated deformylation reactions#JOURNAL:J Steroid Biochem Mol Biol#YEAR:1993# GENE ASSOCIATION: (1588.1) or (1588.2)
Abbreviation: R_GALOR Synonyms: _0 EC Number: 1.1.1.21 SUBSYSTEM: Galactose metabolism Equation: [c] : gal + h + nadph <==> galt + nadp Confidence Level: 2 NCD genes: LOCUS:_10327#TRANSCRIPT:1#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# LOCUS:_231#TRANSCRIPT:1#ABBREVIATION:AKR1B1#ECNUMBERS:1.1.1.21# LOCUS:_10327#TRANSCRIPT:2#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# proteins: NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.1# NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.2# NAME:aldo-keto reductase family 1, member B1 (aldose reductase)#ABBREVIATION:Akr1b1.1# citations: AUTHORS:Leslie ND#TITLE:Insights into the pathogenesis of galactosemia.#JOURNAL:Annu Rev Nutr#YEAR:2003#PUBMED:12704219# AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# GENE ASSOCIATION: (231.1) or (10327.2) or (10327.1)
Abbreviation: R_O2St Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: o2s[c] <==> o2s[e] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Archibald F.#TITLE:Oxygen toxicity and the health and survival of eukaryote cells: a new piece is added to the puzzle. #JOURNAL:Proc Natl Acad Sci U S A#YEAR:2003#PUBMED:12939409# GENE ASSOCIATION:
superoxide anion transport via diffusion (extracellular)
Abbreviation: R_EX_ump_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ump <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FA180ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + ocdcaACP <==> ACP + h + ocdca Confidence Level: 4 NCD genes: LOCUS:_55301#TRANSCRIPT:1#ABBREVIATION:THEDC1# proteins: NAME:s-acyl fatty acid synthase thioesterase#ABBREVIATION:Thedc1.1# citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION: (55301.1)
Abbreviation: R_RTOTAL2CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: Rtotal2crn[c] --> Rtotal2crn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (788.1)
Abbreviation: R_EX_utp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : utp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_COAtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: coa[c] <==> coa[l] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Seetharam B, Alpers DH.#TITLE:Absorption and transport of cobalamin (vitamin B12).#JOURNAL:Annu Rev Nutr.#YEAR:1982#PUBMED:6313022# GENE ASSOCIATION:
_5,10-methylenetetrahydrofolatereductase (NADPH)
Abbreviation: R_MTHFR3 Synonyms: _0 EC Number: 1.5.1.20 SUBSYSTEM: Folate Metabolism Equation: [c] : (2) h + mlthf + nadph --> 5mthf + nadp Confidence Level: 4 NCD genes: LOCUS:_4524#TRANSCRIPT:1#ABBREVIATION:MTHFR#ECNUMBERS:1.5.1.20# proteins: NAME:_5,10-methylenetetrahydrofolate reductase (NADPH) #ABBREVIATION:Mthfr1.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (4524.1)
Abbreviation: R_EX_fru_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fru <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GSNtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: gsn[l] <==> gsn[c] Confidence Level: 4 IT genes: LOCUS:_55315#TRANSCRIPT:1#ABBREVIATION:SLC29A3# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 3 #ABBREVIATION:Slc29a3.1-l# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Pisoni RL, Thoene JG.#TITLE:Detection and characterization of a nucleoside transport system in human fibroblast lysosomes.#JOURNAL:J Biol Chem.#YEAR:1989#PUBMED:2925670# AUTHORS:Baldwin SA, Yao SY, Hyde RJ, Ng AM, Foppolo S, Barnes K, Ritzel MW, Cass CE, Young JD.#TITLE:Functional characterization of novel human and mouse equilibrative nucleoside transporters (hENT3 and mENT3) located in intracellular membranes.#JOURNAL:J Biol Chem. #YEAR:2005#PUBMED:15701636# GENE ASSOCIATION: (55315.1)
guanosine facilated transport from lysosome
Abbreviation: R_Htm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h[c] --> h[m] Confidence Level: 4 TDV genes: LOCUS:_9016#TRANSCRIPT:1#ABBREVIATION:UCP5# LOCUS:_7350#TRANSCRIPT:1#ABBREVIATION:UCP1# LOCUS:_7351#TRANSCRIPT:1#ABBREVIATION:UCP2# LOCUS:_7352#TRANSCRIPT:1#ABBREVIATION:UCP3# LOCUS:_9481#TRANSCRIPT:1#ABBREVIATION:UCP4# LOCUS:_9016#TRANSCRIPT:2#ABBREVIATION:SLC25A14# LOCUS:_7352#TRANSCRIPT:2#ABBREVIATION:UCP3# proteins: NAME:Uncoupling protein 1, mitochondrial#ABBREVIATION:Ucp1.1-m# NAME:Uncoupling protein 2, mitochondrial#ABBREVIATION:Ucp2.1-m# NAME:Uncoupling protein 3 (long isoform), mitochondrial#ABBREVIATION:Ucp3.1-m# NAME:Uncoupling protein 3 (shorter isoform), mitochondrial#ABBREVIATION:Ucp3.2-m# NAME:Uncoupling protein 4, mitochondrial#ABBREVIATION:Ucp4.1-m# NAME:Uncoupling protein 5 (long isoform), mitochondrial#ABBREVIATION:Ucp5.1-m# NAME:Uncoupling protein (short isoform), mitochondrial#ABBREVIATION:Ucp5.2-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (9016.1) or (7352.2) or (7352.1) or (7351.1) or (9016.2) or (7350.1) or (9481.1)
Abbreviation: R_PMI1346PHn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + ppmi1346p --> h + mi13456p + pi Confidence Level: 4 NCD genes: LOCUS:_9807#TRANSCRIPT:1#ABBREVIATION:IHPK1# LOCUS:_117283#TRANSCRIPT:1#ABBREVIATION:IHPK3# LOCUS:_51447#TRANSCRIPT:1#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:3#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:2#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:5#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:4#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:6#ABBREVIATION:IHPK2# LOCUS:_9807#TRANSCRIPT:2#ABBREVIATION:IHPK1# proteins: NAME:inositol hexaphosphate kinase 1, nuclear#ABBREVIATION:Ihpk1.1-n# NAME:inositol hexaphosphate kinase 1#ABBREVIATION:Ihpk1.2-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.1-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.2-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.3-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.4-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.5-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.6-n# NAME:inositol hexaphosphate kinase 3 , nuclear#ABBREVIATION:Ihpk3.1-n# citations: AUTHORS:Saiardi A, Nagata E, Luo HR, Snowman AM, Snyder SH#TITLE:Identification and characterization of a novel inositol hexakisphosphate kinase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11502751# GENE ASSOCIATION: (117283.1) or (51447.4) or (9807.1) or (51447.1) or (51447.3) or (9807.2) or (51447.2) or (51447.5) or (51447.6)
diphosphoinositol-1,3,4,6-tetrakisphosphate diphosphohydrolase, nucleus
Abbreviation: R_CRNtuIR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: crn[e] --> crn[c] Confidence Level: 4 SAB genes: LOCUS:_6583#TRANSCRIPT:1#ABBREVIATION:SLC22A4# proteins: NAME:solute carrier family 22 (organic cation transporter), member 4#ABBREVIATION:Slc22a4.1# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# GENE ASSOCIATION: (6583.1)
Abbreviation: R_H4ETer Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : m3gacpail_hs + pe_hs --> dag_hs + m2emgacpail_hs Confidence Level: 2 NCD genes: LOCUS:_23556#TRANSCRIPT:1#ABBREVIATION:PIGN# LOCUS:_23556#TRANSCRIPT:2#ABBREVIATION:PIGN# proteins: NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.1-r# NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.2-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Hong Y, Maeda Y, Watanabe R, Ohishi K, Mishkind M, Riezman H, Kinoshita T#TITLE:Pig-n, a mammalian homologue of yeast Mcd4p, is involved in transferring phosphoethanolamine to the first mannose of the glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10574991# GENE ASSOCIATION: (23556.1) or (23556.2)
Abbreviation: R_TREHe Synonyms: _0 EC Number: 3.2.1.28 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [e] : h2o + tre --> (2) glc-D Confidence Level: 4 JLR genes: LOCUS:_11181#TRANSCRIPT:1#ABBREVIATION:TREH# proteins: NAME:trehalase (brush-border membrane glycoprotein), extracellular (sm intestine)#ABBREVIATION:Treh.1-e# citations: AUTHORS:Richards AB, Krakowka S, Dexter LB, Schmid H, Wolterbeek AP, Waalkens-Berendsen DH, Shigoyuki A, Kurimoto M#TITLE:Trehalose: a review of properties, history of use and human tolerance, and results of multiple safety studies#JOURNAL:Food Chem Toxicol#YEAR:2002#PUBMED:12065209# AUTHORS:Ishihara R, Taketani S, Sasai-Takedatsu M, Kino M, Tokunaga R, Kobayashi Y#TITLE:Molecular cloning, sequencing and expression of cDNA encoding human trehalase#JOURNAL:Gene#YEAR:1997#PUBMED:9427547# GENE ASSOCIATION: (11181.1)
Abbreviation: R_DURAD2 Synonyms: _0 EC Number: 1.3.1.2 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : 56dthm + nadp <==> h + nadph + thym Confidence Level: 4 _01-26-05 IT based on Lu et al., JBC,1992,24,17102-17109 genes: LOCUS:_1806#TRANSCRIPT:1#ABBREVIATION:DPYD#ECNUMBERS:1.3.1.2# proteins: NAME:dihydropyrimidine dehydrogenase #ABBREVIATION:DpyD.1# citations: AUTHORS:Lu Z, Zhang R, Diasio RB. #TITLE:Dihydropyrimidine dehydrogenase activity in human peripheral blood mononuclear cells and liver: population characteristics, newly identified deficient patients, and clinical implication in 5-fluorouracil chemotherapy. #JOURNAL:Cancer Res.#YEAR:1993#PUBMED:8221682# AUTHORS:Lu ZH, Zhang R, Diasio RB.#TITLE:Purification and characterization of dihydropyrimidine dehydrogenase from human liver.#JOURNAL:J Biol Chem. #YEAR:1992#PUBMED:1512248# GENE ASSOCIATION: (1806.1)
L-Phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase (4-hydroxylating)
Abbreviation: R_PHETHPTOX2 Synonyms: _0 EC Number: 1.14.16.1 SUBSYSTEM: Tyr, Phe, Trp Biosynthesis Equation: [c] : o2 + phe-L + thbpt --> thbpt4acam + tyr-L Confidence Level: 4 SAB unlumped form of PHETHPTOX according to PMID 10727395 genes: LOCUS:_5053#TRANSCRIPT:1#ABBREVIATION:PAH#ECNUMBERS:1.14.16.1# proteins: NAME:phenylalanine hydroxylase#ABBREVIATION:Pah.1# citations: AUTHORS:Waters PJ, Parniak MA, Nowacki P, Scriver CR#TITLE:In vitro expression analysis of mutations in phenylalanine hydroxylase: linking genotype to phenotype and structure to function#JOURNAL:Hum Mutat#YEAR:1998#PUBMED:9450897# AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (5053.1)
Abbreviation: R_FACOAL226i Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + crvnc --> amp + c226coa + ppi Confidence Level: 4 TV catalyzed by acyl-CoA synthetases genes: none proteins: none citations: none GENE ASSOCIATION:
N-Acetylneuraminate 9-phosphate pyruvate-lyase (pyruvate-phosphorylating)
Abbreviation: R_ACNAM9PL2 Synonyms: _0 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : h2o + man6p + pep --> kdnp + pi Confidence Level: 4 EC-2.5.1.56 NCD genes: LOCUS:_54187#TRANSCRIPT:1#ABBREVIATION:NANS# proteins: NAME:N-acetylneuraminic acid synthase (sialic acid synthase)#ABBREVIATION:Nans.1# citations: AUTHORS:Lawrence SM, Huddleston KA, Pitts LR, Nguyen N, Lee YC, Vann WF, Coleman TA, Betenbaugh MJ#TITLE:Cloning and expression of the human N-acetylneuraminic acid phosphate synthase gene with 2-keto-3-deoxy-D-glycero- D-galacto-nononic acid biosynthetic ability#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10749855# GENE ASSOCIATION: (54187.1)
Abbreviation: R_TSTSTERONESte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tststerones[c] <==> tststerones[e] Confidence Level: 1 unknown mechanism - presumed excretion of sulfonated testosterone NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HPYRDC Synonyms: _0 EC Number: 4.1.1.40 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : h + hpyr --> co2 + gcald Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Rofe AM, James HM, Bais R, Conyers RA#TITLE:Hepatic oxalate production: the role of hydroxypyruvate#JOURNAL:Biochem Med Metab Biol#YEAR:1986#PUBMED:3778681# GENE ASSOCIATION:
Abbreviation: R_ORNTArm Synonyms: _0 EC Number: 2.6.1.13 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : akg + orn <==> glu-L + glu5sa Confidence Level: 4 kh 1/7/03 SAB m genes: LOCUS:_4942#TRANSCRIPT:1#ABBREVIATION:OAT#ECNUMBERS:2.6.1.13# proteins: NAME:ornithine aminotransferase#ABBREVIATION:Oat.1-m# citations: AUTHORS:Shen BW, Hennig M, Hohenester E, Jansonius JN, Schirmer T#TITLE:Crystal structure of human recombinant ornithine aminotransferase#JOURNAL:J Mol Biol#YEAR:1998#PUBMED:9514741# GENE ASSOCIATION: (4942.1)
Abbreviation: R_EX_duri_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : duri <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FPGSm Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [m] : (4) atp + (4) glu-L + thf --> 5thf + (4) adp + (4) h + (4) pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_FUCGALGBSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucgalgbside_hs[c] <==> fucgalgbside_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_EX_dcsptn1_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dcsptn1 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
docosa-4,7,10,13,16-pentaenoic acid (n-6) exchange
Abbreviation: R_ASNNm Synonyms: _0 EC Number: 3.5.1.1 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [m] : asn-L + h2o --> asp-L + nh4 Confidence Level: 2 PMID: 11984834 (denotes it as potentially mitochondrial) mm genes: LOCUS:_80150#TRANSCRIPT:1#ABBREVIATION:ASRGL1# proteins: NAME:asparaginase 1 like protein#ABBREVIATION:Asrgl1.1-m# citations: AUTHORS:Bush LA, Herr JC, Wolkowicz M, Sherman NE, Shore A, Flickinger CJ. #TITLE:A novel asparaginase-like protein is a sperm autoantigen in rats.#YEAR:2002#PUBMED:11984834# GENE ASSOCIATION: (80150.1)
Abbreviation: R_CAATPS Synonyms: _0 EC Number: 3.6.3.6 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + (2) ca2[c] + h2o[c] --> adp[c] + (2) ca2[e] + h[e] + pi[c] Confidence Level: 4 plasma membrane version (SR and ER versions also exist) stoichiometry actually varies (number of Ca/ATP transported). See PMID: 7876199 NJ genes: LOCUS:_490#TRANSCRIPT:1#ABBREVIATION:ATP2B1#ECNUMBERS:3.6.1.38# LOCUS:_491#TRANSCRIPT:1#ABBREVIATION:ATP2B2#ECNUMBERS:3.6.1.38# LOCUS:_492#TRANSCRIPT:1#ABBREVIATION:ATP2B3#ECNUMBERS:3.6.1.38# LOCUS:_493#TRANSCRIPT:1#ABBREVIATION:ATP2B4#ECNUMBERS:3.6.1.38# proteins: NAME:ATPase, Ca transporting, plasma membrane 2#ABBREVIATION:Atp2b2.1# NAME:ATPase, Ca++ transporting, plasma membrane 4#ABBREVIATION:Atp2b4.1# NAME:ATPase, Ca++ transporting, plasma membrane 1#ABBREVIATION:Atp2b1.1# NAME:ATPase, Ca++ tranpsorting, plasma membrane 3#ABBREVIATION:Atp2b3.1# citations: AUTHORS:Hilfiker H, Strehler-Page MA, Stauffer TP, Carafoli E, Strehler EE.#TITLE:Structure of the gene encoding the human plasma membrane calcium pump isoform 1.#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8396145# AUTHORS:Yu X, Inesi G.#TITLE:Variable stoichiometric efficiency of Ca2+ and Sr2+ transport by the sarcoplasmic reticulum ATPase.#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7876199# GENE ASSOCIATION: (492.1) or (491.1) or (490.1) or (493.1)
Abbreviation: R_GALGALGALTHCRMte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: galgalgalthcrm_hs[c] <==> galgalgalthcrm_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_RTOT1 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : R1coa_hs --> Rtotalcoa Confidence Level: 0 User defined, tissue specific fluxes that can be customized w/ various ratios of R1 through R5 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_XOLESTte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: xolest_hs[e] <==> xolest_hs[c] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_UDPDOLPT_U Synonyms: _0 EC Number: 2.4.1.117 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dolp_U + udpg --> (0.1) dolglcp_U + udp Confidence Level: 0 NCD genes: LOCUS:_29880#TRANSCRIPT:1#ABBREVIATION:ALG5#ECNUMBERS:2.4.1.117# proteins: NAME:dolichyl-phosphate beta-glucosyltransferase (ubiquitous)#ABBREVIATION:Alg5.1# citations: AUTHORS:Imbach T, Burda P, Kuhnert P, Wevers RA, Aebi M, Berger EG, Hennet T.#TITLE:A mutation in the human ortholog of the Saccharomyces cerevisiae ALG6 gene causes carbohydrate-deficient glycoprotein syndrome type-Ic.#JOURNAL:Proc Natl Acad Sci USA#YEAR:1999#PUBMED:10359825# AUTHORS:Bossuyt X, Blanckaert N.#TITLE:Topology of nucleotide-sugar:dolichyl phosphate glycosyltransferases involved in the dolichol pathway for protein glycosylation in native rat liver microsomes#JOURNAL:Biochem J#YEAR:1993#PUBMED:8280060# GENE ASSOCIATION: (29880.1)
UDPglucose:dolichyl-phosphate beta-D-glucosyltransferase (uterus)
Abbreviation: R_CYTK12n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : dcmp + dctp <==> (2) dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_UDPGD Synonyms: _9 EC Number: 1.1.1.22 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : h2o + (2) nad + udpg --> (3) h + (2) nadh + udpglcur Confidence Level: 2 JLR genes: LOCUS:_7358#TRANSCRIPT:1#ABBREVIATION:UGDH#ECNUMBERS:1.1.1.22# proteins: NAME:UDP-glucose dehydrogenase#ABBREVIATION:Ugdh.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Spicer AP, Kaback LA, Smith TJ, Seldin MF#TITLE:Molecular cloning and characterization of the human and mouse UDP-glucose dehydrogenase genes#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9737970# AUTHORS:Banhegyi G, Braun L, Csala M, Puskas F, Mandl J#TITLE:Ascorbate metabolism and its regulation in animals#JOURNAL:Free Radic Biol Med#YEAR:1997#PUBMED:9296457# GENE ASSOCIATION: (7358.1)
Abbreviation: R_DHPR Synonyms: _0 SUBSYSTEM: Tetrahydrobiopterin Equation: [c] : dhbpt + h + nadh --> nad + thbpt Confidence Level: 4 IT EC 1.5.1.34 genes: LOCUS:_5860#TRANSCRIPT:1#ABBREVIATION:QDPR#ECNUMBERS:1.6.99.7# proteins: NAME:quinoid dihydropteridine reductase #ABBREVIATION:Qdpr1.1# citations: AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (5860.1)
Abbreviation: R_SGALSIDEtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: sgalside_hs[c] <==> sgalside_hs[l] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DNDPt53m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dctp[c] + dgdp[m] --> dctp[m] + dgdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_PNP Synonyms: _0 EC Number: 2.4.2.1 SUBSYSTEM: NAD Metabolism Equation: [c] : pi + rnam <==> h + ncam + r1p Confidence Level: 4 IT genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# citations: AUTHORS:Magni G, Amici A, Emanuelli M, Orsomando G, Raffaelli N, Ruggieri S.#TITLE:Enzymology of NAD+ homeostasis in man.#JOURNAL:Cell Mol Life Sci#YEAR:2004#PUBMED:14704851# AUTHORS:Wielgus-Kutrowska B, Kulikowska E, Wierzchowski J, Bzowska A, Shugar D.#TITLE:Nicotinamide riboside, an unusual, non-typical, substrate of purified purine-nucleoside phosphorylases.#JOURNAL:Eur J Biochem.#YEAR:1997#PUBMED:9030766# GENE ASSOCIATION: (4860.1)
Abbreviation: R_NRVNCCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: nrvnccrn[c] --> nrvnccrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_ATPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: atp[c] <==> atp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MI145PP Synonyms: _15 EC Number: 3.1.3.56 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi145p --> mi14p + pi Confidence Level: 4 NCD genes: LOCUS:_8867#TRANSCRIPT:1#ABBREVIATION:SYNJ1#ECNUMBERS:3.1.3.56# LOCUS:_3632#TRANSCRIPT:1#ABBREVIATION:INPP5A#ECNUMBERS:3.1.3.56# LOCUS:_3633#TRANSCRIPT:1#ABBREVIATION:INPP5B#ECNUMBERS:3.1.3.56# LOCUS:_3636#TRANSCRIPT:1#ABBREVIATION:INPPL1# LOCUS:_27124#TRANSCRIPT:1#ABBREVIATION:PIB5PA# LOCUS:_3635#TRANSCRIPT:1#ABBREVIATION:INPP5D# LOCUS:_56623#TRANSCRIPT:1#ABBREVIATION:INPP5E# LOCUS:_27124#TRANSCRIPT:2#ABBREVIATION:PIB5PA# LOCUS:_8867#TRANSCRIPT:2#ABBREVIATION:SYNJ1# proteins: NAME:synaptojanin 1#ABBREVIATION:Synj1.1# NAME:inositol polyphosphate-5-phosphatase, 75kDa#ABBREVIATION:Inpp5b.1# NAME:inositol polyphosphate-5-phosphatase, 72 kDa#ABBREVIATION:Inpp5e.1# NAME:synaptojanin 1#ABBREVIATION:Synj1.2# NAME:inositol polyphosphate-5-phosphatase, 40kDa#ABBREVIATION:Inpp5a.1# NAME:inositol polyphosphate-5-phosphatase, 145kDa#ABBREVIATION:Inpp5d.1# NAME:inositol polyphosphate phosphatase-like 1#ABBREVIATION:Inppl1.1# NAME:phosphatidylinositol (4,5) bisphosphate 5-phosphatase, A#ABBREVIATION:Pib5pa.1# NAME:phosphatidylinositol (4,5) bisphosphate 5-phosphatase, A#ABBREVIATION:Pib5pa.2# citations: AUTHORS:Haffner C, Takei K, Chen H, Ringstad N, Hudson A, Butler MH, Salcini AE, Di Fiore PP, De Camilli P#TITLE:Synaptojanin 1: localization on coated endocytic intermediates in nerve terminals and interaction of its 170 kDa isoform with Eps15#JOURNAL:FEBS Lett#YEAR:1997#PUBMED:9428629# AUTHORS:Stopkova P, Vevera J, Paclt I, Zukov I, Lachman HM#TITLE:Analysis of SYNJ1, a candidate gene for 21q22 linked bipolar disorder: a replication study#JOURNAL:Psychiatry Res#YEAR:2004#PUBMED:15261714# AUTHORS:Jefferson AB, Majerus PW#TITLE:Properties of type II inositol polyphosphate 5-phosphatase#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7721860# AUTHORS:Kisseleva MV, Wilson MP, Majerus PW#TITLE:The isolation and characterization of a cDNA encoding phospholipid-specific inositol polyphosphate 5-phosphatase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10764818# AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:De Smedt F, Verjans B, Mailleux P, Erneux C#TITLE:Cloning and expression of human brain type I inositol 1,4,5-trisphosphate 5-phosphatase. High levels of mRNA in cerebellar Purkinje cells#JOURNAL:FEBS Lett#YEAR:1994#PUBMED:8013665# AUTHORS:Laxminarayan KM, Chan BK, Tetaz T, Bird PI, Mitchell CA#TITLE:Characterization of a cDNA encoding the 43-kDa membrane-associated inositol-polyphosphate 5-phosphatase#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8006039# AUTHORS:Drayer AL, Pesesse X, De Smedt F, Woscholski R, Parker P, Erneux C#TITLE:Cloning and expression of a human placenta inositol 1,3,4,5-tetrakisphosphate and phosphatidylinositol 3,4,5-trisphosphate 5-phosphatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8769125# AUTHORS:Hejna JA, Saito H, Merkens LS, Tittle TV, Jakobs PM, Whitney MA, Grompe M, Friedberg AS, Moses RE#TITLE:Cloning and characterization of a human cDNA (INPPL1) sharing homology with inositol polyphosphate phosphatases#JOURNAL:Genomics#YEAR:1995#PUBMED:8530088# AUTHORS:Pesesse X, Moreau C, Drayer AL, Woscholski R, Parker P, Erneux C#TITLE:The SH2 domain containing inositol 5-phosphatase SHIP2 displays phosphatidylinositol 3,4,5-trisphosphate and inositol 1,3,4,5-tetrakisphosphate 5-phosphatase activity#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9824312# AUTHORS:Mochizuki Y, Takenawa T#TITLE:Novel inositol polyphosphate 5-phosphatase localizes at membrane ruffles#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10593988# GENE ASSOCIATION: (3635.1) or (8867.2) or (3636.1) or (27124.2) or (8867.1) or (27124.1) or (3633.1) or (56623.1) or (3632.1)
inositol-1,4,5-trisphosphate 5-phosphatase
Abbreviation: R_DESAT22_1p Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [x] : adrncoa + h + nadh + o2 --> dcsptn1coa + (2) h2o + nad Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present Sprecher "An update on the pathways of polyunsaturated fatty acid metabolism" among others describe peroxisomal localization (inner side) n-6 NJ genes: none proteins: none citations: AUTHORS:Sprecher H#TITLE:An update on the pathways of polyunsaturated fatty acid metabolism#JOURNAL:Curr Opn in Clinical Nutrition and Metabolic Care#YEAR:1999# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Ferdinandusse S, Denis S, Mooijer PAW, Zhang Z, Reddy JK, Spector AA, Wanders RJA#TITLE:Identification of the peroxisomal beta-oxidation enzymes involved in the biosynthesis of docosahexaenoic acid#JOURNAL:Journal of Lipid Research#YEAR:2001# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION:
fatty acyl-CoA desaturase (n-C22:4CoA -> n-C22:5CoA)
Abbreviation: R_C204CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : arachdcrn + coa --> arachdcoa + crn Confidence Level: 4 TDV C20:4 transport genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# GENE ASSOCIATION: (1376.1)
Abbreviation: R_PRGNLONEtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: prgnlone[c] <==> prgnlone[m] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLYBt4_LPAREN_2_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glyb[e] + (2) na1[e] <==> glyb[c] + (2) na1[c] Confidence Level: 4 SAB genes: LOCUS:_6539#TRANSCRIPT:1#ABBREVIATION:SLC6A12# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, betaine/GABA), member 12#ABBREVIATION:Slc6a12.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (6539.1)
Betaine transport (sodium symport) (2:1)
Abbreviation: R_G13MT_U Synonyms: _0 EC Number: 2.4.1.132 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : gdpmann + (0.1) mpdol_U --> gdp + h + (0.1) m1mpdol_U Confidence Level: 0 NCD genes: LOCUS:_85365#TRANSCRIPT:1#ABBREVIATION:ALG2# LOCUS:_85365#TRANSCRIPT:2#ABBREVIATION:ALG2# proteins: NAME:alpha-1,3-mannosyltransferase#ABBREVIATION:Alg2.1# NAME:asparagine-linked glycosylation 2 homolog (yeast, alpha-1,3-mannosyltransferase) #ABBREVIATION:Alg2.2# citations: none GENE ASSOCIATION: (85365.2) or (85365.1)
Glycolipid 1,3-alpha-D-mannosyltransferase (uterus)
Abbreviation: R_ADNtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: adn[l] <==> adn[c] Confidence Level: 4 IT genes: LOCUS:_55315#TRANSCRIPT:1#ABBREVIATION:SLC29A3# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 3 #ABBREVIATION:Slc29a3.1-l# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Pisoni RL, Thoene JG.#TITLE:Detection and characterization of a nucleoside transport system in human fibroblast lysosomes.#JOURNAL:J Biol Chem.#YEAR:1989#PUBMED:2925670# AUTHORS:Baldwin SA, Yao SY, Hyde RJ, Ng AM, Foppolo S, Barnes K, Ritzel MW, Cass CE, Young JD.#TITLE:Functional characterization of novel human and mouse equilibrative nucleoside transporters (hENT3 and mENT3) located in intracellular membranes.#JOURNAL:J Biol Chem. #YEAR:2005#PUBMED:15701636# GENE ASSOCIATION: (55315.1)
adenosine facilated transport from lysosome
Abbreviation: R_TMNDNCCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: tmndnccrn[c] --> tmndnccrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_B3GALTg Synonyms: _0 EC Number: 2.4.1.86 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acglcgalgluside_hs + udpgal --> galacglcgalgluside_hs + h + udp Confidence Level: 2 general reaction - no gene association get NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLYC3Ptm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: glyc3p[c] --> glyc3p[m] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LCAT1e Synonyms: _3 EC Number: 2.3.1.43 SUBSYSTEM: Fatty Acid Metabolism Equation: [e] : chsterol + pchol_hs --> lpchol_hs + xolest2_hs Confidence Level: 4 NJ genes: LOCUS:_3931#TRANSCRIPT:1#ABBREVIATION:LCAT#ECNUMBERS:2.3.1.43# proteins: NAME:lecithin-cholesterol acyltransferase#ABBREVIATION:Lcat.1# citations: AUTHORS:Krimbou L, Marcil M, Davignon J, Genest J Jr.#TITLE:Interaction of lecithin:cholesterol acyltransferase (LCAT).alpha 2-macroglobulin complex with low density lipoprotein receptor-related protein (LRP). Evidence for an alpha 2-macroglobulin/LRP receptor-mediated system participating in LCAT clearance.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11435418# AUTHORS:Wang K, Subbaiah PV.#TITLE:Role of the interfacial binding domain in the oxidative susceptibility of lecithin:cholesterol acyltransferase.#JOURNAL:Biochem J#YEAR:2002#PUBMED:11966470# GENE ASSOCIATION: (3931.1)
Abbreviation: R_FACOAL1813 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + elaid <==> amp + od2coa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_PYLALDOX Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Limonene and pinene degradation Equation: [c] : h2o + nad + pylald --> (2) h + nadh + peracd Confidence Level: 1 SAB genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# citations: none GENE ASSOCIATION: (8854.2) or (501.1) or (216.1) or (224.1) or (8854.3) or (223.1) or (8854.1)
Abbreviation: R_EX_thm_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : thm <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LYStm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h[m] + lys-L[c] <==> h[c] + lys-L[m] Confidence Level: 4 TDV genes: LOCUS:_83884#TRANSCRIPT:1#ABBREVIATION:SLC25A2# LOCUS:_10166#TRANSCRIPT:1#ABBREVIATION:SLC25A15# proteins: NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 2#ABBREVIATION:Slc25a2.1-m# NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15#ABBREVIATION:Slc25a15.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (83884.1) or (10166.1)
Lysine mitochondrial transport via ornithine carrier
Abbreviation: R_PTHPSn Synonyms: _0 EC Number: 4.2.3.12 SUBSYSTEM: Tetrahydrobiopterin Equation: [n] : ahdt --> 6pthp + pppi Confidence Level: 4 Formery EC 4.6.1.10; New EC 4.2.3.12 catalyses triphosphate elimination and an intramolecular redox reaction in the presence of Mg2+. It has been identified in human liver. The product is 6-pyruvoyltetrahydrobiopterin genes: LOCUS:_5805#TRANSCRIPT:1#ABBREVIATION:PTS#ECNUMBERS:4.6.1.10# proteins: NAME:_6-pyruvoyltetrahydropterin synthase #ABBREVIATION:Pts1.1-n# citations: AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (5805.1)
_6-pyruvoyltetrahydropterin synthase, nuclear
Abbreviation: R_GLCAT5g Synonyms: _1 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_e_pre2 + udpglcur --> cs_e_pre3 + h + udp Confidence Level: 4 EC 2.4.1.226 NCD genes: LOCUS:_22856#TRANSCRIPT:1#ABBREVIATION:CHSY1# LOCUS:_54480#TRANSCRIPT:1#ABBREVIATION:CSGlcA-T# LOCUS:_79586#TRANSCRIPT:1#ABBREVIATION:CHPF# LOCUS:_337876#TRANSCRIPT:1#ABBREVIATION:CSS3# proteins: NAME:chondroitin sulfate glucuronyltransferase, Golgi apparatus#ABBREVIATION:Csglca-t.1-g# NAME:carbohydrate (chondroitin) synthase 1, Golgi apparatus#ABBREVIATION:Chsy1.1-g# NAME:chondroitin polymerizing factor, Golgi apparatus#ABBREVIATION:Chpf.1-g# NAME:chondroitin sulfate synthase 3, Golgi apparatus#ABBREVIATION:Css3.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Gotoh M, Yada T, Sato T, Akashima T, Iwasaki H, Mochizuki H, Inaba N, Togayachi A, Kudo T, Watanabe H, Kimata K, Narimatsu H#TITLE:Molecular cloning and characterization of a novel chondroitin sulfate glucuronyltransferase that transfers glucuronic acid to N-acetylgalactosamine#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12145278# AUTHORS:Kitagawa H, Uyama T, Sugahara K#TITLE:Molecular cloning and expression of a human chondroitin synthase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11514575# AUTHORS:Kitagawa H, Izumikawa T, Uyama T, Sugahara K#TITLE:Molecular cloning of a chondroitin polymerizing factor that cooperates with chondroitin synthase for chondroitin polymerization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12716890# AUTHORS:Yada T, Sato T, Kaseyama H, Gotoh M, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-3. Molecular cloning and characterization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12907687# AUTHORS:Yada T, Gotoh M, Sato T, Shionyu M, Go M, Kaseyama H, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12761225# GENE ASSOCIATION: (54480.1) or (337876.1) or (79586.1) and (22856.1)
chondroitin glucuronyltransferase II, Golgi apparatus
chondroitin sulfate E (GalNAc4,6diS-GlcA), precursor 2_C31H45NO37S4X
Abbreviation: R_THMTPt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) oh1[e] + thmtp[c] --> (3) oh1[c] + thmtp[e] Confidence Level: 4 IT genes: LOCUS:_6573#TRANSCRIPT:1#ABBREVIATION:SLC19A1# LOCUS:_6573#TRANSCRIPT:2#ABBREVIATION:SLC19A1# proteins: NAME:solute carrier family 19 (folate transporter), member 1 #ABBREVIATION:Slc19a1.1# NAME:solute carrier family 19 (folate transporter), member 1#ABBREVIATION:Slc19a1.2# citations: AUTHORS:Dutta B, Huang W, Molero M, Kekuda R, Leibach FH, Devoe LD, Ganapathy V, Prasad PD. #TITLE:Cloning of the human thiamine transporter, a member of the folate transporter family.#JOURNAL:J Biol Chem. #YEAR:1999#PUBMED:10542220# AUTHORS:Ganapathy V, Smith SB, Prasad PD.#TITLE:SLC19: the folate/thiamine transporter family.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:14770311# GENE ASSOCIATION: (6573.1) or (6573.2)
Thiamine triphosphate transport in via anion antiport
Abbreviation: R_P4503A5 Synonyms: _3 SUBSYSTEM: CYP Metabolism Equation: [c] : aflatoxin + h + nadph + o2 --> eaflatoxin + h2o + nadp Confidence Level: 4 _8,9 epoxidation of aflatoxin assumed to take place on outer membrane NJ genes: LOCUS:_1577#TRANSCRIPT:1#ABBREVIATION:CYP3A5# proteins: NAME:cytochrome P450, family 3, subfamily A, polypeptide 5#ABBREVIATION:Cyp3a5.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# GENE ASSOCIATION: (1577.1)
Abbreviation: R_RETHe Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [e] : h2o + retfa --> Rtotal2 + h + retinol Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_H8TAer Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : gpi_hs + pre_prot --> gpi_prot_hs + gpi_sig Confidence Level: 4 EC 2.6.-.- NCD genes: LOCUS:_10026#TRANSCRIPT:1#ABBREVIATION:PIGK# LOCUS:_8733#TRANSCRIPT:1#ABBREVIATION:GPAA1# LOCUS:_94005#TRANSCRIPT:1#ABBREVIATION:PIGS# LOCUS:_128869#TRANSCRIPT:1#ABBREVIATION:CDC91L1# LOCUS:_51604#TRANSCRIPT:1#ABBREVIATION:PIGT# proteins: NAME:GPAA1P anchor attachment protein 1 homolog (yeast), endoplasmic reticulum#ABBREVIATION:Gpaa1.1-r# NAME:phosphatidylinositol glycan, class K, endoplasmic reticulum#ABBREVIATION:Pigk.1-r# NAME:phosphatidylinositol glycan, class S, endoplasmic reticulum#ABBREVIATION:Pigs.1-r# NAME:phosphatidylinositol glycan, class T, endoplasmic reticulum#ABBREVIATION:Pigt.1-r# NAME:CDC91 cell division cycle 91-like 1 (S. cerevisiae), endoplasmic reticulum#ABBREVIATION:Cdc91l1.1-r# citations: AUTHORS:Shishioh N, Hong Y, Ohishi K, Ashida H, Maeda Y, Kinoshita T#TITLE:GPI7 is the second partner of PIG-F and involved in modification of glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15632136# AUTHORS:Hiroi Y, Komuro I, Chen R, Hosoda T, Mizuno T, Kudoh S, Georgescu SP, Medof ME, Yazaki Y#TITLE:Molecular cloning of human homolog of yeast GAA1 which is required for attachment of glycosylphosphatidylinositols to proteins#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9468317# AUTHORS:Yu J, Nagarajan S, Knez JJ, Udenfriend S, Chen R, Medof ME#TITLE:The affected gene underlying the class K glycosylphosphatidylinositol (GPI) surface protein defect codes for the GPI transamidase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9356492# AUTHORS:Meyer U, Benghezal M, Imhof I, Conzelmann A#TITLE:Active site determination of Gpi8p, a caspase-related enzyme required for glycosylphosphatidylinositol anchor addition to proteins#JOURNAL:Biochemistry#YEAR:2000#PUBMED:10727241# AUTHORS:Ohishi K, Inoue N, Kinoshita T#TITLE:PIG-S and PIG-T, essential for GPI anchor attachment to proteins, form a complex with GAA1 and GPI8#JOURNAL:EMBO J#YEAR:2001#PUBMED:11483512# AUTHORS:Hong Y, Ohishi K, Kang JY, Tanaka S, Inoue N, Nishimura J, Maeda Y, Kinoshita T#TITLE:Human PIG-U and yeast Cdc91p are the fifth subunit of GPI transamidase that attaches GPI-anchors to proteins#JOURNAL:Mol Biol Cell#YEAR:2003#PUBMED:12802054# GENE ASSOCIATION: (10026.1) and (128869.1) and (8733.1) and (94005.1) and (51604.1)
acyl-dihydroxyacetone phosphate_C3H4O6PFULLRCO
Abbreviation: R_DHAPAx Synonyms: _4 EC Number: 2.3.1.42 SUBSYSTEM: Triacylglycerol Synthesis Equation: [x] : Rtotalcoa + dhap --> adhap_hs + coa Confidence Level: 4 NJ genes: LOCUS:_8443#TRANSCRIPT:1#ABBREVIATION:GNPAT#ECNUMBERS:2.3.1.15# proteins: NAME:glyceronephosphate O-acyltransferase#ABBREVIATION:Gnpat.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Coleman RA, Lee DP#TITLE:Enzymes of triacylglycerol synthesis and their regulation#JOURNAL:Prog in Lipid Research#YEAR:2004# AUTHORS:Thai TP, Heid H, Rackwitz HR, Hunziker A, Gorgas K, Just WW.#TITLE:Ether lipid biosynthesis: isolation and molecular characterization of human dihydroxyacetonephosphate acyltransferase.#JOURNAL:FEBS Lett.#YEAR:1997#PUBMED:9459311# GENE ASSOCIATION: (8443.1)
dihydroxyacetone phosphate acyltransferase
Abbreviation: R_FAOXC246226x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : coa + h2o + nad + o2 + tethex3coa --> accoa + c226coa + h + h2o2 + nadh Confidence Level: 4 peroxisomal (very long chain FA) - - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) c24:6 -> c22:6 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1) or (51.1 and 30.1 and 1962.1)
Abbreviation: R_HOMt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hom-L[e] + na1[e] --> hom-L[c] + na1[c] Confidence Level: 4 SAB genes: LOCUS:_84889#TRANSCRIPT:1#ABBREVIATION:SLC7A3# LOCUS:_6542#TRANSCRIPT:1#ABBREVIATION:SLC7A2# LOCUS:_6541#TRANSCRIPT:1#ABBREVIATION:SLC7A1# proteins: NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 1#ABBREVIATION:Slc7a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 2#ABBREVIATION:Slc7a2.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 3#ABBREVIATION:Slc7a3.1# citations: AUTHORS:Albritton LM, Bowcock AM, Eddy RL, Morton CC, Tseng L, Farrer LA, Cavalli-Sforza LL, Shows TB, Cunningham JM#TITLE:The human cationic amino acid transporter (ATRC1): physical and genetic mapping to 13q12-q14#JOURNAL:Genomics#YEAR:1992#PUBMED:1348489# AUTHORS:Cariappa R, Heath-Monnig E, Furesz TC, Kamath SG, Smith CH#TITLE:Stable polarized expression of hCAT-1 in an epithelial cell line#JOURNAL:J Membr Biol#YEAR:2002#PUBMED:11891586# AUTHORS:Hoshide R, Ikeda Y, Karashima S, Matsuura T, Komaki S, Kishino T, Niikawa N, Endo F, Matsuda I#TITLE:Molecular cloning, tissue distribution, and chromosomal localization of human cationic amino acid transporter 2 (HCAT2)#JOURNAL:Genomics#YEAR:1996#PUBMED:8954799# AUTHORS:Furesz TC, Moe AJ, Smith CH#TITLE:Lysine uptake by human placental microvillous membrane: comparison of system y+ with basal membrane#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7534987# AUTHORS:Wolf S, Janzen A, Vekony N, Martine U, Strand D, Closs EI#TITLE:Expression of solute carrier 7A4 (SLC7A4) in the plasma membrane is not sufficient to mediate amino acid transport activity#JOURNAL:Biochem J#YEAR:2002#PUBMED:12049641# AUTHORS:Vekony N, Wolf S, Boissel JP, Gnauert K, Closs EI#TITLE:Human cationic amino acid transporter hCAT-3 is preferentially expressed in peripheral tissues#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11591158# AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (6541.1) or (84889.1) or (6542.1)
Abbreviation: R_PPAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ppa[e] --> ppa[c] Confidence Level: 2 TDV genes: none proteins: none citations: AUTHORS:Tassani V, Cattapan F, Magnanimi L, Peschechera A.#TITLE:Anaplerotic effect of propionyl carnitine in rat heart mitochondria.#JOURNAL:Biochem Biophys Res Commun#YEAR:1994#PUBMED:8135845# GENE ASSOCIATION:
Abbreviation: R_RAI4 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : oretn <==> 13-cis-oretn Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_ARTFR46 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + od2coa[c] --> (1.125) R4coa_hs[c] + fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GASNASE2ly Synonyms: _8 EC Number: 3.5.1.26 SUBSYSTEM: N-Glycan Degradation Equation: [l] : h2o + n2m2nmasn --> Asn-X-Ser/Thr + n2m2nm Confidence Level: 0 NCD genes: LOCUS:_175#TRANSCRIPT:1#ABBREVIATION:AGA#ECNUMBERS:3.5.1.26# proteins: NAME:aspartylglucosaminidase, lysosomal#ABBREVIATION:Aga.1-l# citations: none GENE ASSOCIATION: (175.1)
Abbreviation: R_AG13T1g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre2 + uacgam --> h + ksii_core2_pre3 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (79369.1) or (374907.1) or (11041.1) or (284208.1) or (374907.2) or (93010.1) or (10331.1) or (10678.1) or (10678.2)
Abbreviation: R_B3GALT5g Synonyms: _6 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gbside_hs + udpgal --> galgbside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10317#TRANSCRIPT:1#ABBREVIATION:B3GALT5# LOCUS:_10317#TRANSCRIPT:3#ABBREVIATION:B3GALT5# LOCUS:_10317#TRANSCRIPT:2#ABBREVIATION:B3GALT5# LOCUS:_10317#TRANSCRIPT:4#ABBREVIATION:B3GALT5# LOCUS:_10317#TRANSCRIPT:5#ABBREVIATION:B3GALT5# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 5#ABBREVIATION:B3galt5.1# NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 5#ABBREVIATION:B3galt5.2# NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 5#ABBREVIATION:B3galt5.3# NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 5#ABBREVIATION:B3galt5.4# NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 5#ABBREVIATION:B3galt5.5# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Amado M, Almeida R, Carneiro F, Levery ST, Holmes EH, Nomoto M, Hollingsworth MA, Hassan H, Schwientek T, Nielsen PA, Bennett EP, Clausen H#TITLE:A family of human beta3-galactosyltransferases#JOURNAL:The Journal of Biological Chemistry#YEAR:1998# GENE ASSOCIATION: (10317.4) or (10317.1) or (10317.3) or (10317.2) or (10317.5)
Abbreviation: R_GLYATm Synonyms: _0 EC Number: 2.3.1.29 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : accoa + gly <==> 2aobut + coa Confidence Level: 2 genes: LOCUS:_23464#TRANSCRIPT:1#ABBREVIATION:GCAT#ECNUMBERS:2.3.1.29# proteins: NAME:glycine C-acetyltransferase (2-amino-3-ketobutyrate coenzyme A ligase), mitochondrial #ABBREVIATION:Gcat.1-m# citations: AUTHORS:Edgar,A.J. , Polak,J.M. #TITLE:Molecular cloning of the human and murine 2-amino-3-ketobutyrate coenzyme A ligase cDNAs.#YEAR:2000#PUBMED:10712613# GENE ASSOCIATION: (23464.1)
Abbreviation: R_GABAVESSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) 4abut[c] + atp[c] + h2o[c] --> (3) 4abut[e] + adp[c] + h[c] + pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of glu from the cell. It leaves out the vesicle because a compartment is not available for that. ATP demand is not known for sure stoichiometrically, but 1:1 H coupling ratio is assumed, which could translate to 3 GABA/ATP. genes: LOCUS:_140679#TRANSCRIPT:1#ABBREVIATION:SLC32A1# proteins: NAME:solute carrier family 32 (GABA vesicular transporter), member 1#ABBREVIATION:Slc32a1.1# citations: AUTHORS:Gasnier B#TITLE:The SLC32 transporter, a key protein for the synaptic release of inhibitory amino acids#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750892# AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# GENE ASSOCIATION: (140679.1)
GABA secretion via secretory vesicle (ATP driven)
heparan sulfate, degradation product 14_C57H84N3O62S5
Abbreviation: R_GLCNACASE3ly Synonyms: _4 EC Number: 3.2.1.50 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg14 --> acgam + hs_deg15 Confidence Level: 4 NCD genes: LOCUS:_4669#TRANSCRIPT:1#ABBREVIATION:NAGLU#ECNUMBERS:3.2.1.50# proteins: NAME:N-acetylglucosaminidase, alpha- (Sanfilippo disease IIIB), lysosome#ABBREVIATION:Naglu.1-l# citations: AUTHORS:Weber B, Blanch L, Clements PR, Scott HS, Hopwood JJ#TITLE:Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B)#JOURNAL:Hum Mol Genet#YEAR:1996#PUBMED:8776591# AUTHORS:Sasaki T, Sukegawa K, Masue M, Fukuda S, Tomatsu S, Orii T#TITLE:Purification and partial characterization of alpha-N-acetylglucosaminidase from human liver#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1783617# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION: (4669.1)
Abbreviation: R_H6MTer_U Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_U + m2emgacpail_hs --> (0.1) dolp_U + h + m3emgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_80235#TRANSCRIPT:1#ABBREVIATION:SMP3# proteins: NAME:SMP3 mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Smp3.1-r# citations: AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (80235.1)
H6 mannosyltransferase, endoplasmic reticulum
Abbreviation: R_HSPGt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hspg[g] --> hspg[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
heparan sulfate transport, golgi to extracellular
Abbreviation: R_UGT1A5r2 Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Vitamin A Metabolism Equation: [r] : 13-cis-retn + udpglcur --> 13-cis-retnglc + udp Confidence Level: 2 NJ genes: LOCUS:_54575#TRANSCRIPT:1#ABBREVIATION:UGT1A10# LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A10#ABBREVIATION:Ugt1a10.1# NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Barua AB, Sidell N.#TITLE:Retinoyl beta-glucuronide: a biologically active interesting retinoid.#JOURNAL:J Nutr.#YEAR:2004#PUBMED:14704335# GENE ASSOCIATION: (54576.1) or (54575.1)
UDP-glucuronosyltransferase 1-10 precursor, microsomal (13-cis)
Abbreviation: R_FAEL205 Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : (5) h + malcoa + (4) nadph + o2 + tmndnccoa --> clpndcoa + co2 + coa + (3) h2o + (4) nadp Confidence Level: 4 fatty acid elongation step NJ genes: LOCUS:_6785#TRANSCRIPT:1#ABBREVIATION:ELOVL4# proteins: NAME:elongation of very long chain fatty acids - like 4#ABBREVIATION:Elovl4.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Zhang K, Kniazeva M, Han M, Li W, Yu Z, Yang Z, Li Y, Metzker ML, Allikmets R, Zack DJ, Kakuk LE, Lagali PS, Wong PW, MacDonald IM, Sieving PA, Figueroa DJ, Austin CP, Gould RJ, Ayyagari R, Petrukhin K.#TITLE:A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy.#JOURNAL:Nat Genet#YEAR:2001#PUBMED:11138005# AUTHORS:Nakamura MT, Nara TY.#TITLE:Structure, function, and dietary regulation of delta6, delta5, and delta9 desaturases.#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189125# GENE ASSOCIATION: (6785.1)
Acetyl-CoA:aralkylamine N-acetyltransferase
Abbreviation: R_SRTNACT Synonyms: _1 EC Number: 2.3.1.87 SUBSYSTEM: Tryptophan metabolism Equation: [c] : accoa + srtn --> Nacsertn + coa + h Confidence Level: 4 SAB genes: LOCUS:_15#TRANSCRIPT:1#ABBREVIATION:AANAT#ECNUMBERS:2.3.1.87# proteins: NAME:arylalkylamine N-acetyltransferase#ABBREVIATION:Aanat.1# citations: AUTHORS:Coon SL, Mazuruk K, Bernard M, Roseboom PH, Klein DC, Rodriguez IR#TITLE:The human serotonin N-acetyltransferase (EC 2.3.1.87) gene (AANAT): structure, chromosomal localization, and tissue expression#JOURNAL:Genomics#YEAR:1996#PUBMED:8661026# GENE ASSOCIATION: (15.1)
Abbreviation: R_NDPK4n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + dtdp <==> adp + dttp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:dTDP), nuclear
Abbreviation: R_GND Synonyms: _0 EC Number: 1.1.1.44 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : 6pgc + nadp --> co2 + nadph + ru5p-D Confidence Level: 4 SMP Note no proton generation by the the reaction genes: LOCUS:_5226#TRANSCRIPT:1#ABBREVIATION:PGD#ECNUMBERS:1.1.1.43# proteins: NAME:phosphogluconate dehydrogenase#ABBREVIATION:Pgd.1# citations: AUTHORS:Tsui SK, Chan JY, Waye MM, Fung KP, Lee CY#TITLE:Identification of a cDNA encoding 6-phosphogluconate dehydrogenase from a human heart cDNA library.#JOURNAL:Biochem Genet#YEAR:1996#PUBMED:8978909# GENE ASSOCIATION: (5226.1)
Abbreviation: R_ILETAm Synonyms: _0 EC Number: 2.6.1.42 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : akg + ile-L <==> 3mop + glu-L Confidence Level: 4 NCD genes: LOCUS:_587#TRANSCRIPT:1#ABBREVIATION:BCAT2#ECNUMBERS:2.6.1.26# proteins: NAME:branched chain aminotransferase 2, mitochondrial #ABBREVIATION:Bcat2.1-m# citations: AUTHORS:Bledsoe RK, Dawson PA, Hutson SM.#TITLE:Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm). #YEAR:1997#PUBMED:9165094# AUTHORS:Naylor SL, Shows TB.#TITLE:Branched-chain aminotransferase deficiency in Chinese hamster cells complemented by two independent genes on human chromosomes 12 and 19. #YEAR:1980#PUBMED:6933702# GENE ASSOCIATION: (587.1)
beta-1,4-mannosyl-glycoprotein 4-beta-N-acetylglucosaminyltransferase
Abbreviation: R_M14NTg Synonyms: _4 EC Number: 2.4.1.144 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : n2m2masn + uacgam --> h + n2m2nmasn + udp Confidence Level: 0 NCD genes: LOCUS:_4248#TRANSCRIPT:1#ABBREVIATION:MGAT3#ECNUMBERS:2.4.1.144# proteins: NAME:mannosyl (beta-1,4-)-glycoprotein beta-1,4-N-acetylglucosaminyltransferase, Golgi apparatus#ABBREVIATION:Mgat3.1-g# citations: none GENE ASSOCIATION: (4248.1)
Abbreviation: R_FBA5 Synonyms: _0 EC Number: 4.1.2.13 SUBSYSTEM: Galactose metabolism Equation: [c] : tag1p-D <==> dhap + glyald Confidence Level: 4 NCD genes: LOCUS:_229#TRANSCRIPT:1#ABBREVIATION:ALDOB#ECNUMBERS:4.1.2.13# proteins: NAME:aldolase B, fructose-bisphosphate (liver)#ABBREVIATION:Aldob.1# citations: AUTHORS:Bais R, James HM, Rofe AM, Conyers RA.#TITLE:The purification and properties of human liver ketohexokinase. A role for ketohexokinase and fructose-bisphosphate aldolase in the metabolic production of oxalate from xylitol#JOURNAL:Biochem J#YEAR:1985#PUBMED:2996495# AUTHORS:Barngrover DA, Dills WL Jr.#TITLE:The involvement of liver fructokinase in the metabolism of D-xylulose and xylitol in isolated rat hepatocytes#JOURNAL:J Nutr#YEAR:1983#PUBMED:6298387# AUTHORS:James HM, Bais R, Edwards JB, Rofe AM, Conyers AJ.#TITLE:Models for the metabolic production of oxalate from xylitol in humans: a role for fructokinase and aldolase.#JOURNAL:Aust J Exp Biol Med Sci#YEAR:1982#PUBMED:6284103# GENE ASSOCIATION: (229.1)
D-Tagatose 1-phosphate D-glyceraldehyde-3-phosphate-lyase
Abbreviation: R_CYTDtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: cytd[c] <==> cytd[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NDPK8 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + dadp <==> adp + datp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.2) or (10201.1) or (4831.1 and 4830.1) or (4832.1) or (4831.1 and 4830.2) or (29922.1)
nucleoside-diphosphate kinase (ATP:dADP)
Abbreviation: R_PCm Synonyms: _0 EC Number: 6.4.1.1 SUBSYSTEM: Pyruvate Metabolism Equation: [m] : atp + hco3 + pyr --> adp + h + oaa + pi Confidence Level: 4 TV Metabolism at a glance genes: LOCUS:_5091#TRANSCRIPT:1#ABBREVIATION:PC#ECNUMBERS:6.4.1.1# LOCUS:_5091#TRANSCRIPT:2#ABBREVIATION:PC#ECNUMBERS:6.4.1.1# proteins: NAME:pyruvate carboxylase, mitochondrial (liver, kidney, intestine)#ABBREVIATION:Pc.1-m# NAME:pyruvate carboxylase, mitochondrial (liver, kidney, intestine)#ABBREVIATION:Pc.2-m# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Freytag SO, Collier KJ.#TITLE:Molecular cloning of a cDNA for human pyruvate carboxylase. Structural relationship to other biotin-containing carboxylases and regulation of mRNA content in differentiating preadipocytes.#JOURNAL:J Biol Chem.#YEAR:1984#PUBMED:6548474# GENE ASSOCIATION: (5091.1) or (5091.2)
Abbreviation: R_ACN13ACNGALGBSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acn13acngalgbside_hs[c] <==> acn13acngalgbside_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
acn13acngalgbside_hs intracellular transport
Abbreviation: R_ALDD2y Synonyms: _0 EC Number: 1.2.1.4 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : acald + h2o + nadp --> ac + (2) h + nadph Confidence Level: 0 See also ALDH1 (1.2.1.3) genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Sladek NE#TITLE:Human aldehyde dehydrogenases: potential pathological, pharmacological, and toxicological impact#JOURNAL:J Biochem Mol Toxicol#YEAR:2003#PUBMED:12616643# GENE ASSOCIATION: (223.1) or (221.1) or (218.1) or (8854.1) or (8854.3) or (222.1) or (8854.2) or (224.1)
aldehyde dehydrogenase (acetaldehyde, NADP)
Abbreviation: R_NNMT Synonyms: _0 EC Number: 2.1.1.1 SUBSYSTEM: NAD Metabolism Equation: [c] : amet + ncam --> 1mncam + ahcys Confidence Level: 4 IT genes: LOCUS:_4837#TRANSCRIPT:1#ABBREVIATION:NNMT#ECNUMBERS:2.1.1.1# proteins: NAME:nicotinamide N-methyltransferase #ABBREVIATION:Nnmt.1# citations: AUTHORS:Aksoy S, Szumlanski CL, Weinshilboum RM.#TITLE:Human liver nicotinamide N-methyltransferase. cDNA cloning, expression, and biochemical characterization. #JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8182091# GENE ASSOCIATION: (4837.1)
Abbreviation: R_MALTe Synonyms: _0 EC Number: 3.2.1.20 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [e] : h2o + malt --> (2) glc-D Confidence Level: 4 IF genes: LOCUS:_8972#TRANSCRIPT:1#ABBREVIATION:MGAM# proteins: NAME:maltase-glucoamylase (alpha-glucosidase), extracellular (intestine)#ABBREVIATION:Mgam.1-e# citations: AUTHORS:Naim HY, Sterchi EE, Lentze MJ#TITLE:Structure, biosynthesis, and glycosylation of human small intestinal maltase-glucoamylase#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3143729# GENE ASSOCIATION: (8972.1)
Abbreviation: R_MI1345PP Synonyms: _15 EC Number: 3.1.3.56 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi1345p --> mi134p + pi Confidence Level: 4 NCD genes: LOCUS:_8867#TRANSCRIPT:1#ABBREVIATION:SYNJ1#ECNUMBERS:3.1.3.56# LOCUS:_3632#TRANSCRIPT:1#ABBREVIATION:INPP5A#ECNUMBERS:3.1.3.56# LOCUS:_3633#TRANSCRIPT:1#ABBREVIATION:INPP5B#ECNUMBERS:3.1.3.56# LOCUS:_3636#TRANSCRIPT:1#ABBREVIATION:INPPL1# LOCUS:_27124#TRANSCRIPT:1#ABBREVIATION:PIB5PA# LOCUS:_3635#TRANSCRIPT:1#ABBREVIATION:INPP5D# LOCUS:_56623#TRANSCRIPT:1#ABBREVIATION:INPP5E# LOCUS:_27124#TRANSCRIPT:2#ABBREVIATION:PIB5PA# LOCUS:_8867#TRANSCRIPT:2#ABBREVIATION:SYNJ1# proteins: NAME:synaptojanin 1#ABBREVIATION:Synj1.1# NAME:inositol polyphosphate-5-phosphatase, 75kDa#ABBREVIATION:Inpp5b.1# NAME:inositol polyphosphate-5-phosphatase, 72 kDa#ABBREVIATION:Inpp5e.1# NAME:synaptojanin 1#ABBREVIATION:Synj1.2# NAME:inositol polyphosphate-5-phosphatase, 40kDa#ABBREVIATION:Inpp5a.1# NAME:inositol polyphosphate-5-phosphatase, 145kDa#ABBREVIATION:Inpp5d.1# NAME:inositol polyphosphate phosphatase-like 1#ABBREVIATION:Inppl1.1# NAME:phosphatidylinositol (4,5) bisphosphate 5-phosphatase, A#ABBREVIATION:Pib5pa.1# NAME:phosphatidylinositol (4,5) bisphosphate 5-phosphatase, A#ABBREVIATION:Pib5pa.2# citations: AUTHORS:Haffner C, Takei K, Chen H, Ringstad N, Hudson A, Butler MH, Salcini AE, Di Fiore PP, De Camilli P#TITLE:Synaptojanin 1: localization on coated endocytic intermediates in nerve terminals and interaction of its 170 kDa isoform with Eps15#JOURNAL:FEBS Lett#YEAR:1997#PUBMED:9428629# AUTHORS:Stopkova P, Vevera J, Paclt I, Zukov I, Lachman HM#TITLE:Analysis of SYNJ1, a candidate gene for 21q22 linked bipolar disorder: a replication study#JOURNAL:Psychiatry Res#YEAR:2004#PUBMED:15261714# AUTHORS:Jefferson AB, Majerus PW#TITLE:Properties of type II inositol polyphosphate 5-phosphatase#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7721860# AUTHORS:Kisseleva MV, Wilson MP, Majerus PW#TITLE:The isolation and characterization of a cDNA encoding phospholipid-specific inositol polyphosphate 5-phosphatase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10764818# AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:De Smedt F, Verjans B, Mailleux P, Erneux C#TITLE:Cloning and expression of human brain type I inositol 1,4,5-trisphosphate 5-phosphatase. High levels of mRNA in cerebellar Purkinje cells#JOURNAL:FEBS Lett#YEAR:1994#PUBMED:8013665# AUTHORS:Laxminarayan KM, Chan BK, Tetaz T, Bird PI, Mitchell CA#TITLE:Characterization of a cDNA encoding the 43-kDa membrane-associated inositol-polyphosphate 5-phosphatase#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8006039# AUTHORS:Drayer AL, Pesesse X, De Smedt F, Woscholski R, Parker P, Erneux C#TITLE:Cloning and expression of a human placenta inositol 1,3,4,5-tetrakisphosphate and phosphatidylinositol 3,4,5-trisphosphate 5-phosphatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8769125# AUTHORS:Hejna JA, Saito H, Merkens LS, Tittle TV, Jakobs PM, Whitney MA, Grompe M, Friedberg AS, Moses RE#TITLE:Cloning and characterization of a human cDNA (INPPL1) sharing homology with inositol polyphosphate phosphatases#JOURNAL:Genomics#YEAR:1995#PUBMED:8530088# AUTHORS:Pesesse X, Moreau C, Drayer AL, Woscholski R, Parker P, Erneux C#TITLE:The SH2 domain containing inositol 5-phosphatase SHIP2 displays phosphatidylinositol 3,4,5-trisphosphate and inositol 1,3,4,5-tetrakisphosphate 5-phosphatase activity#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9824312# AUTHORS:Mochizuki Y, Takenawa T#TITLE:Novel inositol polyphosphate 5-phosphatase localizes at membrane ruffles#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10593988# GENE ASSOCIATION: (3635.1) or (8867.2) or (3636.1) or (27124.2) or (8867.1) or (27124.1) or (3633.1) or (56623.1) or (3632.1)
inositol-1,3,4,5-trisphosphate 5-phosphatase
Abbreviation: R_GSNKm Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [m] : atp + gsn --> adp + gmp + h Confidence Level: 1 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UGT1A2r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Steroid Metabolism Equation: [r] : bilirub + (2) udpglcur --> bildglcur + (2) udp Confidence Level: 4 NJ genes: LOCUS:_54575#TRANSCRIPT:1#ABBREVIATION:UGT1A10# LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A10#ABBREVIATION:Ugt1a10.1# NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# citations: AUTHORS:Basu NK, Kubota S, Meselhy MR, Ciotti M, Chowdhury B, Hartori M, Owens IS.#TITLE:Free Full Text Gastrointestinally distributed UDP-glucuronosyltransferase 1A10, which metabolizes estrogens and nonsteroidal anti-inflammatory drugs, depends upon phosphorylation.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117964# AUTHORS:Mackenzie PI, Owens IS, Burchell B, Bock KW, Bairoch A, Belanger A, Fournel-Gigleux S, Green M, Hum DW, Iyanagi T, Lancet D, Louisot P, Magdalou J, Chowdhury JR, Ritter JK, Schachter H, Tephly TR, Tipton KF, Nebert DW.#TITLE:The UDP glycosyltransferase gene superfamily: recommended nomenclature update based on evolutionary divergence.#JOURNAL:Pharmacogenetics#YEAR:1997#PUBMED:9295054# GENE ASSOCIATION: (54575.1) or (54576.1)
Abbreviation: R_DNDPt63m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[m] + dgtp[c] --> dcdp[c] + dgtp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_GGT5r Synonyms: _3 EC Number: 2.3.2.2 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : h2o + leuktrC4 <==> glu-L + leuktrD4 Confidence Level: 4 Cleaves the gamma-glutamyl peptide bond of glutathione conjugates, but maybe not glutathione itself. Converts leukotriene C4 (LTC4) to leukotriene D4 (LTD4). NJ genes: LOCUS:_2687#TRANSCRIPT:1#ABBREVIATION:GGTLA1# proteins: NAME:gamma-glutamyltransferase-like activity 1#ABBREVIATION:Ggtla1.1# citations: TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# GENE ASSOCIATION: (2687.1)
_17-beta-hydroxysteroid dehydrogenase (type 7)
Abbreviation: R_HSD17B9r Synonyms: _0 SUBSYSTEM: Steroid Metabolism Equation: [r] : andrstrn + h + nadph + (1.5) o2 --> eandrstrn + (2) h2o + nadp Confidence Level: 2 see PMID: 14643063 inferred use of O2 for oxidation - biochemical details have not yet been experimentally elucidated NJ genes: LOCUS:_51478#TRANSCRIPT:1#ABBREVIATION:HSD17B7#ECNUMBERS:1.1.1.62# proteins: NAME:hydroxysteroid (17-beta) dehydrogenase 7#ABBREVIATION:Hsd17b7.1# citations: AUTHORS:Belanger A, Pelletier G, Labrie F, Barbier O, Chouinard S.#TITLE:Inactivation of androgens by UDP-glucuronosyltransferase enzymes in humans.#JOURNAL:Trends Endocrinol Metab.#YEAR:2003#PUBMED:14643063# GENE ASSOCIATION: (51478.1)
Abbreviation: R_GGH_DASH_7DHFe Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [e] : 7dhf + h2o --> 6dhf + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-e# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (7DHF), extracellular
chondroitin sulfate D (GlcNAc6S-GlcA2S), precursor 4_C45H66N2O45S3X
chondroitin sulfate D (GlcNAc6S-GlcA2S), precursor 5_C45H65N2O48S4X
Abbreviation: R_S6T21g Synonyms: _3 EC Number: 2.8.2.17 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_d_pre4 + paps --> cs_d_pre5 + h + pap Confidence Level: 4 NCD genes: LOCUS:_56548#TRANSCRIPT:1#ABBREVIATION:CHST7# LOCUS:_9469#TRANSCRIPT:1#ABBREVIATION:CHST3# proteins: NAME:carbohydrate (chondroitin 6) sulfotransferase 3, Golgi#ABBREVIATION:Chst3.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 7, Golgi apparatus#ABBREVIATION:Chst7.1-g# citations: AUTHORS:Habuchi O, Hirahara Y, Uchimura K, Fukuta M#TITLE:Enzymatic sulfation of galactose residue of keratan sulfate by chondroitin 6-sulfotransferase#JOURNAL:Glycobiology#YEAR:1996#PUBMED:8991509# AUTHORS:Fukuta M, Kobayashi Y, Uchimura K, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of human chondroitin 6-sulfotransferase#JOURNAL:Biochim Biophys Acta#YEAR:1998#PUBMED:9714738# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Tsutsumi K, Shimakawa H, Kitagawa H, Sugahara K#TITLE:Functional expression and genomic structure of human chondroitin 6-sulfotransferase#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9883891# AUTHORS:Kitagawa H, Fujita M, Ito N, Sugahara K#TITLE:Molecular cloning and expression of a novel chondroitin 6-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781596# AUTHORS:Bhakta S, Bartes A, Bowman KG, Kao WM, Polsky I, Lee JK, Cook BN, Bruehl RE, Rosen SD, Bertozzi CR, Hemmerich S#TITLE:Sulfation of N-acetylglucosamine by chondroitin 6-sulfotransferase 2 (GST-5)#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10956661# GENE ASSOCIATION: (9469.1) or (56548.1)
Abbreviation: R_PYRt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h[c] + pyr[c] <==> h[m] + pyr[m] Confidence Level: 0 IF changed name from "tm" to "t2m" - NCD genes: LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1 #ABBREVIATION:Slc16a1.1-m# citations: AUTHORS:Halestrap AP, Scott RD, Thomas AP#TITLE:Mitochondrial pyruvate transport and its hormonal regulation#JOURNAL:Int J Biochem#YEAR:1980#PUBMED:6987111# AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (6566.1)
pyruvate mitochondrial transport via proton symport
Abbreviation: R_UMPK3 Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : ump + utp <==> (2) udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_ARTFR13 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : tdcoa --> (0.875) R1coa_hs Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups - add C14 here too ... R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DHCR242r Synonyms: _3 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : chlstol + fadh2 --> fad + lthstrl Confidence Level: 4 lanosterol -> cholesterol pathway FAD known to be cofactor, other details missing NJ genes: LOCUS:_1718#TRANSCRIPT:1#ABBREVIATION:DHCR24# proteins: NAME:_24-dehydrocholesterol reductase#ABBREVIATION:Dhcr24.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (1718.1)
_24-dehydrocholesterol reductase [Precursor]
Abbreviation: R_NACHEX22ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg41 --> acgam + m2mn Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3074.1) or (3073.1 and 3074.1) or (3073.1)
Abbreviation: R_HMGLx Synonyms: _0 EC Number: 4.1.3.4 SUBSYSTEM: Cholesterol Metabolism Equation: [x] : hmgcoa --> acac + accoa Confidence Level: 4 TV NJ added peroxisomal version - see Olivier Biochimica et Biophysica Act 1529 (2000) 89-102 genes: LOCUS:_3155#TRANSCRIPT:1#ABBREVIATION:HMGCL#ECNUMBERS:4.1.3.4# proteins: NAME:_3-hydroxylmethyl-3-methylglutaryl-Coenzyme A lyase#ABBREVIATION:Hmgcl.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (3155.1)
Abbreviation: R_EX_acnacngalgbside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acnacngalgbside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
disialyl galactosylgloboside (homo sapiens) exchange
Abbreviation: R_VITD2Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : h + nadph + o2 + vitd2 --> 25hvitd2 + h2o + nadp Confidence Level: 4 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.196 IT genes: LOCUS:_1593#TRANSCRIPT:1#ABBREVIATION:CYP27A1# proteins: NAME:cytochrome P450, family 27, subfamily A, polypeptide 1#ABBREVIATION:Cyp27a1.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Guo YD, Strugnell S, Back DW, Jones G.#TITLE:Transfected human liver cytochrome P-450 hydroxylates vitamin D analogs at different side-chain positions.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1993#PUBMED:7690968# GENE ASSOCIATION: (1593.1)
Abbreviation: R_VLCSr Synonyms: _5 EC Number: 6.2.1.7 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : atp + coa + thcholstoic --> amp + cholcoar + ppi Confidence Level: 4 from uniprot and lit - see human recon lit refs divalent cation requirement - unclear which one, reaction inhibited in the presence of EDTA (may just be MgATP or MgATP) - see Kelley and Vessey Biochimica et Biophysica Acta (1994) 1209: 51-55 temporarily will have H+ balance reaction NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# proteins: NAME:bile acid CoA ligase#ABBREVIATION:Slc27a1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Steinberg SJ, Wang SJ, Kim DG, Mihalik J, Watkins PA#TITLE:Human very-long-chain-acyl-CoA synthetase#JOURNAL:Biochemical and biophysical research communications#YEAR:1999# AUTHORS:Steinberg SJ, Mihalik SJ, Kim DG, Cuebas DA, Watkins PA#TITLE:The human liver-specific homolog of very long-chain acyl-CoA synthetase is cholate: CoA ligase#JOURNAL:Journal of Biological Chemistry#YEAR:2000# AUTHORS:Mihalik SJ, Steinberg SJ, Pei Z, Park J, Kim DG, Heinzer AK, Dacremont G, Wanders JAR, Cuebas DA, Smith KD, Watkins PA#TITLE:Participation of two members of the veryl long-chain acyl-CoA synthetase family in bile acid synthesis and recycling#JOURNAL:Journal of Biological Chemistry#YEAR:2002# AUTHORS:Kelley M, Vessey DA#TITLE:Dual role of diavlent cations in the bile acid: CoA ligase catalyzed reaction#JOURNAL:Biochimica et Biophysica Acta#YEAR:1994# GENE ASSOCIATION: (11001.1)
Abbreviation: R_CYTK12 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : dcmp + dctp <==> (2) dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_EX_tre_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tre <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLCAASE9ly Synonyms: _3 EC Number: 3.2.1.31 SUBSYSTEM: Hyaluronan Metabolism Equation: [l] : (2) h2o + ha_pre1 --> acgam + glcur Confidence Level: 4 NCD genes: LOCUS:_2990#TRANSCRIPT:1#ABBREVIATION:GUSB#ECNUMBERS:3.2.1.31# proteins: NAME:glucuronidase, beta, lysosomal#ABBREVIATION:Gusb.1-l# citations: AUTHORS:Guise KS, Korneluk RG, Waye J, Lamhonwah AM, Quan F, Palmer R, Ganschow RE, Sly WS, Gravel RA#TITLE:Isolation and expression in Escherichia coli of a cDNA clone encoding human beta-glucuronidase#JOURNAL:Gene#YEAR:1985#PUBMED:3924735# AUTHORS:Ho YC, Ho LH, Ho KJ#TITLE:Human hepatic beta-glucuronidase: an enzyme kinetic study#JOURNAL:Enzyme#YEAR:1985#PUBMED:3987656# AUTHORS:Oshima A, Kyle JW, Miller RD, Hoffmann JW, Powell PP, Grubb JH, Sly WS, Tropak M, Guise KS, Gravel RA#TITLE:Cloning, sequencing, and expression of cDNA for human beta-glucuronidase#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3468507# GENE ASSOCIATION: (2990.1)
cis-hexadec-9-enoyl-[acyl-carrier protein] (n-C16:1)_C27H49N2O8PRS
Abbreviation: R_EX_dhdascb_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dhdascb <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ADK1 Synonyms: _0 EC Number: 2.7.4.3 SUBSYSTEM: Nucleotides Equation: [c] : amp + atp <==> (2) adp Confidence Level: 4 Inorganic triphosphate can also act as donor genes: LOCUS:_26289#TRANSCRIPT:1#ABBREVIATION:AK5# LOCUS:_203#TRANSCRIPT:1#ABBREVIATION:AK1#ECNUMBERS:2.7.4.3# LOCUS:_122481#TRANSCRIPT:1#ABBREVIATION:AK7# LOCUS:_26289#TRANSCRIPT:2#ABBREVIATION:AK5# proteins: NAME:adenylate kinase 1 #ABBREVIATION:Ak1.1# NAME:adenylate kinase 5 #ABBREVIATION:Ak5.1# NAME:adenylate kinase 5 #ABBREVIATION:Ak5.2# NAME:adenylate kinase 7 #ABBREVIATION:Ak7.1# citations: AUTHORS:Matsuura S, Igarashi M, Tanizawa Y, Yamada M, Kishi F, Kajii T, Fujii H, Miwa S, Sakurai M, Nakazawa A.#TITLE:Human adenylate kinase deficiency associated with hemolytic anemia. A single base substitution affecting solubility and catalytic activity of the cytosolic adenylate kinase.#JOURNAL:J Biol Chem. #YEAR:1989#PUBMED:2542324# AUTHORS:Zuffardi O, Caiulo A, Maraschio P, Tupler R, Bianchi E, Amisano P, Beluffi G, Moratti R, Liguri G.#TITLE:Regional assignment of the loci for adenylate kinase to 9q32 and for alpha 1-acid glycoprotein to 9q31-q32. A locus for Goltz syndrome in region 9q32-qter?#JOURNAL:Hum Genet.#YEAR:1989#PUBMED:2541064# AUTHORS:Miwa S, Fujii H, Tani K, Takahashi K, Takizawa T, Igarashi T.#TITLE:Red cell adenylate kinase deficiency associated with hereditary nonspherocytic hemolytic anemia: clinical and biochemical studies. #JOURNAL:Am J Hematol. #YEAR:1983#PUBMED:6305188# AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Identification of a novel human adenylate kinase. cDNA cloning, expression analysis, chromosome localization and characterization of the recombinant protein. #JOURNAL:Eur J Biochem. #YEAR:1999#PUBMED:10215863# GENE ASSOCIATION: (122481.1) or (203.1) or (26289.1) or (26289.2)
Abbreviation: R_4HDEBRISOQUINEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _4hdebrisoquine[e] <==> 4hdebrisoquine[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HXANtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: hxan[c] --> hxan[x] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SELt4_3 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) na1[e] + sel[e] <==> (3) na1[c] + sel[c] Confidence Level: 4 NCD genes: LOCUS:_6561#TRANSCRIPT:1#ABBREVIATION:SLC13A1# proteins: NAME:solute carrier family 13 (sodium/sulfate symporters), member 1#ABBREVIATION:Slc13a1.1-e# citations: AUTHORS:Lee A, Beck L, Markovich D#TITLE:The human renal sodium sulfate cotransporter (SLC13A1; hNaSi-1) cDNA and gene: organization, chromosomal localization, and functional characterization#JOURNAL:Genomics#YEAR:2000#PUBMED:11161786# AUTHORS:Pajor AM#TITLE:Molecular properties of sodium/dicarboxylate cotransporters#JOURNAL:J Membr Biol#YEAR:2000#PUBMED:10811962# GENE ASSOCIATION: (6561.1)
Abbreviation: R_LS3 Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [c] : pd3 <==> lum3 Confidence Level: 4 This reaction takes place in skin and needs UV 290-305. based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.194 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_FACOAL191 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + prist --> amp + ppi + pristcoa Confidence Level: 4 current models suggest COAL activity occurs on outer peroxisomal membrane NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 2#ABBREVIATION:Slc27a2.1# citations: AUTHORS:Steinberg SJ, Wang SJ, Kim DG, Mihalik J, Watkins PA#TITLE:Human very-long-chain-acyl-CoA synthetase#JOURNAL:Biochemical and biophysical research communications#YEAR:1999# AUTHORS:Verhoeven NM, Jakobs C.#TITLE:Human metabolism of phytanic acid and pristanic acid.#JOURNAL:Prog Lipid Res#YEAR:2001#PUBMED:11591435# GENE ASSOCIATION: (11001.1)
aldo-keto reductase family 1, member D1 (delta 4-3-ketosteroid-5-beta-reductase)
Abbreviation: R_AKR1D Synonyms: _4 SUBSYSTEM: Bile Acid Biosynthesis Equation: [c] : h + nadph + xol7aone --> nadp + xol7ah Confidence Level: 4 refs: uniprot, KEGG DW Russell bile acid synthesis review paper (see human recon journal citations) NJ genes: LOCUS:_6718#TRANSCRIPT:1#ABBREVIATION:AKR1D1#ECNUMBERS:1.3.99.6# proteins: NAME:aldo-keto reductase (delta 4-3-ketosteroid-5-beta-reductase)#ABBREVIATION:Akr1d1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (6718.1)
Abbreviation: R_AHEXASE2ly Synonyms: _13 EC Number: 3.2.1.52 SUBSYSTEM: N-Glycan Degradation Equation: [l] : (3) h2o + n2m2nmn --> (3) acgam + m2mn Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3074.1) or (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_LEUKTRD4tr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: leuktrD4[c] <==> leuktrD4[r] Confidence Level: 1 unknown mechanism, presumed diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_H2Ot Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h2o[e] <==> h2o[c] Confidence Level: 4 renamed from H20t5 to H2Ot NCD genes: LOCUS:_6523#TRANSCRIPT:1#ABBREVIATION:SLC5A1# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 1, extracellular#ABBREVIATION:Slc5a1.1-e# citations: AUTHORS:Hirayama BA, Loo DD, Wright EM.#TITLE:Protons drive sugar transport through the Na+/glucose cotransporter (SGLT1)#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8063771# AUTHORS:Hediger MA, Turk E, Wright EM#TITLE:Homology of the human intestinal Na+/glucose and Escherichia coli Na+/proline cotransporters#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1989#PUBMED:2490366# AUTHORS:Quick M, Loo DD, Wright EM#TITLE:Neutralization of a conserved amino acid residue in the human Na+/glucose transporter (hSGLT1) generates a glucose-gated H+ channel#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11024018# AUTHORS:Wright EM, Hirayama BA, Loo DDF, Turk E, Hager K#TITLE:Intestinal sugar transport#YEAR:1994# AUTHORS:Leung DW, Loo DD, Hirayama BA, Zeuthen T, Wright EM#TITLE:Urea transport by cotransporters#JOURNAL:J Physiol#YEAR:2000#PUBMED:11034615# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# GENE ASSOCIATION: (6523.1)
Abbreviation: R_EX_fucgalgbside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucgalgbside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
fucosyl galactosylgloboside (homo sapiens) exchange
Glycolaldehyde dehydrogenase, mitochondrial
Abbreviation: R_GCALDDm Synonyms: _0 EC Number: 1.2.1.21 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [m] : gcald + h2o + nad --> glyclt + (2) h + nadh Confidence Level: 2 JLR genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_7915#TRANSCRIPT:1#ABBREVIATION:ALDH5A1#ECNUMBERS:1.2.1.24# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# LOCUS:_4329#TRANSCRIPT:1#ABBREVIATION:ALDH6A1#ECNUMBERS:1.2.1.27# LOCUS:_5832#TRANSCRIPT:1#ABBREVIATION:ALDH18A1# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_7915#TRANSCRIPT:2#ABBREVIATION:ALDH5A1# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# NAME:aldehyde dehydrogenase 5 family, member A1 (succinate-semialdehyde dehydrogenase), mitochondrial#ABBREVIATION:Aldh5a1.1-m# NAME:aldehyde dehydrogenase 5 family, member A1 (succinate-semialdehyde dehydrogenase), mitochondrial#ABBREVIATION:Aldh5a1.2-m# NAME:aldehyde dehydrogenase 18 family, member A1#ABBREVIATION:Aldh18a1.1-m# NAME:Aldehyde dehydrogenase 6 family, member A1, mitochondrial#ABBREVIATION:Aldh6a1.1-m# citations: AUTHORS:Brent J#TITLE:Current management of ethylene glycol poisoning#JOURNAL:Drugs#YEAR:2001#PUBMED:11434452# AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# GENE ASSOCIATION: (4329.1) or (7915.1) or (8659.1) or (5832.1) or (217.1) or (7915.2) or (219.1) or (8659.2)
Abbreviation: R_LEUKTRC4t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hco3[c] + leuktrC4[e] <==> hco3[e] + leuktrC4[c] Confidence Level: 4 NJ genes: LOCUS:_28234#TRANSCRIPT:1#ABBREVIATION:SLCO1B3# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# NAME:solute carrier organic anion transporter family, member 1B3#ABBREVIATION:Slco1b3.1# citations: AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# AUTHORS:Abe T, Kakyo M, Tokui T, Nakagomi R, Nishio T, Nakai D, Nomura H, Unno M, Suzuki M, Naitoh T, Matsuno S, Yawo H.#TITLE:Identification of a novel gene family encoding human liver-specific organic anion transporter LST-1.#JOURNAL:J Biol Chem.#YEAR:1999#PUBMED:10358072# GENE ASSOCIATION: (10599.1) or (28234.1)
leukotriene C4 transport via bicarbonate countertransport
Abbreviation: R_PUNP2 Synonyms: _0 EC Number: 2.4.2.1 SUBSYSTEM: Purine Catabolism Equation: [c] : dad-2 + pi <==> 2dr1p + ade Confidence Level: 4 Specificity not completely determined. Can also catalyse ribosyltransferase reactions of the type catalysed by EC 2.4.2.5, nucleoside ribosyltransferase genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# citations: AUTHORS:Erion MD, Stoeckler JD, Guida WC, Walter RL, Ealick SE.#TITLE:Purine nucleoside phosphorylase. 2. Catalytic mechanism. #JOURNAL:Biochemistry#YEAR:1997#PUBMED:9305963# AUTHORS:Williams SR, Goddard JM, Martin DW Jr. #TITLE:Human purine nucleoside phosphorylase cDNA sequence and genomic clone characterization.#JOURNAL:Nucleic Acids Res.#YEAR:1984#PUBMED:6087295# GENE ASSOCIATION: (4860.1)
purine-nucleoside phosphorylase (Deoxyadenosine)
Abbreviation: R_GUAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gua[e] <==> gua[c] Confidence Level: 4 JLR genes: LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Yao SY, Ng AM, Vickers MF, Sundaram M, Cass CE, Baldwin SA, Young JD.#TITLE:Functional and molecular characterization of nucleobase transport by recombinant human and rat equilibrative nucleoside transporters 1 and 2.#JOURNAL:J Biol Chem. #YEAR:2002#PUBMED:12006583# GENE ASSOCIATION: (3177.1)
Abbreviation: R_FUMSO3tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: fum[c] + so3[m] <==> fum[m] + so3[c] Confidence Level: 4 MM genes: LOCUS:_1468#TRANSCRIPT:1#ABBREVIATION:SLC25A10# proteins: NAME:Solute carrier family 25 (dicarboxylate transporter) member 10, mitochondrial #ABBREVIATION:Slc25a10.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# AUTHORS:Crompton M, Palmieri F, Capano M, Quagliariello E.#TITLE:The transport of sulphate and sulphite in rat liver mitochondria. #YEAR:1974#PUBMED:4441366# GENE ASSOCIATION: (1468.1)
Fumarate:sulfite antiport, mitochondrial
Abbreviation: R_EX_cspg_a_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cspg_a <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate A (GalNAc4S-GlcA) proteoglycan exchange
Abbreviation: R_H6ET3er Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : m2emgacpail_hs + pe_hs --> dag_hs + em2emgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_5281#TRANSCRIPT:1#ABBREVIATION:PIGF# LOCUS:_84720#TRANSCRIPT:1#ABBREVIATION:PIGO# LOCUS:_84720#TRANSCRIPT:2#ABBREVIATION:PIGO# LOCUS:_5281#TRANSCRIPT:2#ABBREVIATION:PIGF# proteins: NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.1-r# NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.2-r# NAME:phosphatidylinositol glycan, class O, endoplasmic reticulum#ABBREVIATION:Pigo.1-r# NAME:phosphatidylinositol glycan, class O, endoplasmic reticulum#ABBREVIATION:Pigo.2-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Hong Y, Maeda Y, Watanabe R, Inoue N, Ohishi K, Kinoshita T#TITLE:Requirement of PIG-F and PIG-O for transferring phosphoethanolamine to the third mannose in glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781593# AUTHORS:Inoue N, Kinoshita T, Orii T, Takeda J#TITLE:Cloning of a human gene, PIG-F, a component of glycosylphosphatidylinositol anchor biosynthesis, by a novel expression cloning strategy#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8463218# GENE ASSOCIATION: (84720.2) and (5281.1) or (84720.1) and (5281.2) or (5281.1) and (84720.1) or (84720.2) and (5281.2)
H6 phosphoethanolaminyl transferase, endoplasmic reticulum
Abbreviation: R_EX_gua_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gua <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GFUCS Synonyms: _0 EC Number: 1.1.1.271 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : gdpddman + h + nadph --> gdpfuc + nadp Confidence Level: 0 Both human and Escherichia coli enzymes can use NADH in place of NADPH to a slight extent. IT genes: LOCUS:_7264#TRANSCRIPT:1#ABBREVIATION:TSTA3# proteins: NAME:tissue specific transplantation antigen P35B#ABBREVIATION:Tsta3.1# citations: none GENE ASSOCIATION: (7264.1)
Abbreviation: R_EX_cspg_d_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cspg_d <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate D (GlcNAc6S-GlcA2S) proteoglycan exchange
Abbreviation: R_NCNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ca2[e] + (4) na1[c] + nh4[e] <==> ca2[c] + (4) na1[e] + nh4[c] Confidence Level: 4 NCD genes: LOCUS:_25769#TRANSCRIPT:1#ABBREVIATION:SLC24A2# LOCUS:_9187#TRANSCRIPT:1#ABBREVIATION:SLC24A1# proteins: NAME:solute carrier family 24 (sodium/potassium/calcium exchanger), member 1#ABBREVIATION:Slc24a1.1-e# NAME:solute carrier family 24 (sodium/potassium/calcium exchanger), member 2#ABBREVIATION:Slc24a2.1-e# citations: AUTHORS:Tucker JE, Winkfein RJ, Cooper CB, Schnetkamp PP#TITLE:cDNA cloning of the human retinal rod Na-Ca + K exchanger: comparison with a revised bovine sequence#JOURNAL:Invest Ophthalmol Vis Sci#YEAR:1998#PUBMED:9478004# AUTHORS:Kimura M, Jeanclos EM, Donnelly RJ, Lytton J, Reeves JP, Aviv A.#TITLE:Physiological and molecular characterization of the Na+/Ca2+ exchanger in human platelets#JOURNAL:Am J Physiol#YEAR:1999#PUBMED:10484410# AUTHORS:Schnetkamp PP#TITLE:The SLC24 Na+/Ca2+-K+ exchanger family: vision and beyond.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770312# AUTHORS:Prinsen CF, Szerencsei RT, Schnetkamp PP#TITLE:Molecular cloning and functional expression of the potassium-dependent sodium-calcium exchanger from human and chicken retinal cone photoreceptors#JOURNAL:J Neurosci#YEAR:2000#PUBMED:10662833# AUTHORS:Kraev A, Quednau BD, Leach S, Li XF, Dong H, Winkfein R, Perizzolo M, Cai X, Yang R, Philipson KD, Lytton J#TITLE:Molecular cloning of a third member of the potassium-dependent sodium-calcium exchanger gene family, NCKX3#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11294880# AUTHORS:Li XF, Kraev AS, Lytton J#TITLE:Molecular cloning of a fourth member of the potassium-dependent sodium-calcium exchanger gene family, NCKX4#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12379639# GENE ASSOCIATION: (9187.1) or (25769.1)
Abbreviation: R_11DOCRTSTRNtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: _11docrtstrn[c] <==> 11docrtstrn[r] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
_11-deoxycorticosterone intracellular transport
Abbreviation: R_DGNSKm Synonyms: _0 EC Number: 2.7.1.113 SUBSYSTEM: Nucleotides Equation: [m] : atp + dgsn --> adp + dgmp + h Confidence Level: 4 genes: LOCUS:_1716#TRANSCRIPT:1#ABBREVIATION:DGUOK#ECNUMBERS:2.7.1.113# LOCUS:_1716#TRANSCRIPT:3#ABBREVIATION:DGUOK#ECNUMBERS:2.7.1.113# LOCUS:_1716#TRANSCRIPT:4#ABBREVIATION:DGUOK#ECNUMBERS:2.7.1.113# LOCUS:_1716#TRANSCRIPT:5#ABBREVIATION:DGUOK#ECNUMBERS:2.7.1.113# LOCUS:_1716#TRANSCRIPT:2#ABBREVIATION:DGUOK#ECNUMBERS:2.7.1.113# proteins: NAME:deoxyguanosine kinase #ABBREVIATION:DguoK.1-m# NAME:deoxyguanosine kinase #ABBREVIATION:DguoK.2-m# NAME:deoxyguanosine kinase #ABBREVIATION:DguoK.3-m# NAME:deoxyguanosine kinase #ABBREVIATION:DguoK.4-m# NAME:deoxyguanosine kinase #ABBREVIATION:DguoK.5-m# citations: AUTHORS:Krawiec K, Kierdaszuk B, Shugar D.#TITLE:Inorganic tripolyphosphate (PPP(i)) as a phosphate donor for human deoxyribonucleoside kinases. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12535661# AUTHORS:Johansson M, Karlsson A.#TITLE:Cloning and expression of human deoxyguanosine kinase cDNA. #JOURNAL:Proc Natl Acad Sci U S A. #YEAR:1996#PUBMED:8692979# GENE ASSOCIATION: (1716.5) or (1716.1) or (1716.4) or (1716.2) or (1716.3)
Abbreviation: R_PPA2 Synonyms: _3 EC Number: 3.6.1.1 SUBSYSTEM: Others Equation: [c] : h2o + pppi --> h + pi + ppi Confidence Level: 1 AMF genes: none proteins: none citations: none GENE ASSOCIATION:
glutamate dehydrogenase (NADP), mitochondrial
Abbreviation: R_GLUDym Synonyms: _0 EC Number: 1.4.1.4 SUBSYSTEM: Glutamate metabolism Equation: [m] : glu-L + h2o + nadp <==> akg + h + nadph + nh4 Confidence Level: 4 genes: LOCUS:_2746#TRANSCRIPT:1#ABBREVIATION:GLUD1#ECNUMBERS:1.4.1.3# LOCUS:_2747#TRANSCRIPT:1#ABBREVIATION:GLUD2# proteins: NAME:glutamate dehydrogenase 1, mitochondrial#ABBREVIATION:Glud1.1-m# NAME:glutamate dehydrogenase 2, mitochondrial#ABBREVIATION:Glud2.1-m# citations: AUTHORS:Jie FANG , Betty Y. L. HSU , Courtney M. MACMULLEN , Mortimer PONCZ , Thomas J. SMITH and Charles A. STANLEY#TITLE:Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations #YEAR:2002# AUTHORS:Zaganas I, Spanaki C, Karpusas M, Plaitakis A.#TITLE:Substitution of Ser for Arg-443 in the regulatory domain of human housekeeping (GLUD1) glutamate dehydrogenase virtually abolishes basal activity and markedly alters the activation of the enzyme by ADP and L-leucine.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12324473# AUTHORS:Mavrothalassitis G, Tzimagiorgis G, Mitsialis A, Zannis V, Plaitakis A, Papamatheakis J, Moschonas N.#TITLE:Isolation and characterization of cDNA clones encoding human liver glutamate dehydrogenase: evidence for a small gene family. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1988#PUBMED:3368458# GENE ASSOCIATION: (2747.1) or (2746.1)
fatty acyl-CoA synthase (n-C8:0CoA), lumped reaction
Abbreviation: R_FAS80COA_L Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : accoa + (9) h + (3) malcoa + (6) nadph --> (3) co2 + (3) coa + (3) h2o + (6) nadp + occoa Confidence Level: 4 NCD genes: LOCUS:_2194#TRANSCRIPT:1#ABBREVIATION:FASN#ECNUMBERS:2.3.1.85# proteins: NAME:fatty acid synthase#ABBREVIATION:Fasn.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Chakravarty B, Gu Z, Chirala SS, Wakil SJ, Quiocho FA.#TITLE:Human fatty acid synthase: structure and substrate selectivity of the thioesterase domain.#YEAR:2004#PUBMED:15507492# AUTHORS:Brink J, Ludtke SJ, Yang CY, Gu ZW, Wakil SJ, Chiu W.#TITLE:Quaternary structure of human fatty acid synthase by electron cryomicroscopy.#JOURNAL:Proc Natl Acad Sci#YEAR:2002#PUBMED:11756679# AUTHORS:Wakil SJ.#TITLE:Fatty acid synthase, a proficient multifunctional enzyme.#JOURNAL:Biochemistry#YEAR:1989#PUBMED:2669958# GENE ASSOCIATION: (2194.1)
Abbreviation: R_5MTHFt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _5mthf[e] + oh1[c] <==> 5mthf[c] + oh1[e] Confidence Level: 4 IT genes: LOCUS:_6573#TRANSCRIPT:1#ABBREVIATION:SLC19A1# LOCUS:_6573#TRANSCRIPT:2#ABBREVIATION:SLC19A1# proteins: NAME:solute carrier family 19 (folate transporter), member 1 #ABBREVIATION:Slc19a1.1# NAME:solute carrier family 19 (folate transporter), member 1#ABBREVIATION:Slc19a1.2# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Ganapathy V, Smith SB, Prasad PD.#TITLE:SLC19: the folate/thiamine transporter family.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:14770311# AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION: (6573.1) or (6573.2)
_5-methyltetrahydrofolate transport via anion exchange
Beta oxidation of long chain fatty acid (odd chain)
Abbreviation: R_FAOXC170m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (7) coa + (7) fad + (7) h2o + hpdcacoa + (7) nad --> (7) accoa + (7) fadh2 + (7) h + (7) nadh + ppcoa Confidence Level: 4 by extension from even chain lumped FAO - should double-check stoich in future NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# GENE ASSOCIATION: (34.1)
Abbreviation: R_TYMSFt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tymsf[c] --> tymsf[e] Confidence Level: 1 SAB excretion of tyramine sulfate genes: none proteins: none citations: none GENE ASSOCIATION:
Tyramine O-sulfate transport (diffusion)
Abbreviation: R_XOLTRI27tc Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: xoltri27[r] <==> xoltri27[c] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CRTSLtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: crtsl[c] <==> crtsl[r] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_gthrd_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gthrd <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ELAIDCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + elaidcrn --> crn + od2coa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_FORMCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: formcoa[x] <==> formcoa[c] Confidence Level: 2 unknown mechanism see Verhoeven 11591435 and clayton 11356171 - added for peroxisomal oxidation of fatty acids NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6TASE15ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg18 <==> h + ksi_deg19 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_SLCBK1 Synonyms: _0 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : atp + sphgn --> adp + h + sph1p Confidence Level: 4 NCD genes: LOCUS:_56848#TRANSCRIPT:1#ABBREVIATION:SPHK2# proteins: NAME:sphingosine kinase 2 #ABBREVIATION:Sphk2.1# citations: AUTHORS:Liu H, Sugiura M, Nava VE, Edsall LC, Kono K, Poulton S, Milstien S, Kohama T, Spiegel S.#TITLE:Molecular cloning and functional characterization of a novel mammalian sphingosine kinase type 2 isoform.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10751414# GENE ASSOCIATION: (56848.1)
sphingolipid long chain base kinase (sphinganine)
Abbreviation: R_CBPPer Synonyms: _0 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [r] : cbp + glc-D + h --> co2 + g6p + nh4 Confidence Level: 2 See PMID: 11946414 NCD genes: LOCUS:_2538#TRANSCRIPT:1#ABBREVIATION:G6PC#ECNUMBERS:3.1.3.9# LOCUS:_92579#TRANSCRIPT:1#ABBREVIATION:G6PC3# LOCUS:_57818#TRANSCRIPT:1#ABBREVIATION:G6PC2# proteins: NAME:glucose-6-phosphatase, catalytic (glycogen storage disease type I, von Gierke disease), endoplasmic reticulum (liver, kidney, intestine)#ABBREVIATION:G6pc.1-r# NAME:glucose-6-phosphatase, catalytic, 2, endoplasmic reticulum (pancreatic B islets)#ABBREVIATION:G6pc2.1-r# NAME:glucose 6 phosphatase, catalytic, 3, endoplasmic reticulum (ubiquitous)#ABBREVIATION:G6pc3.1-r# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Lueck JD, Nordlie RC.#TITLE:The utilization of intramitochondrially generated carbamyl phosphate for microsomal glucose 6-phosphate biosynthesis.#JOURNAL:FEBS Lett#YEAR:1972#PUBMED:11946414# AUTHORS:Lucius RW, Waddell ID, Burchell A, Nordlie RC#TITLE:The hepatic glucose-6-phosphatase system in Ehrlich-ascites-tumour-bearing mice#JOURNAL:Biochem J.#YEAR:1993#PUBMED:8384451# GENE ASSOCIATION: (2538.1) or (57818.1) or (92579.1)
Carbamoyl phosphate phosphotransferase, endoplasmic reticulum
Abbreviation: R_EX_cgly_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cgly <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAOXC150m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (6) coa + (6) fad + (6) h2o + (6) nad + ptdcacoa --> (6) accoa + (6) fadh2 + (6) h + (6) nadh + ppcoa Confidence Level: 4 by extension from even chain lumped FAO - should double-check stoich in future NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_RIBt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: rib-D[e] <==> rib-D[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Huck JH, Roos B, Jakobs C, van der Knaap MS, Verhoeven NM#TITLE:Evaluation of pentitol metabolism in mammalian tissues provides new insight into disorders of human sugar metabolism#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15234337# GENE ASSOCIATION:
Abbreviation: R_34DHPHEt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _34dhphe[e] <==> 34dhphe[c] Confidence Level: 4 NCD genes: LOCUS:_117247#TRANSCRIPT:1#ABBREVIATION:SLC16A10# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 10#ABBREVIATION:Slc16a10.1-e# citations: AUTHORS:Kim do K, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H#TITLE:The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location#JOURNAL:Genomics#YEAR:2002#PUBMED:11827462# GENE ASSOCIATION: (117247.1)
_3,4-Dihydroxy-L-phenylalanine transport
Abbreviation: R_NAGA2ly Synonyms: _3 EC Number: 3.2.1.49 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : Tn_antigen + h + udp --> Ser/Thr + udpacgal Confidence Level: 4 Alpha-galactosidase B NJ genes: LOCUS:_4668#TRANSCRIPT:1#ABBREVIATION:NAGA#ECNUMBERS:3.2.1.49# proteins: NAME:N-acetylgalactosaminidase, alpha-, lysosomal#ABBREVIATION:Naga.1-l# citations: AUTHORS:Wang AM, Bishop DF, Desnick RJ.#TITLE:Human alpha-N-acetylgalactosaminidase-molecular cloning, nucleotide sequence, and expression of a full-length cDNA. Homology with human alpha-galactosidase A suggests evolution from a common ancestral gene. #JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2174888# AUTHORS:Tsuji S, Yamauchi T, Hiraiwa M, Isobe T, Okuyama T, Sakimura K, Takahashi Y, Nishizawa M, Uda Y, Miyatake T.#TITLE:Molecular cloning of a full-length cDNA for human alpha-N-acetylgalactosaminidase (alpha-galactosidase B)#JOURNAL:Biochem Biophys Res Commun#YEAR:1989#PUBMED:2551294# GENE ASSOCIATION: (4668.1)
Abbreviation: R_DESAT22_2p Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [x] : clpndcoa + h + nadh + o2 --> c226coa + (2) h2o + nad Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present Sprecher "An update on the pathways of polyunsaturated fatty acid metabolism" among others describe peroxisomal localization (inner side) n-3 NJ genes: none proteins: none citations: AUTHORS:Sprecher H#TITLE:An update on the pathways of polyunsaturated fatty acid metabolism#JOURNAL:Curr Opn in Clinical Nutrition and Metabolic Care#YEAR:1999# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Ferdinandusse S, Denis S, Mooijer PAW, Zhang Z, Reddy JK, Spector AA, Wanders RJA#TITLE:Identification of the peroxisomal beta-oxidation enzymes involved in the biosynthesis of docosahexaenoic acid#JOURNAL:Journal of Lipid Research#YEAR:2001# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION:
fatty acyl-CoA desaturase (n-C22:5CoA -> n-C22:6CoA)
_3-sulfinopyruvate hydrolase (spotaneous reaction), mitochondrial
Abbreviation: R_3SPYRSPm Synonyms: _0 SUBSYSTEM: Cysteine Metabolism Equation: [m] : 3snpyr + h2o --> h + pyr + so3 Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Weinstein CL, Griffith OW.#TITLE:Cysteinesulfonate and beta-sulfopyruvate metabolism. Partitioning between decarboxylation, transamination, and reduction pathways.#YEAR:1988#PUBMED:3346220# GENE ASSOCIATION:
Abbreviation: R_2MCITt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _2mcit[c] <==> 2mcit[e] Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Weidman SW, Drysdale GR.#TITLE:he biosynthesis of methylcitrate.#JOURNAL:Biochem J#YEAR:1979#PUBMED:426765# GENE ASSOCIATION:
_2-methylcitrate transport via diffusion
Abbreviation: R_XOL7AH2tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: xol7ah2[c] <==> xol7ah2[m] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_PA_HStn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: pa_hs[c] <==> pa_hs[n] Confidence Level: 2 it genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PAFHe Synonyms: _3 EC Number: 3.1.1.47 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [e] : h2o + paf_hs --> ac + ak2lgchol_hs + h Confidence Level: 4 NJ genes: LOCUS:_7941#TRANSCRIPT:1#ABBREVIATION:PLA2G7#ECNUMBERS:3.1.1.47# proteins: NAME:phospholipase A2, group VII (platelet-activating factor acetylhydrolase)#ABBREVIATION:Pla2g7.1# citations: AUTHORS:Stafforini DM, Satoh K, Atkinson DL, Tjoelker LW, Eberhardt C, Yoshida H, Imaizumi T, Takamatsu S, Zimmerman GA, McIntyre TM, Gray PW, Prescott SM.#TITLE:Platelet-activating factor acetylhydrolase deficiency. A missense mutation near the active site of an anti-inflammatory phospholipase.#JOURNAL:J Clin Invest#YEAR:2005#PUBMED:8675689# GENE ASSOCIATION: (7941.1)
Platelet-activating factor acetylhydrolase
Abbreviation: R_EX_caro_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : caro <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MMEm Synonyms: _0 EC Number: 5.1.99.1 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : mmcoa-R <==> mmcoa-S Confidence Level: 4 TDV genes: LOCUS:_84693#TRANSCRIPT:1#ABBREVIATION:MCEE#ECNUMBERS:5.1.99.1# proteins: NAME:methylmalonyl CoA epimerase, mitochondrial#ABBREVIATION:Mcee.1-m# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Bobik TA, Rasche ME.#TITLE:Identification of the human methylmalonyl-CoA racemase gene based on the analysis of prokaryotic gene arrangements. Implications for decoding the human genome.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11481338# GENE ASSOCIATION: (84693.1)
Abbreviation: R_PIPLC Synonyms: _9 EC Number: 3.1.4.3 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail_hs --> dag_hs + h + mi1p-D Confidence Level: 4 NCD genes: LOCUS:_5335#TRANSCRIPT:1#ABBREVIATION:PLCG1#ECNUMBERS:3.1.4.11# LOCUS:_5331#TRANSCRIPT:1#ABBREVIATION:PLCB3# LOCUS:_23236#TRANSCRIPT:1#ABBREVIATION:PLCB1#ECNUMBERS:3.1.4.11# LOCUS:_5330#TRANSCRIPT:1#ABBREVIATION:PLCB2#ECNUMBERS:3.1.4.11# LOCUS:_5333#TRANSCRIPT:1#ABBREVIATION:PLCD1# LOCUS:_5332#TRANSCRIPT:1#ABBREVIATION:PLCB4# LOCUS:_5336#TRANSCRIPT:1#ABBREVIATION:PLCG2#ECNUMBERS:3.1.4.3# LOCUS:_89869#TRANSCRIPT:1#ABBREVIATION:PLCZ1# LOCUS:_257068#TRANSCRIPT:1#ABBREVIATION:PLCXD2# LOCUS:_5334#TRANSCRIPT:1#ABBREVIATION:PLCL1# LOCUS:_5332#TRANSCRIPT:2#ABBREVIATION:PLCB4# LOCUS:_84812#TRANSCRIPT:1#ABBREVIATION:PLCD4# LOCUS:_23007#TRANSCRIPT:1#ABBREVIATION:PLCL3# LOCUS:_51196#TRANSCRIPT:1#ABBREVIATION:PLCE1# LOCUS:_23236#TRANSCRIPT:2#ABBREVIATION:PLCB1# LOCUS:_113026#TRANSCRIPT:1#ABBREVIATION:PLCD3# LOCUS:_5335#TRANSCRIPT:2#ABBREVIATION:PLCG1# LOCUS:_9651#TRANSCRIPT:1#ABBREVIATION:PLCL4# proteins: NAME:phospholipase C, delta 4#ABBREVIATION:Plcd4.1# NAME:phospholipase C, delta 1#ABBREVIATION:Plcd1.1# NAME:phospholipase C-like 3#ABBREVIATION:Plcl3.1# NAME:phospholipase C, epsilon 1#ABBREVIATION:Plce1.1# NAME:phospholipase C, gamma 2 (phosphatidylinositol-specific)#ABBREVIATION:Plcg2.1# NAME:phospholipase C, beta 4#ABBREVIATION:Plcb4.1# NAME:phospholipase C, beta 4#ABBREVIATION:Plcb4.2# NAME:phospholipase C, beta 1 (phosphoinositide-specific)#ABBREVIATION:Plcb1.1# NAME:phospholipase C, beta 1 (phosphoinositide-specific)#ABBREVIATION:Plcb1.2# NAME:phospholipase C, zeta 1#ABBREVIATION:Plcz1.1# NAME:phospholipase C, delta 3#ABBREVIATION:Plcd3.1# NAME:phosphatidylinositol-specific phospholipase C, X domain containing 2 #ABBREVIATION:Plcxd2.1# NAME:phospholipase C, beta 3 (phosphatidylinositol-specific)#ABBREVIATION:Plcb3.1# NAME:phospholipase C, gamma 1#ABBREVIATION:Plcg1.1# NAME:phospholipase C, gamma 1#ABBREVIATION:Plcg1.2# NAME:phospholipase C-like 1#ABBREVIATION:Plcl1.1# NAME:phospholipase C, beta 2#ABBREVIATION:Plcb2.1# NAME:phospholipase C-like 4#ABBREVIATION:Plcl4.1# citations: AUTHORS:Cheng HF, Jiang MJ, Chen CL, Liu SM, Wong LP, Lomasney JW, King K#TITLE:Cloning and identification of amino acid residues of human phospholipase C delta 1 essential for catalysis#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7890667# AUTHORS:Ishikawa S, Takahashi T, Ogawa M, Nakamura Y#TITLE:Genomic structure of the human PLCD1 (phospholipase C delta 1) locus on 3p22-->p21.3#JOURNAL:Cytogenet Cell Genet#YEAR:1997#PUBMED:9345909# AUTHORS:Kim H, Suh PG, Ryu SH, Park SH#TITLE:Assignment of the human PLC delta4 gene (PLCD4) to human chromosome band 2q35 by fluorescence in situ hybridization#JOURNAL:Cytogenet Cell Genet.#YEAR:1999#PUBMED:10702683# AUTHORS:Leung DW, Tompkins C, Brewer J, Ball A, Coon M, Morris V, Waggoner D, Singer JW#TITLE:Phospholipase C delta-4 overexpression upregulates ErbB1/2 expression, Erk signaling pathway, and proliferation in MCF-7 cells#JOURNAL:Mol Cancer#YEAR:2004#PUBMED:15140260# AUTHORS:Song C, Hu CD, Masago M, Kariyai K, Yamawaki-Kataoka Y, Shibatohge M, Wu D, Satoh T, Kataoka T#TITLE:Regulation of a novel human phospholipase C, PLCepsilon, through membrane targeting by Ras#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11022048# AUTHORS:Lopez I, Mak EC, Ding J, Hamm HE, Lomasney JW#TITLE:A novel bifunctional phospholipase c that is regulated by Galpha 12 and stimulates the Ras/mitogen-activated protein kinase pathway#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11022047# AUTHORS:Hernandez D, Egan SE, Yulug IG, Fisher EM#TITLE:Mapping the gene that encodes phosphatidylinositol-specific phospholipase C-gamma 2 in the human and the mouse#JOURNAL:Genomics#YEAR:1994#PUBMED:7835906# AUTHORS:Alvarez RA, Ghalayini AJ, Xu P, Hardcastle A, Bhattacharya S, Rao PN, Pettenati MJ, Anderson RE, Baehr W.#TITLE:cDNA sequence and gene locus of the human retinal phosphoinositide-specific phospholipase-C beta 4 (PLCB4)#JOURNAL:Genomics#YEAR:1995#PUBMED:8530101# AUTHORS:Peruzzi D, Aluigi M, Manzoli L, Billi AM, Di Giorgio FP, Morleo M, Martelli AM, Cocco L#TITLE:Molecular characterization of the human PLC beta1 gene#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12213492# AUTHORS:Caricasole A, Sala C, Roncarati R, Formenti E, Terstappen GC#TITLE:Cloning and characterization of the human phosphoinositide-specific phospholipase C-beta 1 (PLC beta 1)#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:11118617# AUTHORS:Peruzzi D, Calabrese G, Faenza I, Manzoli L, Matteucci A, Gianfrancesco F, Billi AM, Stuppia L, Palka G, Cocco L#TITLE:Identification and chromosomal localisation by fluorescence in situ hybridisation of human gene of phosphoinositide-specific phospholipase C beta(1)#JOURNAL:Biochim Biophy Acta#YEAR:2000#PUBMED:10760467# AUTHORS:Saunders CM, Larman MG, Parrington J, Cox LJ, Royse J, Blayney LM, Swann K, Lai FA#TITLE:PLC zeta: a sperm-specific trigger of Ca(2+) oscillations in eggs and embryo development#JOURNAL:Development#YEAR:2002#PUBMED:12117804# AUTHORS:Cox LJ, Larman MG, Saunders CM, Hashimoto K, Swann K, Lai FA#TITLE:Sperm phospholipase Czeta from humans and cynomolgus monkeys triggers Ca2+ oscillations, activation and development of mouse oocytes#JOURNAL:Reproduction#YEAR:2002#PUBMED:12416999# AUTHORS:Kim H, Suh PG, Ryu SH, Park SH#TITLE:Assignment of the human PLC delta3 gene (PLCD3) to human chromosome band 17q21 by fluorescence in situ hybridization#JOURNAL:Cytogenet Cell Genet#YEAR:1999#PUBMED:10702670# AUTHORS:Carozzi AJ, Kriz RW, Webster C, Parker PJ#TITLE:Identification, purification and characterization of a novel phosphatidylinositol-specific phospholipase C, a third member of the beta subfamily#JOURNAL:Eur J Biochem#YEAR:1992#PUBMED:13333955# AUTHORS:Weber G, Friedman E, Grimmond S, Hayward NK, Phelan C, Skogseid B, Gobl A, Zedenius J, Sandelin K, Teh BT, et al.#TITLE:The phospholipase C beta 3 gene located in the MEN1 region shows loss of expression in endocrine tumours#JOURNAL:Hum Mol Genet#YEAR:1994#PUBMED:7849701# AUTHORS:Sinke RJ, Geurts van Kessel AG#TITLE:Localization of the human phosphatidylinositol-specific phospholipase c beta 3 gene (PLCB3) within chromosome band 11q13#JOURNAL:Genoics#YEAR:1995#PUBMED:7789993# AUTHORS:Lagercrantz J, Carson E, Phelan C, Grimmond S, Rosen A, Dare E, Nordenskjold M, Hayward NK, Larsson C, Weber G#TITLE:Genomic organization and complete cDNA sequence of the human phosphoinositide-specific phospholipase C beta 3 gene (PLCB3)#JOURNAL:Genomics#YEAR:1995#PUBMED:7607669# AUTHORS:Mazuruk K, Schoen TJ, Chader GJ, Rodriguez IR#TITLE:Structural organization and expression of the human phosphatidylinositol-specific phospholipase C beta-3 gene#JOURNAL:Biochem Biophys Res Commun#YEAR:1995#PUBMED:7612006# AUTHORS:Burgess WH, Dionne CA, Kaplow J, Mudd R, Friesel R, Zilberstein A, Schlessinger J, Jaye M#TITLE:Characterization and cDNA cloning of phospholipase C-gamma, a major substrate for heparin-binding growth factor 1 (acidic fibroblast growth factor)-activated tyrosine kinase#JOURNAL:Mol Cell Biol#YEAR:1990#PUBMED:2167438# AUTHORS:Kohno T, Otsuka T, Takano H, Yamamoto T, Hamaguchi M, Terada M, Yokota J#TITLE:Identification of a novel phospholipase C family gene at chromosome 2q33 that is homozygously deleted in human small cell lung carcinoma#JOURNAL:Hum Mol Genet#YEAR:1995#PUBMED:7633416# AUTHORS:Park D, Jhon DY, Kriz R, Knopf J, Rhee SG#TITLE:Cloning, sequencing, expression, and Gq-independent activation of phospholipase C-beta 2#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1644792# GENE ASSOCIATION: (5331.1) or (5332.1) or (9651.1) or (113026.1) or (89869.1) or (23007.1) or (5330.1) or (5335.2) or (5336.1) or (257068.1) or (23236.1) or (5335.1) or (5334.1) or (84812.1) or (5332.2) or (5333.1) or (23236.2) or (51196.1)
chondroitin sulfate C (GalNAc6S-GlcA), precursor 3_C45H67N2O42S2X
Abbreviation: R_GTHRDt Synonyms: _0 SUBSYSTEM: Glutathione Metabolism Equation: atp[c] + gthrd[c] + h2o[c] <==> adp[c] + gthrd[m] + h[c] + pi[c] Confidence Level: 2 TV (8-15-2003) Meister, A, Mitochondrial changes associated with glutathione deficiency, Biochimica et Biophysica Acta 1271 (1995) 35-42. The article reports that this is an active transport but doesn't not specify if it uses atp. genes: none proteins: none citations: AUTHORS:J Martensson, J C Lai, and A Meister#TITLE:High-affinity transport of glutathione is part of a multicomponent system essential for mitochondrial function.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990# GENE ASSOCIATION:
cytochrome c oxidase, mitochondrial Complex IV
Abbreviation: R_CYOOm3 Synonyms: _0 EC Number: 1.9.3.1 SUBSYSTEM: Oxidative Phosphorylation Equation: (4) focytC[m] + (7.92) h[m] + o2[m] --> (4) ficytC[m] + (4) h[c] + (1.96) h2o[m] + (0.02) o2s[m] Confidence Level: 4 TV, 2% O2 lost in O2- genes: LOCUS:_4514#TRANSCRIPT:1#ABBREVIATION:MTCO3# LOCUS:_4513#TRANSCRIPT:1#ABBREVIATION:MTCO2#ECNUMBERS:1.9.3.1# LOCUS:_9377#TRANSCRIPT:1#ABBREVIATION:COX5A#ECNUMBERS:1.9.3.1# LOCUS:_9167#TRANSCRIPT:1#ABBREVIATION:COX7A2L# LOCUS:_1337#TRANSCRIPT:1#ABBREVIATION:COX6A1#ECNUMBERS:1.9.3.1# LOCUS:_1347#TRANSCRIPT:1#ABBREVIATION:COX7A2#ECNUMBERS:1.9.3.1# LOCUS:_1346#TRANSCRIPT:1#ABBREVIATION:COX7A1#ECNUMBERS:1.9.3.1# LOCUS:_1329#TRANSCRIPT:1#ABBREVIATION:COX5B#ECNUMBERS:1.9.3.1# LOCUS:_1339#TRANSCRIPT:1#ABBREVIATION:COX6A2#ECNUMBERS:1.9.3.1# LOCUS:_1327#TRANSCRIPT:1#ABBREVIATION:COX4I1#ECNUMBERS:1.9.3.1# LOCUS:_1345#TRANSCRIPT:1#ABBREVIATION:COX6C#ECNUMBERS:1.9.3.1# LOCUS:_1350#TRANSCRIPT:1#ABBREVIATION:COX7C# LOCUS:_84701#TRANSCRIPT:1#ABBREVIATION:COX4I2# LOCUS:_4512#TRANSCRIPT:1#ABBREVIATION:MTCO1# LOCUS:_1340#TRANSCRIPT:1#ABBREVIATION:COX6B1#ECNUMBERS:1.9.3.1# LOCUS:_1351#TRANSCRIPT:1#ABBREVIATION:COX8#ECNUMBERS:1.9.3.1# LOCUS:_1349#TRANSCRIPT:1#ABBREVIATION:COX7B# LOCUS:_341947#TRANSCRIPT:1#ABBREVIATION:COX8C# LOCUS:_125965#TRANSCRIPT:1#ABBREVIATION:COX6B2# LOCUS:_170712#TRANSCRIPT:1#ABBREVIATION:COX7B2#ECNUMBERS:1.9.3.1# proteins: NAME:cytochrome c oxidase subunit IV isoform 1#ABBREVIATION:Cox4i1.1-m# NAME:cytochrome c oxidase subunit Vb #ABBREVIATION:Cox5b.1-m# NAME:cytochrome c oxidase subunit VIa polypeptide 1#ABBREVIATION:Cox6a1.1-m# NAME:cytochrome c oxidase subunit VIa polypeptide 2#ABBREVIATION:Cox6a2.1-m# NAME:cytochrome c oxidase subunit Vib polypeptide 1 (ubiquitous) #ABBREVIATION:Cox6b1.1-m# NAME:cytochrome c oxidase subunit VIc#ABBREVIATION:Cox6c.1-m# NAME:cytochrome c oxidase subunit VIIa polypeptide 1 (muscle)#ABBREVIATION:Cox7a1.1-m# NAME:cytochrome c oxidase subunit VIIa polypeptide 2 (liver)#ABBREVIATION:Cox7a2.1-m# NAME:cytochrome c oxidase subunit VIIb#ABBREVIATION:Cox7b.1-m# NAME:cytochrome c oxidase subunit IV isoform 2 (lung)#ABBREVIATION:Cox4i2.1-m# NAME:cytochrome c oxidase subunit VIIa polypeptide 2 like#ABBREVIATION:Cox7a2l.1-m# NAME:cytochrome c oxidase subunit Va#ABBREVIATION:Cox5a.1-m# NAME:cytochrome c oxidase subunit VIIc #ABBREVIATION:Cox7c.1-m# NAME:cytochrome c oxidase subunit 8A (ubiquitous)#ABBREVIATION:Cox8a.1-m# NAME:cytochrome c oxidase I#ABBREVIATION:Cox1.1-m# NAME:cytochrome c oxidase II#ABBREVIATION:Cox2.1-m# NAME:cytochrome c oxidase III#ABBREVIATION:Cox3.1-m# NAME:cytochrome c oxidase subunit 8C#ABBREVIATION:Cox8c.1-m# NAME:cytochrome c oxidase subunit VIb, testis-specific isoform#ABBREVIATION:Cox6b2.1-m# NAME:cytochrome c oxidase subunit VIIb2 #ABBREVIATION:Cox7b2.1-m# citations: none GENE ASSOCIATION: (1351.1) and (1329.1) and (1347.1) and (1327.1) and (9167.1) and (341947.1) and (4512.1) and (1350.1) and (1349.1) and (1339.1) and (4513.1) and (9377.1) and (1345.1) and (125965.1) and (4514.1) and (170712.1) and (1346.1) and (1340.1) and (1337.1) and (84701.1)
Abbreviation: R_PPA Synonyms: _3 EC Number: 3.6.1.1 SUBSYSTEM: Oxidative Phosphorylation Equation: [c] : h2o + ppi --> h + (2) pi Confidence Level: 4 genes: LOCUS:_5464#TRANSCRIPT:1#ABBREVIATION:PP#ECNUMBERS:3.6.1.1# proteins: NAME:Pyrophosphatase (inorganic), cytosolic#ABBREVIATION:Pp.1# citations: AUTHORS:Fairchild TA, Patejunas G.#TITLE:Cloning and expression profile of human inorganic pyrophosphatase.#JOURNAL:Biochim Biophys Acta#YEAR:1999#PUBMED:10542310# AUTHORS:Fabrichniy IP, Lehtio L, Salminen A, Zyryanov AB, Baykov AA, Lahti R, Goldman A.#TITLE:Structural studies of metal ions in family II pyrophosphatases: the requirement for a Janus ion.#JOURNAL:Biochemistry#YEAR:2004#PUBMED:15533045# GENE ASSOCIATION: (5464.1)
Abbreviation: R_EX_dgsn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dgsn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PA_HSter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: pa_hs[c] <==> pa_hs[r] Confidence Level: 2 for assumed reversible, non-energy dep scramblase mechanism (PMID: 10856717). NJ genes: none proteins: none citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# GENE ASSOCIATION:
Abbreviation: R_10FTHFtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _10fthf[c] <==> 10fthf[l] Confidence Level: 2 NCD, IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_10-Formyltetrahydrofolate lysosomal transport via diffusion
Abbreviation: R_SUCOAS1m Synonyms: _0 EC Number: 6.2.1.4 SUBSYSTEM: Citric Acid Cycle Equation: [m] : coa + gtp + succ <==> gdp + pi + succoa Confidence Level: 0 IF genes: LOCUS:_8802#TRANSCRIPT:1#ABBREVIATION:SUCLG1#ECNUMBERS:6.2.1.4# LOCUS:_8801#TRANSCRIPT:1#ABBREVIATION:SUCLG2#ECNUMBERS:6.2.1.4# proteins: NAME:Succinate-CoA ligase (GDP-forming), mitochondiral#ABBREVIATION:Suclg-m# citations: AUTHORS:Johnson JD, Mehus JG, Tews K, Milavetz BI, Lambeth DO#TITLE:Genetic evidence for the expression of ATP- and GTP-specific succinyl-CoA synthetases in multicellular eucaryotes#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9765291# GENE ASSOCIATION: (8802.1 and 8801.1)
Abbreviation: R_GLYOX Synonyms: _1 EC Number: 3.1.2.6 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : h2o + lgt-S --> gthrd + h + lac-D Confidence Level: 4 IF genes: LOCUS:_3029#TRANSCRIPT:1#ABBREVIATION:HAGH#ECNUMBERS:3.1.2.6# LOCUS:_84264#TRANSCRIPT:1#ABBREVIATION:HAGHL# LOCUS:_84264#TRANSCRIPT:2#ABBREVIATION:HAGHL# proteins: NAME:hydroxyacylglutathione hydrolase #ABBREVIATION:Hagh.1# NAME:hydroxyacylglutathione hydrolase-like#ABBREVIATION:Haghl.1# NAME:hydroxyacylglutathione hydrolase-like#ABBREVIATION:Haghl.2# citations: AUTHORS:Cordell PA, Futers TS, Grant PJ, Pease RJ#TITLE:The Human hydroxyacylglutathione hydrolase (HAGH) gene encodes both cytosolic and mitochondrial forms of glyoxalase II.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117945# AUTHORS:Ridderstrom M, Saccucci F, Hellman U, Bergman T, Principato G, Mannervik B#TITLE:Molecular cloning, heterologous expression, and characterization of human glyoxalase II#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8550579# GENE ASSOCIATION: (3029.1) or (84264.2) or (84264.1)
Abbreviation: R_SERGLYexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gly[c] + ser-L[e] <==> gly[e] + ser-L[c] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_CHTNASE Synonyms: _0 EC Number: 3.2.1.14 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : chtn + (2) h2o --> (3) acgam Confidence Level: 4 NCD genes: LOCUS:_27159#TRANSCRIPT:1#ABBREVIATION:CHIA# LOCUS:_54682#TRANSCRIPT:1#ABBREVIATION:MANSC1# LOCUS:_1118#TRANSCRIPT:1#ABBREVIATION:CHIT1# proteins: NAME:eosinophil chemotactic cytokine #ABBREVIATION:Chia.1# NAME:MANSC domain containing 1 #ABBREVIATION:Mansc1.1# NAME:chitinase 1 (chitotriosidase) (macrophage)#ABBREVIATION:Chit.1# citations: AUTHORS:Boot RG, Blommaart EF, Swart E, Ghauharali-van der Vlugt K, Bijl N, Moe C, Place A, Aerts JM#TITLE:Identification of a novel acidic mammalian chitinase distinct from chitotriosidase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11085997# GENE ASSOCIATION: (27159.1) or (54682.1) or (1118.1)
Abbreviation: R_EX_elaid_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : elaid <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_3AIBTm Synonyms: _4 EC Number: 2.6.1.22 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 2mop + glu-L <==> 3aib + akg Confidence Level: 2 NCD genes: LOCUS:_18#TRANSCRIPT:1#ABBREVIATION:ABAT#ECNUMBERS:2.6.19.4# LOCUS:_18#TRANSCRIPT:2#ABBREVIATION:ABAT#ECNUMBERS:2.6.19.4# proteins: NAME:_4-aminobutyrate aminotransferase, mitochondrial#ABBREVIATION:Abat.1-m# NAME:_4-aminobutyrate aminotransferase, mitochondrial#ABBREVIATION:Abat.2-m# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (18.1) or (18.2)
L-3-aminoisobutyrate transaminase, mitochondrial
Abbreviation: R_S4T4g Synonyms: _1 EC Number: 2.8.2.5 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_c,d,e_pre1 + (2) paps --> cs_e_pre2 + (2) h + (2) pap Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Hiraoka N, Nakagawa H, Ong E, Akama TO, Fukuda MN, Fukuda M#TITLE:Molecular cloning and expression of two distinct human chondroitin 4-O-sulfotransferases that belong to the HNK-1 sulfotransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781601# GENE ASSOCIATION:
Abbreviation: R_SLDx Synonyms: _3 EC Number: 1.1.1.272 SUBSYSTEM: Cysteine Metabolism Equation: [c] : nad + sl-L <==> 3spyr + h + nadh Confidence Level: 2 AMF EC: 1.1.1.272 genes: LOCUS:_4190#TRANSCRIPT:1#ABBREVIATION:MDH1#ECNUMBERS:1.1.1.37# LOCUS:_130752#TRANSCRIPT:1#ABBREVIATION:MDH1B# proteins: NAME:malate dehydrogenase 1 (NAD), soluble#ABBREVIATION:Mdh1.1# NAME:malate dehydrogenase 1b (NAD), soluble#ABBREVIATION:Mdh1b.1# citations: AUTHORS:Weinstein CL, Griffith OW.#TITLE:Cysteinesulfonate and beta-sulfopyruvate metabolism. Partitioning between decarboxylation, transamination, and reduction pathways.#YEAR:1988#PUBMED:3346220# AUTHORS:Rein U, Gueta R, Denger K, Ruff J, Hollemeyer K, Cook AM.#TITLE:Dissimilation of cysteate via 3-sulfolactate sulfo-lyase and a sulfate exporter in Paracoccus pantotrophus NKNCYSA.#YEAR:2005#PUBMED:15758220# GENE ASSOCIATION: (4190.1) or (130752.1)
Abbreviation: R_PE_HSter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: pe_hs[c] <==> pe_hs[r] Confidence Level: 2 NCD for assumed reversible, non-energy dep scramblase mechanism (PMID: 10856717). NJ genes: none proteins: none citations: AUTHORS:Chang QL, Gummadi SN, Menon AK#TITLE:Chemical modification identifies two populations of glycerophospholipid flippase in rat liver ER#JOURNAL:Biochemistry#YEAR:2004#PUBMED:15311932# AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# GENE ASSOCIATION:
Abbreviation: R_EX_ach_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ach <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RETI1 Synonyms: _0 EC Number: 5.2.1.7 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : retinol <==> retinol-cis-11 Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_PSDm_hs Synonyms: _0 EC Number: 4.1.1.65 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [m] : h + ps_hs --> co2 + pe_hs Confidence Level: 4 modified from TV version for hs NJ genes: LOCUS:_23761#TRANSCRIPT:1#ABBREVIATION:PISD#ECNUMBERS:4.1.1.65# proteins: NAME:phosphatidylserine decarboxylase, mitochondrial #ABBREVIATION:Pisd.1-m# citations: AUTHORS:Voelker DR.#TITLE:Phosphatidylserine decarboxylase.#JOURNAL:Biochim Biophys Acta#YEAR:1997#PUBMED:9370338# AUTHORS:Hatch GM.#TITLE:Cell biology of cardiac mitochondrial phospholipids.#JOURNAL:Biochem Cell Biol#YEAR:2004#PUBMED:15052331# GENE ASSOCIATION: (23761.1)
Abbreviation: R_NACHEX19ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg34 --> acgam + ksi_deg35 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3074.1) or (3073.1 and 3074.1) or (3073.1)
phosphatidylinositol 3-phosphate 4-kinase, nuclear
Abbreviation: R_PI3P4Kn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + pail3p_hs --> adp + h + pail34p_hs Confidence Level: 2 NCD FULLR corresponds to acyl/R groups in texts. NJ changed EC from 2.7.1.149 (pail5p) to 2.7.1.68 (pail4p) - NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
Abbreviation: R_LGTHL Synonyms: _1 EC Number: 4.4.1.5 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : gthrd + mthgxl --> lgt-S Confidence Level: 4 IF genes: LOCUS:_2739#TRANSCRIPT:1#ABBREVIATION:GLO1#ECNUMBERS:4.4.1.5# proteins: NAME:glyoxalase I#ABBREVIATION:Glo1.1# citations: AUTHORS:Kim NS, Umezawa Y, Ohmura S, Kato S#TITLE:Human glyoxalase I. cDNA cloning, expression, and sequence similarity to glyoxalase I from Pseudomonas putida#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:7684374# AUTHORS:Ranganathan S, Walsh ES, Godwin AK, Tew KD#TITLE:Cloning and characterization of human colon glyoxalase-I#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8449929# GENE ASSOCIATION: (2739.1)
Abbreviation: R_P45017A1r Synonyms: _4 EC Number: 1.14.99.9 SUBSYSTEM: Steroid Metabolism Equation: [r] : h + nadph + o2 + prgnlone --> 17ahprgnlone + h2o + nadp Confidence Level: 4 cytosolic by default - RETURN AND CHECK AFTER DEEPER LIT SEARCH NJ genes: LOCUS:_1586#TRANSCRIPT:1#ABBREVIATION:CYP17A1#ECNUMBERS:1.14.99.9# proteins: NAME:cytochrome P450, family 17, subfamily A, polypeptide 1#ABBREVIATION:Cyp17a1.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# AUTHORS:Korzekwa KR, Trager WF, Mancewicz J, Osawa Y#TITLE:Studies on the mechanism of aromatase and other cytochrome P450 mediated deformylation reactions#JOURNAL:J Steroid Biochem Mol Biol#YEAR:1993# GENE ASSOCIATION: (1586.1)
Abbreviation: R_B3GNT37g Synonyms: _9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gm2a_hs + udpgal --> gm1a_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10331.1)
GM2alpha (homo sapiens)_C49H85N3O25FULLRCO
Abbreviation: R_CHOLD2m Synonyms: _0 EC Number: 1.1.99.1 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : chol + fad --> betald + fadh2 Confidence Level: 4 genes: LOCUS:_55349#TRANSCRIPT:1#ABBREVIATION:CHDH#ECNUMBERS:1.1.99.1# proteins: NAME:choline dehydrogenase, mitochondrial#ABBREVIATION:Chdh.1-m# citations: AUTHORS:Craig SA.#TITLE:Betaine in human nutrition.#YEAR:2004#PUBMED:15321791# GENE ASSOCIATION: (55349.1)
choline dehydrogenase (FAD acceptor), mitochondrial
Abbreviation: R_NDP8ex Synonyms: _0 EC Number: 3.6.1.6 SUBSYSTEM: Nucleotides Equation: [e] : h2o + utp --> h + pi + udp Confidence Level: 4 genes: LOCUS:_124583#TRANSCRIPT:1#ABBREVIATION:CANT1# proteins: NAME:calcium activated nucleotidase 1 #ABBREVIATION:Cant1.1-e# citations: AUTHORS:Smith TM, Hicks-Berger CA, Kim S, Kirley TL.#TITLE:Cloning, expression, and characterization of a soluble calcium-activated nucleotidase, a human enzyme belonging to a new family of extracellular nucleotidases.#JOURNAL:Arch Biochem Biophys. #YEAR:2002#PUBMED:12234496# AUTHORS:Murphy DM, Ivanenkov VV, Kirley TL. #TITLE:Bacterial expression and characterization of a novel, soluble, calcium-binding, and calcium-activated human nucleotidase. #JOURNAL:Biochemistry#YEAR:2003#PUBMED:12600208# GENE ASSOCIATION: (124583.1)
nucleoside-diphosphatase (UTP), extracellular
Abbreviation: R_NACHEXA10ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg9 --> acgam + h + ksi_deg11 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_GLCtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: glc-D[c] <==> glc-D[g] Confidence Level: 4 NCD genes: LOCUS:_6513#TRANSCRIPT:1#ABBREVIATION:SLC2A1# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 1, Golgi apparatus#ABBREVIATION:Slc2a1.1-g# citations: AUTHORS:Haney PM#TITLE:Glucose transport in lactation#JOURNAL:Adv Exp Med Biol#YEAR:2004#PUBMED:15384581# GENE ASSOCIATION: (6513.1)
Abbreviation: R_3AIBtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _3aib[m] --> 3aib[c] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION:
_3-amino-isobutyrate transport, mitochondrial
Abbreviation: R_NS26Tg Synonyms: _0 EC Number: 2.4.99.3 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : Tn_antigen + cmpacna --> cmp + h + sTn_antigen Confidence Level: 0 NCD genes: LOCUS:_55808#TRANSCRIPT:1#ABBREVIATION:SIAT7A# proteins: NAME:sialyltransferase 7, Golgi apparatus (intestine)#ABBREVIATION:Siat7a.1-g# citations: none GENE ASSOCIATION: (55808.1)
alpha-N-acetylgalactosaminide alpha-2,6-sialyltransferase
Abbreviation: R_SPHK21c Synonyms: _3 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : atp + sphings --> adp + h + sphs1p Confidence Level: 4 NJ genes: LOCUS:_56848#TRANSCRIPT:1#ABBREVIATION:SPHK2# proteins: NAME:sphingosine kinase 2 #ABBREVIATION:Sphk2.1# citations: AUTHORS:Liu H, Sugiura M, Nava VE, Edsall LC, Kono K, Poulton S, Milstien S, Kohama T, Spiegel S.#TITLE:Molecular cloning and functional characterization of a novel mammalian sphingosine kinase type 2 isoform.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10751414# GENE ASSOCIATION: (56848.1)
Abbreviation: R_NRPPHRtu Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: nrpphr[e] <==> nrpphr[c] Confidence Level: 4 SAB electrogenic, Na indepdendent & rev NJ genes: LOCUS:_6582#TRANSCRIPT:1#ABBREVIATION:SLC22A2# LOCUS:_6582#TRANSCRIPT:2#ABBREVIATION:SLC22A2# LOCUS:_6581#TRANSCRIPT:1#ABBREVIATION:SLC22A3# proteins: NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.2# NAME:solute carrier family 22 (extraneuronal monoamine transporter), member 3#ABBREVIATION:Slc22a3.1# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# GENE ASSOCIATION: (6582.1) or (6581.1) or (6582.2)
Abbreviation: R_UTPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: utp[c] <==> utp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MM8Ber Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : h2o + m8masn --> m7masnB + man Confidence Level: 0 NCD genes: LOCUS:_11253#TRANSCRIPT:1#ABBREVIATION:MAN1B1#ECNUMBERS:3.2.1.113# proteins: NAME:mannosidase, alpha, class 1B, member 1, endoplasmic reticulum (ubiquitous)#ABBREVIATION:Man1b1.1-er# citations: AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# AUTHORS:Gonzalez DS, Karaveg K, Vandersall-Nairn AS, Lal A, Moremen KW.#TITLE:Identification, expression, and characterization of a cDNA encoding human endoplasmic reticulum mannosidase I, the enzyme that catalyzes the first mannose trimming step in mammalian Asn-linked oligosaccharide biosynthesis.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10409699# GENE ASSOCIATION: (11253.1)
mannosyl-oligosaccharide 1,2-alpha-mannosidase, endoplasmic reticulum
Abbreviation: R_DGCHOLte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dgchol[e] <==> dgchol[c] Confidence Level: 1 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NDPK10m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + didp <==> adp + ditp Confidence Level: 2 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:dIDP), mitochondrial
Abbreviation: R_EX_crvnc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : crvnc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TSTSTERONEGLCtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: tststeroneglc[c] <==> tststeroneglc[r] Confidence Level: 2 unknown mechanism, however glucuronidated metabolites need to get out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_4HBZCOAFm Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : coa + coucoa + h2o + nad --> 4hbzcoa + accoa + h + nadh Confidence Level: 2 IT reaction involved in formation of 4-hydroxybenzoate from tyrosine. genes: none proteins: none citations: AUTHORS:Meganathan R.#TITLE:Ubiquinone biosynthesis in microorganisms.#JOURNAL:FEMS Microbiol Lett. #YEAR:2001#PUBMED:11583838# AUTHORS:Loscher R, Heide L.#TITLE:Biosynthesis of p-Hydroxybenzoate from p-Coumarate and p-Coumaroyl-Coenzyme A in Cell-Free Extracts of Lithospermum erythrorhizon Cell Cultures. #JOURNAL:Plant Physiol.#YEAR:1994#PUBMED:12232327# AUTHORS:Booth, A. N., Masri, M. S., Robbins, D. J., Emerson, O. H., Jones, F. T. & DeEds, F.#TITLE:Urinary Phenolic Acid Metabolites of Tyrosine#JOURNAL:J. Biol. Chem#YEAR:1960# GENE ASSOCIATION:
Abbreviation: R_5HOXINDACTOX Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 5hoxindact + h2o + nad --> 5hoxindoa + (2) h + nadh Confidence Level: 2 SAB genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# citations: AUTHORS:Keung WM#TITLE:Biogenic aldehyde(s) derived from the action of monoamine oxidase may mediate the antidipsotropic effect of daidzin#JOURNAL:Chem Biol Interact#YEAR:2001#PUBMED:11306106# GENE ASSOCIATION: (501.1) or (8854.3) or (216.1) or (8854.2) or (223.1) or (8854.1) or (224.1)
_5-Hydroxyindoleacetaldehyde:NAD+ oxidoreductase
Abbreviation: R_DM_Asn_DASH_X_DASH_Ser_FSLASH_Thr_LPAREN_ly_RPAREN_ Synonyms: _0 Equation: [l] : Asn-X-Ser/Thr --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RIBFLVt3 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + ribflv[e] --> adp[c] + h[c] + pi[c] + ribflv[c] Confidence Level: 4 _01-25-05 IT based on SAid et al., 1994, Am. Physiol. Soc., G15-G21. genes: none proteins: none citations: AUTHORS:Said HM, Ma TY. #TITLE:Mechanism of riboflavine uptake by Caco-2 human intestinal epithelial cells.#JOURNAL:Am J Physiol. #YEAR:2003#PUBMED:8304455# GENE ASSOCIATION:
Abbreviation: R_SUCCt4_2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) na1[e] + succ[e] <==> (2) na1[c] + succ[c] Confidence Level: 4 NCD genes: LOCUS:_9058#TRANSCRIPT:1#ABBREVIATION:SLC13A2# proteins: NAME:solute carrier family 13 (sodium-dependent dicarboxylate transporter), member 2#ABBREVIATION:Slc13a2.1-e# citations: AUTHORS:Pajor AM, Sun N#TITLE:Functional differences between rabbit and human Na(+)-dicarboxylate cotransporters, NaDC-1 and hNaDC-1.#JOURNAL:Am J Physiol#YEAR:1996#PUBMED:8946005# AUTHORS:Pajor AM#TITLE:Molecular cloning and functional expression of a sodium-dicarboxylate cotransporter from human kidney#JOURNAL:Am J Physiol#YEAR:1996#PUBMED:8967342# GENE ASSOCIATION: (9058.1)
Abbreviation: R_KHK Synonyms: _1 EC Number: 2.7.1.3 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : atp + fru --> adp + f1p + h Confidence Level: 0 NCD genes: LOCUS:_3795#TRANSCRIPT:1#ABBREVIATION:KHK#ECNUMBERS:2.7.1.3# LOCUS:_3795#TRANSCRIPT:2#ABBREVIATION:KHK#ECNUMBERS:2.7.1.3# proteins: NAME:ketohexokinase (fructokinase)#ABBREVIATION:Khk.1# NAME:ketohexokinase (fructokinase) (liver, kidney, sm intestine)#ABBREVIATION:Khk.2# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (3795.1) or (3795.2)
Abbreviation: R_THBPT4ACAMDASE Synonyms: _0 EC Number: 4.2.1.96 SUBSYSTEM: Tetrahydrobiopterin Equation: [c] : thbpt4acam --> dhbpt + h2o Confidence Level: 4 SAB based on PMID 10727395 genes: LOCUS:_5092#TRANSCRIPT:1#ABBREVIATION:PCBD#ECNUMBERS:4.2.1.96# LOCUS:_5092#TRANSCRIPT:2#ABBREVIATION:PCBD#ECNUMBERS:4.2.1.96# proteins: NAME:_6-pyruvoyl-tetrahydropterin synthase#ABBREVIATION:Pcbd1.1# NAME:_6-pyruvoyl-tetrahydropterin synthase#ABBREVIATION:Pcbd1.2# citations: AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (5092.1) or (5092.2)
Tetrahydrobiopterin-4a-carbinolamine dehydratase
Abbreviation: R_24_COMMA_25VITD2Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : 25hvitd2 + h + nadph + o2 --> 2425dhvitd2 + h2o + nadp Confidence Level: 4 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.196 IT genes: LOCUS:_1591#TRANSCRIPT:1#ABBREVIATION:CYP24A1# proteins: NAME:cytochrome P450, family 24, subfamily A, polypeptide 1 #ABBREVIATION:Cyp24a1.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Kusudo T, Sakaki T, Abe D, Fujishima T, Kittaka A, Takayama H, Hatakeyama S, Ohta M, Inouye K.#TITLE:Metabolism of A-ring diastereomers of 1alpha,25-dihydroxyvitamin D3 by CYP24A1. #JOURNAL:Biochem Biophys Res Commun.#YEAR:2004#PUBMED:15358094# AUTHORS:Labuda M, Lemieux N, Tihy F, Prinster C, Glorieux FH.#TITLE:Human 25-hydroxyvitamin D 24-hydroxylase cytochrome P450 subunit maps to a different chromosomal location than that of pseudovitamin D-deficient rickets. #JOURNAL:J Bone Miner Res.#YEAR:1993#PUBMED:8266831# GENE ASSOCIATION: (1591.1)
Abbreviation: R_MI1PS Synonyms: _0 EC Number: 5.5.1.4 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : g6p --> mi1p-D Confidence Level: 4 NCD genes: LOCUS:_51477#TRANSCRIPT:1#ABBREVIATION:ISYNA1#ECNUMBERS:5.5.1.4# proteins: NAME:myo-inositol 1-phosphate synthase A1#ABBREVIATION:Isyna1.1# citations: AUTHORS:Guan G, Dai P, Shechter I.#TITLE:cDNA cloning and gene expression analysis of human myo-inositol 1-phosphate synthase#JOURNAL:Arch Biochem Biophys#YEAR:2003#PUBMED:12941308# GENE ASSOCIATION: (51477.1)
cob(I)alamin adenosyltransferase, mitochondrial
Abbreviation: R_CBLATm Synonyms: _0 EC Number: 2.5.1.17 SUBSYSTEM: Vitamin B12 Metabolism Equation: [m] : atp + cbl1 + h <==> adocbl + pppi Confidence Level: 4 JLR (EC 2.5.1.17) IT genes: LOCUS:_166785#TRANSCRIPT:1#ABBREVIATION:MMAA# proteins: NAME:methylmalonic aciduria (cobalamin deficiency) type A #ABBREVIATION:Mmaa1.1-m# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Seetharam B, Alpers DH.#TITLE:Absorption and transport of cobalamin (vitamin B12).#JOURNAL:Annu Rev Nutr.#YEAR:1982#PUBMED:6313022# AUTHORS:Carmel R, Green R, Rosenblatt DS, Watkins D.#TITLE:Update on cobalamin, folate, and homocysteine.#JOURNAL:Hematology (Am Soc Hematol Educ Program).#YEAR:2003#PUBMED:14633777# AUTHORS:Dobson CM, Wai T, Leclerc D, Wilson A, Wu X, Dore C, Hudson T, Rosenblatt DS, Gravel RA.#TITLE:Identification of the gene responsible for the cblA complementation group of vitamin B12-responsive methylmalonic acidemia based on analysis of prokaryotic gene arrangements. #JOURNAL:Proc Natl Acad Sci U S A. #YEAR:2002#PUBMED:12438653# GENE ASSOCIATION: (166785.1)
Abbreviation: R_DNDPt34m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgdp[c] + dtdp[m] --> dgdp[m] + dtdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
beta-D-Glucosyl-2-coumarinate glucohydrolase
Abbreviation: R_BDG2HCGHD Synonyms: _0 EC Number: 3.2.1.21 SUBSYSTEM: Stilbene, coumarine and lignin biosynthesis Equation: [c] : bdg2hc + h2o --> 2coum + glc-D + h Confidence Level: 1 SAB genes: LOCUS:_57733#TRANSCRIPT:1#ABBREVIATION:GBA3#ECNUMBERS:3.2.1.21# proteins: NAME:glucosidase, beta, acid 3 (cytosolic)#ABBREVIATION:Gba3.1# citations: AUTHORS:Daniels LB, Coyle PJ, Chiao YB, Glew RH, Labow RS.#TITLE:Purification and characterization of a cytosolic broad specificity beta-glucosidase from human liver#JOURNAL:J Biol Chem#YEAR:1981#PUBMED:6796580# GENE ASSOCIATION: (57733.1)
Abbreviation: R_ALAGLNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + gln-L[c] + na1[e] --> ala-L[c] + gln-L[e] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-alanine/L-glutamine Na-dependent exchange (Ala-L in)
Abbreviation: R_CHLPCTD Synonyms: _0 EC Number: 2.7.7.15 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : cholp + ctp + h --> cdpchol + ppi Confidence Level: 4 NCD genes: LOCUS:_5130#TRANSCRIPT:1#ABBREVIATION:PCYT1A# LOCUS:_9468#TRANSCRIPT:1#ABBREVIATION:PCYT1B#ECNUMBERS:2.7.7.15# proteins: NAME:phosphate cytidylyltransferase 1, choline, beta isoform#ABBREVIATION:Pcyt1b.1# NAME:phosphate cytidylyltransferase 1, choline, alpha isoform#ABBREVIATION:Pcyt1a.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Kent C#TITLE:Eukaryotic phospholipid biosynthesis#JOURNAL:Annu Rev Biochem#YEAR:1995# GENE ASSOCIATION: (5130.1) or (9468.1)
Abbreviation: R_TSTSTERONEGLCte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + tststeroneglc[c] --> adp[c] + h[c] + pi[c] + tststeroneglc[e] Confidence Level: 2 for MDR transport NJ genes: LOCUS:_4363#TRANSCRIPT:1#ABBREVIATION:ABCC1.1# proteins: NAME:ATP-binding cassette, subfamily C (CFTR/MRP), member 1#ABBREVIATION:ABCC1.1# citations: none GENE ASSOCIATION: (4363.1)
Abbreviation: R_DOLPMT2_Uer Synonyms: _2 EC Number: 2.4.1.130 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolmanp_U + (0.1) m5mpdol_U --> (0.1) dolp_U + h + (0.1) m6mpdol_U Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
(alpha-D-Mannosyl)5-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1545H2540N20O470P20
Abbreviation: R_FUCFUC12GAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucfuc12gal14acglcgalgluside_hs[e] <==> fucfuc12gal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_OAGT3te Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: oagt3_hs[c] <==> oagt3_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_ASAH1 Synonyms: _4 EC Number: 3.5.1.23 SUBSYSTEM: Sphingolipid Metabolism Equation: [l] : crm_hs + h2o --> Rtotal + sphings Confidence Level: 4 NJ genes: LOCUS:_427#TRANSCRIPT:1#ABBREVIATION:ASAH1#ECNUMBERS:3.5.1.23# LOCUS:_427#TRANSCRIPT:2#ABBREVIATION:ASAH1# proteins: NAME:N-acylsphingosine amidohydrolase (acid ceramidase) 1 #ABBREVIATION:Asah1.1# NAME:N-acylsphingosine amihydrolase (acid ceramidase 1)#ABBREVIATION:Asah1.2# citations: AUTHORS:Koch J, Gartner S, Li CM, Quintern LE, Bernardo K, Levran O, Schnabel D, Desnick RJ, Schuchman EH, Sandhoff K.#TITLE:Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification Of the first molecular lesion causing Farber disease.#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8955159# GENE ASSOCIATION: (427.1) or (427.2)
Abbreviation: R_DOLASNT_Uer Synonyms: _0 EC Number: 2.4.1.119 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : Asn-X-Ser/Thr + (0.1) g3m8mpdol_U --> (0.1) doldp_U + g3m8masn + h Confidence Level: 0 NCD genes: LOCUS:_6185#TRANSCRIPT:1#ABBREVIATION:RPN2# LOCUS:_6184#TRANSCRIPT:1#ABBREVIATION:RPN1# LOCUS:_1650#TRANSCRIPT:1#ABBREVIATION:DDOST# LOCUS:_1603#TRANSCRIPT:1#ABBREVIATION:DAD1# proteins: NAME:oligosaccharyltransferase complex, endoplasmic reticulum (ubiquitous)#ABBREVIATION:Ost-er# citations: AUTHORS:Nakashima T, Sekiguchi T, Kuraoka A, Fukushima K, Shibata Y, Komiyama S, Nishimoto T.#TITLE:Molecular cloning of a human cDNA encoding a novel protein, DAD1, whose defect causes apoptotic cell death in hamster BHK21 cells.#JOURNAL:Mol Cell Biol#YEAR:1993#PUBMED:8413235# AUTHORS:Yamagata T, Tsuru T, Momoi MY, Suwa K, Nozaki Y, Mukasa T, Ohashi H, Fukushima Y, Momoi T#TITLE:Genome organization of human 48-kDa oligosaccharyltransferase (DDOST)#JOURNAL:Genomics#YEAR:1997#PUBMED:9367678# GENE ASSOCIATION: (6185.1 and 1650.1 and 6184.1 and 1603.1)
(alpha-D-Glucosyl)3-(alpha-D-mannosyl)8-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human uterine homolog_C1905H3140N20O770P20
Dolichyl-diphosphooligosaccharide:protein-L-asparagine oligopolysaccharidotransferase (uterus)
Abbreviation: R_AATAi Synonyms: _5 EC Number: 2.6.1.39 SUBSYSTEM: Lysine Metabolism Equation: [c] : L2aadp + akg --> 2oxoadp + glu-L Confidence Level: 4 MM genes: LOCUS:_51166#TRANSCRIPT:1#ABBREVIATION:AADAT# LOCUS:_51166#TRANSCRIPT:2#ABBREVIATION:AADAT# proteins: NAME:aminoadipate aminotransferase#ABBREVIATION:Aadat.1# NAME:aminoadipate aminotransferase#ABBREVIATION:Aadat.2# citations: AUTHORS:Cox, RP#TITLE:Errors of lysine metabolism#JOURNAL:The Metabolic and Molecular Bases of Inherited Disease, 8th ed#YEAR:2001# AUTHORS:Goh,D.L.M., Patel,A., Thomas,G.H., Salomons,G.S., Schor,D.S.M., Jakobs,C., Geraghty,M.T., #TITLE:Characterization of the human gene encoding alpha-aminoadipate aminotransferase (AADAT). #YEAR:2002#PUBMED:12126930# GENE ASSOCIATION: (51166.1) or (51166.2)
_2-aminoadipate transaminase, irreversible
Abbreviation: R_EX_phyQ_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : phyQ <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
UDP-GlcNAc:N-acetyl-D-glucosaminyl diphosphodolichol N-acetyl-D-glucosaminyltransferase (liver)
N,N'-Diacetylchitobiosyldiphosphodolichol, human liver homolog_C1240H2028N20O170P20
N-Acetyl-D-glucosaminyldiphosphodolichol, human liver homolog_C1160H1898N10O120P20
Abbreviation: R_GLCNACT_L Synonyms: _1 EC Number: 2.4.1.141 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) naglc2p_L + uacgam --> (0.1) chito2pdol_L + h + udp Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SELCYSLY2 Synonyms: _2 SUBSYSTEM: Selenoamino acid metabolism Equation: [c] : pdx5p + selcys --> ala-L + h + pydx5p + seln Confidence Level: 4 Added based on PMID: 1483038 - pyridoxal 5 phosphate is required, it is not completely clear however if it is also the reduced/oxidized compound NJ genes: LOCUS:_51540#TRANSCRIPT:1#ABBREVIATION:SCLY# proteins: NAME:selenocysteine lyase#ABBREVIATION:Scly.1# citations: AUTHORS:Daher R, Van Lente F.#TITLE:Characterization of selenocysteine lyase in human tissues and its relationship to tissue selenium concentrations#JOURNAL:J Trace Elem Electrolytes Health Dis#YEAR:1992#PUBMED:1483038# GENE ASSOCIATION: (51540.1)
Abbreviation: R_MGSA2 Synonyms: _0 EC Number: 4.2.3.3 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : g3p --> mthgxl + pi Confidence Level: 4 this rxn also represents the enzymatic conversion or non-enzymatic fragmentation of g3p to mthgxl - NCD genes: none proteins: none citations: AUTHORS:Vander Jagt DL, Robinson B, Taylor KK, Hunsaker LA.#TITLE:Reduction of trioses by NADPH-dependent aldo-keto reductases. Aldose reductase, methylglyoxal, and diabetic complications#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1537826# AUTHORS:Thornalley PJ#TITLE:Pharmacology of methylglyoxal: formation, modification of proteins and nucleic acids, and enzymatic detoxification--a role in pathogenesis and antiproliferative chemotherapy.#JOURNAL:Gen Pharmacol#YEAR:1996#PUBMED:8853285# AUTHORS:Beisswenger BG, Delucia EM, Lapoint N, Sanford RJ, Beisswenger PJ.#TITLE:Ketosis leads to increased methylglyoxal production on the atkins diet#JOURNAL:Ann N Y Acad Sci#YEAR:2005#PUBMED:16037240# GENE ASSOCIATION:
Abbreviation: R_UGT1A5r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Vitamin A Metabolism Equation: [r] : retn + udpglcur --> retnglc + udp Confidence Level: 4 NJ genes: LOCUS:_54575#TRANSCRIPT:1#ABBREVIATION:UGT1A10# LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A10#ABBREVIATION:Ugt1a10.1# NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# citations: AUTHORS:Basu NK, Kubota S, Meselhy MR, Ciotti M, Chowdhury B, Hartori M, Owens IS.#TITLE:Free Full Text Gastrointestinally distributed UDP-glucuronosyltransferase 1A10, which metabolizes estrogens and nonsteroidal anti-inflammatory drugs, depends upon phosphorylation.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117964# AUTHORS:Mackenzie PI, Owens IS, Burchell B, Bock KW, Bairoch A, Belanger A, Fournel-Gigleux S, Green M, Hum DW, Iyanagi T, Lancet D, Louisot P, Magdalou J, Chowdhury JR, Ritter JK, Schachter H, Tephly TR, Tipton KF, Nebert DW.#TITLE:The UDP glycosyltransferase gene superfamily: recommended nomenclature update based on evolutionary divergence.#JOURNAL:Pharmacogenetics#YEAR:1997#PUBMED:9295054# GENE ASSOCIATION: (54575.1) or (54576.1)
Abbreviation: R_PROt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[e] + pro-L[e] <==> h[c] + pro-L[c] Confidence Level: 2 NCD genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
L-proline reversible transport via proton symport
Abbreviation: R_CTPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: ctp[c] <==> ctp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MI14P4P Synonyms: _1 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi14p --> mi1p-D + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# GENE ASSOCIATION:
Abbreviation: R_FAOXC16080m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (4) coa + (4) fad + (4) h2o + (4) nad + pmtcoa --> (4) accoa + (4) fadh2 + (4) h + (4) nadh + occoa Confidence Level: 4 _4 rounds of beta ox c16:0 -> c8:0, 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_UGALNACtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: udpacgal[c] + ump[g] <==> udpacgal[g] + ump[c] Confidence Level: 4 NCD genes: LOCUS:_7355#TRANSCRIPT:1#ABBREVIATION:SLC35A2# proteins: NAME:solute carrier family 35 (UDP-galactose transporter), member A2#ABBREVIATION:Slc35a2.1-g# citations: AUTHORS:Ishida N, Miura N, Yoshioka S, Kawakita M#TITLE:Molecular cloning and characterization of a novel isoform of the human UDP-galactose transporter, and of related complementary DNAs belonging to the nucleotide-sugar transporter gene family#JOURNAL:J Biochem (Tokyo)#YEAR:1996#PUBMED:9010752# AUTHORS:Miura N, Ishida N, Hoshino M, Yamauchi M, Hara T, Ayusawa D, Kawakita M#TITLE:Human UDP-galactose translocator: molecular cloning of a complementary DNA that complements the genetic defect of a mutant cell line deficient in UDP-galactose translocator#JOURNAL:J Biochem (Tokyo)#YEAR:1996#PUBMED:8889805# AUTHORS:Yoshioka S, Sun-Wada GH, Ishida N, Kawakita M#TITLE:Expression of the human UDP-galactose transporter in the Golgi membranes of murine Had-1 cells that lack the endogenous transporter#JOURNAL:J Biochem (Tokyo)#YEAR:1997#PUBMED:9399569# AUTHORS:Segawa H, Kawakita M, Ishida N#TITLE:Human and Drosophila UDP-galactose transporters transport UDP-N-acetylgalactosamine in addition to UDP-galactose#JOURNAL:Eur J Biochem.#YEAR:2002#PUBMED:11784306# AUTHORS:Sun-Wada GH, Yoshioka S, Ishida N, Kawakita M#TITLE:Functional expression of the human UDP-galactose transporters in the yeast Saccharomyces cerevisiae#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9562625# GENE ASSOCIATION: (7355.1)
UDP-GalNAc Golgi transport via CMP antiport
Abbreviation: R_METAT Synonyms: _0 EC Number: 2.5.1.6 SUBSYSTEM: Methionine Metabolism Equation: [c] : atp + h2o + met-L --> amet + pi + ppi Confidence Level: 4 genes: LOCUS:_4144#TRANSCRIPT:1#ABBREVIATION:MAT2A# LOCUS:_4143#TRANSCRIPT:1#ABBREVIATION:MAT1A# LOCUS:_27430#TRANSCRIPT:1#ABBREVIATION:MAT2B# LOCUS:_27430#TRANSCRIPT:2#ABBREVIATION:MAT2B# proteins: NAME:methionine adenosyltransferase I, alpha #ABBREVIATION:Mat1a.1# NAME:methionine adenosyltransferase II 1#ABBREVIATION:Mat2ab1# NAME:methionine adenosyltransferase II 2#ABBREVIATION:Mat2ab2# citations: AUTHORS:Ubagai,T. , Lei,K.J. , Huang,S. , Mudd,S.H. , Levy,H.L. , Chou,J.Y. #TITLE:Molecular mechanisms of an inborn error of methionine pathway. Methionine adenosyltransferase deficiency.#YEAR:1995#PUBMED:7560086# AUTHORS:Horikawa,S., Tsukada,K., #TITLE:Molecular cloning and developmental expression of a human kidney S- adenosylmethionine synthetase. #YEAR:1992#PUBMED:1426236# AUTHORS:Alvarez,L., Corrales,F., Mato,J.M., #TITLE:Characterization of a full-length cDNA encoding human liver S- adenosylmethionine synthetase: tissue-specific gene expression and mRNA levels in hepatopathies. #YEAR:1993#PUBMED:8393662# GENE ASSOCIATION: (4143.1) or (27430.2 and 4144.1) or (27430.1 and 4144.1)
Abbreviation: R_HISD Synonyms: _0 EC Number: 4.3.1.3 SUBSYSTEM: Histidine Metabolism Equation: [c] : his-L --> nh4 + urcan Confidence Level: 4 genes: LOCUS:_3034#TRANSCRIPT:1#ABBREVIATION:HAL#ECNUMBERS:4.3.1.3# proteins: NAME:histidine ammonia-lyase#ABBREVIATION:Hal.1# citations: AUTHORS:Suchi M, Sano H, Mizuno H, Wada Y. #TITLE:Molecular cloning and structural characterization of the human histidase gene (HAL). #JOURNAL:Genomics#YEAR:1995#PUBMED:8530107# GENE ASSOCIATION: (3034.1)
Abbreviation: R_GCCbim Synonyms: _0 EC Number: 2.1.2.10 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : alpro + thf --> dhlpro + mlthf + nh4 Confidence Level: 4 NCD genes: LOCUS:_2731#TRANSCRIPT:1#ABBREVIATION:GLDC#ECNUMBERS:1.4.4.2# LOCUS:_275#TRANSCRIPT:1#ABBREVIATION:AMT#ECNUMBERS:2.1.2.10# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_2653#TRANSCRIPT:1#ABBREVIATION:GCSH# proteins: NAME:glycine dehydrogenase (decarboxylating; glycine decarboxylase, glycine cleavage system protein P), mitochondrial #ABBREVIATION:Gldc.1-m# NAME:aminomethyltransferase (glycine cleavage system protein T), mitochondrial #ABBREVIATION:Amt.1-m# NAME:glycine cleavage system protein H (aminomethyl carrier) #ABBREVIATION:Gcsh.1-m# NAME:dihydrolipoamide dehydrogenase, mitochondrial#ABBREVIATION:Dld.1-m# citations: AUTHORS:Lee HH, Kim do J, Ahn HJ, Ha JY, Suh SW.#TITLE:Crystal structure of T-protein of the glycine cleavage system. Cofactor binding, insights into H-protein recognition, and molecular basis for understanding nonketotic hyperglycinemia.#YEAR:2004#PUBMED:15355973# GENE ASSOCIATION: (275.1) and (1738.1) and (2731.1) and (2653.1)
glycine-cleavage complex (lipoylprotein) irreversible, mitochondrial
Abbreviation: R_PPDOx Synonyms: _0 EC Number: 1.1.1.21 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : h + lald-D + nadh --> 12ppd-R + nad Confidence Level: 4 NCD genes: LOCUS:_8574#TRANSCRIPT:1#ABBREVIATION:AKR7A2# proteins: NAME:aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)#ABBREVIATION:Akr7a2.1# citations: AUTHORS:Kelly VP, Sherratt PJ, Crouch DH, Hayes JD#TITLE:Novel homodimeric and heterodimeric rat gamma-hydroxybutyrate synthases that associate with the Golgi apparatus define a distinct subclass of aldo-keto reductase 7 family proteins.#JOURNAL:Biochem J#YEAR:2002#PUBMED:12071861# AUTHORS:Praml C, Savelyeva L, Perri P, Schwab M.#TITLE:Cloning of the human aflatoxin B1-aldehyde reductase gene at 1p35-1p36.1 in a region frequently altered in human tumor cells.#JOURNAL:Cancer Res#YEAR:1998#PUBMED:9823300# GENE ASSOCIATION: (8574.1)
Abbreviation: R_EX_o2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : o2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DIDPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: didp[c] <==> didp[n] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ESTRADIOLtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: estradiol[r] <==> estradiol[c] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_vitd3_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : vitd3 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DUTPDPn Synonyms: _0 EC Number: 3.6.1.23 SUBSYSTEM: Nucleotides Equation: [n] : dutp + h2o --> dump + h + ppi Confidence Level: 4 genes: LOCUS:_1854#TRANSCRIPT:1#ABBREVIATION:DUT#ECNUMBERS:3.6.1.23# proteins: NAME:dUTP pyrophosphatase #ABBREVIATION:Dut.1-n# citations: AUTHORS:Tinkelenberg BA, Fazzone W, Lynch FJ, Ladner RD. #TITLE:Identification of sequence determinants of human nuclear dUTPase isoform localization.#JOURNAL:Exp Cell Res#YEAR:2003#PUBMED:12799180# AUTHORS:Cohen D, Heng HH, Shi XM, McIntosh EM, Tsui LC, Pearlman RE.#TITLE:Assignment of the human dUTPase gene (DUT) to chromosome 15q15-q21. 1 by fluorescence in situ hybridization. #JOURNAL:Genomics#YEAR:1997#PUBMED:9070952# AUTHORS:Ladner RD, McNulty DE, Carr SA, Roberts GD, Caradonna SJ.#TITLE:Characterization of distinct nuclear and mitochondrial forms of human deoxyuridine triphosphate nucleotidohydrolase.#JOURNAL:J Biol Chem. #YEAR:1996#PUBMED:8631816# GENE ASSOCIATION: (1854.1)
Abbreviation: R_PItg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: pi[g] --> pi[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
glyceraldehyde-3-phosphate dehydrogenase
Abbreviation: R_GAPD Synonyms: _0 EC Number: 1.2.1.12 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : g3p + nad + pi <==> 13dpg + h + nadh Confidence Level: 0 SMP genes: LOCUS:_2597#TRANSCRIPT:1#ABBREVIATION:GAPDH#ECNUMBERS:1.2.1.12# LOCUS:_26330#TRANSCRIPT:1#ABBREVIATION:GAPDS#ECNUMBERS:1.2.1.12# proteins: NAME:glyceraldehyde-3-phosphate dehydrogenase#ABBREVIATION:Gapdh.1# NAME:spermatogenic glyceraldehyde-3-phosphate dehydrogenase (testis)#ABBREVIATION:Gapds.1# citations: none GENE ASSOCIATION: (2597.1) or (26330.1)
Abbreviation: R_EX_co2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : co2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_3-Methoxytyramine:oxygen oxidoreductase (deaminating)
Abbreviation: R_3MOXTYROX Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 3moxtyr + h2o + o2 --> 3mox4hpac + h2o2 + nh4 Confidence Level: 4 SAB genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# proteins: NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Jonsson EG, Norton N, Gustavsson JP, Oreland L, Owen MJ, Sedvall GC#TITLE:A promoter polymorphism in the monoamine oxidase A gene and its relationships to monoamine metabolite concentrations in CSF of healthy volunteers#JOURNAL:J Psychiatr Res#YEAR:2000#PUBMED:10867119# GENE ASSOCIATION: (4128.1) or (4129.1)
Abbreviation: R_ALAASNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + asn-L[c] + na1[e] --> ala-L[c] + asn-L[e] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-alanine/L-asparagine Na-dependent exchange (Ala-L in)
Abbreviation: R_FAOXC226205m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : c226coa + coa + h2o + nad --> accoa + h + nadh + tmndnccoa Confidence Level: 4 _1 round of beta-oxidation, loses degree of desat, so no fad peroxisomal (very long chain FA) c22:6,n-3 -> c20:5, n-3 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Kelly DP, Kim JJ, Billadello JJ, Hainline BE, Chu TW, Strauss AW#TITLE:Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3035565# AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# AUTHORS:Weiping Le, Azfar S. Abbas, Howard Sprecher, Jerry Vockley and Horst Schulz#TITLE:Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial [beta]-oxidation of unsaturated fatty acids#JOURNAL:Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids#YEAR:2000# GENE ASSOCIATION: (35.1) or (34.1)
chondroitin sulfate D (GlcNAc6S-GlcA2S), precursor 3_C37H53NO40S3X
Abbreviation: R_GALNACT4g Synonyms: _6 EC Number: 2.4.1.175 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_d_pre3 + uacgam --> cs_d_pre4 + h + udp Confidence Level: 4 NCD genes: LOCUS:_22856#TRANSCRIPT:1#ABBREVIATION:CHSY1# LOCUS:_55454#TRANSCRIPT:1#ABBREVIATION:GALNACT-2# LOCUS:_55790#TRANSCRIPT:1#ABBREVIATION:ChGn# LOCUS:_79586#TRANSCRIPT:1#ABBREVIATION:CHPF# LOCUS:_337876#TRANSCRIPT:1#ABBREVIATION:CSS3# proteins: NAME:chondroitin sulfate GalNAcT-2, Golgi apparatus#ABBREVIATION:Galnact-2.1-g# NAME:chondroitin beta1,4 N-acetylgalactosaminyltransferase, Golgi apparatus#ABBREVIATION:Chgn.1-g# NAME:carbohydrate (chondroitin) synthase 1, Golgi apparatus#ABBREVIATION:Chsy1.1-g# NAME:chondroitin polymerizing factor, Golgi apparatus#ABBREVIATION:Chpf.1-g# NAME:chondroitin sulfate synthase 3, Golgi apparatus#ABBREVIATION:Css3.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Sato T, Gotoh M, Kiyohara K, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Togayachi A, Kudo T, Asada M, Watanabe H, Imamura T, Kimata K, Narimatsu H#TITLE:Differential roles of two N-acetylgalactosaminyltransferases, CSGalNAcT-1, and a novel enzyme, CSGalNAcT-2. Initiation and elongation in synthesis of chondroitin sulfate#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12446672# AUTHORS:Uyama T, Kitagawa H, Tamura Ji J, Sugahara K#TITLE:Molecular cloning and expression of human chondroitin N-acetylgalactosaminyltransferase: the key enzyme for chain initiation and elongation of chondroitin/dermatan sulfate on the protein linkage region tetrasaccharide shared by heparin/heparan sulfate#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11788602# AUTHORS:Gotoh M, Sato T, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Zhang Y, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Nishihara S, Watanabe H, Kimata K, Narimatsu H#TITLE:Enzymatic synthesis of chondroitin with a novel chondroitin sulfate N-acetylgalactosaminyltransferase that transfers N-acetylgalactosamine to glucuronic acid in initiation and elongation of chondroitin sulfate synthesis#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12163485# AUTHORS:Kitagawa H, Uyama T, Sugahara K#TITLE:Molecular cloning and expression of a human chondroitin synthase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11514575# AUTHORS:Kitagawa H, Izumikawa T, Uyama T, Sugahara K#TITLE:Molecular cloning of a chondroitin polymerizing factor that cooperates with chondroitin synthase for chondroitin polymerization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12716890# AUTHORS:Yada T, Sato T, Kaseyama H, Gotoh M, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-3. Molecular cloning and characterization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12907687# AUTHORS:Yada T, Gotoh M, Sato T, Shionyu M, Go M, Kaseyama H, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12761225# GENE ASSOCIATION: (55790.1) or (79586.1) and (22856.1) or (55454.1) or (337876.1)
Abbreviation: R_NACHEX12ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg13 --> acgam + ksi_deg14 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3074.1) or (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_DNDPt26m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[c] + dudp[m] --> dcdp[m] + dudp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_SOAT12 Synonyms: _9 SUBSYSTEM: Cholesterol Metabolism Equation: [c] : R2coa_hs + chsterol --> coa + xolest_hs Confidence Level: 4 SOAT11 and SOAT12 are templates, customized rxns can be formed particular ester moieties. NJ genes: LOCUS:_6646#TRANSCRIPT:1#ABBREVIATION:SOAT1#ECNUMBERS:2.3.1.26# proteins: NAME:sterol O-acetyltransferase (acyl-Coenzyme A: cholesterol acyltransferase) 1#ABBREVIATION:Soat1.1# citations: AUTHORS:Guo ZY, Chang CC, Lu X, Chen J, Li BL, Chang TY.#TITLE:The disulfide linkage and the free sulfhydryl accessibility of acyl-coenzyme A:cholesterol acyltransferase 1 as studied by using mPEG5000-maleimide.#JOURNAL:Biochemistry#YEAR:2005#PUBMED:15850387# AUTHORS:Chang CC, Huh HY, Cadigan KM, Chang TY.#TITLE:Molecular cloning and functional expression of human acyl-coenzyme A:cholesterol acyltransferase cDNA in mutant Chinese hamster ovary cells.#JOURNAL:J Biol Chem.#YEAR:1993#PUBMED:8407899# GENE ASSOCIATION: (6646.1)
Abbreviation: R_THFtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: thf[c] <==> thf[m] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_5,6,7,8-Tetrahydrofolate transport, diffusion, mitochondrial
Abbreviation: R_FAOXC204 Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : arachdcoa + (9) coa + (5) fad + (9) h2o + (9) nad --> (10) accoa + (5) fadh2 + (9) h + (9) nadh Confidence Level: 4 TV Other enzymes: 1.3.3.6, 4.2.1.74, 4.2.1.17, 1.1.1.35, 1.1.1.211 genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Kelly DP, Kim JJ, Billadello JJ, Hainline BE, Chu TW, Strauss AW#TITLE:Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3035565# AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# AUTHORS:Weiping Le, Azfar S. Abbas, Howard Sprecher, Jerry Vockley and Horst Schulz#TITLE:Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial [beta]-oxidation of unsaturated fatty acids#JOURNAL:Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids#YEAR:2000# GENE ASSOCIATION: (34.1) or (34.1) or (35.1) or (35.1)
Abbreviation: R_EX_gchola_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gchola <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RTOTAL3CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: Rtotal3crn[c] --> Rtotal3crn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (788.1)
Abbreviation: R_P45017A3r Synonyms: _4 EC Number: 1.14.99.9 SUBSYSTEM: Steroid Metabolism Equation: [r] : h + nadph + o2 + prgstrn --> 17ahprgstrn + h2o + nadp Confidence Level: 4 ER inferred from lit ref - RETURN AND CHECK AFTER DEEPER LIT SEARCH NJ genes: LOCUS:_1586#TRANSCRIPT:1#ABBREVIATION:CYP17A1#ECNUMBERS:1.14.99.9# proteins: NAME:cytochrome P450, family 17, subfamily A, polypeptide 1#ABBREVIATION:Cyp17a1.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# AUTHORS:Korzekwa KR, Trager WF, Mancewicz J, Osawa Y#TITLE:Studies on the mechanism of aromatase and other cytochrome P450 mediated deformylation reactions#JOURNAL:J Steroid Biochem Mol Biol#YEAR:1993# GENE ASSOCIATION: (1586.1)
Abbreviation: R_AOBUTDsm Synonyms: _0 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : 2aobut + h --> aact + co2 Confidence Level: 2 Spontaneous reaction genes: none proteins: none citations: AUTHORS:Edgar AJ. #TITLE:Molecular cloning and tissue distribution of mammalian L-threonine 3-dehydrogenases.#YEAR:2002#PUBMED:12097150# GENE ASSOCIATION:
L-2-amino-3-oxobutanoate decarboxylation, mitochondrial (spontaneous)
Abbreviation: R_UMPK Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : atp + ump <==> adp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_ST6GALNAC28 Synonyms: _5 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gq1c_hs --> cmp + gp1c_hs + h Confidence Level: 2 NJ genes: LOCUS:_10610#TRANSCRIPT:1#ABBREVIATION:SIAT7B#ECNUMBERS:2.4.99.7# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 2#ABBREVIATION:St6galnac2.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10610.1)
Abbreviation: R_HPYRtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: hpyr[c] <==> hpyr[x] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Johnson SA, Rumsby G, Cregeen D, Hulton SA#TITLE:Primary hyperoxaluria type 2 in children.#JOURNAL:Pediatr Nephrol#YEAR:2002#PUBMED:12185464# GENE ASSOCIATION:
Abbreviation: R_DNDPt6m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: datp[c] + dgdp[m] --> datp[m] + dgdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_UDPXYLter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: udpxyl[c] + ump[r] <==> udpxyl[r] + ump[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
UDP-Xyl endoplasmic reticular transport via CMP antiport
Abbreviation: R_CYTDK1 Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [c] : atp + cytd --> adp + cmp + h Confidence Level: 2 genes: LOCUS:_7371#TRANSCRIPT:1#ABBREVIATION:UCK2#ECNUMBERS:2.7.4.*# LOCUS:_83549#TRANSCRIPT:1#ABBREVIATION:UCK1#ECNUMBERS:2.7.1.48# proteins: NAME:uridine-cytidine kinase 2 #ABBREVIATION:Uck2.1# NAME:uridine-cytidine kinase 1 #ABBREVIATION:Uck1.1# citations: AUTHORS:Kashuba E, Kashuba V, Sandalova T, Klein G, Szekely L#TITLE:Epstein-Barr virus encoded nuclear protein EBNA-3 binds a novel human uridine kinase/uracil phosphoribosyltransferase. #JOURNAL:BMC Cell Biol.#YEAR:2002#PUBMED:12199906# AUTHORS:Ozaki K, Kuroki T, Hayashi S, Nakamura Y.#TITLE:Isolation of three testis-specific genes (TSA303, TSA806, TSA903) by a differential mRNA display method. #JOURNAL:Genomics#YEAR:1996#PUBMED:8812458# AUTHORS:Van Rompay AR, Norda A, Linden K, Johansson M, Karlsson A.#TITLE:Phosphorylation of uridine and cytidine nucleoside analogs by two human uridine-cytidine kinases.#JOURNAL:Mol Pharmacol. #YEAR:2001#PUBMED:11306702# GENE ASSOCIATION: (7371.1) or (83549.1)
Abbreviation: R_RDH4 Synonyms: _0 EC Number: 1.1.1.105 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : nad + retinol-cis-13 <==> h + nadh + retinal-cis-13 Confidence Level: 4 IT genes: LOCUS:_5959#TRANSCRIPT:1#ABBREVIATION:RDH5#ECNUMBERS:1.1.1.105# proteins: NAME:retinol dehydrogenase 5 (11-cis and 9-cis) #ABBREVIATION:Rdh5.1# citations: AUTHORS:Mertz JR, Shang E, Piantedosi R, Wei S, Wolgemuth DJ, Blaner WS. #TITLE:Identification and characterization of a stereospecific human enzyme that catalyzes 9-cis-retinol oxidation. A possible role in 9-cis-retinoic acid formation.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9115228# GENE ASSOCIATION: (5959.1)
Abbreviation: R_ABUTt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _4abut[e] + h[e] <==> 4abut[c] + h[c] Confidence Level: 2 SAB r genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
_4-aminobutyrate reversible transport in via proton symport
fucosyltransferase 3 (galactoside 3(4)-L-fucosyltransferase, Lewis blood group included) 1
Abbreviation: R_FUT31g Synonyms: _5 EC Number: 2.4.1.65 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacglcgalgluside_hs + gdpfuc --> fuc14galacglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2525#TRANSCRIPT:1#ABBREVIATION:FUT3#ECNUMBERS:2.4.1.65# proteins: NAME:fucosyltransferase 3 (galactoside 3(4)-L-fucosyltransferase, Lewis blood group)#ABBREVIATION:Fut3.1# citations: AUTHORS:Cakir B, Pankow JS, Salomaa V, Couper D, Morris TL, Brantley KR, Hiller KM, Heiss G, Weston BW.#TITLE:Distribution of Lewis (FUT3)genotype and allele: frequencies in a biethnic United States population.#JOURNAL:Ann Hematol#YEAR:2002#PUBMED:12424536# AUTHORS:Sousa VL, Brito C, Costa T, Lanoix J, Nilsson T, Costa J.#TITLE:Importance of Cys, Gln, and Tyr from the transmembrane domain of human alpha 3/4 fucosyltransferase III for its localization and sorting in the Golgi of baby hamster kidney cells.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12493760# AUTHORS:Kukowska-Latallo JF, Larsen RD, Nair RP, Lowe JB.#TITLE:A cloned human cDNA determines expression of a mouse stage-specific embryonic antigen and the Lewis blood group alpha(1,3/1,4)fucosyltransferase.#JOURNAL:Genes Dev#YEAR:1990#PUBMED:1977660# GENE ASSOCIATION: (2525.1)
Abbreviation: R_FUCFUCFUCGALACGLC13GALACGLCGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucfucfucgalacglc13galacglcgal14acglcgalgluside_hs[e] <==> fucfucfucgalacglc13galacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PROSTGD2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hco3[c] + prostgd2[e] <==> hco3[e] + prostgd2[c] Confidence Level: 4 NJ genes: LOCUS:_6578#TRANSCRIPT:1#ABBREVIATION:SLCO2A1# proteins: NAME:solute carrier organic anion transporter family, member 2A1#ABBREVIATION:Slco2a1.1# citations: AUTHORS:Lu R, Kanai N, Bao Y, Schuster VL.#TITLE:Cloning, in vitro expression, and tissue distribution of a human prostaglandin transporter cDNA(hPGT).#JOURNAL:J Clin Invest.#YEAR:1996#PUBMED:8787677# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (6578.1)
prostaglandin transport via bicarbonate countertransport
Abbreviation: R_EX_Rtotal2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : Rtotal2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NCKt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ca2[e] + k[e] + (4) na1[c] <==> ca2[c] + k[c] + (4) na1[e] Confidence Level: 4 NCD genes: LOCUS:_57419#TRANSCRIPT:1#ABBREVIATION:SLC24A3# LOCUS:_123041#TRANSCRIPT:1#ABBREVIATION:SLC24A4# LOCUS:_25769#TRANSCRIPT:1#ABBREVIATION:SLC24A2# LOCUS:_9187#TRANSCRIPT:1#ABBREVIATION:SLC24A1# LOCUS:_123041#TRANSCRIPT:2#ABBREVIATION:SLC24A4# LOCUS:_123041#TRANSCRIPT:3#ABBREVIATION:SLC24A4# proteins: NAME:solute carrier family 24 (sodium/potassium/calcium exchanger), member 1#ABBREVIATION:Slc24a1.1-e# NAME:solute carrier family 24 (sodium/potassium/calcium exchanger), member 2#ABBREVIATION:Slc24a2.1-e# NAME:solute carrier family 24 (sodium/potassium/calcium exchanger), member 3#ABBREVIATION:Slc24a3.1-e# NAME:solute carrier family 24 (sodium/potassium/calcium exchanger), member 4#ABBREVIATION:Slc24a4.1-e# NAME:solute carrier family 24 (sodium/potassium/calcium exchanger), member 4#ABBREVIATION:Slc24a4.3-e# NAME:solute carrier family 24 (sodium/potassium/calcium exchanger), member 4#ABBREVIATION:Slc24a4.2-e# citations: AUTHORS:Tucker JE, Winkfein RJ, Cooper CB, Schnetkamp PP#TITLE:cDNA cloning of the human retinal rod Na-Ca + K exchanger: comparison with a revised bovine sequence#JOURNAL:Invest Ophthalmol Vis Sci#YEAR:1998#PUBMED:9478004# AUTHORS:Kimura M, Jeanclos EM, Donnelly RJ, Lytton J, Reeves JP, Aviv A.#TITLE:Physiological and molecular characterization of the Na+/Ca2+ exchanger in human platelets#JOURNAL:Am J Physiol#YEAR:1999#PUBMED:10484410# AUTHORS:Schnetkamp PP#TITLE:The SLC24 Na+/Ca2+-K+ exchanger family: vision and beyond.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770312# AUTHORS:Prinsen CF, Szerencsei RT, Schnetkamp PP#TITLE:Molecular cloning and functional expression of the potassium-dependent sodium-calcium exchanger from human and chicken retinal cone photoreceptors#JOURNAL:J Neurosci#YEAR:2000#PUBMED:10662833# AUTHORS:Kraev A, Quednau BD, Leach S, Li XF, Dong H, Winkfein R, Perizzolo M, Cai X, Yang R, Philipson KD, Lytton J#TITLE:Molecular cloning of a third member of the potassium-dependent sodium-calcium exchanger gene family, NCKX3#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11294880# AUTHORS:Li XF, Kraev AS, Lytton J#TITLE:Molecular cloning of a fourth member of the potassium-dependent sodium-calcium exchanger gene family, NCKX4#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12379639# GENE ASSOCIATION: (123041.2) or (57419.1) or (9187.1) or (123041.3) or (25769.1) or (123041.1)
Abbreviation: R_PGMT Synonyms: _0 EC Number: 5.4.2.2 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : g1p <==> g6p Confidence Level: 0 genes: LOCUS:_55276#TRANSCRIPT:1#ABBREVIATION:PGM2#ECNUMBERS:5.4.2.2# LOCUS:_5236#TRANSCRIPT:1#ABBREVIATION:PGM1#ECNUMBERS:5.4.2.2# proteins: NAME:phosphoglucomutase 1#ABBREVIATION:Pgm1.1# NAME:phosphoglucomutase 2#ABBREVIATION:Pgm2# citations: none GENE ASSOCIATION: (5236.1) or (55276.1)
Abbreviation: R_STRDNCCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + strdnccrn --> crn + strdnccoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_MG1er Synonyms: _1 EC Number: 3.2.1.106 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : g3m8masn + h2o --> g2m8masn + glc-D Confidence Level: 0 NCD genes: LOCUS:_7841#TRANSCRIPT:1#ABBREVIATION:GCS1#ECNUMBERS:3.2.1.106# proteins: NAME:Glucosidase I, endoplasmic reticulum#ABBREVIATION:Gcs1.1-er# citations: none GENE ASSOCIATION: (7841.1)
aldo-keto reductase family 1, member C4 (chlordecone reductase; 3-alpha hydroxysteroid dehydrogenase, type I; dihydrodiol dehydrogenase 4)
Abbreviation: R_AKR1C41 Synonyms: _7 EC Number: 1.1.1.50 SUBSYSTEM: Bile Acid Biosynthesis Equation: [c] : h + nadh + xol7ah --> nad + xol7ah2 Confidence Level: 4 NJ genes: LOCUS:_1109#TRANSCRIPT:1#ABBREVIATION:AKR1C4#ECNUMBERS:1.1.1.225# proteins: NAME:aldo-keto reductase family 1, member C4#ABBREVIATION:Akr1c4.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1109.1)
Abbreviation: R_GLYt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gly[e] + na1[e] --> gly[c] + na1[c] Confidence Level: 4 SAB irreversible genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# GENE ASSOCIATION: (81539.1) or (11254.1) or (54407.1) or (55089.1)
Abbreviation: R_LNELDCCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: lneldccrn[c] --> lneldccrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_6DHFtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _6dhf[c] --> 6dhf[l] Confidence Level: 2 carrier-mediated transport - but which and how is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
Abbreviation: R_FAOXC2031836m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : coa + dlnlcgcoa + fad + h2o + nad --> accoa + fadh2 + h + lnlncgcoa + nadh Confidence Level: 4 c20:3 -> c18:3 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_EX_estroneglc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : estroneglc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LNLCCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: lnlccrn[c] --> lnlccrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_FACOAL161 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + hdcea <==> amp + hdcoa + ppi Confidence Level: 4 NCD genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_XANDp Synonyms: _0 EC Number: 1.1.1.204 SUBSYSTEM: Purine Catabolism Equation: [x] : h2o + nad + xan --> h + nadh + urate Confidence Level: 4 Acts on a variety of purines and aldehydes. The animal enzyme can be interconverted to EC 1.1.3.22 xanthine oxidase (the oxidase form). IT genes: LOCUS:_7498#TRANSCRIPT:1#ABBREVIATION:XDH#ECNUMBERS:1.1.1.204# proteins: NAME:xanthine dehydrogenase #ABBREVIATION:Xdh.1-x# citations: AUTHORS:Saksela M, Raivio KO.#TITLE:Cloning and expression in vitro of human xanthine dehydrogenase/oxidase. #JOURNAL:Biochem J#YEAR:1996#PUBMED:8670112# AUTHORS:Linder N, Martelin E, Lapatto R, Raivio KO.#TITLE:Posttranslational inactivation of human xanthine oxidoreductase by oxygen under standard cell culture conditions.#JOURNAL:Am J Physiol Cell Physiol.#YEAR:2003#PUBMED:12637268# GENE ASSOCIATION: (7498.1)
Abbreviation: R_CSNATer Synonyms: _0 EC Number: 2.3.1.7 SUBSYSTEM: Fatty Acid Metabolism Equation: [r] : accoa + crn <==> acrn + coa Confidence Level: 2 mm genes: LOCUS:_1384#TRANSCRIPT:3#ABBREVIATION:CRAT#ECNUMBERS:2.3.1.7# proteins: NAME:carnitine acetyltransferase, endoplasmic reticulum#ABBREVIATION:Crat.3-r# citations: AUTHORS:Lysiak W, Toth PP, Suelter CH, Bieber LL.#TITLE:Quantitation of the efflux of acylcarnitines from rat heart, brain, and liver mitochondria.#JOURNAL:J Biol Chem#YEAR:1986#PUBMED:3759988# AUTHORS:Ramsay, Gandour, van der Leij#TITLE:Molecular enzymology of carnitine transfer and transport#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11257506# GENE ASSOCIATION: (1384.3)
carnitine O-acetyltransferase, endolasmic reticulum
Abbreviation: R_GALGALFUCFUCGALACGLCGALACGLCGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: galgalfucfucgalacglcgalacglcgal14acglcgalgluside_hs[g] <==> galgalfucfucgalacglcgalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SCPx Synonyms: _2 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : cholcoaone + coa --> cholcoa + ppcoa Confidence Level: 4 SCP has two transcripts, second isoform specific to peroxisomes, first is specific for cyt and mit need to double check stoichiometry - reaction cofactors (additional coa + release of prop coa) from BM (Roche) Biochemical Pathways NJ genes: LOCUS:_6342#TRANSCRIPT:1#ABBREVIATION:SCP2# proteins: NAME:sterol carrier protein 2#ABBREVIATION:Scp2x.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Mukherji M, Kershaw NJ, Schofield CH, Wierzbicki AS, Lloyd MD#TITLE:Utilization of sterol carrier protein-2 by phytanoyl-CoA 2-hydroxylase in the peroxisomal alpha oxidation of phytanic acid#JOURNAL:Chemistry and Biology#YEAR:2002# GENE ASSOCIATION: (6342.1)
Abbreviation: R_TETTET6CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + tettet6coa --> coa + tettet6crn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_4MOPt2im Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _4mop[c] + h[c] --> 4mop[m] + h[m] Confidence Level: 4 MM genes: none proteins: none citations: AUTHORS:Hutson SM, Hall TR.#TITLE:Identification of the mitochondrial branched chain aminotransferase as a branched chain alpha-keto acid transport protein.#YEAR:1993#PUBMED:8428987# GENE ASSOCIATION:
_4-methyl-2-oxopentanoate mitochondrial transport via proton symport
R group 6 Coenzyme A homo sapiens_XCO2C21H31N7O15P3S
Abbreviation: R_ARTFR61 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + hdd2coa[c] --> R6coa_hs[c] + fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LGNCCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + lgnccrn --> crn + lgnccoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_PRPNCOAHYDx Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: beta-Alanine metabolism Equation: [x] : h2o + prpncoa --> 3hpcoa Confidence Level: 4 SAB made up name because name in KEGG seemed wrong genes: LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# proteins: NAME:enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase (x)#ABBREVIATION:Ehhadh.1-x# citations: AUTHORS:Hoefler G, Forstner M, McGuinness MC, Hulla W, Hiden M, Krisper P, Kenner L, Ried T, Lengauer C, Zechner R, et al.#TITLE:DNA cloning of the human peroxisomal enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase bifunctional enzyme and localization to chromosome 3q26.3-3q28: a free left Alu Arm is inserted in the 3' noncoding region.#JOURNAL:Genomics#YEAR:1994#PUBMED:8188243# GENE ASSOCIATION: (1962.1)
Abbreviation: R_ARACHCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: arachcoa[c] <==> arachcoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FA160ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + palmACP <==> ACP + h + hdca Confidence Level: 4 NCD genes: LOCUS:_55301#TRANSCRIPT:1#ABBREVIATION:THEDC1# proteins: NAME:s-acyl fatty acid synthase thioesterase#ABBREVIATION:Thedc1.1# citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION: (55301.1)
Palmitoyl-ACP (n-C16:0ACP)_C27H51N2O8PRS
Abbreviation: R_LNLNCACRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: lnlncacrn[c] --> lnlncacrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_IDOURtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: h[c] + idour[c] <==> h[l] + idour[l] Confidence Level: 4 H symport into lysososome. See PMID: 2768261 and PMID: 10581036 (for human evidence). Sialic acid storage disorders (due to transporter mutations) require import and export e.g. PMID: 2768266 NJ renamed from GLCURlt to GLCURtly - NCD added IdoA reaction - NCD genes: LOCUS:_26503#TRANSCRIPT:1#ABBREVIATION:SLC17A5# proteins: NAME:solute carrier family 17 (anion/sugar transporter), member 5#ABBREVIATION:Slc17a5.1# citations: AUTHORS:Mancini GM, de Jonge HR, Galjaard H, Verheijen FW.#TITLE:Characterization of a proton-driven carrier for sialic acid in the lysosomal membrane. Evidence for a group-specific transport system for acidic monosaccharides.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2768261# AUTHORS:Verheijen FW, Verbeek E, Aula N, Beerens CE, Havelaar AC, Joosse M, Peltonen L, Aula P, Galjaard H, van der Spek PJ, Mancini GM.#TITLE:A new gene, encoding an anion transporter, is mutated in sialic acid storage diseases.#JOURNAL:Nat Genet#YEAR:1999#PUBMED:10581036# AUTHORS:Havelaar AC, Mancini GM, Beerens CE, Souren RM, Verheijen FW. #TITLE:Purification of the lysosomal sialic acid transporter. Functional characteristics of a monocarboxylate transporter#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9852127# GENE ASSOCIATION: (26503.1)
Abbreviation: R_CYTDtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: cytd[l] <==> cytd[c] Confidence Level: 4 IT genes: LOCUS:_55315#TRANSCRIPT:1#ABBREVIATION:SLC29A3# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 3 #ABBREVIATION:Slc29a3.1-l# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Pisoni RL, Thoene JG.#TITLE:Detection and characterization of a nucleoside transport system in human fibroblast lysosomes.#JOURNAL:J Biol Chem.#YEAR:1989#PUBMED:2925670# AUTHORS:Baldwin SA, Yao SY, Hyde RJ, Ng AM, Foppolo S, Barnes K, Ritzel MW, Cass CE, Young JD.#TITLE:Functional characterization of novel human and mouse equilibrative nucleoside transporters (hENT3 and mENT3) located in intracellular membranes.#JOURNAL:J Biol Chem. #YEAR:2005#PUBMED:15701636# GENE ASSOCIATION: (55315.1)
Cytidine facilated transport from lysosome
Abbreviation: R_FUCFUCFUCGALACGLC13GALACGLCGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucfucfucgalacglc13galacglcgal14acglcgalgluside_hs[g] <==> fucfucfucgalacglc13galacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_4hphac_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 4hphac <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NCAMUP Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ncam[e] --> ncam[c] Confidence Level: 2 SAB mechanism copied from nac--not certain genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Reyes AM, Bustamante F, Rivas CI, Ortega M, Donnet C, Rossi JP, Fischbarg J, Vera JC. #TITLE:Nicotinamide is not a substrate of the facilitative hexose transporter GLUT1.#JOURNAL:Biochemistry. #YEAR:2002#PUBMED:12069599# AUTHORS:Sofue M, Yoshimura Y, Nishida M, Kawada J.#TITLE:Possible multifunction of glucose transporter. Transport of nicotinamide by reconstituted liposomes.#JOURNAL:Biochem J. #YEAR:1992#PUBMED:1463467# GENE ASSOCIATION:
Abbreviation: R_G6PDH1er Synonyms: _0 SUBSYSTEM: Pentose Phosphate Pathway Equation: [r] : g6p + nad --> 6pgl + h + nadh Confidence Level: 4 tv, jp NCD genes: LOCUS:_9563#TRANSCRIPT:1#ABBREVIATION:H6PD#ECNUMBERS:1.1.1.47# proteins: NAME:hexose-6-phosphate dehydrogenase, endoplasmic reticulum#ABBREVIATION:H6pd.1-r# citations: AUTHORS:Mason PJ, Stevens D, Diez A, Knight SW, Scopes DA, Vulliamy TJ.#TITLE:Human hexose-6-phosphate dehydrogenase (glucose 1-dehydrogenase) encoded at 1p36: coding sequence and expression.#JOURNAL: Blood Cells Mol Dis#YEAR:1999#PUBMED:10349511# AUTHORS:Clarke JL, Mason PJ#TITLE:Murine hexose-6-phosphate dehydrogenase: a bifunctional enzyme with broad substrate specificity and 6-phosphogluconolactonase activity.#JOURNAL:Arch Biochem Biophys#YEAR:2003#PUBMED:12831846# AUTHORS:Barash V, Erlich T, Bashan N.#TITLE:Microsomal hexose-6-phosphate and 6-phosphogluconate dehydrogenases in extrahepatic tissues: human placenta and pig kidney cortex.#JOURNAL:Biochem Int.#YEAR:1990#PUBMED:2156506# GENE ASSOCIATION: (9563.1)
Abbreviation: R_SPODMx Synonyms: _1 EC Number: 1.15.1.1 SUBSYSTEM: ROS Detoxification Equation: [x] : (2) h + (2) o2s --> h2o2 + o2 Confidence Level: 4 JLR genes: LOCUS:_6647#TRANSCRIPT:1#ABBREVIATION:SOD1#ECNUMBERS:1.15.1.1# proteins: NAME:superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult)), peroxisome#ABBREVIATION:Sod1.1-x# citations: AUTHORS:Crapo JD, Oury T, Rabouille C, Slot JW, Chang LY#TITLE:Copper,zinc superoxide dismutase is primarily a cytosolic protein in human cells#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1992#PUBMED:1332049# AUTHORS:Hallewell RA, Masiarz FR, Najarian RC, Puma JP, Quiroga MR, Randolph A, Sanchez-Pescador R, Scandella CJ, Smith B, Steimer KS, et al#TITLE:Human Cu/Zn superoxide dismutase cDNA: isolation of clones synthesising high levels of active or inactive enzyme from an expression library#JOURNAL:Nucleic Acids Res#YEAR:1985#PUBMED:3889846# GENE ASSOCIATION: (6647.1)
Abbreviation: R_GGH_DASH_7THFl Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [l] : 7thf + h2o --> 6thf + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-l# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (7THF), lysosomal
coproporphyrinogen oxidase (O2 required)
Abbreviation: R_CPPPGO Synonyms: _0 EC Number: 1.3.3.3 SUBSYSTEM: Heme Biosynthesis Equation: [c] : cpppg3 + (2) h + o2 --> (2) co2 + (2) h2o + pppg9 Confidence Level: 2 JLR genes: LOCUS:_1371#TRANSCRIPT:1#ABBREVIATION:CPOX#ECNUMBERS:1.3.3.3# proteins: NAME:coproporphyrinogen oxidase#ABBREVIATION:Cpox.1# citations: AUTHORS:Kohno H, Furukawa T, Yoshinaga T, Tokunaga R, Taketani S.#TITLE:Coproporphyrinogen oxidase. Purification, molecular cloning, and induction of mRNA during erythroid differentiation.#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:8407975# GENE ASSOCIATION: (1371.1)
keratan sulfate I, degradation product 7_C210H337N15O181S10
Abbreviation: R_GSNt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gsn[e] + na1[e] --> gsn[c] + na1[c] Confidence Level: 4 genes: LOCUS:_9153#TRANSCRIPT:1#ABBREVIATION:SLC28A2# LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 2, extracellular #ABBREVIATION:Slc28a2.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (9153.1) or (64078.1)
guanosine transport in via sodium symport
Abbreviation: R_NTD9 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [c] : gmp + h2o --> gsn + pi Confidence Level: 2 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_22978#TRANSCRIPT:1#ABBREVIATION:NT5C2# proteins: NAME:_5'-nucleotidase, cytosolic II #ABBREVIATION:Nt5C2.1# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# GENE ASSOCIATION: (22978.1)
Abbreviation: R_M4BET2er Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : mem2emgacpail_hs + pe_hs --> dag_hs + m(em)3gacpail_hs Confidence Level: 4 NCD genes: LOCUS:_5281#TRANSCRIPT:1#ABBREVIATION:PIGF# LOCUS:_5281#TRANSCRIPT:2#ABBREVIATION:PIGF# LOCUS:_54872#TRANSCRIPT:1#ABBREVIATION:GPI7# proteins: NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.1-r# NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.2-r# NAME:GPI protein, endoplasmic reticulum#ABBREVIATION:Gpi7.1-r# citations: AUTHORS:Shishioh N, Hong Y, Ohishi K, Ashida H, Maeda Y, Kinoshita T#TITLE:GPI7 is the second partner of PIG-F and involved in modification of glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15632136# AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (5281.1) and (54872.1) or (5281.2) and (54872.1)
M4B phosphoethanolaminyl transferase, endoplasmic reticulum
Abbreviation: R_THRD_L Synonyms: _1 EC Number: 4.3.1.19 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : thr-L --> 2obut + nh4 Confidence Level: 4 Previous EC number: 4.2.1.16. New EC number 4.3.1.19. Can't enter this.... genes: LOCUS:_10993#TRANSCRIPT:1#ABBREVIATION:SDS# proteins: NAME:serine dehydratase#ABBREVIATION:Sds.1# citations: AUTHORS:Edgar AJ.#TITLE:The human L-threonine 3-dehydrogenase gene is an expressed pseudogene.#YEAR:2002#PUBMED:12361482# GENE ASSOCIATION: (10993.1)
Abbreviation: R_EX_24nph_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 24nph <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NICRNTtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: nicrnt[c] <==> nicrnt[n] Confidence Level: 2 it genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I, degradation product 8_C194H311N13O171S10
Abbreviation: R_MELATNOX Synonyms: _0 EC Number: 1.14.14.1 SUBSYSTEM: Tryptophan metabolism Equation: [c] : h + melatn + nadph + o2 --> 6hoxmelatn + h2o + nadp Confidence Level: 4 SAB made up name genes: LOCUS:_1543#TRANSCRIPT:1#ABBREVIATION:CYP1A1#ECNUMBERS:1.14.14.1# LOCUS:_1557#TRANSCRIPT:1#ABBREVIATION:CYP2C19#ECNUMBERS:1.14.14.1# LOCUS:_1545#TRANSCRIPT:1#ABBREVIATION:CYP1B1#ECNUMBERS:1.14.14.1# LOCUS:_1544#TRANSCRIPT:1#ABBREVIATION:CYP1A2#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 1, subfamily A, polypeptide 1#ABBREVIATION:Cyp1a1.1# NAME:cytochrome P450, family 1, subfamily A, polypeptide 2#ABBREVIATION:Cyp1a2.1# NAME:cytochrome P450, family 1, subfamily B, polypeptide 1#ABBREVIATION:Cyp1b1.1# NAME:cytochrome P450, family 2, subfamily C, polypeptide 19#ABBREVIATION:Cyp2c19.1# citations: AUTHORS:Ma X, Idle JR, Krausz KW, Gonzalez FJ. #TITLE:METABOLISM OF MELATONIN BY HUMAN CYTOCHROMES P450. #JOURNAL:Drug Metab Dispos#YEAR:2004#PUBMED:15616152# GENE ASSOCIATION: (1557.1) or (1543.1) or (1545.1) or (1544.1)
Abbreviation: R_PGI Synonyms: _0 EC Number: 5.3.1.9 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : g6p <==> f6p Confidence Level: 0 genes: LOCUS:_2821#TRANSCRIPT:1#ABBREVIATION:GPI#ECNUMBERS:5.3.1.9# proteins: NAME:glucose phosphate isomerase#ABBREVIATION:Gpi.1# citations: none GENE ASSOCIATION: (2821.1)
Abbreviation: R_EX_ser_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ser-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MCLOR Synonyms: _0 EC Number: 1.1.1.27 SUBSYSTEM: Cysteine Metabolism Equation: [c] : mercplac + nad <==> h + mercppyr + nadh Confidence Level: 4 genes: LOCUS:_3945#TRANSCRIPT:1#ABBREVIATION:LDHB#ECNUMBERS:1.1.1.27# LOCUS:_3948#TRANSCRIPT:1#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_3939#TRANSCRIPT:1#ABBREVIATION:LDHA#ECNUMBERS:1.1.1.27# LOCUS:_160287#TRANSCRIPT:1#ABBREVIATION:LDHAL6A# LOCUS:_92483#TRANSCRIPT:1#ABBREVIATION:LDHAL6B# LOCUS:_3948#TRANSCRIPT:2#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_55293#TRANSCRIPT:1#ABBREVIATION:UEV3# proteins: NAME:lactate dehydrogenase A (liver, sk muscle)#ABBREVIATION:Ldha.1# NAME:lactate dehydrogenase B (heart, RBC)#ABBREVIATION:Ldhb.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.2# NAME:lactate dehydrogenase A-like 6B (testis)#ABBREVIATION:Ldhal6b.1# NAME:lactate dehydrogenase A-like 6A#ABBREVIATION:Ldhal6a.1# NAME:ubiquitin-conjugating enzyme E2-like#ABBREVIATION:Uev3.1# NAME:lactate dehydrogenase (heart, RBC, brain, kidney, liver, sk muscle)#ABBREVIATION:LdhAB# citations: AUTHORS:Cooper AJ, Haber MT, Meister A. #TITLE:On the chemistry and biochemistry of 3-mercaptopyruvic acid, the alpha-keto acid analog of cysteine#YEAR:1982#PUBMED:7054184# GENE ASSOCIATION: (92483.1) or (3945.1 and 3939.1) or (55293.1) or (3948.2) or (3948.1) or (3945.1) or (160287.1) or (3939.1)
Abbreviation: R_PROSTGE1t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hco3[c] + prostge1[e] <==> hco3[e] + prostge1[c] Confidence Level: 4 NJ genes: LOCUS:_6578#TRANSCRIPT:1#ABBREVIATION:SLCO2A1# proteins: NAME:solute carrier organic anion transporter family, member 2A1#ABBREVIATION:Slco2a1.1# citations: AUTHORS:Lu R, Kanai N, Bao Y, Schuster VL.#TITLE:Cloning, in vitro expression, and tissue distribution of a human prostaglandin transporter cDNA(hPGT).#JOURNAL:J Clin Invest.#YEAR:1996#PUBMED:8787677# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (6578.1)
Abbreviation: R_GLCNACPT_L Synonyms: _1 EC Number: 2.7.8.15 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dolp_L + uacgam --> (0.1) naglc2p_L + ump Confidence Level: 0 NCD genes: LOCUS:_1798#TRANSCRIPT:1#ABBREVIATION:DPAGT1#ECNUMBERS:2.7.8.15# LOCUS:_1798#TRANSCRIPT:2#ABBREVIATION:DPAGT1#ECNUMBERS:2.7.8.15# proteins: NAME:dolichyl-phosphate N-acetylglucosaminephosphotransferase 1 #ABBREVIATION:Dpagt1.1# NAME:dolichyl-phosphate N-acetylglucosaminephosphotransferase 1 #ABBREVIATION:Dpagt1.2# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (1798.2) or (1798.1)
UDP-GlcNAc:dolichol-phosphate GlcNAc phosphotransferase (liver)
Abbreviation: R_MACACI Synonyms: _3 EC Number: 5.2.1.2 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 4mlacac --> 4fumacac Confidence Level: 4 NCD genes: LOCUS:_2954#TRANSCRIPT:1#ABBREVIATION:GSTZ1#ECNUMBERS:5.2.1.2,2.5.1.18# LOCUS:_2954#TRANSCRIPT:2#ABBREVIATION:GSTZ1# LOCUS:_2954#TRANSCRIPT:3#ABBREVIATION:GSTZ1# proteins: NAME:glutathione transferase zeta 1 (maleylacetoacetate isomerase)#ABBREVIATION:Gstz1.1# NAME:glutathione transferase zeta 1 (maleylacetoacetate isomerase)#ABBREVIATION:Gstz1.2# NAME:glutathione transferase zeta 1 (maleylacetoacetate isomerase)#ABBREVIATION:Gstz1.3# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# AUTHORS:Fernandez-Canon JM, Hejna J, Reifsteck C, Olson S, Grompe M#TITLE:Gene structure, chromosomal location, and expression pattern of maleylacetoacetate isomerase#JOURNAL:Genomics#YEAR:1999#PUBMED:10373324# GENE ASSOCIATION: (2954.1) or (2954.3) or (2954.2)
Abbreviation: R_EX_h2o2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : h2o2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DDPGAm Synonyms: _0 EC Number: 4.1.2.14 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : 4h2oglt <==> glx + pyr Confidence Level: 2 IT rxn is done by 4.1.2.14 and 4.1.3.16 mitochondrial isozyme - NCD genes: none proteins: none citations: AUTHORS:Holmes RP, Assimos DG#TITLE:Glyoxylate synthesis, and its modulation and influence on oxalate synthesis#JOURNAL:J Urol#YEAR:1998#PUBMED:9783918# AUTHORS:Takayama T, Fujita K, Suzuki K, Sakaguchi M, Fujie M, Nagai E, Watanabe S, Ichiyama A, Ogawa Y#TITLE:Control of oxalate formation from L-hydroxyproline in liver mitochondria#JOURNAL:J Am Soc Nephrol#YEAR:2003#PUBMED:12660328# AUTHORS:MAITRA U, DEKKER EE#TITLE:PURIFICATION AND PROPERTIES OF RAT LIVER 2-KETO-4-HYDROXYGLUTARATE ALDOLASE#JOURNAL:J Biol Chem#YEAR:1964#PUBMED:14193832# AUTHORS:Knight J, Holmes RP#TITLE:Mitochondrial hydroxyproline metabolism: implications for primary hyperoxaluria#JOURNAL:Am J Nephrol#YEAR:2005#PUBMED:15849464# GENE ASSOCIATION:
_2-dehydro-3-deoxy-phosphogluconate aldolase, mitochondrial
Abbreviation: R_DCK2n Synonyms: _0 EC Number: 2.7.1.74 SUBSYSTEM: Nucleotides Equation: [n] : dcyt + utp <==> dcmp + h + udp Confidence Level: 4 IT (2.7.1.74) genes: LOCUS:_1633#TRANSCRIPT:1#ABBREVIATION:DCK#ECNUMBERS:2.7.1.74# proteins: NAME:deoxycytidine kinase #ABBREVIATION:Dck.1# citations: AUTHORS:Turk B, Awad R, Usova EV, Bjork I, Eriksson S. #TITLE:A pre-steady-state kinetic analysis of substrate binding to human recombinant deoxycytidine kinase: a model for nucleoside kinase action. #JOURNAL:Biochemistry#YEAR:1999#PUBMED:10387103# AUTHORS:Johansson M, Brismar S, Karlsson A.#TITLE:Human deoxycytidine kinase is located in the cell nucleus.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9342341# AUTHORS:Krawiec K, Kierdaszuk B, Shugar D.#TITLE:Inorganic tripolyphosphate (PPP(i)) as a phosphate donor for human deoxyribonucleoside kinases. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12535661# GENE ASSOCIATION: (1633.1)
Abbreviation: R_ADRNCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : adrncoa + crn --> adrncrn + coa Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_S2T3g Synonyms: _0 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_b_pre5 + paps --> cspg_b + h + pap Confidence Level: 4 EC 2.8.2- NCD genes: LOCUS:_10090#TRANSCRIPT:1#ABBREVIATION:UST# proteins: NAME:uronyl-2-sulfotransferase, Golgi apparatus#ABBREVIATION:Ust.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Kobayashi M, Sugumaran G, Liu J, Shworak NW, Silbert JE, Rosenberg RD#TITLE:Molecular cloning and characterization of a human uronyl 2-sulfotransferase that sulfates iduronyl and glucuronyl residues in dermatan/chondroitin sulfate#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10187838# GENE ASSOCIATION: (10090.1)
Abbreviation: R_GLCAT6g Synonyms: _2 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre1 + udpglcur --> h + hs_pre2 + udp Confidence Level: 4 EC 2.4.1.225 NCD genes: LOCUS:_2131#TRANSCRIPT:1#ABBREVIATION:EXT1# LOCUS:_2132#TRANSCRIPT:1#ABBREVIATION:EXT2#ECNUMBERS:2.4.1.-# LOCUS:_2132#TRANSCRIPT:2#ABBREVIATION:EXT2# proteins: NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.1-g# NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.2-g# citations: AUTHORS:McCormick C, Duncan G, Goutsos KT, Tufaro F#TITLE:The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10639137# AUTHORS:Kobayashi S, Morimoto K, Shimizu T, Takahashi M, Kurosawa H, Shirasawa T#TITLE:Association of EXT1 and EXT2, hereditary multiple exostoses gene products, in Golgi apparatus#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679296# AUTHORS:Ahn J, Ludecke HJ, Lindow S, Horton WA, Lee B, Wagner MJ, Horsthemke B, Wells DE#TITLE:Cloning of the putative tumour suppressor gene for hereditary multiple exostoses (EXT1)#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7550340# AUTHORS:McCormick C, Leduc Y, Martindale D, Mattison K, Esford LE, Dyer AP, Tufaro F#TITLE:The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate#JOURNAL:Nat Genet#YEAR:1998#PUBMED:9620772# AUTHORS:Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K#TITLE:The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9756849# AUTHORS:Stickens D, Clines G, Burbee D, Ramos P, Thomas S, Hogue D, Hecht JT, Lovett M, Evans GA#TITLE:The EXT2 multiple exostoses gene defines a family of putative tumour suppressor genes#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8782816# GENE ASSOCIATION: (2131.1 and 2132.1) or (2131.1 and 2132.2)
heparan sulfate, precursor 2_C37H56NO31X
Abbreviation: R_SELADT Synonyms: _0 EC Number: 2.7.7.4 SUBSYSTEM: Selenoamino acid metabolism Equation: [c] : atp + h + sel --> adsel + ppi Confidence Level: 1 SAB genes: LOCUS:_9061#TRANSCRIPT:1#ABBREVIATION:PAPSS1#ECNUMBERS:2.7.1.25,2.7.7.4# LOCUS:_9060#TRANSCRIPT:1#ABBREVIATION:PAPSS2#ECNUMBERS:2.7.7.4,&,2.7.1# proteins: NAME:_3'-phosphoadenosine 5'-phosphosulfate synthase 2 #ABBREVIATION:Papss2.1# NAME:_3'-phosphoadenosine 5'-phosphosulfate synthase 1 #ABBREVIATION:Papss1.1# citations: AUTHORS:Xu ZH, Otterness DM, Freimuth RR, Carlini EJ, Wood TC, Mitchell S, Moon E, Kim UJ, Xu JP, Siciliano MJ, Weinshilboum RM#TITLE:Human 3'-phosphoadenosine 5'-phosphosulfate synthetase 1 (PAPSS1) and PAPSS2: gene cloning, characterization and chromosomal localization#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679223# GENE ASSOCIATION: (9061.1) or (9060.1)
Abbreviation: R_PIPLCn Synonyms: _9 EC Number: 3.1.4.3 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail_hs --> dag_hs + h + mi1p-D Confidence Level: 4 NCD genes: LOCUS:_23236#TRANSCRIPT:1#ABBREVIATION:PLCB1#ECNUMBERS:3.1.4.11# LOCUS:_23236#TRANSCRIPT:2#ABBREVIATION:PLCB1# proteins: NAME:phospholipase C, beta 1 (phosphoinositide-specific), nucleus#ABBREVIATION:Plcb1.2-n# NAME:phospholipase C, beta 1 (phosphoinositide-specific), nucleus#ABBREVIATION:Plcb1.1-n# citations: AUTHORS:Peruzzi D, Aluigi M, Manzoli L, Billi AM, Di Giorgio FP, Morleo M, Martelli AM, Cocco L#TITLE:Molecular characterization of the human PLC beta1 gene#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12213492# AUTHORS:Caricasole A, Sala C, Roncarati R, Formenti E, Terstappen GC#TITLE:Cloning and characterization of the human phosphoinositide-specific phospholipase C-beta 1 (PLC beta 1)#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:11118617# AUTHORS:Peruzzi D, Calabrese G, Faenza I, Manzoli L, Matteucci A, Gianfrancesco F, Billi AM, Stuppia L, Palka G, Cocco L#TITLE:Identification and chromosomal localisation by fluorescence in situ hybridisation of human gene of phosphoinositide-specific phospholipase C beta(1)#JOURNAL:Biochim Biophy Acta#YEAR:2000#PUBMED:10760467# GENE ASSOCIATION: (23236.2) or (23236.1)
phosphatidylinositol phospholipase C, nucleus
Abbreviation: R_ALADGLYexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-D[e] + gly[c] <==> ala-D[c] + gly[e] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_PROD2 Synonyms: _0 EC Number: 1.5.99.8 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : fad + pro-L --> 1pyr5c + fadh2 + h Confidence Level: 2 JLR genes: LOCUS:_58510#TRANSCRIPT:1#ABBREVIATION:PRODH2# proteins: NAME:proline dehydrogenase (oxidase) 2#ABBREVIATION:Prodh2.1# citations: AUTHORS:Gogos JA, Santha M, Takacs Z, Beck KD, Luine V, Lucas LR, Nadler JV, Karayiorgou M.#TITLE:The gene encoding proline dehydrogenase modulates sensorimotor gating in mice. #JOURNAL:Nat Genet#YEAR:1999#PUBMED:10192398# GENE ASSOCIATION: (58510.1)
Abbreviation: R_EX_hcoumarin_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hcoumarin <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ATP1ter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: adp[r] + atp[c] <==> adp[c] + atp[r] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
ADP/ATP transporter, endoplasmic reticulum
Abbreviation: R_KCCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[e] + k[e] <==> cl[c] + k[c] Confidence Level: 4 NCD genes: LOCUS:_10723#TRANSCRIPT:1#ABBREVIATION:SLC12A7# LOCUS:_57468#TRANSCRIPT:1#ABBREVIATION:SLC12A5# LOCUS:_6560#TRANSCRIPT:1#ABBREVIATION:SLC12A4# LOCUS:_9990#TRANSCRIPT:1#ABBREVIATION:SLC12A6# proteins: NAME:solute carrier family 12 (potassium/chloride transporters), member 4#ABBREVIATION:Slc12a4.1-e# NAME:solute carrier family 12, (potassium-chloride transporter) member 5#ABBREVIATION:Slc12a5.1-e# NAME:solute carrier family 12 (potassium/chloride transporters), member 6#ABBREVIATION:Slc12a6.1-e# NAME:solute carrier family 12 (potassium/chloride transporters), member 7#ABBREVIATION:Slc12a7.1-e# citations: AUTHORS:Hebert SC, Mount DB, Gamba G#TITLE:Molecular physiology of cation-coupled Cl- cotransport: the SLC12 family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739168# AUTHORS:Gillen CM, Brill S, Payne JA, Forbush B 3rd#TITLE:Molecular cloning and functional expression of the K-Cl cotransporter from rabbit, rat, and human. A new member of the cation-chloride cotransporter family#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8663127# AUTHORS:Song L, Mercado A, Vazquez N, Xie Q, Desai R, George AL Jr, Gamba G, Mount DB#TITLE:Molecular, functional, and genomic characterization of human KCC2, the neuronal K-Cl cotransporter#JOURNAL:Brain Res Mol Brain Res#YEAR:2002#PUBMED:121106695# AUTHORS:Mount DB, Mercado A, Song L, Xu J, George AL Jr, Delpire E, Gamba G#TITLE:Cloning and characterization of KCC3 and KCC4, new members of the cation-chloride cotransporter gene family#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10347194# AUTHORS:Hiki K, D'Andrea RJ, Furze J, Crawford J, Woollatt E, Sutherland GR, Vadas MA, Gamble JR#TITLE:Cloning, characterization, and chromosomal location of a novel human K+-Cl- cotransporter#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10187864# AUTHORS:Race JE, Makhlouf FN, Logue PJ, Wilson FH, Dunham PB, Holtzman EJ#TITLE:Molecular cloning and functional characterization of KCC3, a new K-Cl cotransporter#JOURNAL:Am J Physiol#YEAR:1999#PUBMED:10600773# AUTHORS:Bergeron MJ, Gagnon E, Wallendorff B, Lapointe JY, Isenring P.#TITLE:Ammonium transport and pH regulation by K(+)-Cl(-) cotransporters#JOURNAL:Am J Physiol Renal Physiol#YEAR:2003#PUBMED:12657561# GENE ASSOCIATION: (10723.1) or (57468.1) or (9990.1) or (6560.1)
Abbreviation: R_RETNtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: retn[c] --> retn[r] Confidence Level: 1 IT genes: none proteins: none citations: AUTHORS:Barua AB, Sidell N.#TITLE:Retinoyl beta-glucuronide: a biologically active interesting retinoid.#JOURNAL:J Nutr.#YEAR:2004#PUBMED:14704335# GENE ASSOCIATION:
Abbreviation: R_EX_ins_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ins <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ORNtiDF Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: orn[e] --> orn[c] Confidence Level: 4 SAB genes: LOCUS:_84889#TRANSCRIPT:1#ABBREVIATION:SLC7A3# LOCUS:_6542#TRANSCRIPT:1#ABBREVIATION:SLC7A2# LOCUS:_6541#TRANSCRIPT:1#ABBREVIATION:SLC7A1# proteins: NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 1#ABBREVIATION:Slc7a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 2#ABBREVIATION:Slc7a2.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 3#ABBREVIATION:Slc7a3.1# citations: AUTHORS:Albritton LM, Bowcock AM, Eddy RL, Morton CC, Tseng L, Farrer LA, Cavalli-Sforza LL, Shows TB, Cunningham JM#TITLE:The human cationic amino acid transporter (ATRC1): physical and genetic mapping to 13q12-q14#JOURNAL:Genomics#YEAR:1992#PUBMED:1348489# AUTHORS:Cariappa R, Heath-Monnig E, Furesz TC, Kamath SG, Smith CH#TITLE:Stable polarized expression of hCAT-1 in an epithelial cell line#JOURNAL:J Membr Biol#YEAR:2002#PUBMED:11891586# AUTHORS:Hoshide R, Ikeda Y, Karashima S, Matsuura T, Komaki S, Kishino T, Niikawa N, Endo F, Matsuda I#TITLE:Molecular cloning, tissue distribution, and chromosomal localization of human cationic amino acid transporter 2 (HCAT2)#JOURNAL:Genomics#YEAR:1996#PUBMED:8954799# AUTHORS:Furesz TC, Moe AJ, Smith CH#TITLE:Lysine uptake by human placental microvillous membrane: comparison of system y+ with basal membrane#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7534987# AUTHORS:Wolf S, Janzen A, Vekony N, Martine U, Strand D, Closs EI#TITLE:Expression of solute carrier 7A4 (SLC7A4) in the plasma membrane is not sufficient to mediate amino acid transport activity#JOURNAL:Biochem J#YEAR:2002#PUBMED:12049641# AUTHORS:Vekony N, Wolf S, Boissel JP, Gnauert K, Closs EI#TITLE:Human cationic amino acid transporter hCAT-3 is preferentially expressed in peripheral tissues#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11591158# AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (6541.1) or (84889.1) or (6542.1)
ornithine transport via diffusion (extracellular to cytosol)
Abbreviation: R_GALGT3 Synonyms: _3 EC Number: 2.4.1.92 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gd3_hs + udpacgal --> gd2_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_2583#TRANSCRIPT:1#ABBREVIATION:GALGT#ECNUMBERS:2.4.1.92# proteins: NAME:UDP-N-acetyl-alpha-D-galactosamine:(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase (GalNAc-T)#ABBREVIATION:Galgt.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Nagata Y, Yamashiro S, Yodoi J, Lloyd KO, Shiku H, Furukawa K.#TITLE:Expression cloning of beta 1,4 N-acetylgalactosaminyltransferase cDNAs that determine the expression of GM2 and GD2 gangliosides.#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1601877# GENE ASSOCIATION: (2583.1)
Abbreviation: R_AKGt4_3 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: akg[e] + (3) na1[e] <==> akg[c] + (3) na1[c] Confidence Level: 4 NCD genes: LOCUS:_64849#TRANSCRIPT:1#ABBREVIATION:SLC13A3# LOCUS:_64849#TRANSCRIPT:2#ABBREVIATION:SLC13A3# proteins: NAME:solute carrier family 13 (sodium-dependent dicarboxylate transporter), member 3#ABBREVIATION:Slc13a3.1-e# NAME:solute carrier family 13 (sodium-dependent dicarboxylate transporter), member 3#ABBREVIATION:Slc13a3.2-e# citations: AUTHORS:Wang H, Fei YJ, Kekuda R, Yang-Feng TL, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Structure, function, and genomic organization of human Na(+)-dependent high-affinity dicarboxylate transporter#JOURNAL:Am J Physiol Cell Physiol#YEAR:2000#PUBMED:0794676# AUTHORS:Pajor AM#TITLE:Sodium-coupled transporters for Krebs cycle intermediates#JOURNAL:Annu Rev Physiol#YEAR:1999#PUBMED:10099705# GENE ASSOCIATION: (64849.1) or (64849.2)
Abbreviation: R_GALACGLCGALGBSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: galacglcgalgbside_hs[c] <==> galacglcgalgbside_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_PROt2rL Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: h[l] + pro-L[l] <==> h[c] + pro-L[c] Confidence Level: 4 NCD SAB lyso genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
L-proline reversible transport via proton symport (lysosome)
Abbreviation: R_G14T19g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core4_pre3 + udpgal --> h + ksii_core4_pre4 + udp Confidence Level: 4 Keratan sulfate II biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
aldehyde dehydrogenase (phenylacetaldehyde, NAD)
Abbreviation: R_ALDD19x Synonyms: _0 EC Number: 1.2.1.39 SUBSYSTEM: Phenylalanine metabolism Equation: [c] : h2o + nad + pacald --> (2) h + nadh + pac Confidence Level: 2 JLR genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# GENE ASSOCIATION: (222.1) or (218.1) or (221.1) or (220.1)
Abbreviation: R_ACN13ACNGALGBSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acn13acngalgbside_hs[c] <==> acn13acngalgbside_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_LPSe Synonyms: _1 EC Number: 3.1.1.3 SUBSYSTEM: Triacylglycerol Synthesis Equation: [e] : h2o + tag_hs --> Rtotal3 + dag_hs + h Confidence Level: 4 FULLR corresponds to acyl/R groups in texts. NJ genes: LOCUS:_3990#TRANSCRIPT:1#ABBREVIATION:LIPC#ECNUMBERS:3.1.1.3# LOCUS:_8513#TRANSCRIPT:1#ABBREVIATION:LIPF#ECNUMBERS:3.1.1.3# LOCUS:_5406#TRANSCRIPT:1#ABBREVIATION:PNLIP#ECNUMBERS:3.1.1.3# LOCUS:_5407#TRANSCRIPT:1#ABBREVIATION:PNLIPRP1# LOCUS:_5408#TRANSCRIPT:1#ABBREVIATION:PNLIPRP2# LOCUS:_9388#TRANSCRIPT:1#ABBREVIATION:LIPG#ECNUMBERS:3.1.1.3# proteins: NAME:pancreatic lipase#ABBREVIATION:Pnlip.1# NAME:lipase, hepatic#ABBREVIATION:Lipc.1# NAME:pancreatic lipase-related protein 1#ABBREVIATION:Pnliprp1.1# NAME:pancreatic lipase-related protein 2#ABBREVIATION:Pnliprp2.1# NAME:lipase, gastric#ABBREVIATION:Lipf.1# NAME:lipase, endothelial#ABBREVIATION:Lipg.1# citations: AUTHORS:Davis RC, Diep A, Hunziker W, Klisak I, Mohandas T, Schotz MC, Sparkes RS, Lusis AJ.#TITLE:Assignment of human pancreatic lipase gene (PNLIP) to chromosome 10q24-q26.#JOURNAL:Genomics#YEAR:1991#PUBMED:1783385# AUTHORS:Giller T, Buchwald P, Blum-Kaelin D, Hunziker W.#TITLE:Two novel human pancreatic lipase related proteins, hPLRP1 and hPLRP2. Differences in colipase dependence and in lipase activity.#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1379598# AUTHORS:Bodmer MW, Angal S, Yarranton GT, Harris TJ, Lyons A, King DJ, Pieroni G, Riviere C, Verger R, Lowe PA.#TITLE:Molecular cloning of a human gastric lipase and expression of the enzyme in yeast.#JOURNAL:Biochim Biophys Acta#YEAR:1987#PUBMED:3304425# AUTHORS:Bernback S, Blackberg L.#TITLE:Human gastric lipase. The N-terminal tetrapeptide is essential for lipid binding and lipase activity.#JOURNAL:Eur J Biochem#YEAR:1989#PUBMED:2753032# AUTHORS:Hirata K, Dichek HL, Cioffi JA, Choi SY, Leeper NJ, Quintana L, Kronmal GS, Cooper AD, Quertermous T.#TITLE:Cloning of a unique lipase from endothelial cells extends the lipase gene family.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10318835# GENE ASSOCIATION: (3990.1) or (9388.1) or (5407.1) or (8513.1) or (5408.1) or (5406.1)
Abbreviation: R_LSTO2r Synonyms: _4 EC Number: 1.3.3.2 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : h + lthstrl + nadph + o2 --> 7dhchsterol + (2) h2o + nadp Confidence Level: 4 lanosterol -> cholesterol pathway NJ genes: LOCUS:_6309#TRANSCRIPT:1#ABBREVIATION:SC5DL# proteins: NAME:sterol-C5-desaturase#ABBREVIATION:Sc5dl.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# AUTHORS:Taton M, Husselstein T, Benveniste P, Rahier A#TITLE:Role of highly conserved residues in the reaction catalyzed by recombinant delta-sterol-C5(6)-desaturase studied by site-directed mutagensis#JOURNAL:Biochemistry#YEAR:2000# GENE ASSOCIATION: (6309.1)
Abbreviation: R_PRGNLONESULT Synonyms: _0 EC Number: 2.8.2.2 SUBSYSTEM: Steroid Metabolism Equation: [c] : paps + prgnlone --> h + pap + prgnlones Confidence Level: 4 NJ genes: LOCUS:_6822#TRANSCRIPT:1#ABBREVIATION:SULT2A1#ECNUMBERS:2.8.2.2# LOCUS:_6820#TRANSCRIPT:2#ABBREVIATION:SULT2B1# proteins: NAME:sulfotransferase family, cytosolic, 2A, dehydroepiandrosterone#ABBREVIATION:Sult2a1.1# NAME:sulfotransferase family, cytosolic, 2B, member 1#ABBREVIATION:Sult2b1.2# citations: AUTHORS:Her C, Wood TC, Eichler EE, Mohrenweiser HW, Ramagli LS, Siciliano MJ, Weinshilboum RM.#TITLE:Human hydroxysteroid sulfotransferase SULT2B1: two enzymes encoded by a single chromosome 19 gene.#JOURNAL:Genomics#YEAR:1998#PUBMED:9799594# AUTHORS:Fuda H, Lee YC, Shimizu C, Javitt NB, Strott CA.#TITLE:Mutational analysis of human hydroxysteroid sulfotransferase SULT2B1 isoforms reveals that exon 1B of the SULT2B1 gene produces cholesterol sulfotransferase, whereas exon 1A yields pregnenolone sulfotransferase.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12145317# GENE ASSOCIATION: (6822.1) or (6820.2)
keratan sulfate II (core 2-linked), degradation product 1_C70H113N5O59S3X
Abbreviation: R_UREAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: urea[e] <==> urea[c] Confidence Level: 4 JLR genes: LOCUS:_6563#TRANSCRIPT:1#ABBREVIATION:SLC14A1# LOCUS:_6528#TRANSCRIPT:1#ABBREVIATION:SLC5A5# LOCUS:_6523#TRANSCRIPT:1#ABBREVIATION:SLC5A1# LOCUS:_8170#TRANSCRIPT:1#ABBREVIATION:SLC14A2# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 1, extracellular#ABBREVIATION:Slc5a1.1-e# NAME:solute carrier family 14 (urea transporter), member 1 (Kidd blood group), extracellular #ABBREVIATION:Slc14a1.1-e# NAME:solute carrier family 14 (urea transporter), member 2, extracellular #ABBREVIATION:Slc14a2.1-e# NAME:solute carrier family 5 (sodium iodide symporter), member 5, extacellular#ABBREVIATION:Slc5a5.1-e# citations: AUTHORS:Hirayama BA, Loo DD, Wright EM.#TITLE:Protons drive sugar transport through the Na+/glucose cotransporter (SGLT1)#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8063771# AUTHORS:Hediger MA, Turk E, Wright EM#TITLE:Homology of the human intestinal Na+/glucose and Escherichia coli Na+/proline cotransporters#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1989#PUBMED:2490366# AUTHORS:Quick M, Loo DD, Wright EM#TITLE:Neutralization of a conserved amino acid residue in the human Na+/glucose transporter (hSGLT1) generates a glucose-gated H+ channel#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11024018# AUTHORS:Wright EM, Hirayama BA, Loo DDF, Turk E, Hager K#TITLE:Intestinal sugar transport#YEAR:1994# AUTHORS:Leung DW, Loo DD, Hirayama BA, Zeuthen T, Wright EM#TITLE:Urea transport by cotransporters#JOURNAL:J Physiol#YEAR:2000#PUBMED:11034615# AUTHORS:Shayakul C, Hediger MA.#TITLE:The SLC14 gene family of urea transporters.#YEAR:2004#PUBMED:12856182# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# AUTHORS:Wapnir IL, van de Rijn M, Nowels K, Amenta PS, Walton K, Montgomery K, Greco RS, Dohan O, Carrasco N#TITLE:Immunohistochemical profile of the sodium/iodide symporter in thyroid, breast, and other carcinomas using high density tissue microarrays and conventional sections#JOURNAL:J Clin Endocrinol Metab#YEAR:2003#PUBMED:12679487# AUTHORS:Dai G, Levy O, Carrasco N#TITLE:Cloning and characterization of the thyroid iodide transporter#JOURNAL:Nature#YEAR:1996#PUBMED:8559252# AUTHORS:Smanik PA, Liu Q, Furminger TL, Ryu K, Xing S, Mazzaferri EL, Jhiang SM#TITLE:Cloning of the human sodium lodide symporter#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8806637# AUTHORS:De La Vieja A, Dohan O, Levy O, Carrasco N#TITLE:Molecular analysis of the sodium/iodide symporter: impact on thyroid and extrathyroid pathophysiology#JOURNAL:Physiol Rev#YEAR:2000#PUBMED:10893432# AUTHORS:Eskandari S, Loo DD, Dai G, Levy O, Wright EM, Carrasco N.#TITLE:Thyroid Na+/I- symporter. Mechanism, stoichiometry, and specificity#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9341168# GENE ASSOCIATION: (6523.1) or (6563.1) or (6528.1) or (8170.1)
Abbreviation: R_P4502F1 Synonyms: _1 SUBSYSTEM: CYP Metabolism Equation: [c] : h + nadph + npthl + o2 --> h2o + nadp + onpthl Confidence Level: 4 naphthalene epoxidation assumed to take place on outer membrane NJ genes: LOCUS:_1572#TRANSCRIPT:1#ABBREVIATION:CYP2F1# proteins: NAME:cytochrome P450, family 2, subfamily F, polypeptide 1#ABBREVIATION:Cyp2f1.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Nhamburo PT, Kimura S, McBride OW, Kozak CA, Gelboin HV, Gonzalez FJ.#TITLE:The human CYP2F gene subfamily: identification of a cDNA encoding a new cytochrome P450, cDNA-directed expression, and chromosome mapping.#JOURNAL:Biochemistry#YEAR:1990#PUBMED:1974816# GENE ASSOCIATION: (1572.1)
Abbreviation: R_34DHOXPEGt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _34dhoxpeg[c] <==> 34dhoxpeg[e] Confidence Level: 2 SAB genes: none proteins: none citations: AUTHORS:Kurz T, Richardt G, Hagl S, Seyfarth M, Schomig A#TITLE:Two different mechanisms of noradrenaline release during normoxia and simulated ischemia in human cardiac tissue#JOURNAL:J Mol Cell Cardiol#YEAR:1995#PUBMED:7473774# GENE ASSOCIATION:
_3,4-Dihydroxyphenylethyleneglycol transport (diffusion)
Abbreviation: R_BETALDHx Synonyms: _3 EC Number: 1.2.1.8 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : betald + h2o + nad --> glyb + (2) h + nadh Confidence Level: 4 JLR genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# proteins: NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# citations: AUTHORS:Chern MK, Pietruszko R.#TITLE:Human aldehyde dehydrogenase E3 isozyme is a betaine aldehyde dehydrogenase. #YEAR:1995#PUBMED:7646513# AUTHORS:Craig SA.#TITLE:Betaine in human nutrition.#YEAR:2004#PUBMED:15321791# GENE ASSOCIATION: (223.1)
Abbreviation: R_OCDCEAtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ocdcea[e] <==> ocdcea[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_EX_urate_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : urate <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_vitd2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : vitd2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SO4CLtex2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[c] + (2) so4[e] --> cl[e] + (2) so4[c] Confidence Level: 2 SAB genes: LOCUS:_65010#TRANSCRIPT:1#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:2#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:3#ABBREVIATION:SLC26A6# proteins: NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.1# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.2# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.3# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (65010.1) or (65010.3) or (65010.2)
sulfate transport via chloride countertransport (2:1)
Abbreviation: R_3HPCOAHYD Synonyms: _0 EC Number: 3.1.2.4 SUBSYSTEM: beta-Alanine metabolism Equation: [c] : 3hpcoa + h2o --> 3hpp + coa + h Confidence Level: 4 SAB genes: LOCUS:_26275#TRANSCRIPT:1#ABBREVIATION:HIBCH# LOCUS:_26275#TRANSCRIPT:2#ABBREVIATION:HIBCH# proteins: NAME:_3-hydroxyisobutyryl-Coenzyme A hydrolase#ABBREVIATION:Hibch.1# NAME:_3-hydroxyisobutyryl-Coenzyme A hydrolase#ABBREVIATION:Hibch.2# citations: AUTHORS:Hawes JW, Jaskiewicz J, Shimomura Y, Huang B, Bunting J, Harper ET, Harris RA. #TITLE:Primary structure and tissue-specific expression of human beta-hydroxyisobutyryl-coenzyme A hydrolase. #JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8824301# GENE ASSOCIATION: (26275.1) or (26275.2)
_3-hydroxyisobutyryl-Coenzyme A hydrolase
linoelaidic acid ACP (all trans)_C29H51N2O8PRS
Abbreviation: R_FA1822ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + lneldcACP <==> ACP + h + lneldc Confidence Level: 4 NJ genes: none proteins: none citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION:
Abbreviation: R_ADMDC Synonyms: _0 EC Number: 4.1.1.50 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : amet + h --> ametam + co2 Confidence Level: 4 genes: LOCUS:_262#TRANSCRIPT:1#ABBREVIATION:AMD1#ECNUMBERS:4.1.1.50# proteins: NAME:adenosylmethionine decarboxylase 1#ABBREVIATION:Amd1.1# citations: AUTHORS:Pajunen A, Crozat A, Janne OA, Ihalainen R, Laitinen PH, Stanley B, Madhubala R, Pegg AE#TITLE:Structure and regulation of mammalian S-adenosylmethionine decarboxylase#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:2460457# GENE ASSOCIATION: (262.1)
Abbreviation: R_FUMTSULtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: fum[c] + tsul[m] <==> fum[m] + tsul[c] Confidence Level: 4 MM genes: LOCUS:_1468#TRANSCRIPT:1#ABBREVIATION:SLC25A10# proteins: NAME:Solute carrier family 25 (dicarboxylate transporter) member 10, mitochondrial #ABBREVIATION:Slc25a10.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# AUTHORS:Crompton M, Palmieri F, Capano M, Quagliariello E.#TITLE:The transport of sulphate and sulphite in rat liver mitochondria. #YEAR:1974#PUBMED:4441366# GENE ASSOCIATION: (1468.1)
Fumarate:thiosulfate antiport, mitochondrial
Abbreviation: R_HMBS Synonyms: _0 EC Number: 4.3.1.8 SUBSYSTEM: Heme Biosynthesis Equation: [c] : h2o + (4) ppbng --> hmbil + (4) nh4 Confidence Level: 2 genes: LOCUS:_3145#TRANSCRIPT:1#ABBREVIATION:HMBS#ECNUMBERS:4.3.1.8# proteins: NAME:hydroxymethylbilane synthase#ABBREVIATION:Hmbs.1# citations: AUTHORS:Raich N, Romeo PH, Dubart A, Beaupain D, Cohen-Solal M, Goossens M.#TITLE:Molecular cloning and complete primary sequence of human erythrocyte porphobilinogen deaminase.#JOURNAL:Nucleic Acids Res#YEAR:1986#PUBMED:2875434# GENE ASSOCIATION: (3145.1)
Abbreviation: R_GALASE19ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : f1a + h2o --> core6 + gal Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_GLCt4_2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glc-D[e] + (2) na1[e] <==> glc-D[c] + (2) na1[c] Confidence Level: 4 NCD genes: LOCUS:_6523#TRANSCRIPT:1#ABBREVIATION:SLC5A1# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 1, extracellular#ABBREVIATION:Slc5a1.1-e# citations: AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# AUTHORS:Hirayama BA, Loo DD, Wright EM.#TITLE:Protons drive sugar transport through the Na+/glucose cotransporter (SGLT1)#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8063771# AUTHORS:Hediger MA, Turk E, Wright EM#TITLE:Homology of the human intestinal Na+/glucose and Escherichia coli Na+/proline cotransporters#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1989#PUBMED:2490366# AUTHORS:Quick M, Loo DD, Wright EM#TITLE:Neutralization of a conserved amino acid residue in the human Na+/glucose transporter (hSGLT1) generates a glucose-gated H+ channel#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11024018# AUTHORS:Wright EM, Hirayama BA, Loo DDF, Turk E, Hager K#TITLE:Intestinal sugar transport#YEAR:1994# AUTHORS:Leung DW, Loo DD, Hirayama BA, Zeuthen T, Wright EM#TITLE:Urea transport by cotransporters#JOURNAL:J Physiol#YEAR:2000#PUBMED:11034615# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# AUTHORS:Tazawa S, Yamato T, Fujikura H, Hiratochi M, Itoh F, Tomae M, Takemura Y, Maruyama H, Sugiyama T, Wakamatsu A, Isogai T, Isaji M#TITLE:SLC5A9/SGLT4, a new Na+-dependent glucose transporter, is an essential transporter for mannose, 1,5-anhydro-D-glucitol, and fructose#JOURNAL:Life Sci#YEAR:2005#PUBMED:15607332# GENE ASSOCIATION: (6523.1)
Abbreviation: R_EX_na1_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : na1 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NACASPtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: Nacasp[m] --> Nacasp[c] Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Moreno A, Ross BD, Bluml S.#TITLE:Direct determination of the N-acetyl-L-aspartate synthesis rate in the human brain by (13)C MRS and [1-(13)C]glucose infusion.#YEAR:2001#PUBMED:11279290# GENE ASSOCIATION:
N-acetyl-L-aspartate transport (mitochondria to cytosol)
Abbreviation: R_MM8Ag Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m8masn --> m7masnA + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
Abbreviation: R_MERCPLACCYSt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: mercplaccys[c] --> mercplaccys[e] Confidence Level: 2 excretion of 3-mercaptolactate-cysteine disulfide (PMID: 6945862) MM genes: none proteins: none citations: none GENE ASSOCIATION:
_3-mercaptolactate-cysteine disulfide transport, extracellular
Abbreviation: R_MEOHtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: meoh[c] <==> meoh[r] Confidence Level: 1 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Methanol transporter, endoplasmic reticulum
Abbreviation: R_GAL3ST11 Synonyms: _4 EC Number: 2.8.2.11 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : galside_hs + paps --> h + pap + sgalside_hs Confidence Level: 4 NJ genes: LOCUS:_9514#TRANSCRIPT:1#ABBREVIATION:CST#ECNUMBERS:2.8.2.11# proteins: NAME:galactose-3-O-sulfotransferase 1#ABBREVIATION:Gal3st1.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Honke K, Tsuda M, Hirahara Y, Ishii A, Makita A, Wada Y.#TITLE:Molecular cloning and expression of cDNA encoding human 3'-phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase.#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9030544# GENE ASSOCIATION: (9514.1)
Abbreviation: R_ECOAH9m Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 2mb2coa + h2o <==> 3hmbcoa Confidence Level: 2 MM genes: LOCUS:_1892#TRANSCRIPT:1#ABBREVIATION:ECHS1# LOCUS:_3030#TRANSCRIPT:1#ABBREVIATION:HADHA#ECNUMBERS:4.2.1.17,1.1.1.35# LOCUS:_3032#TRANSCRIPT:1#ABBREVIATION:HADHB# proteins: NAME:enoyl Coenzyme A hydratase, short chain, 1, mitochondrial#ABBREVIATION:Echs1.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit #ABBREVIATION:Hadha.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit #ABBREVIATION:Hadhb.1-m# citations: AUTHORS:Sweetman, L, Williams, JC#TITLE:Branched chain organic acidurias#JOURNAL:The Metabolic and Molecular Bases of Inherited Disease, 8th ed (Scriver CR, et al., editors)#YEAR:2001# GENE ASSOCIATION: (1892.1) or (3030.1) and (3032.1)
_2-Methylprop-2-enoyl-CoA (2-Methylbut-2-enoyl-CoA), mitochondrial
Abbreviation: R_CH25H Synonyms: _0 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : chsterol + h + nadph + o2 --> h2o + nadp + xol25oh Confidence Level: 4 NJ genes: LOCUS:_9023#TRANSCRIPT:1#ABBREVIATION:CH25H# proteins: NAME:cholesterol 25-hydroxylase#ABBREVIATION:Ch25h.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (9023.1)
Abbreviation: R_GLCAT8g Synonyms: _2 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre5 + udpglcur --> h + hs_pre6 + udp Confidence Level: 4 EC 2.4.1.225 NCD genes: LOCUS:_2131#TRANSCRIPT:1#ABBREVIATION:EXT1# LOCUS:_2132#TRANSCRIPT:1#ABBREVIATION:EXT2#ECNUMBERS:2.4.1.-# LOCUS:_2132#TRANSCRIPT:2#ABBREVIATION:EXT2# proteins: NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.1-g# NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.2-g# citations: AUTHORS:McCormick C, Duncan G, Goutsos KT, Tufaro F#TITLE:The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10639137# AUTHORS:Kobayashi S, Morimoto K, Shimizu T, Takahashi M, Kurosawa H, Shirasawa T#TITLE:Association of EXT1 and EXT2, hereditary multiple exostoses gene products, in Golgi apparatus#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679296# AUTHORS:Ahn J, Ludecke HJ, Lindow S, Horton WA, Lee B, Wagner MJ, Horsthemke B, Wells DE#TITLE:Cloning of the putative tumour suppressor gene for hereditary multiple exostoses (EXT1)#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7550340# AUTHORS:McCormick C, Leduc Y, Martindale D, Mattison K, Esford LE, Dyer AP, Tufaro F#TITLE:The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate#JOURNAL:Nat Genet#YEAR:1998#PUBMED:9620772# AUTHORS:Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K#TITLE:The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9756849# AUTHORS:Stickens D, Clines G, Burbee D, Ramos P, Thomas S, Hogue D, Hecht JT, Lovett M, Evans GA#TITLE:The EXT2 multiple exostoses gene defines a family of putative tumour suppressor genes#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8782816# GENE ASSOCIATION: (2131.1 and 2132.1) or (2131.1 and 2132.2)
heparan sulfate, precursor 6_C65H96N3O53X
Abbreviation: R_DNDPt50m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[m] + dttp[c] --> dcdp[c] + dttp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_TRPt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: trp-L[e] <==> trp-L[c] Confidence Level: 4 genes: LOCUS:_117247#TRANSCRIPT:1#ABBREVIATION:SLC16A10# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 10#ABBREVIATION:Slc16a10.1-e# citations: AUTHORS:Kim do K, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H#TITLE:The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location#JOURNAL:Genomics#YEAR:2002#PUBMED:11827462# GENE ASSOCIATION: (117247.1)
Abbreviation: R_C3STKR2r Synonyms: _0 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : h + nadph + zym_int2 --> nadp + zymst Confidence Level: 4 NCD ER - NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# proteins: NAME:hydroxysteroid (17-beta) dehydrogenase 4 er#ABBREVIATION:Hsd17b4.1-r# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (3295.1)
Abbreviation: R_IPDDIx Synonyms: _0 EC Number: 5.3.3.2 SUBSYSTEM: Cholesterol Metabolism Equation: [x] : ipdp <==> dmpp Confidence Level: 4 peroxisomal version - NJ genes: LOCUS:_3422#TRANSCRIPT:1#ABBREVIATION:IDI1#ECNUMBERS:5.3.3.2# LOCUS:_91734#TRANSCRIPT:1#ABBREVIATION:IDI2# proteins: NAME:isopentyl-diphosphate isomerase#ABBREVIATION:Idi1.1# NAME:isopentenyl diphosphate isomerase (type 2)#ABBREVIATION:Idi2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# AUTHORS:Breitling R, Laubner D, Clizbe D, Adamski J, Krisans SK#TITLE:Isopentenyl-diphosphate isomerases in human and mouse: evolutionary analysis of a mammalian gene duplication#JOURNAL:J Mol Evol#YEAR:2003# AUTHORS:Bonanno JB, Edo C, Eswar N, Pieper U, Romanowski MJ, Ilyin V, Gerchman SE, Kycia H, Studier W, Sali A, Burley SK#TITLE:Structural genomics of enzymes involved in sterol/isoprenoid biosynthesis#JOURNAL:Proceedings of the National Academy of Sciences#YEAR:2001# GENE ASSOCIATION: (3422.1) or (91734.1)
Abbreviation: R_DCSPTN1t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dcsptn1[e] <==> dcsptn1[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_EX_s2l2n2m2masn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : s2l2n2m2masn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DM_dgtp_LPAREN_n_RPAREN_ Synonyms: _0 Equation: [n] : dgtp --> Confidence Level: IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_cytd_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cytd <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_INSTt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[e] + inost[e] <==> h[c] + inost[c] Confidence Level: 4 genes: LOCUS:_114134#TRANSCRIPT:1#ABBREVIATION:SLC2A13# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 13, extracellular#ABBREVIATION:Slc2a13.1-e# citations: AUTHORS:Uldry M, Thorens B#TITLE:The SLC2 family of facilitated hexose and polyol transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750891# AUTHORS:Uldry M, Ibberson M, Horisberger JD, Chatton JY, Riederer BM, Thorens B#TITLE:Identification of a mammalian H(+)-myo-inositol symporter expressed predominantly in the brain#JOURNAL:EMBO J#YEAR:2001#PUBMED:11500374# GENE ASSOCIATION: (114134.1)
inositol transport in via proton symport
Abbreviation: R_ACGBGBSIDEtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: acgbgbside_hs[c] <==> acgbgbside_hs[l] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# GENE ASSOCIATION:
Abbreviation: R_L_DASH_LACt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[e] + lac-L[e] <==> h[c] + lac-L[c] Confidence Level: 4 NCD genes: LOCUS:_23539#TRANSCRIPT:1#ABBREVIATION:SLC16A8# LOCUS:_9194#TRANSCRIPT:1#ABBREVIATION:SLC16A7# LOCUS:_9123#TRANSCRIPT:1#ABBREVIATION:SLC16A3# LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 7#ABBREVIATION:Slc16a7.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1#ABBREVIATION:Slc16a1.1# NAME:solute carrier 16 (monocarboxylic acid transporters), member 8#ABBREVIATION:Slc16a8.1-e# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 3#ABBREVIATION:Slc16a3.1-e# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Lin RY, Vera JC, Chaganti RS, Golde DW#TITLE:Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9786900# AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# AUTHORS:Halestrap AP, Price NT#TITLE:The proton-linked monocarboxylate transporter (MCT) family: structure, function and regulation#JOURNAL:Biochem J#YEAR:1999#PUBMED:10510291# AUTHORS:Garcia CK, Li X, Luna J, Francke U#TITLE:cDNA cloning of the human monocarboxylate transporter 1 and chromosomal localization of the SLC16A1 locus to 1p13.2-p12#JOURNAL:Genomics#YEAR:1994#PUBMED:7835905# AUTHORS:Poole RC, Halestrap AP#TITLE:Transport of lactate and other monocarboxylates across mammalian plasma membranes#JOURNAL:Am J Physiol#YEAR:1993#PUBMED:8476015# AUTHORS:Ritzhaupt A, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:The characterization of butyrate transport across pig and human colonic luminal membrane#JOURNAL:J Physiol#YEAR:1998#PUBMED:9508842# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification of a monocarboxylate transporter isoform type 1 (MCT1) on the luminal membrane of human and pig colon#JOURNAL:Biochem Soc Trans#YEAR:1998#PUBMED:9649795# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification and characterization of a monocarboxylate transporter (MCT1) in pig and human colon: its potential to transport L-lactate as well as butyrate#JOURNAL:J Physiol#YEAR:1998#PUBMED:9824713# AUTHORS:Halestrap AP, Meredith D#TITLE:The SLC16 gene family-from monocarboxylate transporters (MCTs) to aromatic amino acid transporters and beyond#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739169# AUTHORS:Yoon H, Donoso LA, Philp NJ#TITLE:Cloning of the human monocarboxylate transporter MCT3 gene: localization to chromosome 22q12.3-q13.2.#JOURNAL:Genomics#YEAR:1999#PUBMED:10493836# AUTHORS:Manning Fox JE, Meredith D, Halestrap AP#TITLE:Characterisation of human monocarboxylate transporter 4 substantiates its role in lactic acid efflux from skeletal muscle#JOURNAL:J Physiol#YEAR:2000#PUBMED:11101640# AUTHORS:Price NT, Jackson VN, Halestrap AP#TITLE:Cloning and sequencing of four new mammalian monocarboxylate transporter (MCT) homologues confirms the existence of a transporter family with an ancient past#JOURNAL:Biochem J#YEAR:1998#PUBMED:9425115# AUTHORS:Kim do K, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H#TITLE:The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location#JOURNAL:Genomics#YEAR:2002#PUBMED:11827462# GENE ASSOCIATION: (9123.1) or (6566.1) or (23539.1) or (9194.1)
L-lactate reversible transport via proton symport
Abbreviation: R_NADS2 Synonyms: _0 EC Number: 6.3.5.1 SUBSYSTEM: NAD Metabolism Equation: [c] : atp + dnad + gln-L + h2o --> amp + glu-L + h + nad + ppi Confidence Level: 4 genes: LOCUS:_55191#TRANSCRIPT:1#ABBREVIATION:NADSYN1# proteins: NAME:NAD synthetase 1 (glutamine-dependent)#ABBREVIATION:Nadsyn1.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Hara N, Yamada K, Terashima M, Osago H, Shimoyama M, Tsuchiya M. #TITLE:Molecular identification of human glutamine- and ammonia-dependent NAD synthetases. Carbon-nitrogen hydrolase domain confers glutamine dependency.#JOURNAL: J Biol Chem.#YEAR:2003#PUBMED:12547821# GENE ASSOCIATION: (55191.1)
_3 beta-hydroxysteroid dehydrogenase type 1
Abbreviation: R_HSD3B2r Synonyms: _0 SUBSYSTEM: Steroid Metabolism Equation: [r] : eandrstrn + h + nadh --> andrstandn + h2o + nad Confidence Level: 4 see PMID: 14643063 specific cofactors/etc have not yet been determined - largely inferred NJ genes: LOCUS:_3284#TRANSCRIPT:1#ABBREVIATION:HSD3B2#ECNUMBERS:1.1.1.145,,5.3.# proteins: NAME:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 2#ABBREVIATION:Hsd3b2.1# citations: AUTHORS:Belanger A, Pelletier G, Labrie F, Barbier O, Chouinard S.#TITLE:Inactivation of androgens by UDP-glucuronosyltransferase enzymes in humans.#JOURNAL:Trends Endocrinol Metab.#YEAR:2003#PUBMED:14643063# AUTHORS:Luu-The V.#TITLE:Analysis and characteristics of multiple types of human 17beta-hydroxysteroid dehydrogenase.#JOURNAL:J Steroid Biochem Mol Biol. #YEAR:2001#PUBMED:11384872# GENE ASSOCIATION: (3284.1)
Abbreviation: R_GLYt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gly[e] + h[e] <==> gly[c] + h[c] Confidence Level: 2 NCD genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
glycine reversible transport via proton symport
phosphatidylinositol 4-phosphate 5-kinase
Abbreviation: R_PI4P5K Synonyms: _5 EC Number: 2.7.1.68 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : atp + pail4p_hs --> adp + h + pail45p_hs Confidence Level: 4 Notice that contrary to some standards in texts, KEGG, etc, the Rtotal group accounts for the whole fatty acyl CoA (so the CHO only accounts for the core backbone), manner in which Rtotal was produced accounts for O and H balances. Formation of the Rtotal group (rxn flux)accounted for mass balance for CoA NJ changed EC from 2.7.1.149 (pail5p) to 2.7.1.68 (pail4p) - NCD genes: LOCUS:_5305#TRANSCRIPT:1#ABBREVIATION:PIP5K2A# LOCUS:_8394#TRANSCRIPT:1#ABBREVIATION:PIP5K1A#ECNUMBERS:2.7.1.68# LOCUS:_8396#TRANSCRIPT:1#ABBREVIATION:PIP5K2B#ECNUMBERS:2.7.1.68# LOCUS:_79837#TRANSCRIPT:1#ABBREVIATION:PIP5K2C# LOCUS:_23396#TRANSCRIPT:1#ABBREVIATION:PIP5K1C# LOCUS:_200576#TRANSCRIPT:1#ABBREVIATION:PIP5K3# LOCUS:_200576#TRANSCRIPT:2#ABBREVIATION:PIP5K3# LOCUS:_200576#TRANSCRIPT:3#ABBREVIATION:PIP5K3# LOCUS:_8395#TRANSCRIPT:1#ABBREVIATION:PIP5K1B# LOCUS:_8396#TRANSCRIPT:2#ABBREVIATION:PIP5K2B# proteins: NAME:phosphatidylinositol-3-phosphate/phosphatidylinositol 5-kinase, type III#ABBREVIATION:Pip5k3.1# NAME:phosphatidylinositol-3-phosphate/phosphatidylinositol 5-kinase, type III#ABBREVIATION:Pip5k3.2# NAME:phosphatidylinositol-3-phosphate/phosphatidylinositol 5-kinase, type III#ABBREVIATION:Pip5k3.3# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, gamma #ABBREVIATION:Pip5k1c.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type II, gamma#ABBREVIATION:Pip5k2c.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type II, alpha#ABBREVIATION:Pip5k2a.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, beta#ABBREVIATION:Pip5k1b.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, alpha#ABBREVIATION:Pip5k1a.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type II, beta#ABBREVIATION:Pip5k2b.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type II, beta#ABBREVIATION:Pip5k2b.2# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Boronenkov IV, Anderson RA#TITLE:The sequence of phosphatidylinositol-4-phosphate 5-kinase defines a novel family of lipid kinases#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7852364# AUTHORS:Carvajal JJ, Pook MA, dos Santos M, Doudney K, Hillermann R, Minogue S, Williamson R, Hsuan JJ, Chamberlain S#TITLE:The Friedreich's ataxia gene encodes a novel phosphatidylinositol-4- phosphate 5-kinase#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8841185# AUTHORS:Loijens JC, Anderson RA#TITLE:Type I phosphatidylinositol-4-phosphate 5-kinases are distinct members of this novel lipid kinase family#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8955136# AUTHORS:Burriss Garrett RJ, Redman CM#TITLE:Localization of enzymes involved in polyphosphoinositids metabolism on the cytoplasmic surface of the human erythrocyte membrane#JOURNAL:Biochim Biophys Acta#YEAR:1975#PUBMED:164238# GENE ASSOCIATION: (5305.1) or (200576.3) or (8395.1) or (8396.1) or (200576.2) or (8394.1) or (200576.1) or (23396.1) or (8396.2) or (79837.1)
Abbreviation: R_GALACGLCGALGBSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: galacglcgalgbside_hs[c] <==> galacglcgalgbside_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
galacglcgalgbside_hs intracellular transport
Abbreviation: R_DURIK1m Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [m] : atp + duri --> adp + dump + h Confidence Level: 2 genes: LOCUS:_7084#TRANSCRIPT:1#ABBREVIATION:TK2#ECNUMBERS:2.7.1.21# proteins: NAME:thymidine kinase 2, mitochondrial #ABBREVIATION:Tk2.1-m# citations: AUTHORS:Johansson M, Brismar S, Karlsson A.#TITLE:Human deoxycytidine kinase is located in the cell nucleus.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9342341# AUTHORS:Krawiec K, Kierdaszuk B, Shugar D.#TITLE:Inorganic tripolyphosphate (PPP(i)) as a phosphate donor for human deoxyribonucleoside kinases. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12535661# GENE ASSOCIATION: (7084.1)
deoxyuridine kinase (ATP:Deoxyuridine), mitochondrial
Abbreviation: R_S4TASE4ly Synonyms: _5 EC Number: 3.1.6.12 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e + h2o <==> cs_e_deg1 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_411#TRANSCRIPT:1#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# LOCUS:_411#TRANSCRIPT:2#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# proteins: NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.1-l# NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.2-l# citations: AUTHORS:Schuchman EH, Jackson CE, Desnick RJ#TITLE:Human arylsulfatase B: MOPAC cloning, nucleotide sequence of a full-length cDNA, and regions of amino acid identity with arylsulfatases A and C#JOURNAL:Genomics#YEAR:1990#PUBMED:1968043# AUTHORS:Peters C, Schmidt B, Rommerskirch W, Rupp K, Zuhlsdorf M, Vingron M, Meyer HE, Pohlmann R, von Figura K#TITLE:Phylogenetic conservation of arylsulfatases. cDNA cloning and expression of human arylsulfatase B.#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2303452# GENE ASSOCIATION: (411.1) or (411.2)
chondroitin sulfate E (GalNAc4,6diS-GlcA), degradation product 1_C45H65N2O52S5
Abbreviation: R_BTND1 Synonyms: _0 EC Number: 3.5.1.12 SUBSYSTEM: Biotin Metabolism Equation: [c] : biocyt + h2o --> btn + lys-L Confidence Level: 4 IT genes: LOCUS:_686#TRANSCRIPT:1#ABBREVIATION:BTD#ECNUMBERS:3.5.1.12# proteins: NAME:Biotinidase #ABBREVIATION:Btd.2# citations: AUTHORS:Stanley CM, Hymes J, Wolf B.#TITLE:Identification of alternatively spliced human biotinidase mRNAs and putative localization of endogenous biotinidase.#JOURNAL:Mol Genet Metab.#YEAR:2004#PUBMED:15059618# AUTHORS:Pacheco-Alvarez D, Solorzano-Vargas RS, Del Rio AL.#TITLE:Biotin in metabolism and its relationship to human disease.#JOURNAL:Arch Med Res. #YEAR:2002#PUBMED:12459313# GENE ASSOCIATION: (686.1)
Abbreviation: R_DIGALSGALSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: digalsgalside_hs[c] <==> digalsgalside_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
digalsgalside_hs intracellular transport
Abbreviation: R_CRNtHa Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: crn[c] + h[e] --> crn[e] + h[c] Confidence Level: 4 SAB H+ stoichiometry unknown genes: LOCUS:_6583#TRANSCRIPT:1#ABBREVIATION:SLC22A4# proteins: NAME:solute carrier family 22 (organic cation transporter), member 4#ABBREVIATION:Slc22a4.1# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# GENE ASSOCIATION: (6583.1)
L-carnitine outward transport (H+ antiport)
Abbreviation: R_PYDAMtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: pydam[e] <==> pydam[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# AUTHORS:Mehansho H, Henderson LM.#TITLE:Transport and accumulation of pyridoxine and pyridoxal by erythrocytes.#JOURNAL:J Biol Chem. #YEAR:1980#PUBMED:J Biol Chem.# GENE ASSOCIATION:
Abbreviation: R_NTD7 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [c] : amp + h2o --> adn + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_93034#TRANSCRIPT:1#ABBREVIATION:NT5C1B#ECNUMBERS:3.1.3.5# LOCUS:_22978#TRANSCRIPT:1#ABBREVIATION:NT5C2# LOCUS:_93034#TRANSCRIPT:2#ABBREVIATION:NT5C1B#ECNUMBERS:3.1.3.5# proteins: NAME:_5'-nucleotidase, cytosolic II #ABBREVIATION:Nt5C2.1# NAME:_5'-nucleotidase, cytosolic IB #ABBREVIATION:Nt5c1B.1# NAME:_5'-nucleotidase, cytosolic IB #ABBREVIATION:Nt5c1B.2# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# AUTHORS:Hunsucker SA, Spychala J, Mitchell BS.#TITLE:Human cytosolic 5'-nucleotidase I: characterization and role in nucleoside analog resistance. #JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11133996# GENE ASSOCIATION: (22978.1) or (93034.2) or (93034.1)
Abbreviation: R_C226CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: c226crn[c] --> c226crn[m] Confidence Level: 4 TDV genes: none proteins: none citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION:
Abbreviation: R_RETNCOA Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : atp + coa + retn --> amp + ppi + retncoa Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_EX_met_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : met-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ETOHt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: etoh[e] <==> etoh[c] Confidence Level: 4 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_VLCS2p Synonyms: _5 EC Number: 6.2.1.7 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : atp + coa + dhcholestanate --> amp + dhcholestancoa + ppi Confidence Level: 2 from uniprot and lit - see human recon lit refs divalent cation requirement - unclear which one, reaction inhibited in the presence of EDTA (may just be MgATP or MgATP) - see Kelley and Vessey Biochimica et Biophysica Acta (1994) 1209: 51-55 temporarily will have H+ balance reaction NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# proteins: NAME:bile acid CoA ligase#ABBREVIATION:Slc27a1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Steinberg SJ, Wang SJ, Kim DG, Mihalik J, Watkins PA#TITLE:Human very-long-chain-acyl-CoA synthetase#JOURNAL:Biochemical and biophysical research communications#YEAR:1999# AUTHORS:Mihalik SJ, Steinberg SJ, Pei Z, Park J, Kim DG, Heinzer AK, Dacremont G, Wanders JAR, Cuebas DA, Smith KD, Watkins PA#TITLE:Participation of two members of the veryl long-chain acyl-CoA synthetase family in bile acid synthesis and recycling#JOURNAL:Journal of Biological Chemistry#YEAR:2002# AUTHORS:Kelley M, Vessey DA#TITLE:Dual role of diavlent cations in the bile acid: CoA ligase catalyzed reaction#JOURNAL:Biochimica et Biophysica Acta#YEAR:1994# AUTHORS:Steinberg SJ, Wang SJ, McGuinness MC, Watkins PA.#TITLE:Human liver-specific very-long-chain acyl-coenzyme A synthetase: cDNA cloning and characterization of a second enzymatically active protein.#JOURNAL:Mol Genet Metab.#YEAR:1999#PUBMED:10479480# GENE ASSOCIATION: (11001.1)
Abbreviation: R_PEPCK Synonyms: _5 EC Number: 4.1.1.32 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : gtp + oaa --> co2 + gdp + pep Confidence Level: 0 genes: LOCUS:_5105#TRANSCRIPT:1#ABBREVIATION:PCK1#ECNUMBERS:4.1.1.32# proteins: NAME:phosphoenolpyruvate carboxykinase 1, soluble (liver, kidney, intestine)#ABBREVIATION:Pck1.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (5105.1)
Abbreviation: R_GLCNACPT_U Synonyms: _1 EC Number: 2.7.8.15 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dolp_U + uacgam --> (0.1) naglc2p_U + ump Confidence Level: 0 NCD genes: LOCUS:_1798#TRANSCRIPT:1#ABBREVIATION:DPAGT1#ECNUMBERS:2.7.8.15# LOCUS:_1798#TRANSCRIPT:2#ABBREVIATION:DPAGT1#ECNUMBERS:2.7.8.15# proteins: NAME:dolichyl-phosphate N-acetylglucosaminephosphotransferase 1 #ABBREVIATION:Dpagt1.1# NAME:dolichyl-phosphate N-acetylglucosaminephosphotransferase 1 #ABBREVIATION:Dpagt1.2# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (1798.1) or (1798.2)
UDP-GlcNAc:dolichol-phosphate GlcNAc phosphotransferase (uterus)
Abbreviation: R_URAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ura[e] <==> ura[c] Confidence Level: 4 IT genes: LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Yao SY, Ng AM, Vickers MF, Sundaram M, Cass CE, Baldwin SA, Young JD.#TITLE:Functional and molecular characterization of nucleobase transport by recombinant human and rat equilibrative nucleoside transporters 1 and 2.#JOURNAL:J Biol Chem. #YEAR:2002#PUBMED:12006583# GENE ASSOCIATION: (3177.1)
uracil transport via facilated diffusion
Abbreviation: R_AMPDA Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [c] : amp + h + h2o --> imp + nh4 Confidence Level: 4 genes: LOCUS:_272#TRANSCRIPT:1#ABBREVIATION:AMPD3#ECNUMBERS:3.5.4.6# LOCUS:_271#TRANSCRIPT:1#ABBREVIATION:AMPD2#ECNUMBERS:3.5.4.6# LOCUS:_270#TRANSCRIPT:1#ABBREVIATION:AMPD1#ECNUMBERS:3.5.4.6# LOCUS:_271#TRANSCRIPT:2#ABBREVIATION:AMPD2#ECNUMBERS:3.5.4.6# LOCUS:_271#TRANSCRIPT:3#ABBREVIATION:AMPD2#ECNUMBERS:3.5.4.6# proteins: NAME:adenosine monophosphate deaminase 1 (isoform M) #ABBREVIATION:AmpD1.1# NAME:adenosine monophosphate deaminase 2 (isoform L) #ABBREVIATION:AmpD2.1# NAME:adenosine monophosphate deaminase 2 (isoform L) #ABBREVIATION:AmpD2.2# NAME:adenosine monophosphate deaminase 2 (isoform L) #ABBREVIATION:AmpD2.3# NAME:adenosine monophosphate deaminase (isoform E) #ABBREVIATION:AmpD3.1# citations: AUTHORS:Mahnke-Zizelman DK, Sabina RL.#TITLE:Cloning of human AMP deaminase isoform E cDNAs. Evidence for a third AMPD gene exhibiting alternatively spliced 5'-exons. #JOURNAL:J Biol Chem. #YEAR:1992#PUBMED:1400401# AUTHORS:Sabina RL, Morisaki T, Clarke P, Eddy R, Shows TB, Morton CC, Holmes EW. #TITLE:Characterization of the human and rat myoadenylate deaminase genes. #JOURNAL:J Biol Chem.#YEAR:1990#PUBMED:2345176# GENE ASSOCIATION: (271.2) or (270.1) or (271.1) or (272.1) or (271.3)
Abbreviation: R_HSD3B7P Synonyms: _2 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : nadp + xol7a --> h + nadph + xol7aone Confidence Level: 4 genes: LOCUS:_80270#TRANSCRIPT:1#ABBREVIATION:HSD3B7# proteins: NAME:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7#ABBREVIATION:Hsd3b7.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (80270.1)
hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7
Abbreviation: R_KSII_CORE4t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ksii_core4[g] --> ksii_core4[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
keratan sulfate II (core4) transport, golgi to extracellular
Abbreviation: R_DNDPt55m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[m] + dctp[c] --> adp[c] + dctp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_FORtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: for[c] <==> for[r] Confidence Level: 1 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAOXC2452256x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : coa + h2o + nad + o2 + tetpent6coa --> accoa + dcsptn1coa + h + h2o2 + nadh Confidence Level: 4 peroxisomal (very long chain FA) - - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) c24:5,n-6 -> c22:5, n-6 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1) or (51.1 and 30.1 and 1962.1)
Abbreviation: R_PPMI12346Ptn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: ppmi12346p[c] <==> ppmi12346p[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
_5-diphosphatidylinositol pentakisphosphate nuclear transport (diffusion)
Abbreviation: R_ETF Synonyms: _0 SUBSYSTEM: Fatty acid oxidation Equation: [m] : etfox + fadh2 --> etfrd + fad Confidence Level: 4 TV (5/4/05) Voet p.571 genes: LOCUS:_2108#TRANSCRIPT:1#ABBREVIATION:ETFA# LOCUS:_2109#TRANSCRIPT:1#ABBREVIATION:ETFB# LOCUS:_2109#TRANSCRIPT:2#ABBREVIATION:ETFB# proteins: NAME:Electron-transfer-flavoprotein, beta polypeptide, mitochondrial #ABBREVIATION:Etfb.1-m# NAME:Electron-transfer-flavoprotein, alpha polypeptide, mitochondrial #ABBREVIATION:Etfa.1-m# NAME:electron-transfer-flavoprotein, beta polypeptide #ABBREVIATION:Etfb.2-m# citations: AUTHORS:Finocchiaro G, Ito M, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of cDNAs encoding the alpha-subunit of human electron transfer flavoprotein.#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3170610# AUTHORS:Thorpe C, Kim JJ.#TITLE:Structure and mechanism of action of the acyl-CoA dehydrogenases.#JOURNAL:FASEB J#YEAR:1995#PUBMED:7601336# GENE ASSOCIATION: (2109.2) and (2108.1) or (2109.1) and (2108.1)
Abbreviation: R_ACTLMO Synonyms: _0 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : acetol + h + nadph + o2 --> (2) h2o + mthgxl + nadp Confidence Level: 4 stoichiometry described in Thornalley PJ. Gen Pharmacol. 1996 Jun;27(4):565-73. PMID: 8853285 NCD genes: LOCUS:_1571#TRANSCRIPT:1#ABBREVIATION:CYP2E1# proteins: NAME:cytochrome P450, family 2, subfamily E, polypeptide 1#ABBREVIATION:Cyp2e1.1# citations: AUTHORS:Vander Jagt DL, Robinson B, Taylor KK, Hunsaker LA.#TITLE:Reduction of trioses by NADPH-dependent aldo-keto reductases. Aldose reductase, methylglyoxal, and diabetic complications#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1537826# AUTHORS:Thornalley PJ#TITLE:Pharmacology of methylglyoxal: formation, modification of proteins and nucleic acids, and enzymatic detoxification--a role in pathogenesis and antiproliferative chemotherapy.#JOURNAL:Gen Pharmacol#YEAR:1996#PUBMED:8853285# AUTHORS:Beisswenger BG, Delucia EM, Lapoint N, Sanford RJ, Beisswenger PJ.#TITLE:Ketosis leads to increased methylglyoxal production on the atkins diet#JOURNAL:Ann N Y Acad Sci#YEAR:2005#PUBMED:16037240# GENE ASSOCIATION: (1571.1)
Abbreviation: R_NTD8 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [c] : dgmp + h2o --> dgsn + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_30833#TRANSCRIPT:1#ABBREVIATION:NT5C#ECNUMBERS:3.1.3.5# LOCUS:_22978#TRANSCRIPT:1#ABBREVIATION:NT5C2# proteins: NAME:_5'-nucleotidase, cytosolic II #ABBREVIATION:Nt5C2.1# NAME:_5', 3'-nucleotidase, cytosolic #ABBREVIATION:Nt5C.1# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION: (22978.1) or (30833.1)
Abbreviation: R_EX_9_DASH_cis_DASH_retfa_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 9-cis-retfa <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_thym_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : thym <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RTOT6 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : R6coa_hs --> Rtotalcoa Confidence Level: 0 User defined, tissue specific fluxes that can be customized w/ various ratios of R1 through R6 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_3SPYRSP Synonyms: _0 SUBSYSTEM: Cysteine Metabolism Equation: [c] : 3snpyr + h2o --> h + pyr + so3 Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Weinstein CL, Griffith OW.#TITLE:Cysteinesulfonate and beta-sulfopyruvate metabolism. Partitioning between decarboxylation, transamination, and reduction pathways.#YEAR:1988#PUBMED:3346220# GENE ASSOCIATION:
_3-sulfinopyruvate hydrolase (spotaneous reaction)
Abbreviation: R_PRODt2rL Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: h[l] + pro-D[l] <==> h[c] + pro-D[c] Confidence Level: 4 NCD SAB D form, lyso genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
D-proline reversible transport via proton symport (lysosome)
Abbreviation: R_NADH2_DASH_u10m Synonyms: _0 EC Number: 1.6.99.3 SUBSYSTEM: Oxidative Phosphorylation Equation: (5) h[m] + nadh[m] + q10[m] --> (4) h[c] + nad[m] + q10h2[m] Confidence Level: 4 TV genes: LOCUS:_4694#TRANSCRIPT:1#ABBREVIATION:NDUFA1# LOCUS:_4712#TRANSCRIPT:1#ABBREVIATION:NDUFB6#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4702#TRANSCRIPT:1#ABBREVIATION:NDUFA8# LOCUS:_4728#TRANSCRIPT:1#ABBREVIATION:NDUFS8#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4538#TRANSCRIPT:1#ABBREVIATION:MTND4#ECNUMBERS:1.6.5.3# LOCUS:_4713#TRANSCRIPT:1#ABBREVIATION:NDUFB7#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4537#TRANSCRIPT:1#ABBREVIATION:MTND3# LOCUS:_4539#TRANSCRIPT:1#ABBREVIATION:MTND4L# LOCUS:_4697#TRANSCRIPT:1#ABBREVIATION:NDUFA4# LOCUS:_4729#TRANSCRIPT:1#ABBREVIATION:NDUFV2#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4710#TRANSCRIPT:1#ABBREVIATION:NDUFB4# LOCUS:_4696#TRANSCRIPT:1#ABBREVIATION:NDUFA3# LOCUS:_4700#TRANSCRIPT:1#ABBREVIATION:NDUFA6#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4726#TRANSCRIPT:1#ABBREVIATION:NDUFS6# LOCUS:_4711#TRANSCRIPT:1#ABBREVIATION:NDUFB5# LOCUS:_4716#TRANSCRIPT:1#ABBREVIATION:NDUFB10#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4698#TRANSCRIPT:1#ABBREVIATION:NDUFA5#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4717#TRANSCRIPT:1#ABBREVIATION:NDUFC1#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4540#TRANSCRIPT:1#ABBREVIATION:MTND5# LOCUS:_4701#TRANSCRIPT:1#ABBREVIATION:NDUFA7# LOCUS:_4714#TRANSCRIPT:1#ABBREVIATION:NDUFB8# LOCUS:_4704#TRANSCRIPT:1#ABBREVIATION:NDUFA9# LOCUS:_4715#TRANSCRIPT:1#ABBREVIATION:NDUFB9# LOCUS:_4723#TRANSCRIPT:1#ABBREVIATION:NDUFV1#ECNUMBERS:1.6.5.3# LOCUS:_4707#TRANSCRIPT:1#ABBREVIATION:NDUFB1# LOCUS:_4705#TRANSCRIPT:1#ABBREVIATION:NDUFA10#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4718#TRANSCRIPT:1#ABBREVIATION:NDUFC2# LOCUS:_4541#TRANSCRIPT:1#ABBREVIATION:MTND6# LOCUS:_4706#TRANSCRIPT:1#ABBREVIATION:NDUFAB1#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4708#TRANSCRIPT:1#ABBREVIATION:NDUFB2#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4719#TRANSCRIPT:1#ABBREVIATION:NDUFS1#ECNUMBERS:1.6.5.3# LOCUS:_4724#TRANSCRIPT:1#ABBREVIATION:NDUFS4#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4731#TRANSCRIPT:1#ABBREVIATION:NDUFV3#ECNUMBERS:1.6.5.3# LOCUS:_4709#TRANSCRIPT:1#ABBREVIATION:NDUFB3# LOCUS:_4720#TRANSCRIPT:1#ABBREVIATION:NDUFS2#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_4535#TRANSCRIPT:1#ABBREVIATION:MTND1# LOCUS:_4725#TRANSCRIPT:1#ABBREVIATION:NDUFS5#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_55967#TRANSCRIPT:1#ABBREVIATION:DAP13#ECNUMBERS:1.6.99.3# LOCUS:_4536#TRANSCRIPT:1#ABBREVIATION:MTND2# LOCUS:_4722#TRANSCRIPT:1#ABBREVIATION:NDUFS3# LOCUS:_4695#TRANSCRIPT:1#ABBREVIATION:NDUFA2#ECNUMBERS:1.6.5.3,1.6.99.3# LOCUS:_51079#TRANSCRIPT:1#ABBREVIATION:GRIM19# LOCUS:_126328#TRANSCRIPT:1#ABBREVIATION:NDUFA11# LOCUS:_374291#TRANSCRIPT:1#ABBREVIATION:NDUFS7# LOCUS:_4731#TRANSCRIPT:2#ABBREVIATION:NDUFV3#ECNUMBERS:1.6.5.3# LOCUS:_4712#TRANSCRIPT:2#ABBREVIATION:NDUFB6#ECNUMBERS:1.6.99.3# LOCUS:_7991#TRANSCRIPT:1#ABBREVIATION:TUSC3# LOCUS:_7991#TRANSCRIPT:2#ABBREVIATION:TUSC3# proteins: NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1#ABBREVIATION:Ndufa1.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4#ABBREVIATION:Ndufa4.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5#ABBREVIATION:Ndufa5.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8#ABBREVIATION:Ndufa8.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10#ABBREVIATION:Ndufa10.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2#ABBREVIATION:Ndufb2.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3#ABBREVIATION:Ndufb3.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4#ABBREVIATION:Ndufb4.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5#ABBREVIATION:Ndufb5.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7#ABBREVIATION:Ndufb7.1-m# NAME:NADH dehydrogenase (ubiquinone) Fe-S protein 1#ABBREVIATION:Ndufs1.1-m# NAME:NADH dehydrogenase (ubiquinone) Fe-S protein 2#ABBREVIATION:Ndufs2.1-m# NAME:NADH dehydrogenase (ubiquinone) Fe-S protein 4#ABBREVIATION:Ndufs4.1-m# NAME:NADH dehydrogenase subunit 1#ABBREVIATION:Nd1.1-m# NAME:NADH dehydrogenase subunit 2#ABBREVIATION:Nd2.1-m# NAME:NADH dehydrogenase subunit 3#ABBREVIATION:Nd3.1-m# NAME:NADH dehydrogenase subunit 4#ABBREVIATION:Nd4.1-m# NAME:NADH dehydrogenase subunit 4L#ABBREVIATION:Nd4l.1-m# NAME:NADH dehydrogenase subunit 5#ABBREVIATION:Nd5.1-m# NAME:NADH dehydrogenase subunit 6#ABBREVIATION:Nd6.1-m# NAME:NADH dehydrogenase (ubiquinone) flavoprotein 1#ABBREVIATION:Ndufv1.1-m# NAME:NADH dehydrogenase (ubiquinone) flavoprotein 2#ABBREVIATION:Ndufv2.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11#ABBREVIATION:Ndufa11.1-m# NAME:NADH dehydrogenase (ubiquinone) Fe-S protein 7#ABBREVIATION:Ndufs7.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2#ABBREVIATION:Ndufa2.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3#ABBREVIATION:Ndufa3.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6#ABBREVIATION:Ndufa6.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7#ABBREVIATION:Ndufa7.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9#ABBREVIATION:Ndufa9.1-m# NAME:NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1#ABBREVIATION:Ndufab1.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 1#ABBREVIATION:Ndufb1.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 8#ABBREVIATION:Ndufb8.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9#ABBREVIATION:Ndufb9.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 10#ABBREVIATION:Ndufb10.1-m# NAME:NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1#ABBREVIATION:Ndufc1.1-m# NAME:NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2#ABBREVIATION:Ndufc2.1-m# NAME:NADH dehydrogenase (ubiquinone) Fe-S protein 3#ABBREVIATION:Ndufs3.1-m# NAME:NADH dehydrogenase (ubiquinone) Fe-S protein 5#ABBREVIATION:Ndufs5.1-m# NAME:NADH dehydrogenase (ubiquinone) Fe-S protein 6#ABBREVIATION:Ndufs6.1-m# NAME:NADH dehydrogenase (ubiquinone) Fe-S protein 8#ABBREVIATION:Ndufs8.1-m# NAME:cell death-regulatory protein GRIM19#ABBREVIATION:Grim19.1-m# NAME:_13kDa differentiation-associated protein#ABBREVIATION:Dap13.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6#ABBREVIATION:Ndufb6.1-m# NAME:NADH-ubiquinone oxidoreductase flavoprotein 3 isoform a #ABBREVIATION:Ndufv3.1-m# NAME:NADH-ubiquinone oxidoreductase flavoprotein 3 isoform b#ABBREVIATION:Ndufv3.2-m# NAME:tumor suppressor candidate 3 isoform b #ABBREVIATION:Tusc3.2-m# NAME:tumor suppressor candidate 3 isoform a #ABBREVIATION:Tusc3.1-m# NAME:NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa #ABBREVIATION:Ndufb6.2-m# citations: none GENE ASSOCIATION: (4715.1) and (4720.1) and (4719.1) and (55967.1) and (4700.1) and (4709.1) and (4710.1) and (4726.1) and (4696.1) and (4718.1) and (4694.1) and (374291.1) and (4728.1) and (4717.1) and (4731.1) and (4705.1) and (4725.1) and (4716.1) and (4537.1) and (4722.1) and (7991.1) and (4541.1) and (4539.1) and (4712.1) and (4707.1) and (4701.1) and (4723.1) and (4698.1) and (4538.1) and (4697.1) and (51079.1) and (4714.1) and (4704.1) and (4711.1) and (126328.1) and (4724.1) and (4713.1) and (4536.1) and (4695.1) and (4702.1) and (4708.1) and (4706.1) and (4535.1) and (4540.1) and (4729.1) or (4715.1) and (4720.1) and (4719.1) and (4700.1) and (55967.1) and (4709.1) and (4710.1) and (4726.1) and (4718.1) and (4696.1) and (4694.1) and (374291.1) and (4728.1) and (4712.2) and (4717.1) and (4705.1) and (4725.1) and (4716.1) and (4537.1) and (7991.1) and (4722.1) and (4541.1) and (4539.1) and (4707.1) and (4701.1) and (4723.1) and (4698.1) and (4538.1) and (4697.1) and (51079.1) and (4714.1) and (4704.1) and (126328.1) and (4711.1) and (4724.1) and (4713.1) and (4536.1) and (4695.1) and (4702.1) and (4708.1) and (4706.1) and (4535.1) and (4731.2) and (4729.1) and (4540.1) or (4715.1) and (4720.1) and (4719.1) and (4700.1) and (55967.1) and (4709.1) and (4710.1) and (4726.1) and (4718.1) and (4696.1) and (4694.1) and (374291.1) and (4728.1) and (7991.2) and (4717.1) and (4705.1) and (4725.1) and (4537.1) and (4716.1) and (4722.1) and (4541.1) and (4712.1) and (4539.1) and (4707.1) and (4701.1) and (4723.1) and (4698.1) and (4538.1) and (4697.1) and (51079.1) and (4714.1) and (4704.1) and (4711.1) and (126328.1) and (4724.1) and (4713.1) and (4536.1) and (4695.1) and (4702.1) and (4708.1) and (4706.1) and (4731.2) and (4535.1) and (4540.1) and (4729.1) or (4715.1) and (4720.1) and (4719.1) and (55967.1) and (4700.1) and (4709.1) and (4710.1) and (4726.1) and (4696.1) and (4718.1) and (374291.1) and (4694.1) and (4728.1) and (4712.2) and (4717.1) and (4731.1) and (4705.1) and (4725.1) and (4537.1) and (4716.1) and (4722.1) and (7991.1) and (4541.1) and (4539.1) and (4707.1) and (4701.1) and (4723.1) and (4698.1) and (4538.1) and (4697.1) and (51079.1) and (4714.1) and (4704.1) and (126328.1) and (4711.1) and (4724.1) and (4713.1) and (4536.1) and (4695.1) and (4702.1) and (4708.1) and (4535.1) and (4706.1) and (4729.1) and (4540.1) or (4715.1) and (4720.1) and (4719.1) and (4700.1) and (55967.1) and (4709.1) and (4710.1) and (4726.1) and (4696.1) and (4718.1) and (4694.1) and (374291.1) and (4728.1) and (7991.2) and (4717.1) and (4731.1) and (4705.1) and (4725.1) and (4537.1) and (4716.1) and (4722.1) and (4541.1) and (4539.1) and (4712.1) and (4707.1) and (4701.1) and (4723.1) and (4698.1) and (4538.1) and (4697.1) and (51079.1) and (4714.1) and (4704.1) and (126328.1) and (4711.1) and (4724.1) and (4713.1) and (4536.1) and (4695.1) and (4702.1) and (4708.1) and (4706.1) and (4535.1) and (4540.1) and (4729.1) or (4715.1) and (4720.1) and (4719.1) and (4700.1) and (55967.1) and (4709.1) and (4710.1) and (4726.1) and (4718.1) and (4696.1) and (374291.1) and (4694.1) and (4728.1) and (7991.2) and (4712.2) and (4717.1) and (4705.1) and (4725.1) and (4716.1) and (4537.1) and (4722.1) and (4541.1) and (4539.1) and (4707.1) and (4701.1) and (4698.1) and (4723.1) and (4538.1) and (4697.1) and (51079.1) and (4714.1) and (4704.1) and (4711.1) and (126328.1) and (4724.1) and (4536.1) and (4713.1) and (4695.1) and (4702.1) and (4708.1) and (4706.1) and (4731.2) and (4535.1) and (4540.1) and (4729.1) or (4715.1) and (4720.1) and (4719.1) and (4700.1) and (55967.1) and (4709.1) and (4710.1) and (4726.1) and (4718.1) and (4696.1) and (374291.1) and (4694.1) and (4728.1) and (4717.1) and (4705.1) and (4725.1) and (4537.1) and (4716.1) and (4722.1) and (7991.1) and (4541.1) and (4712.1) and (4539.1) and (4707.1) and (4701.1) and (4723.1) and (4698.1) and (4538.1) and (4697.1) and (51079.1) and (4714.1) and (4704.1) and (4711.1) and (126328.1) and (4724.1) and (4713.1) and (4536.1) and (4695.1) and (4702.1) and (4708.1) and (4706.1) and (4535.1) and (4731.2) and (4540.1) and (4729.1) or (4715.1) and (4720.1) and (4719.1) and (4700.1) and (55967.1) and (4709.1) and (4710.1) and (4726.1) and (4718.1) and (4696.1) and (374291.1) and (4694.1) and (4728.1) and (7991.2) and (4712.2) and (4717.1) and (4731.1) and (4705.1) and (4725.1) and (4537.1) and (4716.1) and (4722.1) and (4541.1) and (4539.1) and (4707.1) and (4701.1) and (4698.1) and (4723.1) and (4538.1) and (4697.1) and (51079.1) and (4714.1) and (4704.1) and (4711.1) and (126328.1) and (4724.1) and (4713.1) and (4536.1) and (4695.1) and (4702.1) and (4708.1) and (4706.1) and (4535.1) and (4540.1) and (4729.1)
Abbreviation: R_DNDPt8m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[c] + dudp[m] --> dadp[m] + dudp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_CYSGLYexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[c] + gly[e] <==> cys-L[e] + gly[c] Confidence Level: 2 SAB genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_H6MTer_L Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_L + m2emgacpail_hs --> (0.1) dolp_L + h + m3emgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_80235#TRANSCRIPT:1#ABBREVIATION:SMP3# proteins: NAME:SMP3 mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Smp3.1-r# citations: AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (80235.1)
Abbreviation: R_CRNtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: dmnoncrn[x] <==> dmnoncrn[m] Confidence Level: 2 mechanism not currently elucidated NJ genes: none proteins: none citations: AUTHORS:Mukherji M, Schofield CJ, Wierzbicki AS, Jansen GA, Wanders RJ, Lloyd MD.#TITLE:The chemical biology of branched-chain lipid metabolism.#JOURNAL:Prog Lipid Res.#YEAR:2003#PUBMED:12814641# AUTHORS:Wanders RJ.#TITLE:Peroxisomes, lipid metabolism, and peroxisomal disorders.#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15464416# GENE ASSOCIATION:
carnitine transport peroxisome to mitochondria
keratan sulfate I, degradation product 25_C118H192N8O98S4
Abbreviation: R_CYTK6n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : cmp + ctp <==> (2) cdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_DCSPTN1CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: dcsptn1crn[c] --> dcsptn1crn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_ACCOACm Synonyms: _0 EC Number: 6.4.1.2 SUBSYSTEM: Fatty Acid Metabolism Equation: [m] : accoa + atp + hco3 --> adp + h + malcoa + pi Confidence Level: 4 TDV (9/14/2004) genes: LOCUS:_32#TRANSCRIPT:1#ABBREVIATION:ACACB# proteins: NAME:acetyl-Coenzyme A carboxylase beta#ABBREVIATION:Acacb.1-m# citations: AUTHORS:Abu-Elheiga L, Brinkley WR, Zhong L, Chirala SS, Woldegiorgis G, Wakil SJ.#TITLE:The subcellular localization of acetyl-CoA carboxylase 2.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2000#PUBMED:10677481# GENE ASSOCIATION: (32.1)
Abbreviation: R_SBPP1er Synonyms: _0 SUBSYSTEM: Sphingolipid Metabolism Equation: [r] : h2o + sph1p --> pi + sphgn Confidence Level: 4 NCD genes: LOCUS:_81537#TRANSCRIPT:1#ABBREVIATION:SGPP1# proteins: NAME:sphingosine-1-phosphate phosphatase 1 #ABBREVIATION:Sgpp1.1# citations: AUTHORS:Johnson KR, Johnson KY, Becker KP, Bielawski J, Mao C, Obeid LM.#TITLE:Role of human sphingosine-1-phosphate phosphatase 1 in the regulation of intra- and extracellular sphingosine-1-phosphate levels and cell viability.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12815058# GENE ASSOCIATION: (81537.1)
Abbreviation: R_GTHPm Synonyms: _0 EC Number: 1.11.1.9 SUBSYSTEM: Glutathione Metabolism Equation: [m] : (2) gthrd + h2o2 <==> gthox + (2) h2o Confidence Level: 4 TV (8-15-2003) genes: LOCUS:_2879#TRANSCRIPT:1#ABBREVIATION:GPX4# LOCUS:_10935#TRANSCRIPT:1#ABBREVIATION:PRDX3# LOCUS:_10935#TRANSCRIPT:2#ABBREVIATION:PRDX3# proteins: NAME:Glutathione peroxidase 4 (phospholipid hydroperoxidase), mitochondrial&cytoplasmic#ABBREVIATION:Gpx4.1-m# NAME:Peroxiredoxin3#ABBREVIATION:Prdx3.1# NAME:Peroxiredoxin 3#ABBREVIATION:Prdx3.2# citations: AUTHORS:Borchert A, Savaskan NE, Kuhn H.#TITLE:Regulation of expression of the phospholipid hydroperoxide/sperm nucleus glutathione peroxidase gene. Tissue-specific expression pattern and identification of functional cis- and trans-regulatory elements.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:12427732# AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# GENE ASSOCIATION: (10935.1) or (2879.1) or (10935.2)
Abbreviation: R_GALtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: gal[l] --> gal[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Pisoni RL, Thoene JG#TITLE:The transport systems of mammalian lysosomes#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1751541# AUTHORS:Lloyd JB#TITLE:Metabolite efflux and influx across the lysosome membrane#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993166# GENE ASSOCIATION:
Abbreviation: R_F1PGT Synonyms: _5 EC Number: 2.7.7.30 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : fuc1p-L + gtp + h <==> gdpfuc + ppi Confidence Level: 0 NCD genes: LOCUS:_8790#TRANSCRIPT:1#ABBREVIATION:FPGT#ECNUMBERS:2.7.7.30# proteins: NAME:fucose-1-phosphate guanylyltransferase#ABBREVIATION:Fpgt.1# citations: none GENE ASSOCIATION: (8790.1)
Abbreviation: R_ACGALFUCGALACGALFUCGALACGLCGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acgalfucgalacgalfucgalacglcgal14acglcgalgluside_hs[e] <==> acgalfucgalacgalfucgalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CYOR_DASH_u10m Synonyms: _0 EC Number: 1.10.2.2 SUBSYSTEM: Oxidative Phosphorylation Equation: (2) ficytC[m] + (2) h[m] + q10h2[m] --> (2) focytC[m] + (4) h[c] + q10[m] Confidence Level: 4 TV genes: LOCUS:_7381#TRANSCRIPT:1#ABBREVIATION:UQCRB# LOCUS:_7385#TRANSCRIPT:1#ABBREVIATION:UQCRC2#ECNUMBERS:1.10.2.2# LOCUS:_7384#TRANSCRIPT:1#ABBREVIATION:UQCRC1#ECNUMBERS:1.10.2.2# LOCUS:_4519#TRANSCRIPT:1#ABBREVIATION:MTCYB# LOCUS:_7386#TRANSCRIPT:1#ABBREVIATION:UQCRFS1# LOCUS:_10975#TRANSCRIPT:1#ABBREVIATION:UQCR#ECNUMBERS:1.10.2.2# LOCUS:_7388#TRANSCRIPT:1#ABBREVIATION:UQCRH#ECNUMBERS:1.10.2.2# LOCUS:_1537#TRANSCRIPT:1#ABBREVIATION:CYC1# LOCUS:_27089#TRANSCRIPT:1#ABBREVIATION:QP-C#ECNUMBERS:1.10.2.2# LOCUS:_29796#TRANSCRIPT:1#ABBREVIATION:HSPC051# LOCUS:_29796#TRANSCRIPT:2#ABBREVIATION:HSPC051#ECNUMBERS:1.10.2.2# proteins: NAME:low molecular mass ubiquinone-binding protein #ABBREVIATION:Qp-c.1-m# NAME:ubiquinol-cytochrome c reductase binding protein#ABBREVIATION:Uqcrb.1-m# NAME:cytochrome b#ABBREVIATION:Cytb.1-m# NAME:ubiquinol-cytochrome c reductase core protein I#ABBREVIATION:Uqcrc1.1-m# NAME:ubiquinol-cytochrome c reductase core protein II #ABBREVIATION:Uqcrc2.1-m# NAME:ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1#ABBREVIATION:Uqcrfs1.1-m# NAME:ubiquinol-cytochrome c reductase hinge protein#ABBREVIATION:Uqcrh.1-m# NAME:ubiquinol-cytochrome c reductase (6.4kD) subunit #ABBREVIATION:Uqcr.1-m# NAME:cytochrome c-1#ABBREVIATION:Cyc1.1-m# NAME:ubiquinol-cytochrome c reductase complex protein#ABBREVIATION:Hspc051.2-m# NAME:ubiquinol-cytochrome c reductase complex protein#ABBREVIATION:Hspc051.1-m# citations: none GENE ASSOCIATION: (7384.1) and (7388.1) and (4519.1) and (10975.1) and (29796.2) and (7385.1) and (1537.1) and (7386.1) and (27089.1) and (7381.1) or (7384.1) and (7388.1) and (29796.1) and (10975.1) and (4519.1) and (7385.1) and (7386.1) and (1537.1) and (27089.1) and (7381.1)
ubiquinol-6 cytochrome c reductase, Complex III
Abbreviation: R_EX_lnlnca_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lnlnca <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GTPCI Synonyms: _0 EC Number: 3.5.4.16 SUBSYSTEM: Tetrahydrobiopterin Equation: [c] : gtp + h2o --> ahdt + for + h Confidence Level: 4 The reaction involves hydrolysis of two C-N bonds and isomerization of the pentose unit; the recyclization may be non-enzymic genes: LOCUS:_2643#TRANSCRIPT:1#ABBREVIATION:GCH1#ECNUMBERS:3.5.4.16# proteins: NAME:GTP cyclohydrolase 1 (dopa-responsive dystonia) #ABBREVIATION:Gch1.1# citations: AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (2643.1)
Abbreviation: R_WHTSTSTERONEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: whtststerone[c] <==> whtststerone[e] Confidence Level: 1 details of metabolism, etc are unknown - allwoed to be exported presently - unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PYK Synonyms: _7 EC Number: 2.7.1.40 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : adp + h + pep --> atp + pyr Confidence Level: 0 SMP genes: LOCUS:_5315#TRANSCRIPT:1#ABBREVIATION:PKM2#ECNUMBERS:2.7.1.40# LOCUS:_5313#TRANSCRIPT:1#ABBREVIATION:PKLR#ECNUMBERS:2.7.1.40# LOCUS:_113452#TRANSCRIPT:1#ABBREVIATION:BCLP# LOCUS:_5313#TRANSCRIPT:2#ABBREVIATION:PKLR#ECNUMBERS:2.7.1.40# LOCUS:_5315#TRANSCRIPT:2#ABBREVIATION:PKM2#ECNUMBERS:2.7.1.40# LOCUS:_5315#TRANSCRIPT:3#ABBREVIATION:PKM2#ECNUMBERS:2.7.1.40# proteins: NAME:liver and RBS pyruvate kinase (liver, RBC)#ABBREVIATION:Pklr.1# NAME:liver and RBC pyruvate kinase (liver, RBC)#ABBREVIATION:Pklr.2# NAME:muscle pyruvate kinase (fetal)#ABBREVIATION:Pkm2.1# NAME:muscle pyruvate kinase (fetal)#ABBREVIATION:Pkm2.2# NAME:muscle pyruvate kinase (fetal)#ABBREVIATION:Pkm2.3# NAME:beta-casein-like protein#ABBREVIATION:Bclp.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (113452.1) or (5315.2) or (5313.1) or (5315.1) or (5313.2) or (5315.3)
Abbreviation: R_PROt4_LPAREN_2_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) na1[e] + pro-L[e] <==> (2) na1[c] + pro-L[c] Confidence Level: 4 SAB genes: LOCUS:_6534#TRANSCRIPT:1#ABBREVIATION:SLC6A7# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, L-proline), member 7#ABBREVIATION:Slc6a7.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (6534.1)
Proline transport (sodium symport) (2:1)
Abbreviation: R_EX_rib_DASH_D_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : rib-D <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HEXCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hexc[e] <==> hexc[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_NTD7e Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [e] : amp + h2o --> adn + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_4907#TRANSCRIPT:1#ABBREVIATION:NT5E#ECNUMBERS:3.1.3.5# proteins: NAME:_5'-nucleotidase, ecto (CD73) #ABBREVIATION:Nt5E.1-e# citations: AUTHORS:Hashikawa T, Takedachi M, Terakura M, Saho T, Yamada S, Thompson LF, Shimabukuro Y, Murakami S.#TITLE:Involvement of CD73 (ecto-5'-nucleotidase) in adenosine generation by human gingival fibroblasts.#JOURNAL:J Dent Res.#YEAR:2003#PUBMED:14578500# GENE ASSOCIATION: (4907.1)
Abbreviation: R_MTHGXLt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: mthgxl[c] --> mthgxl[e] Confidence Level: 2 MM genes: none proteins: none citations: none GENE ASSOCIATION:
Methylglyoxal transport (cytosol to extracellular)
Abbreviation: R_EX_fucfuc12gal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucfuc12gal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GBA Synonyms: _5 EC Number: 3.2.1.45 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : gluside_hs + h2o --> crm_hs + glc-D Confidence Level: 0 NJ genes: LOCUS:_2629#TRANSCRIPT:1#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# LOCUS:_2629#TRANSCRIPT:5#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# LOCUS:_2629#TRANSCRIPT:3#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# LOCUS:_2629#TRANSCRIPT:4#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# LOCUS:_2629#TRANSCRIPT:2#ABBREVIATION:GBA#ECNUMBERS:3.2.1.21# proteins: NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.1# NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.2# NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.3# NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.4# NAME:glucosidase, beta; acid (includes glucosylceramidase)#ABBREVIATION:Gba.5# citations: AUTHORS:Schmitz M, Alfalah M, Aerts JM, Naim HY, Zimmer KP.#TITLE:Impaired trafficking of mutants of lysosomal glucocerebrosidase in Gaucher's disease.#JOURNAL:Int J Biochem Cell Biol#YEAR:2005#PUBMED:15982918# GENE ASSOCIATION: (2629.2) or (2629.3) or (2629.4) or (2629.5) or (2629.1)
keratan sulfate I biosynthesis, precursor 13_C143H231N10O111S3X
Abbreviation: R_AG13T8g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre12 + uacgam --> h + ksi_pre13 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
Abbreviation: R_EX_chol_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : chol <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_apnnox_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : apnnox <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CO2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: co2[e] <==> co2[c] Confidence Level: 1 tv renamed from CO2TPr to CO2t NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DALAt2rL Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: ala-D[l] + h[l] <==> ala-D[c] + h[c] Confidence Level: 4 JLR SAB lyso genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
D-alanine transport via proton symport (lysosome)
Abbreviation: R_EX_gdchola_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gdchola <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6TASE11ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg6 <==> h + ksi_deg7 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_P450LTB4r Synonyms: _1 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : leuktrB4woh + nadph + (1.5) o2 --> (2) h2o + leuktrB4wcooh + nadp Confidence Level: 2 further oxidation of omega hydroxy LTB4 (leukotriene B4) stoichiometry inferrered - explicity primary literature refs not found NJ genes: none proteins: none citations: AUTHORS:Leff AR.#TITLE:Regulation of leukotrienes in the management of asthma: biology and clinical therapy.#JOURNAL:Annu Rev Med#YEAR:2001#PUBMED:11160764# AUTHORS:Hammarstrom S, Orning L, Bernstrom K.#TITLE:Metabolism of leukotrienes.#JOURNAL:Mol Cell Biochem.#YEAR:1985#PUBMED:3001504# AUTHORS:Soberman RJ.#TITLE:Cytochrome P-450LTB and inactivation of leukotriene B4.#JOURNAL:Methods Enzymol#YEAR:1990#PUBMED:2172735# GENE ASSOCIATION:
Abbreviation: R_NMNATn Synonyms: _0 EC Number: 2.7.7.1 SUBSYSTEM: NAD Metabolism Equation: [n] : atp + h + nmn --> nad + ppi Confidence Level: 4 IT genes: LOCUS:_64802#TRANSCRIPT:1#ABBREVIATION:NMNAT1#ECNUMBERS:2.7.7.1# proteins: NAME:Nicotinamide nucleotide adenylyltransferase 1 #ABBREVIATION:Nmnat1.1# citations: AUTHORS:Zhang X, Kurnasov OV, Karthikeyan S, Grishin NV, Osterman AL, Zhang H. #TITLE:Structural characterization of a human cytosolic NMN/NaMN adenylyltransferase and implication in human NAD biosynthesis.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:12574164# AUTHORS: Emanuelli M, Carnevali F, Saccucci F, Pierella F, Amici A, Raffaelli N, Magni G. #TITLE:Molecular cloning, chromosomal localization, tissue mRNA levels, bacterial expression, and enzymatic properties of human NMN adenylyltransferase.#JOURNAL:J Biol Chem.#YEAR:2001#PUBMED:11027696# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Schweiger M, Hennig K, Lerner F, Niere M, Hirsch-Kauffmann M, Specht T, Weise C, Oei SL, Ziegler M.#TITLE:Characterization of recombinant human nicotinamide mononucleotide adenylyl transferase (NMNAT), a nuclear enzyme essential for NAD synthesis.#JOURNAL:FEBS Lett. #YEAR:2001#PUBMED:11248244# GENE ASSOCIATION: (64802.1)
Abbreviation: R_DOLGLCP_Lter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: (0.1) dolglcp_L[c] --> (0.1) dolglcp_L[r] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION:
Dolichyl beta-D-glucosyl phosphate flippase (liver)
Abbreviation: R_AMACR2r Synonyms: _0 EC Number: 5.1.99.4 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : dhcholestancoa + (0.5) o2 --> dhcholoylcoa + h2o Confidence Level: 2 should be analogous to AMACRr as per KEGG - in this case: reductase not a racemase NJ genes: LOCUS:_23600#TRANSCRIPT:1#ABBREVIATION:AMACR# LOCUS:_23600#TRANSCRIPT:2#ABBREVIATION:AMACR# proteins: NAME:alpha-methylacyl-CoA racemase#ABBREVIATION:Amacr.1# NAME:alpha-methylacyl-CoA racemase#ABBREVIATION:Amacr.2# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (23600.1) or (23600.2)
H2 mannosyltransferase, endoplasmic reticulum
Abbreviation: R_H2MTer_U Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_U + mgacpail_hs --> (0.1) dolp_U + h + m2gacpail_hs Confidence Level: 4 NCD genes: LOCUS:_55650#TRANSCRIPT:1#ABBREVIATION:PIGV# proteins: NAME:phosphatidylinositol glycan, class V, endoplasmic reticulum#ABBREVIATION:Pigv.1-r# citations: AUTHORS:Kang JY, Hong Y, Ashida H, Shishioh N, Murakami Y, Morita YS, Maeda Y, Kinoshita T#TITLE:IG-V involved in transferring the second mannose in glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15623507# GENE ASSOCIATION: (55650.1)
heparan sulfate, degradation product 21_C37H55NO38S2
Abbreviation: R_S2TASE2ly Synonyms: _12 EC Number: 3.1.6.13 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg21 --> h + hs_deg22 + so4 Confidence Level: 4 NCD genes: LOCUS:_3423#TRANSCRIPT:1#ABBREVIATION:IDS#ECNUMBERS:3.1.6.13# LOCUS:_3423#TRANSCRIPT:2#ABBREVIATION:IDS#ECNUMBERS:3.1.6.13# proteins: NAME:iduronate 2-sulfatase (Hunter syndrome), lysosome#ABBREVIATION:Ids.1-l# NAME:iduronate 2-sulfatase (Hunter syndrome), lysosome#ABBREVIATION:Ids.2-l# citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Wilson PJ, Meaney CA, Hopwood JJ, Morris CP#TITLE:Sequence of the human iduronate 2-sulfatase (IDS) gene#JOURNAL:Genomics#YEAR:1993#PUBMED:8244397# AUTHORS:Malmgren H, Carlberg BM, Pettersson U, Bondeson ML#TITLE:dentification of an alternative transcript from the human iduronate-2-sulfatase (IDS) gene#JOURNAL:Genomics#YEAR:1995#PUBMED:8530090# AUTHORS:Wilson PJ, Morris CP, Anson DS, Occhiodoro T, Bielicki J, Clements PR, Hopwood JJ#TITLE:Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990#PUBMED:2122463# GENE ASSOCIATION: (3423.1) or (3423.2)
Abbreviation: R_LALDO2 Synonyms: _1 EC Number: 1.1.1.21 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : h + mthgxl + nadph --> lald-D + nadp Confidence Level: 4 NCD genes: LOCUS:_10327#TRANSCRIPT:1#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# LOCUS:_231#TRANSCRIPT:1#ABBREVIATION:AKR1B1#ECNUMBERS:1.1.1.21# LOCUS:_8574#TRANSCRIPT:1#ABBREVIATION:AKR7A2# LOCUS:_10327#TRANSCRIPT:2#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# proteins: NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.1# NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.2# NAME:aldo-keto reductase family 1, member B1 (aldose reductase)#ABBREVIATION:Akr1b1.1# NAME:aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)#ABBREVIATION:Akr7a2.1# citations: AUTHORS:Bohren KM, Bullock B, Wermuth B, Gabbay KH#TITLE:The aldo-keto reductase superfamily. cDNAs and deduced amino acid sequences of human aldehyde and aldose reductases#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2498333# AUTHORS:Vander Jagt DL, Robinson B, Taylor KK, Hunsaker LA.#TITLE:Reduction of trioses by NADPH-dependent aldo-keto reductases. Aldose reductase, methylglyoxal, and diabetic complications#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1537826# AUTHORS:Kelly VP, Sherratt PJ, Crouch DH, Hayes JD#TITLE:Novel homodimeric and heterodimeric rat gamma-hydroxybutyrate synthases that associate with the Golgi apparatus define a distinct subclass of aldo-keto reductase 7 family proteins.#JOURNAL:Biochem J#YEAR:2002#PUBMED:12071861# AUTHORS:Praml C, Savelyeva L, Perri P, Schwab M.#TITLE:Cloning of the human aflatoxin B1-aldehyde reductase gene at 1p35-1p36.1 in a region frequently altered in human tumor cells.#JOURNAL:Cancer Res#YEAR:1998#PUBMED:9823300# GENE ASSOCIATION: (10327.1) or (8574.1) or (10327.2) or (231.1)
Abbreviation: R_LTA4H Synonyms: _2 EC Number: 3.3.2.6 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : h2o + leuktrA4 --> leuktrB4 Confidence Level: 4 NJ genes: LOCUS:_4048#TRANSCRIPT:1#ABBREVIATION:LTA4H#ECNUMBERS:3.3.2.6# proteins: NAME:leukotriene A4 hydrolase#ABBREVIATION:Lta4h.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# GENE ASSOCIATION: (4048.1)
Abbreviation: R_FAOXC140 Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (6) coa + (6) fad + (6) h2o + (6) nad + tdcoa --> (7) accoa + (6) fadh2 + (6) h + (6) nadh Confidence Level: 4 TV Other enzymes: 1.3.3.6, 4.2.1.74, 4.2.1.17, 1.1.1.35, 1.1.1.211 genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_ST6GALNAC23 Synonyms: _5 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + ga2_hs --> cmp + gm2a_hs + h Confidence Level: 2 NJ genes: LOCUS:_10610#TRANSCRIPT:1#ABBREVIATION:SIAT7B#ECNUMBERS:2.4.99.7# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 2#ABBREVIATION:St6galnac2.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10610.1)
Abbreviation: R_RDH1 Synonyms: _0 EC Number: 1.1.1.105 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : nad + retinol <==> h + nadh + retinal Confidence Level: 4 IT genes: LOCUS:_5959#TRANSCRIPT:1#ABBREVIATION:RDH5#ECNUMBERS:1.1.1.105# LOCUS:_8608#TRANSCRIPT:1#ABBREVIATION:RDH16# proteins: NAME:retinol dehydrogenase 5 (11-cis and 9-cis) #ABBREVIATION:Rdh5.1# NAME:retinol dehydrogenase 16 (all-trans and 13-cis) #ABBREVIATION:Rdh16.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Haeseleer F, Jang GF, Imanishi Y, Driessen CA, Matsumura M, Nelson PS, Palczewski K.#TITLE:Dual-substrate specificity short chain retinol dehydrogenases from the vertebrate retina.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12226107# AUTHORS:Lapshina EA, Belyaeva OV, Chumakova OV, Kedishvili NY.#TITLE:Differential recognition of the free versus bound retinol by human microsomal retinol/sterol dehydrogenases: characterization of the holo-CRBP dehydrogenase activity of RoDH-4.#JOURNAL:Biochemistry#YEAR:2003#PUBMED:12534290# AUTHORS:Jurukovski V, Markova NG, Karaman-Jurukovska N, Randolph RK, Su J, Napoli JL, Simon M.#TITLE:Cloning and characterization of retinol dehydrogenase transcripts expressed in human epidermal keratinocytes.#JOURNAL:Mol Genet Metab.#YEAR:1999#PUBMED:10329026# GENE ASSOCIATION: (8608.1) or (5959.1)
Abbreviation: R_PROtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: pro-L[c] <==> pro-L[m] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_BETALDHxm Synonyms: _3 EC Number: 1.2.1.8 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : betald + h2o + nad --> glyb + (2) h + nadh Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Craig SA.#TITLE:Betaine in human nutrition.#YEAR:2004#PUBMED:15321791# GENE ASSOCIATION:
betaine-aldehyde dehydrogenase, mitochondrial
Abbreviation: R_BAAT3x Synonyms: _1 EC Number: 2.3.1.65 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : dgcholcoa + gly --> coa + dgchol Confidence Level: 2 third version analog added as per KEGG NJ genes: LOCUS:_570#TRANSCRIPT:1#ABBREVIATION:BAAT#ECNUMBERS:2.3.1.65# proteins: NAME:bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase)#ABBREVIATION:Baat.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Falany CN, Johnson MR, Barnes S, Diasio RB#TITLE:Glycine and taurine conugation of bile acids by a single enzyme#JOURNAL:Journal of Biological Chemistry#YEAR:1994# GENE ASSOCIATION: (570.1)
Abbreviation: R_TETTET6CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + tettet6crn --> crn + tettet6coa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_ARTFR209 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: adrncoa[c] + (2) fadh2[m] + (2) h[m] + (2) nadph[m] --> (1.375) R2coa_hs[c] + (2) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
L-sulfolactate dehydrogenase (NAD+), mitochondrial
Abbreviation: R_SLDxm Synonyms: _3 EC Number: 1.1.1.272 SUBSYSTEM: Cysteine Metabolism Equation: [m] : nad + sl-L <==> 3spyr + h + nadh Confidence Level: 2 AMF EC: 1.1.1.272 genes: LOCUS:_4191#TRANSCRIPT:1#ABBREVIATION:MDH2#ECNUMBERS:1.1.1.37# proteins: NAME:malate dehydrogenase 2 (NAD), mitochondrial#ABBREVIATION:Mdh2.1-m# citations: AUTHORS:Weinstein CL, Griffith OW.#TITLE:Cysteinesulfonate and beta-sulfopyruvate metabolism. Partitioning between decarboxylation, transamination, and reduction pathways.#YEAR:1988#PUBMED:3346220# AUTHORS:Rein U, Gueta R, Denger K, Ruff J, Hollemeyer K, Cook AM.#TITLE:Dissimilation of cysteate via 3-sulfolactate sulfo-lyase and a sulfate exporter in Paracoccus pantotrophus NKNCYSA.#YEAR:2005#PUBMED:15758220# GENE ASSOCIATION: (4191.1)
Abbreviation: R_NACHEXA17ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg30 --> acgam + h + ksi_deg32 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_DALAOXx Synonyms: _0 EC Number: 1.4.3.3 SUBSYSTEM: D-alanine metabolism Equation: [x] : ala-D + h2o + o2 --> h2o2 + nh4 + pyr Confidence Level: 4 SAB genes: LOCUS:_1610#TRANSCRIPT:1#ABBREVIATION:DAO#ECNUMBERS:1.4.3.3# proteins: NAME:D-amino-acid oxidase#ABBREVIATION:Dao.1-x# citations: AUTHORS:Vanoni MA, Cosma A, Mazzeo D, Mattevi A, Todone F, Curti B#TITLE:Limited proteolysis and X-ray crystallography reveal the origin of substrate specificity and of the rate-limiting product release during oxidation of D-amino acids catalyzed by mammalian D-amino acid oxidase#JOURNAL:Biochemistry#YEAR:1997#PUBMED:9153402# GENE ASSOCIATION: (1610.1)
Abbreviation: R_FACOAL1831 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + lnlncg <==> amp + lnlncgcoa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_ARSA Synonyms: _2 EC Number: 3.1.6.8 SUBSYSTEM: Sphingolipid Metabolism Equation: [l] : h2o + sgalside_hs --> galside_hs + h + so4 Confidence Level: 4 NJ genes: LOCUS:_410#TRANSCRIPT:1#ABBREVIATION:ARSA#ECNUMBERS:3.1.6.8# proteins: NAME:arylsulfatase A#ABBREVIATION:Arsa.1# citations: AUTHORS:Stein C, Gieselmann V, Kreysing J, Schmidt B, Pohlmann R, Waheed A, Meyer HE, O'Brien JS, von Figura K.#TITLE:Cloning and expression of human arylsulfatase A.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2562955# GENE ASSOCIATION: (410.1)
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Abbreviation: R_5ADTSTSTERONESte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _5adtststerones[c] <==> 5adtststerones[e] Confidence Level: 1 unknown mechanism - presumed excretion of sulfonated testosterone NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_lpchol_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lpchol_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
lysophosphatidylcholine (homo sapiens) exchange
Abbreviation: R_NTPP11 Synonyms: _0 EC Number: 3.6.1.19 SUBSYSTEM: Nucleotides Equation: [c] : h2o + xtp --> h + ppi + xmp Confidence Level: 4 JLR; IT genes: LOCUS:_3704#TRANSCRIPT:1#ABBREVIATION:ITPA#ECNUMBERS:3.6.1.19# LOCUS:_3704#TRANSCRIPT:2#ABBREVIATION:ITPA#ECNUMBERS:3.6.1.19# proteins: NAME:inosine triphosphatase (nucleoside triphosphate pyrophosphatase) #ABBREVIATION:ItpA.1# NAME:inosine triphosphatase (nucleoside triphosphate pyrophosphatase) #ABBREVIATION:ItpA.2# citations: AUTHORS:Lin S, McLennan AG, Ying K, Wang Z, Gu S, Jin H, Wu C, Liu W, Yuan Y, Tang R, Xie Y, Mao Y. #TITLE:Cloning, expression, and characterization of a human inosine triphosphate pyrophosphatase encoded by the itpa gene. #JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11278832# GENE ASSOCIATION: (3704.1) or (3704.2)
Nucleoside triphosphate pyrophosphorylase (xtp)
Abbreviation: R_GLYBtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: glyb[m] <==> glyb[c] Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Porter RK, Scott JM, Brand MD.#TITLE:Characterization of betaine efflux from rat liver mitochondria.#YEAR:1993#PUBMED:8443213# GENE ASSOCIATION:
Glycine betaine transport via diffusion (mitochondria to cytosol)
Abbreviation: R_ALR2 Synonyms: _0 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : h + mthgxl + nadph --> acetol + nadp Confidence Level: 4 NCD genes: LOCUS:_10327#TRANSCRIPT:1#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# LOCUS:_231#TRANSCRIPT:1#ABBREVIATION:AKR1B1#ECNUMBERS:1.1.1.21# LOCUS:_8574#TRANSCRIPT:1#ABBREVIATION:AKR7A2# LOCUS:_10327#TRANSCRIPT:2#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# proteins: NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.1# NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.2# NAME:aldo-keto reductase family 1, member B1 (aldose reductase)#ABBREVIATION:Akr1b1.1# NAME:aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)#ABBREVIATION:Akr7a2.1# citations: AUTHORS:Vander Jagt DL, Robinson B, Taylor KK, Hunsaker LA.#TITLE:Reduction of trioses by NADPH-dependent aldo-keto reductases. Aldose reductase, methylglyoxal, and diabetic complications#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1537826# AUTHORS:Kelly VP, Sherratt PJ, Crouch DH, Hayes JD#TITLE:Novel homodimeric and heterodimeric rat gamma-hydroxybutyrate synthases that associate with the Golgi apparatus define a distinct subclass of aldo-keto reductase 7 family proteins.#JOURNAL:Biochem J#YEAR:2002#PUBMED:12071861# AUTHORS:Praml C, Savelyeva L, Perri P, Schwab M.#TITLE:Cloning of the human aflatoxin B1-aldehyde reductase gene at 1p35-1p36.1 in a region frequently altered in human tumor cells.#JOURNAL:Cancer Res#YEAR:1998#PUBMED:9823300# GENE ASSOCIATION: (10327.2) or (8574.1) or (10327.1) or (231.1)
Abbreviation: R_PS_HSter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: ps_hs[c] <==> ps_hs[r] Confidence Level: 2 for assumed reversible, non-energy dep scramblase mechanism (PMID: 10856717). NJ genes: none proteins: none citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# GENE ASSOCIATION:
Abbreviation: R_P5CRx Synonyms: _0 EC Number: 1.5.1.2 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : 1pyr5c + (2) h + nadh --> nad + pro-L Confidence Level: 4 PRO1x is irreversible in the other direction genes: LOCUS:_65263#TRANSCRIPT:1#ABBREVIATION:PYCRL# LOCUS:_29920#TRANSCRIPT:1#ABBREVIATION:PYCR2# proteins: NAME:pyrroline-5-carboxylate reductase family, member 2#ABBREVIATION:Pycr2.1# NAME:pyrroline-5-carboxylate reductase-like#ABBREVIATION:Pycrl.1# citations: AUTHORS:Merrill MJ, Yeh GC, Phang JM.#TITLE:Purified human erythrocyte pyrroline-5-carboxylate reductase. Preferential oxidation of NADPH.#JOURNAL: J Biol Chem#YEAR:1989#PUBMED:2722838# GENE ASSOCIATION: (29920.1) or (65263.1)
Abbreviation: R_PGDIr Synonyms: _8 EC Number: 5.3.99.2 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : prostgh2 <==> prostgd2 Confidence Level: 4 noted on RER, but also cytoplasmic structures NJ genes: LOCUS:_5730#TRANSCRIPT:1#ABBREVIATION:PTGDS#ECNUMBERS:5.3.99.2# LOCUS:_27306#TRANSCRIPT:1#ABBREVIATION:PGDS#ECNUMBERS:5.3.99.2# proteins: NAME:prostaglandin D2 synthase#ABBREVIATION:Ptgds.1# NAME:prostaglandin D2 synthase, hematopoietic#ABBREVIATION:Pgds.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Yamashima T, Sakuda K, Tohma Y, Yamashita J, Oda H, Irikura D, Eguchi N, Beuckmann CT, Kanaoka Y, Urade Y, Hayaishi O#TITLE:Free Full Text Prostaglandin D synthase (beta-trace) in human arachnoid and meningioma cells: roles as a cell marker or in cerebrospinal fluid absorption, tumorigenesis, and calcification process.#JOURNAL:J Neurosci#YEAR:1997#PUBMED:9065498# AUTHORS:Mahmud I, Ueda N, Yamaguchi H, Yamashita R, Yamamoto S, Kanaoka Y, Urade Y, Hayaishi O.#TITLE:Prostaglandin D synthase in human megakaryoblastic cells.#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9353279# GENE ASSOCIATION: (5730.1) or (27306.1)
Abbreviation: R_ADEtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: ade[l] <==> ade[c] Confidence Level: 4 IT genes: LOCUS:_55315#TRANSCRIPT:1#ABBREVIATION:SLC29A3# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 3 #ABBREVIATION:Slc29a3.1-l# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Pisoni RL, Thoene JG.#TITLE:Detection and characterization of a nucleoside transport system in human fibroblast lysosomes.#JOURNAL:J Biol Chem.#YEAR:1989#PUBMED:2925670# AUTHORS:Baldwin SA, Yao SY, Hyde RJ, Ng AM, Foppolo S, Barnes K, Ritzel MW, Cass CE, Young JD.#TITLE:Functional characterization of novel human and mouse equilibrative nucleoside transporters (hENT3 and mENT3) located in intracellular membranes.#JOURNAL:J Biol Chem. #YEAR:2005#PUBMED:15701636# GENE ASSOCIATION: (55315.1)
adenine facilated transport from lysosome
Abbreviation: R_GDPtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gdp[c] <==> gdp[g] Confidence Level: 1 unknown mechanism for transport - likely translocated, unknown transporter/mechanism though NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LNELDCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lneldc[e] <==> lneldc[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_TMABADH Synonyms: _0 EC Number: 1.2.1.47 SUBSYSTEM: Lysine Metabolism Equation: [c] : 4tmeabut + h2o + nad --> 4tmeabutn + (2) h + nadh Confidence Level: 4 MM genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# proteins: NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# citations: AUTHORS:Vaz,F.M. , Fouchier,S.W. , Ofman,R. , Sommer,M. , Wanders,R.J. #TITLE:Molecular and biochemical characterization of rat gamma-trimethylaminobutyraldehyde dehydrogenase and evidence for the involvement of human aldehyde dehydrogenase 9 in carnitine biosynthesis.#YEAR:2000#PUBMED:10702312# AUTHORS:Lin SW, Chen JC, Hsu LC, Hsieh CL, Yoshida A.#TITLE:Human gamma-aminobutyraldehyde dehydrogenase (ALDH9): cDNA sequence, genomic organization, polymorphism, chromosomal localization, and tissue expression.#YEAR:1996#PUBMED:8786138# AUTHORS:Kikonyogo,A. , Pietruszko,R. #TITLE:Aldehyde dehydrogenase from adult human brain that dehydrogenates gamma-aminobutyraldehyde: purification, characterization, cloning and distribution. #YEAR:1996#PUBMED:8645224# GENE ASSOCIATION: (223.1)
_4-trimethylaminobutyraldehyde dehydrogenase
keratan sulfate II (core 2-linked), degradation product 6_C44H72N4O33SX
Abbreviation: R_S6TASE24ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core2_deg6 <==> h + ksii_core2_deg7 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_ACACt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acac[e] + h[e] <==> acac[c] + h[c] Confidence Level: 4 originally named AACTP NCD genes: LOCUS:_9194#TRANSCRIPT:1#ABBREVIATION:SLC16A7# LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 7#ABBREVIATION:Slc16a7.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1#ABBREVIATION:Slc16a1.1# citations: AUTHORS:Lin RY, Vera JC, Chaganti RS, Golde DW#TITLE:Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9786900# AUTHORS:Halestrap AP, Price NT#TITLE:The proton-linked monocarboxylate transporter (MCT) family: structure, function and regulation#JOURNAL:Biochem J#YEAR:1999#PUBMED:10510291# AUTHORS:Garcia CK, Li X, Luna J, Francke U#TITLE:cDNA cloning of the human monocarboxylate transporter 1 and chromosomal localization of the SLC16A1 locus to 1p13.2-p12#JOURNAL:Genomics#YEAR:1994#PUBMED:7835905# AUTHORS:Poole RC, Halestrap AP#TITLE:Transport of lactate and other monocarboxylates across mammalian plasma membranes#JOURNAL:Am J Physiol#YEAR:1993#PUBMED:8476015# AUTHORS:Ritzhaupt A, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:The characterization of butyrate transport across pig and human colonic luminal membrane#JOURNAL:J Physiol#YEAR:1998#PUBMED:9508842# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification of a monocarboxylate transporter isoform type 1 (MCT1) on the luminal membrane of human and pig colon#JOURNAL:Biochem Soc Trans#YEAR:1998#PUBMED:9649795# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification and characterization of a monocarboxylate transporter (MCT1) in pig and human colon: its potential to transport L-lactate as well as butyrate#JOURNAL:J Physiol#YEAR:1998#PUBMED:9824713# AUTHORS:Halestrap AP, Meredith D#TITLE:The SLC16 gene family-from monocarboxylate transporters (MCTs) to aromatic amino acid transporters and beyond#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739169# AUTHORS:Manning Fox JE, Meredith D, Halestrap AP#TITLE:Characterisation of human monocarboxylate transporter 4 substantiates its role in lactic acid efflux from skeletal muscle#JOURNAL:J Physiol#YEAR:2000#PUBMED:11101640# AUTHORS:Price NT, Jackson VN, Halestrap AP#TITLE:Cloning and sequencing of four new mammalian monocarboxylate transporter (MCT) homologues confirms the existence of a transporter family with an ancient past#JOURNAL:Biochem J#YEAR:1998#PUBMED:9425115# GENE ASSOCIATION: (6566.1) or (9194.1)
acetoacetate transport via proton symport
Abbreviation: R_ANDRSTRNGLCte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: andrstrnglc[c] + atp[c] + h2o[c] --> adp[c] + andrstrnglc[e] + h[c] + pi[c] Confidence Level: 2 for MDR transport mechanism NJ genes: LOCUS:_4363#TRANSCRIPT:1#ABBREVIATION:ABCC1.1# proteins: NAME:ATP-binding cassette, subfamily C (CFTR/MRP), member 1#ABBREVIATION:ABCC1.1# citations: none GENE ASSOCIATION: (4363.1)
Abbreviation: R_CYSTS Synonyms: _0 EC Number: 4.2.1.22 SUBSYSTEM: Methionine Metabolism Equation: [c] : hcys-L + ser-L --> cyst-L + h2o Confidence Level: 4 NCD genes: LOCUS:_875#TRANSCRIPT:1#ABBREVIATION:CBS#ECNUMBERS:4.2.1.22# proteins: NAME:cystathionine-beta-synthase #ABBREVIATION:Cbs.1# citations: AUTHORS:Kraus JP, Oliveriusova J, Sokolova J, Kraus E, Vlcek C, de Franchis R#TITLE:The human cystathionine beta-synthase (CBS) gene: complete sequence, alternative splicing, and polymorphisms.#YEAR:1998#PUBMED:9790750# AUTHORS:Kraus J, Packman S, Fowler B, Rosenberg LE.#TITLE:Purification and properties of cystathionine beta-synthase from human liver. Evidence for identical subunits.#YEAR:1978#PUBMED:681363# GENE ASSOCIATION: (875.1)
Abbreviation: R_DALAt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-D[e] + h[e] <==> ala-D[c] + h[c] Confidence Level: 2 JLR genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
folylpolyglutamate synthetase (10fthf), mitochondrial
Abbreviation: R_FPGS9m Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [m] : 10fthf6glu + atp + glu-L --> 10fthf7glu + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
L-cysteine/L-threonine Na-dependent exchange (Cys-L in)
Abbreviation: R_CYSTHRNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[e] + na1[e] + thr-L[c] --> cys-L[c] + na1[c] + thr-L[e] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
Abbreviation: R_S3T1g Synonyms: _5 EC Number: 2.8.2.23 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre13 + paps --> h + hs_pre14 + pap Confidence Level: 4 NCD genes: LOCUS:_9957#TRANSCRIPT:1#ABBREVIATION:HS3ST1#ECNUMBERS:2.8.2.23# LOCUS:_222537#TRANSCRIPT:1#ABBREVIATION:HS3ST5# LOCUS:_9951#TRANSCRIPT:1#ABBREVIATION:HS3ST4#ECNUMBERS:2.8.2.23# proteins: NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 1#ABBREVIATION:Hs3st1.1-g# NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 4, Golgi apparatus#ABBREVIATION:Hs3st4.1-g# NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 5, Golgi apparatus#ABBREVIATION:Hs3st5.1-g# citations: AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Shworak NW, Liu J, Fritze LM, Schwartz JJ, Zhang L, Logeart D, Rosenberg RD#TITLE:Molecular cloning and expression of mouse and human cDNAs encoding heparan sulfate D-glucosaminyl 3-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9346953# AUTHORS:Shworak NW, Liu J, Petros LM, Zhang L, Kobayashi M, Copeland NG, Jenkins NA, Rosenberg RD#TITLE:Multiple isoforms of heparan sulfate D-glucosaminyl 3-O-sulfotransferase. Isolation, characterization, and expression of human cdnas and identification of distinct genomic loci#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9988767# AUTHORS:Xia G, Chen J, Tiwari V, Ju W, Li JP, Malmstrom A, Shukla D, Liu J#TITLE:Heparan sulfate 3-O-sulfotransferase isoform 5 generates both an antithrombin-binding site and an entry receptor for herpes simplex virus, type 1#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12138164# AUTHORS:Mochizuki H, Yoshida K, Gotoh M, Sugioka S, Kikuchi N, Kwon YD, Tawada A, Maeyama K, Inaba N, Hiruma T, Kimata K, Narimatsu H#TITLE:Characterization of a heparan sulfate 3-O-sulfotransferase-5, an enzyme synthesizing a tetrasulfated disaccharide#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12740361# GENE ASSOCIATION: (9957.1) or (9951.1) or (222537.1)
Abbreviation: R_FUCACNGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucacngal14acglcgalgluside_hs[e] <==> fucacngal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
degradation of proteoglycan linkage region, lysosomal
Abbreviation: R_LINKDEG2ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_a_deg5 + (4) h2o --> (2) gal + glcur + h + so4 + xyl-D Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
Abbreviation: R_DNDPt9m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[c] + dtdp[m] --> dadp[m] + dtdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_UDPGP Synonyms: _0 EC Number: 3.6.1.9 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : h2o + udpg --> g1p + (2) h + ump Confidence Level: 0 NCD genes: LOCUS:_5167#TRANSCRIPT:1#ABBREVIATION:ENPP1#ECNUMBERS:3.1.4.1,3.6.1.9# LOCUS:_5168#TRANSCRIPT:1#ABBREVIATION:ENPP2#ECNUMBERS:3.1.4.1,3.6.1.9# LOCUS:_5169#TRANSCRIPT:1#ABBREVIATION:ENPP3#ECNUMBERS:3.1.4.1,3.6.1.9# proteins: NAME:ectonucleotide pyrophosphatase/phosphodiesterase 1 #ABBREVIATION:EnpP1.1# NAME:ectonucleotide pyrophosphatase/phosphodiesterase 2 (autotaxin)#ABBREVIATION:Enpp2.1# NAME:ectonucleotide pyrophosphatase/phosphodiesterase 3#ABBREVIATION:Enpp3.1# citations: none GENE ASSOCIATION: (5167.1) or (5169.1) or (5168.1)
Abbreviation: R_CGLYt3_LPAREN_2_RPAREN_ Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cgly[e] + (2) h[e] --> cgly[c] + (2) h[c] Confidence Level: 2 SAB genes: LOCUS:_6565#TRANSCRIPT:1#ABBREVIATION:SLC15A2# proteins: NAME:solute carrier family 15 (H+/peptide transporter), member 2#ABBREVIATION:Slc15a2.1# citations: AUTHORS:Daniel H, Kottra G#TITLE:The proton oligopeptide cotransporter family SLC15 in physiology and pharmacology#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12905028# GENE ASSOCIATION: (6565.1)
Abbreviation: R_CSND Synonyms: _0 EC Number: 3.5.4.1 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : csn + h + h2o --> nh4 + ura Confidence Level: 2 JLR genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION:
Abbreviation: R_GAO2 Synonyms: _1 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : accoa + gt3_hs --> coa + oagt3_hs Confidence Level: 4 for humans - seq predicted SLC transporters have been found to have O-acetylation activity on gangliosides NJ genes: LOCUS:_9197#TRANSCRIPT:1#ABBREVIATION:SLC33A1# proteins: NAME:acetyl-CoA transporter/O-acetylator#ABBREVIATION:Slc33a1.1# citations: AUTHORS:Kanamori A, Nakayama J, Fukuda MN, Stallcup WB, Sasaki K, Fukuda M, Hirabayashi Y#TITLE:Expression cloning and characterization of a cDNA encoding a novel membrane protein required for the formation of O-acetylated ganglioside: A putative acetyl-CoA-transporter#JOURNAL:Proc Natl Acad Sci#YEAR:1997# GENE ASSOCIATION: (9197.1)
Abbreviation: R_EX_pheme_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pheme <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_IDOAASE4ly Synonyms: _1 EC Number: 3.2.1.76 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_b_deg3 + h2o --> cs_a_deg3 + idour Confidence Level: 4 NCD genes: LOCUS:_3425#TRANSCRIPT:1#ABBREVIATION:IDUA#ECNUMBERS:3.2.1.76# proteins: NAME:iduronidase, alpha-L-, lysosomal#ABBREVIATION:Idua.1-l# citations: AUTHORS:Scott HS, Anson DS, Orsborn AM, Nelson PV, Clements PR, Morris CP, Hopwood JJ#TITLE:Human alpha-L-iduronidase: cDNA isolation and expression#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1946389# AUTHORS:Clements PR, Brooks DA, McCourt PA, Hopwood JJ#TITLE:Immunopurification and characterization of human alpha-L-iduronidase with the use of monoclonal antibodies#JOURNAL:Biochem J#YEAR:1989#PUBMED:2470345# GENE ASSOCIATION: (3425.1)
_5-Hydroxykynurenamine:oxygen oxidoreductase(deaminating)(flavin-containing)
Abbreviation: R_5HXKYNOXDA Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 5hxkynam + o2 --> 46dhoxquin + h2o2 + nh4 Confidence Level: 1 SAB genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# proteins: NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# GENE ASSOCIATION: (4128.1) or (4129.1)
B-alanine secretion via secretory vesicle (ATP driven)
Abbreviation: R_BALAVECSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) ala-B[c] + atp[c] + h2o[c] --> adp[c] + (3) ala-B[e] + h[c] + pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of glu from the cell. It leaves out the vesicle because a compartment is not available for that. ATP demand is not known for sure stoichiometrically, but 1:1 H coupling ratio is assumed, which could translate to 3 b-ala/ATP. genes: LOCUS:_140679#TRANSCRIPT:1#ABBREVIATION:SLC32A1# proteins: NAME:solute carrier family 32 (GABA vesicular transporter), member 1#ABBREVIATION:Slc32a1.1# citations: AUTHORS:Gasnier B#TITLE:The SLC32 transporter, a key protein for the synaptic release of inhibitory amino acids#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750892# AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# GENE ASSOCIATION: (140679.1)
Abbreviation: R_H8MTer_L Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_L + gpi_hs --> (0.1) dolp_L + h + m(em)3gacpail_hs Confidence Level: 4 NCD genes: LOCUS:_80235#TRANSCRIPT:1#ABBREVIATION:SMP3# proteins: NAME:SMP3 mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Smp3.1-r# citations: AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (80235.1)
Abbreviation: R_D_DASH_LACtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h[c] + lac-D[c] <==> h[m] + lac-D[m] Confidence Level: 0 NCD genes: LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1 #ABBREVIATION:Slc16a1.1-m# citations: none GENE ASSOCIATION: (6566.1)
Abbreviation: R_ACHtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: ach[n] <==> ach[c] Confidence Level: 1 it genes: none proteins: none citations: none GENE ASSOCIATION:
Acetylcholin transport, nuclear trhough pores
Abbreviation: R_URIDK2m Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [m] : atp + dump <==> adp + dudp Confidence Level: 1 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FUT35g Synonyms: _5 EC Number: 2.4.1.65 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galfucgalacglcgalgluside_hs + gdpfuc --> fucgalfucgalacglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2525#TRANSCRIPT:1#ABBREVIATION:FUT3#ECNUMBERS:2.4.1.65# proteins: NAME:fucosyltransferase 3 (galactoside 3(4)-L-fucosyltransferase, Lewis blood group)#ABBREVIATION:Fut3.1# citations: AUTHORS:Cakir B, Pankow JS, Salomaa V, Couper D, Morris TL, Brantley KR, Hiller KM, Heiss G, Weston BW.#TITLE:Distribution of Lewis (FUT3)genotype and allele: frequencies in a biethnic United States population.#JOURNAL:Ann Hematol#YEAR:2002#PUBMED:12424536# AUTHORS:Sousa VL, Brito C, Costa T, Lanoix J, Nilsson T, Costa J.#TITLE:Importance of Cys, Gln, and Tyr from the transmembrane domain of human alpha 3/4 fucosyltransferase III for its localization and sorting in the Golgi of baby hamster kidney cells.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12493760# AUTHORS:Kukowska-Latallo JF, Larsen RD, Nair RP, Lowe JB.#TITLE:A cloned human cDNA determines expression of a mouse stage-specific embryonic antigen and the Lewis blood group alpha(1,3/1,4)fucosyltransferase.#JOURNAL:Genes Dev#YEAR:1990#PUBMED:1977660# GENE ASSOCIATION: (2525.1)
Abbreviation: R_DNDPt27m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[c] + dtdp[m] --> dcdp[m] + dtdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
_1-alpha,25-Dihydroxyvitamin D2_C28H44O3
chondroitin sulfate D (GlcNAc6S-GlcA2S), degradation product 6_C23H35O27S2
Abbreviation: R_DNADDP Synonyms: _0 EC Number: 3.6.1.9 SUBSYSTEM: NAD Metabolism Equation: [c] : dnad + h2o --> amp + (2) h + nicrnt Confidence Level: 2 genes: LOCUS:_5167#TRANSCRIPT:1#ABBREVIATION:ENPP1#ECNUMBERS:3.1.4.1,3.6.1.9# LOCUS:_5168#TRANSCRIPT:1#ABBREVIATION:ENPP2#ECNUMBERS:3.1.4.1,3.6.1.9# LOCUS:_5169#TRANSCRIPT:1#ABBREVIATION:ENPP3#ECNUMBERS:3.1.4.1,3.6.1.9# proteins: NAME:ectonucleotide pyrophosphatase/phosphodiesterase 1 #ABBREVIATION:EnpP1.1# NAME:ectonucleotide pyrophosphatase/phosphodiesterase 2 (autotaxin)#ABBREVIATION:Enpp2.1# NAME:ectonucleotide pyrophosphatase/phosphodiesterase 3#ABBREVIATION:Enpp3.1# citations: AUTHORS:Funakoshi I, Kato H, Horie K, Yano T, Hori Y, Kobayashi H, Inoue T, Suzuki H, Fukui S, Tsukahara M, et al.#TITLE:Molecular cloning of cDNAs for human fibroblast nucleotide pyrophosphatase.#JOURNAL:Arch Biochem Biophys.#YEAR:1992#PUBMED:1315502# GENE ASSOCIATION: (5167.1) or (5169.1) or (5168.1)
Abbreviation: R_URIt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + uri[e] --> na1[c] + uri[c] Confidence Level: 4 SAB genes: LOCUS:_9154#TRANSCRIPT:1#ABBREVIATION:SLC28A1# LOCUS:_9153#TRANSCRIPT:1#ABBREVIATION:SLC28A2# LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# LOCUS:_9154#TRANSCRIPT:2#ABBREVIATION:SLC28A1# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 1, extracellular #ABBREVIATION:Slc28a1.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 2, extracellular #ABBREVIATION:Slc28a2.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 1 #ABBREVIATION:Slc28a1.2-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (9154.2) or (9154.1) or (64078.1) or (9153.1)
Abbreviation: R_RAI1 Synonyms: _0 EC Number: 5.2.1.3 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : retinal <==> retinal-11-cis Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_LPCOXp Synonyms: _0 EC Number: 1.5.3.7 SUBSYSTEM: Lysine Metabolism Equation: [x] : Lpipecol + o2 --> h + h2o2 + thp2c Confidence Level: 4 genes: LOCUS:_51268#TRANSCRIPT:1#ABBREVIATION:PIPOX#ECNUMBERS:1.5.3.1,,1.5.3.7# proteins: NAME:pipecolic acid/sarcosine oxidase, peroxisomal#ABBREVIATION:Pipox.1-x# citations: AUTHORS:IJlst L, de Kromme I, Oostheim W, Wanders RJ.#TITLE:Molecular cloning and expression of human L-pipecolate oxidase.#YEAR:2000#PUBMED:10772957# GENE ASSOCIATION: (51268.1)
carbamoyl-phosphate synthase (ammonia) (mitochondria
Abbreviation: R_CBPSam Synonyms: _0 EC Number: 6.3.4.16 SUBSYSTEM: Glutamate metabolism Equation: [m] : (2) atp + hco3 + nh4 --> (2) adp + cbp + (2) h + pi Confidence Level: 4 MM genes: LOCUS:_1373#TRANSCRIPT:1#ABBREVIATION:CPS1#ECNUMBERS:6.3.4.16# LOCUS:_1373#TRANSCRIPT:2#ABBREVIATION:CPS1# proteins: NAME:carbamoyl-phosphate synthetase 1, mitochondrial#ABBREVIATION:Cps1.1-m# NAME:carbamoyl-phosphate synthetase 1, mitochondrial#ABBREVIATION:Cps1.2-m# citations: AUTHORS:Summar ML, Hall LD, Eeds AM, Hutcheson HB, Kuo AN, Willis AS, Rubio V, Arvin MK, Schofield JP, Dawson EP.#TITLE:Characterization of genomic structure and polymorphisms in the human carbamyl phosphate synthetase I gene.#JOURNAL:Gene#YEAR:2003#PUBMED:12853138# AUTHORS:Uriarte M, Marina A, Ramon-Maiques S, Fita I, Rubio V.#TITLE:The carbamoyl-phosphate synthetase of Pyrococcus furiosus is enzymologically and structurally a carbamate kinase.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10347186# GENE ASSOCIATION: (1373.1) or (1373.2)
Abbreviation: R_PUNP5 Synonyms: _0 EC Number: 2.4.2.1 SUBSYSTEM: Purine Catabolism Equation: [c] : ins + pi <==> hxan + r1p Confidence Level: 4 Specificity not completely determined. Can also catalyse ribosyltransferase reactions of the type catalysed by EC 2.4.2.5, nucleoside ribosyltransferase genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# citations: AUTHORS:Erion MD, Stoeckler JD, Guida WC, Walter RL, Ealick SE.#TITLE:Purine nucleoside phosphorylase. 2. Catalytic mechanism. #JOURNAL:Biochemistry#YEAR:1997#PUBMED:9305963# AUTHORS:Williams SR, Goddard JM, Martin DW Jr. #TITLE:Human purine nucleoside phosphorylase cDNA sequence and genomic clone characterization.#JOURNAL:Nucleic Acids Res.#YEAR:1984#PUBMED:6087295# AUTHORS:Canduri F, dos Santos DM, Silva RG, Mendes MA, Basso LA, Palma MS, de Azevedo WF, Santos DS.#TITLE:Structures of human purine nucleoside phosphorylase complexed with inosine and ddI.#JOURNAL:Biochem Biophys Res Commun. #YEAR:2004#PUBMED:14706628# GENE ASSOCIATION: (4860.1)
purine-nucleoside phosphorylase (Inosine)
Abbreviation: R_FBA4 Synonyms: _0 EC Number: 4.1.2.13 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : xu1p-D <==> dhap + gcald Confidence Level: 4 NCD genes: LOCUS:_226#TRANSCRIPT:1#ABBREVIATION:ALDOA#ECNUMBERS:4.1.2.13# LOCUS:_229#TRANSCRIPT:1#ABBREVIATION:ALDOB#ECNUMBERS:4.1.2.13# LOCUS:_230#TRANSCRIPT:1#ABBREVIATION:ALDOC#ECNUMBERS:4.1.2.13# LOCUS:_226#TRANSCRIPT:2#ABBREVIATION:ALDOA#ECNUMBERS:4.1.2.13# LOCUS:_226#TRANSCRIPT:3#ABBREVIATION:ALDOA#ECNUMBERS:4.1.2.13# proteins: NAME:aldolase A, fructose-bisphosphate (muscle)#ABBREVIATION:Aldoa.1# NAME:aldolase A, fructose-bisphosphate (muscle)#ABBREVIATION:Aldoa.2# NAME:aldolase A, fructose-bisphosphate (muscle)#ABBREVIATION:Aldoa.3# NAME:aldolase B, fructose-bisphosphate (liver)#ABBREVIATION:Aldob.1# NAME:aldolase C, fructose-bisphosphate (brain)#ABBREVIATION:Aldoc.1# citations: AUTHORS:Bais R, James HM, Rofe AM, Conyers RA.#TITLE:The purification and properties of human liver ketohexokinase. A role for ketohexokinase and fructose-bisphosphate aldolase in the metabolic production of oxalate from xylitol#JOURNAL:Biochem J#YEAR:1985#PUBMED:2996495# AUTHORS:Barngrover DA, Dills WL Jr.#TITLE:The involvement of liver fructokinase in the metabolism of D-xylulose and xylitol in isolated rat hepatocytes#JOURNAL:J Nutr#YEAR:1983#PUBMED:6298387# AUTHORS:James HM, Bais R, Edwards JB, Rofe AM, Conyers AJ.#TITLE:Models for the metabolic production of oxalate from xylitol in humans: a role for fructokinase and aldolase.#JOURNAL:Aust J Exp Biol Med Sci#YEAR:1982#PUBMED:6284103# GENE ASSOCIATION: (226.2) or (229.1) or (226.3) or (230.1) or (226.1)
D-Fructose 1-phosphate D-glyceraldehyde-3-phosphate-lyase
Abbreviation: R_BILDGLCURte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + bildglcur[c] + h2o[c] <==> adp[c] + bildglcur[e] + h[c] + pi[c] Confidence Level: 2 NJ genes: LOCUS:_4363#TRANSCRIPT:1#ABBREVIATION:ABCC1.1# proteins: NAME:ATP-binding cassette, subfamily C (CFTR/MRP), member 1#ABBREVIATION:ABCC1.1# citations: none GENE ASSOCIATION: (4363.1)
bilirubin beta-diglucuronide transport MDR
Abbreviation: R_PS_HStg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: ps_hs[c] <==> ps_hs[g] Confidence Level: 2 for assumed reversible, non-energy dep scramblase mechanism (PMID: 10856717). NJ genes: none proteins: none citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# GENE ASSOCIATION:
Abbreviation: R_DESAT18_6 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + nadh + o2 + odecoa --> (2) h2o + lneldccoa + nad Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_9415#TRANSCRIPT:1#ABBREVIATION:FADS2#ECNUMBERS:1.14.99.25# proteins: NAME:fatty acid desaturase 2#ABBREVIATION:Fads2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Cho HP, Nakamura MT, Clarke SD#TITLE:Cloning, expression, and nutritional regulation of the mammalian delta-6 desaturase#JOURNAL:The Journal of Biological Chemistry#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION: (9415.1)
Abbreviation: R_GCNTg Synonyms: _6 EC Number: 2.4.1.150 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacglcgal14acglcgalgluside_hs + uacgam --> acglcgalacglcgal14acglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_2651#TRANSCRIPT:1#ABBREVIATION:GCNT2#ECNUMBERS:2.4.1.150# proteins: NAME:glucosaminyl (N-acetyl) transferase 2, I-branching enzyme#ABBREVIATION:Gcnt2.1# citations: AUTHORS:Pras E, Raz J, Yahalom V, Frydman M, Garzozi HJ, Pras E, Hejtmancik JF.#TITLE:A nonsense mutation in the glucosaminyl (N-acetyl) transferase 2 gene (GCNT2): association with autosomal recessive congenital cataracts.#JOURNAL:Invest Ophthalmol Vis Sci#YEAR:2004#PUBMED:15161861# AUTHORS:Bierhuizen MF, Mattei MG, Fukuda M.#TITLE:Expression of the developmental I antigen by a cloned human cDNA encoding a member of a beta-1,6-N-acetylglucosaminyltransferase gene family.#JOURNAL:Genes Dev#YEAR:1993#PUBMED:8449405# GENE ASSOCIATION: (2651.1)
N-acetyllactosaminide beta-1,6-N-acetylglucosaminyl-transferase
Abbreviation: R_FAOXC16180m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (4) coa + (3) fad + (4) h2o + hdcoa + (4) nad --> (4) accoa + (3) fadh2 + (4) h + (4) nadh + occoa Confidence Level: 4 _4 rounds of beta ox, 1 loss of degree of unsat -> only 3 fad oxidized c16:1 -> c8:0 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (35.1) or (34.1)
Abbreviation: R_PI45P3K Synonyms: _0 EC Number: 2.7.1.153 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + pail45p_hs --> adp + h + pail345p_hs Confidence Level: 4 NCD genes: LOCUS:_5291#TRANSCRIPT:1#ABBREVIATION:PIK3CB#ECNUMBERS:2.7.1.137# LOCUS:_5290#TRANSCRIPT:1#ABBREVIATION:PIK3CA#ECNUMBERS:2.7.1.137# LOCUS:_5293#TRANSCRIPT:1#ABBREVIATION:PIK3CD#ECNUMBERS:2.7.1.137# LOCUS:_5294#TRANSCRIPT:1#ABBREVIATION:PIK3CG#ECNUMBERS:2.7.1.137# LOCUS:_23533#TRANSCRIPT:1#ABBREVIATION:P101-PI3K# LOCUS:_5295#TRANSCRIPT:1#ABBREVIATION:PIK3R1# LOCUS:_8503#TRANSCRIPT:1#ABBREVIATION:PIK3R3# LOCUS:_5296#TRANSCRIPT:1#ABBREVIATION:PIK3R2# LOCUS:_5295#TRANSCRIPT:2#ABBREVIATION:PIK3R1# LOCUS:_5295#TRANSCRIPT:3#ABBREVIATION:PIK3R1# LOCUS:_10870#TRANSCRIPT:1#ABBREVIATION:HCST# proteins: NAME:phosphoinositide-3-kinase (p110a, p85a)#ABBREVIATION:Pik3car1.1# NAME:phosphoinositide-3-kinase (p110a, p55a)#ABBREVIATION:Pik3car1.2# NAME:phosphoinositide-3-kinase (p110a, p50a)#ABBREVIATION:Pik3car.3# NAME:phosphoinositide-3-kinase (p110a, p85b)#ABBREVIATION:Pik3car2.1# NAME:phosphoinositide-3-kinase (p110a, p55c)#ABBREVIATION:Pik3car3.1# NAME:phosphoinositide-3-kinase (p110b, p85a)#ABBREVIATION:Pik3cbr1.1# NAME:phosphoinositide-3-kinase (p110b, p55a)#ABBREVIATION:Pik3cbr1.2# NAME:phosphoinositide-3-kinase (p110b, p50a)#ABBREVIATION:Pik3cbr1.3# NAME:phosphoinositide-3-kinase (p110b, p85b)#ABBREVIATION:Pik3cbr2.1# NAME:phosphoinositide-3-kinase (p110b, p55c)#ABBREVIATION:Pik3cbr3.1# NAME:phosphoinositide-3-kinase (p110c, p85a)#ABBREVIATION:Pik3cdr1.1# NAME:phosphoinositide-3-kinase (p110c, p55a)#ABBREVIATION:Pik3cdr1.2# NAME:phosphoinositide-3-kinase (p110c, p50a)#ABBREVIATION:Pik3cdr1.3# NAME:phosphoinositide-3-kinase (p110c, p85b)#ABBREVIATION:Pik3cdr2.1# NAME:phosphoinositide-3-kinase (p110c, p55c)#ABBREVIATION:Pik3cdr3.1# NAME:phosphoinositide-3-kinase (PIK3cg, p101)#ABBREVIATION:Pik3cgr5.1# NAME:phosphoinositide-3-kinase (PIK3CG, p87 adapter protein)#ABBREVIATION:Pik3cgap.1# citations: AUTHORS:Carpenter CL, Duckworth BC, Auger KR, Cohen B, Schaffhausen BS, Cantley LC#TITLE:Purification and characterization of phosphoinositide 3-kinase from rat liver#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2174051# AUTHORS:Volinia S, Hiles I, Ormondroyd E, Nizetic D, Antonacci R, Rocchi M, Waterfield MD#TITLE:Molecular cloning, cDNA sequence, and chromosomal localization of the human phosphatidylinositol 3-kinase p110 alpha (PIK3CA) gene#JOURNAL:Genomics#YEAR:1994#PUBMED:7713498# AUTHORS:Wymann MP, Marone R#TITLE:Phosphoinositide 3-kinase in disease: timing, location, and scaffolding#JOURNAL:Curr Opin Cell Biol#YEAR:2005#PUBMED:15780590# AUTHORS:Hu P, Mondino A, Skolnik EY, Schlessinger J#TITLE:Cloning of a novel, ubiquitously expressed human phosphatidylinositol 3-kinase and identification of its binding site on p85#JOURNAL:Mol Cell Biol#YEAR:1993#PUBMED:8246984# AUTHORS:Vanhaesebroeck B, Welham MJ, Kotani K, Stein R, Warne PH, Zvelebil MJ, Higashi K, Volinia S, Downward J, Waterfield MD#TITLE:P110delta, a novel phosphoinositide 3-kinase in leukocytes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9113989# AUTHORS:Stoyanov B, Volinia S, Hanck T, Rubio I, Loubtchenkov M, Malek D, Stoyanova S, Vanhaesebroeck B, Dhand R, Nurnberg B, et al#TITLE:Cloning and characterization of a G protein-activated human phosphoinositide-3 kinase#JOURNAL:Science#YEAR:1995#PUBMED:7624799# AUTHORS:Brock C, Schaefer M, Reusch HP, Czupalla C, Michalke M, Spicher K, Schultz G, Nurnberg B#TITLE:Roles of G beta gamma in membrane recruitment and activation of p110 gamma/p101 phosphoinositide 3-kinase gamma.#JOURNAL:J Cell Biol#YEAR:2003#PUBMED:12507995# GENE ASSOCIATION: (5290.1 and 8503.1) or (5293.1 and 5295.3) or (5290.1 and 5295.1) or (5295.2 and 5291.1) or (5293.1 and 5295.1) or (10870.1 and 5294.1) or (5293.1 and 5296.1) or (5291.1 and 5295.1) or (8503.1 and 5291.1) or (5296.1 and 5291.1) or (5291.1 and 5295.3) or (5290.1 and 5295.3) or (5295.2 and 5290.1) or (23533.1 and 5294.1) or (5293.1 and 8503.1) or (5295.2 and 5293.1) or (5296.1 and 5290.1)
phosphatidylinositol 4,5-bisphosphate 3-kinase
Abbreviation: R_GULN3D Synonyms: _6 EC Number: 1.1.1.45 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : guln + nad <==> 3dhguln + h + nadh Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Wang YM, van Eys J#TITLE:Nutritional significance of fructose and sugar alcohols#JOURNAL:Annu Rev Nutr.#YEAR:1981#PUBMED:6821187# GENE ASSOCIATION:
Abbreviation: R_EX_nad_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : nad <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Nicotinamide adenine dinucleotide exchange
Abbreviation: R_NADPtru Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: nadp[r] --> nadp[c] Confidence Level: 1 diffusion - but unidirectional output from ER (to avoid loops w/ NAD/NADP) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
UDP-D-galactose:galactosylxylose galactosyltransferase, Golgi apparatus
Abbreviation: R_GALT2g Synonyms: _3 EC Number: 2.4.1.134 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : lxser + udpgal --> h + l2xser + udp Confidence Level: 4 NCD genes: LOCUS:_126792#TRANSCRIPT:1#ABBREVIATION:B3GALT6# proteins: NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 6, Golgi apparatus#ABBREVIATION:B3galt6.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Bai X, Zhou D, Brown JR, Crawford BE, Hennet T, Esko JD#TITLE:Biosynthesis of the linkage region of glycosaminoglycans: cloning and activity of galactosyltransferase II, the sixth member of the beta 1,3-galactosyltransferase family (beta 3GalT6)#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11551958# GENE ASSOCIATION: (126792.1)
Abbreviation: R_EX_npthl_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : npthl <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_24_COMMA_25VITD3Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : 25hvitd3 + h + nadph + o2 --> 2425dhvitd3 + h2o + nadp Confidence Level: 4 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.196 IT genes: LOCUS:_1591#TRANSCRIPT:1#ABBREVIATION:CYP24A1# proteins: NAME:cytochrome P450, family 24, subfamily A, polypeptide 1 #ABBREVIATION:Cyp24a1.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Kusudo T, Sakaki T, Abe D, Fujishima T, Kittaka A, Takayama H, Hatakeyama S, Ohta M, Inouye K.#TITLE:Metabolism of A-ring diastereomers of 1alpha,25-dihydroxyvitamin D3 by CYP24A1. #JOURNAL:Biochem Biophys Res Commun.#YEAR:2004#PUBMED:15358094# AUTHORS:Labuda M, Lemieux N, Tihy F, Prinster C, Glorieux FH.#TITLE:Human 25-hydroxyvitamin D 24-hydroxylase cytochrome P450 subunit maps to a different chromosomal location than that of pseudovitamin D-deficient rickets. #JOURNAL:J Bone Miner Res.#YEAR:1993#PUBMED:8266831# GENE ASSOCIATION: (1591.1)
Abbreviation: R_EX_meoh_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : meoh <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
keratan sulfate I, degradation product 2_C241H384N17O210S12
Abbreviation: R_URCN Synonyms: _0 EC Number: 4.2.1.49 SUBSYSTEM: Histidine Metabolism Equation: [c] : h2o + urcan --> 4izp Confidence Level: 4 genes: LOCUS:_131669#TRANSCRIPT:1#ABBREVIATION:FLJ31300#ECNUMBERS:4.2.1.49# proteins: NAME:urocanate family protein#ABBREVIATION:Flj31300.1# citations: AUTHORS:Kessler D, Retey J, Schulz GE#TITLE:Structure and action of urocanase#JOURNAL:J Mol Biol#YEAR:2004#PUBMED:15313616# GENE ASSOCIATION: (131669.1)
Abbreviation: R_24_COMMA_25DHVITD2tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _2425dhvitd2[m] --> 2425dhvitd2[c] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_24,25-Dihydroxyvitamin D2 transport from mitochondria
Abbreviation: R_HIBDm Synonyms: _0 EC Number: 1.1.1.31 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 3hmp + nad <==> 2mop + h + nadh Confidence Level: 4 MM genes: LOCUS:_11112#TRANSCRIPT:1#ABBREVIATION:HIBADH# proteins: NAME:_3-hydroxyisobutyrate dehydrogenase, mitochondrial#ABBREVIATION:Hibadh.1-m# citations: AUTHORS:Kedishvili NY, Popov KM, Jaskiewicz JA, Harris RA.#TITLE:Coordinated expression of valine catabolic enzymes during adipogenesis: analysis of activity, mRNA, protein levels, and metabolic consequences.#YEAR:1994#PUBMED:7527207# AUTHORS:Sweetman, L, Williams, JC#TITLE:Branched chain organic acidurias#JOURNAL:The Metabolic and Molecular Bases of Inherited Disease, 8th ed (Scriver CR, et al., editors)#YEAR:2001# GENE ASSOCIATION: (11112.1)
_3-hydroxyisobutyrate dehydrogenase, mitochondrial
Abbreviation: R_CYTDK2m Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [m] : atp + cytd --> adp + cmp + h Confidence Level: 1 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GAO2g Synonyms: _1 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : accoa + gt3_hs --> coa + oagt3_hs Confidence Level: 4 for humans - seq predicted SLC transporters have been found to have O-acetylation activity on gangliosides NJ genes: LOCUS:_9197#TRANSCRIPT:1#ABBREVIATION:SLC33A1# proteins: NAME:acetyl-CoA transporter/O-acetylator#ABBREVIATION:Slc33a1.1# citations: AUTHORS:Kanamori A, Nakayama J, Fukuda MN, Stallcup WB, Sasaki K, Fukuda M, Hirabayashi Y#TITLE:Expression cloning and characterization of a cDNA encoding a novel membrane protein required for the formation of O-acetylated ganglioside: A putative acetyl-CoA-transporter#JOURNAL:Proc Natl Acad Sci#YEAR:1997# GENE ASSOCIATION: (9197.1)
Abbreviation: R_COKECBESr Synonyms: _0 EC Number: 3.1.1.1 SUBSYSTEM: Alkaloid biosynthesis II Equation: [r] : coke + h2o --> bz + egme + h Confidence Level: 4 SAB genes: LOCUS:_8824#TRANSCRIPT:1#ABBREVIATION:CES2# proteins: NAME:carboxylesterase 2#ABBREVIATION:Ces2.1-r# citations: AUTHORS:Pindel EV, Kedishvili NY, Abraham TL, Brzezinski MR, Zhang J, Dean RA, Bosron WF#TITLE:Purification and cloning of a broad substrate specificity human liver carboxylesterase that catalyzes the hydrolysis of cocaine and heroin#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9169443# GENE ASSOCIATION: (8824.1)
Abbreviation: R_GLCNACT2g Synonyms: _3 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre2 + uacgam --> h + hs_pre3 + udp Confidence Level: 4 EC 2.4.1.224 NCD genes: LOCUS:_2131#TRANSCRIPT:1#ABBREVIATION:EXT1# LOCUS:_2132#TRANSCRIPT:1#ABBREVIATION:EXT2#ECNUMBERS:2.4.1.-# LOCUS:_2137#TRANSCRIPT:1#ABBREVIATION:EXTL3#ECNUMBERS:2.4.1.-# LOCUS:_2134#TRANSCRIPT:1#ABBREVIATION:EXTL1# LOCUS:_2132#TRANSCRIPT:2#ABBREVIATION:EXT2# proteins: NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.1-g# NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.2-g# NAME:exostoses (multiple)-like 1, Golgi apparatus#ABBREVIATION:Extl1.1-g# NAME:exostoses (multiple)-like 3, Golgi apparatus#ABBREVIATION:Extl3.1-g# citations: AUTHORS:Saito T, Seki N, Yamauchi M, Tsuji S, Hayashi A, Kozuma S, Hori T#TITLE:Structure, chromosomal location, and expression profile of EXTR1 and EXTR2, new members of the multiple exostoses gene family#JOURNAL:Biochem Biophys Res Commun#YEAR:1998#PUBMED:9473480# AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Kim BT, Kitagawa H, Tamura J, Saito T, Kusche-Gullberg M, Lindahl U, Sugahara K#TITLE:Human tumor suppressor EXT gene family members EXTL1 and EXTL3 encode alpha 1,4- N-acetylglucosaminyltransferases that likely are involved in heparan sulfate/ heparin biosynthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390981# AUTHORS:Van Hul W, Wuyts W, Hendrickx J, Speleman F, Wauters J, De Boulle K, Van Roy N, Bossuyt P, Willems PJ#TITLE:Identification of a third EXT-like gene (EXTL3) belonging to the EXT gene family#JOURNAL:Genomics#YEAR:1998#PUBMED:9479495# AUTHORS:McCormick C, Duncan G, Goutsos KT, Tufaro F#TITLE:The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10639137# AUTHORS:Kobayashi S, Morimoto K, Shimizu T, Takahashi M, Kurosawa H, Shirasawa T#TITLE:Association of EXT1 and EXT2, hereditary multiple exostoses gene products, in Golgi apparatus#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679296# AUTHORS:Ahn J, Ludecke HJ, Lindow S, Horton WA, Lee B, Wagner MJ, Horsthemke B, Wells DE#TITLE:Cloning of the putative tumour suppressor gene for hereditary multiple exostoses (EXT1)#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7550340# AUTHORS:McCormick C, Leduc Y, Martindale D, Mattison K, Esford LE, Dyer AP, Tufaro F#TITLE:The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate#JOURNAL:Nat Genet#YEAR:1998#PUBMED:9620772# AUTHORS:Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K#TITLE:The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9756849# AUTHORS:Stickens D, Clines G, Burbee D, Ramos P, Thomas S, Hogue D, Hecht JT, Lovett M, Evans GA#TITLE:The EXT2 multiple exostoses gene defines a family of putative tumour suppressor genes#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8782816# AUTHORS:Wise CA, Clines GA, Massa H, Trask BJ, Lovett M#TITLE:Identification and localization of the gene for EXTL, a third member of the multiple exostoses gene family#JOURNAL:Genome Res#YEAR:1997#PUBMED:9037597# GENE ASSOCIATION: (2131.1 and 2132.2) or (2137.1) or (2131.1 and 2132.1) or (2134.1)
Abbreviation: R_THMt3 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[c] + thm[e] <==> h[e] + thm[c] Confidence Level: 4 _01-25-05 based on Dutta et al., JBC, 1999, 274(45),31925-31929; Zhao et al, 2002, Am J Physiol, Cell Physiol. 282,C1512-C1517 genes: LOCUS:_80704#TRANSCRIPT:1#ABBREVIATION:SLC19A3# LOCUS:_10560#TRANSCRIPT:1#ABBREVIATION:SLC19A2# proteins: NAME:thiamine transporter I #ABBREVIATION:Slc19a2.1# NAME:solute carrier family 19, member 3 #ABBREVIATION:Slc19a3.1# citations: AUTHORS:Dutta B, Huang W, Molero M, Kekuda R, Leibach FH, Devoe LD, Ganapathy V, Prasad PD. #TITLE:Cloning of the human thiamine transporter, a member of the folate transporter family.#JOURNAL:J Biol Chem. #YEAR:1999#PUBMED:10542220# AUTHORS:Rajgopal A, Edmondnson A, Goldman ID, Zhao R.#TITLE:SLC19A3 encodes a second thiamine transporter ThTr2.#JOURNAL:Biochim Biophys Acta.#YEAR:2001#PUBMED:11731220# AUTHORS:Eudy JD, Spiegelstein O, Barber RC, Wlodarczyk BJ, Talbot J, Finnell RH.#TITLE:Identification and characterization of the human and mouse SLC19A3 gene: a novel member of the reduced folate family of micronutrient transporter genes.#JOURNAL:Mol Genet Metab. #YEAR:2000#PUBMED:11136550# AUTHORS:Ganapathy V, Smith SB, Prasad PD.#TITLE:SLC19: the folate/thiamine transporter family.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:14770311# GENE ASSOCIATION: (80704.1) or (10560.1)
Thiamine transport in via proton antiport
Adrenaline secretion via secretory vesicle (ATP driven)
Abbreviation: R_ADRNLPVESSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) adrnl[c] + (2) atp[c] + (2) h2o[c] --> (2) adp[c] + (3) adrnl[e] + (2) h[c] + (2) pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of adrnl from the cell. It leaves out the vesicle because that is not a compartment. ATP:H ratio is assumed as 1:3 for vacuolar ATPase and substrate:h ratio is 1:2. This gives a 3:2 ratio for substrate:ATP genes: LOCUS:_6571#TRANSCRIPT:1#ABBREVIATION:SLC18A2# LOCUS:_6570#TRANSCRIPT:1#ABBREVIATION:SLC18A1# proteins: NAME:solute carrier family 18 (vesicular monoamine), member 1#ABBREVIATION:Slc18a1.1# NAME:solute carrier family 18 (vesicular monoamine), member 2#ABBREVIATION:Slc18a2.1# citations: AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# AUTHORS:Eiden LE, Schafer MK, Weihe E, Schutz B#TITLE:The vesicular amine transporter family (SLC18): amine/proton antiporters required for vesicular accumulation and regulated exocytotic secretion of monoamines and acetylcholine#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12827358# GENE ASSOCIATION: (6570.1) or (6571.1)
Abbreviation: R_ARACHCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : arachcoa + crn --> arachcrn + coa Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_XYLTer Synonyms: _7 EC Number: 2.4.2.26 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [r] : Ser-Gly/Ala-X-Gly + udpxyl --> h + udp + xser Confidence Level: 4 NCD genes: LOCUS:_64132#TRANSCRIPT:1#ABBREVIATION:XYLT2# LOCUS:_64131#TRANSCRIPT:1#ABBREVIATION:XYLT1# proteins: NAME:xylosyltransferase I, endoplasmic reticulum#ABBREVIATION:Xylt1.1-er# NAME:xylosyltransferase II, endoplasmic reticulum#ABBREVIATION:Xylt2.1-er# citations: AUTHORS:Kuhn J, Gotting C, Schnolzer M, Kempf T, Brinkmann T, Kleesiek K#TITLE:First isolation of human UDP-D-xylose: proteoglycan core protein beta-D-xylosyltransferase secreted from cultured JAR choriocarcinoma cells#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11087729# AUTHORS:Gotting C, Kuhn J, Zahn R, Brinkmann T, Kleesiek K#TITLE:Molecular cloning and expression of human UDP-d-Xylose:proteoglycan core protein beta-d-xylosyltransferase and its first isoform XT-II#JOURNAL:J Mol Biol#YEAR:2000#PUBMED:11099377# AUTHORS:Kearns AE, Vertel BM, Schwartz NB#TITLE:Topography of glycosylation and UDP-xylose production#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8496172# AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# GENE ASSOCIATION: (64131.1) or (64132.1)
UDP-xylose:protein xylosyltransferase, endoplasmic reticulum
Abbreviation: R_EX_xoltri25_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : xoltri25 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_7-alpha,25-Dihydroxycholesterol exchange
Abbreviation: R_TETPENT3CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + tetpent3crn --> crn + tetpent3coa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_ARTFR52 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : hexccoa --> (1.625) R5coa_hs Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TRIODTHYSUFt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: triodthysuf[c] --> triodthysuf[e] Confidence Level: 1 SAB excretion genes: none proteins: none citations: none GENE ASSOCIATION:
Triiodothyronine sulfate transport (diffusion)
glucose-1-phosphate thymidylyltransferase
Abbreviation: R_G1PTT Synonyms: _6 EC Number: 2.7.7.24 SUBSYSTEM: Nucleotide Sugar Metabolism Equation: [c] : dttp + g1p + h --> dtdpglu + ppi Confidence Level: 2 JLR genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_GGH_DASH_6THFl Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [l] : 6thf + h2o --> 5thf + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-l# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (6THF), lysosomal
Abbreviation: R_DNDPt15m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgdp[m] + dudp[c] --> dgdp[c] + dudp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_csn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : csn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CSPG_At Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cspg_a[g] --> cspg_a[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate A transport, golgi to extracellular
Abbreviation: R_EX_aldstrn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : aldstrn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DEDOLP2_L Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dedolp_L + h2o --> (0.1) dedol_L + pi Confidence Level: 0 EC-3.6.1.- NCD genes: none proteins: none citations: AUTHORS:Schenk B, Fernandez F, Waechter CJ.#TITLE:The ins(ide) and out(side) of dolichyl phosphate biosynthesis and recycling in the endoplasmic reticulum.#JOURNAL:Glycobiology#YEAR:2001#PUBMED:11425794# GENE ASSOCIATION:
dehydrodolichol phosphate phosphatase (liver)
Abbreviation: R_GALK Synonyms: _0 EC Number: 2.7.1.6 SUBSYSTEM: Galactose metabolism Equation: [c] : atp + gal --> adp + gal1p + h Confidence Level: 0 genes: LOCUS:_2584#TRANSCRIPT:1#ABBREVIATION:GALK1#ECNUMBERS:2.7.1.6# LOCUS:_2585#TRANSCRIPT:1#ABBREVIATION:GALK2#ECNUMBERS:2.7.1.6# LOCUS:_2585#TRANSCRIPT:2#ABBREVIATION:GALK2#ECNUMBERS:2.7.1.6# proteins: NAME:galactokinase 1#ABBREVIATION:Galk1.1# NAME:galactokinase 2#ABBREVIATION:Galk2.1# NAME:galactokinase 2#ABBREVIATION:Galk2.2# citations: none GENE ASSOCIATION: (2584.1) or (2585.2) or (2585.1)
Abbreviation: R_3SALATAi Synonyms: _0 SUBSYSTEM: Cysteine Metabolism Equation: [c] : 3sala + akg + h --> 3snpyr + glu-L Confidence Level: 2 MM genes: LOCUS:_2805#TRANSCRIPT:1#ABBREVIATION:GOT1#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase #ABBREVIATION:Got1.1# citations: none GENE ASSOCIATION: (2805.1)
_3-sulfino-alanine transaminase (irreversible)
Abbreviation: R_HSD3B3r Synonyms: _0 SUBSYSTEM: Steroid Metabolism Equation: [r] : eandrstrn + h + nadph --> andrstandn + h2o + nadp Confidence Level: 4 see PMID: 14643063 specific cofactors/etc have not yet been determined - largely inferred NJ genes: LOCUS:_3284#TRANSCRIPT:1#ABBREVIATION:HSD3B2#ECNUMBERS:1.1.1.145,,5.3.# proteins: NAME:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 2#ABBREVIATION:Hsd3b2.1# citations: AUTHORS:Belanger A, Pelletier G, Labrie F, Barbier O, Chouinard S.#TITLE:Inactivation of androgens by UDP-glucuronosyltransferase enzymes in humans.#JOURNAL:Trends Endocrinol Metab.#YEAR:2003#PUBMED:14643063# AUTHORS:Luu-The V.#TITLE:Analysis and characteristics of multiple types of human 17beta-hydroxysteroid dehydrogenase.#JOURNAL:J Steroid Biochem Mol Biol. #YEAR:2001#PUBMED:11384872# GENE ASSOCIATION: (3284.1)
Abbreviation: R_GBSIDEtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: gbside_hs[c] <==> gbside_hs[l] Confidence Level: 1 unknown mechanism for transport - not known if it is energy dependent or not, recycling and IC transport is important to get it to other areas of the cell (e.g. plasma membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DNDPt54m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[m] + dctp[c] --> dadp[c] + dctp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_etoh_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : etoh <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S2T2g Synonyms: _0 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_d_pre5 + paps --> cspg_d + h + pap Confidence Level: 4 EC 2.8.2- NCD genes: LOCUS:_10090#TRANSCRIPT:1#ABBREVIATION:UST# proteins: NAME:uronyl-2-sulfotransferase, Golgi apparatus#ABBREVIATION:Ust.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Kobayashi M, Sugumaran G, Liu J, Shworak NW, Silbert JE, Rosenberg RD#TITLE:Molecular cloning and characterization of a human uronyl 2-sulfotransferase that sulfates iduronyl and glucuronyl residues in dermatan/chondroitin sulfate#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10187838# GENE ASSOCIATION: (10090.1)
_3-Methoxy-4-hydroxyphenylacetaldehyde:NAD+ oxidoreductase
Abbreviation: R_3M4HDXPAC Synonyms: _0 EC Number: 1.2.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 3mox4hpac + h2o + nad <==> (2) h + homoval + nadh Confidence Level: 1 SAB genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: none GENE ASSOCIATION: (218.1) or (221.1) or (222.1) or (220.1)
Abbreviation: R_ACCOALm Synonyms: _1 EC Number: 6.2.1.17 SUBSYSTEM: Propanoate Metabolism Equation: [m] : atp + coa + ppa --> amp + ppcoa + ppi Confidence Level: 2 TDV-mitochondrial version JLR- also acts on propanoate AMF- made reaction to create AMP not ADP and changed EC genes: LOCUS:_84532#TRANSCRIPT:1#ABBREVIATION:ACAS2L# proteins: NAME:acetyl-CoA synthetase 2 (AMP-forming)-like, mitochondrial#ABBREVIATION:Acas2l.1-m# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Fujino T, Kondo J, Ishikawa M, Morikawa K, Yamamoto TT#TITLE:Acetyl-CoA synthetase 2, a mitochondrial matrix enzyme involved in the oxidation of acetate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11150295# GENE ASSOCIATION: (84532.1)
Abbreviation: R_FA161ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + hdeACP <==> ACP + h + hdcea Confidence Level: 4 NCD genes: LOCUS:_55301#TRANSCRIPT:1#ABBREVIATION:THEDC1# proteins: NAME:s-acyl fatty acid synthase thioesterase#ABBREVIATION:Thedc1.1# citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION: (55301.1)
Abbreviation: R_ARACHDtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: arachd[c] <==> arachd[r] Confidence Level: 1 unknown mechanism, presumed diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SPODMm Synonyms: _1 EC Number: 1.15.1.1 SUBSYSTEM: ROS Detoxification Equation: [m] : (2) h + (2) o2s --> h2o2 + o2 Confidence Level: 2 TV (8-15-2003) genes: LOCUS:_6648#TRANSCRIPT:1#ABBREVIATION:SOD2#ECNUMBERS:1.15.1.1# proteins: NAME:superoxide dismutase (Mn)#ABBREVIATION:Sod2.1-m# citations: AUTHORS:Folz RJ, Guan J, Seldin MF, Oury TD, Enghild JJ, Crapo JD.#TITLE:Mouse extracellular superoxide dismutase: primary structure, tissue-specific gene expression, chromosomal localization, and lung in situ hybridization.#JOURNAL:Am J Respir Cell Mol Biol.#YEAR:1997#PUBMED:9376114# AUTHORS:Elam JS, Malek K, Rodriguez JA, Doucette PA, Taylor AB, Hayward LJ, Cabelli DE, Valentine JS, Hart PJ.#TITLE:An alternative mechanism of bicarbonate-mediated peroxidation by copper-zinc superoxide dismutase: rates enhanced via proposed enzyme-associated peroxycarbonate intermediate.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12649272# GENE ASSOCIATION: (6648.1)
Abbreviation: R_EX_adprbp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : adprbp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_STRDNCCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + strdnccoa --> coa + strdnccrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_DM_dttp_LPAREN_n_RPAREN_ Synonyms: _0 Equation: [n] : dttp --> Confidence Level: IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ENGASE3ly Synonyms: _15 EC Number: 3.2.1.96 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg2 --> acgam + ksi_deg3 Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FRUt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fru[e] + na1[e] <==> fru[c] + na1[c] Confidence Level: 4 NCD genes: LOCUS:_200010#TRANSCRIPT:1#ABBREVIATION:SLC5A9# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 9, extracellular#ABBREVIATION:Slc5a9.1-e# citations: AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# AUTHORS:Tazawa S, Yamato T, Fujikura H, Hiratochi M, Itoh F, Tomae M, Takemura Y, Maruyama H, Sugiyama T, Wakamatsu A, Isogai T, Isaji M#TITLE:SLC5A9/SGLT4, a new Na+-dependent glucose transporter, is an essential transporter for mannose, 1,5-anhydro-D-glucitol, and fructose#JOURNAL:Life Sci#YEAR:2005#PUBMED:15607332# GENE ASSOCIATION: (200010.1)
D-fructose transport via sodium cotransport
Abbreviation: R_DUTPDPm Synonyms: _0 EC Number: 3.6.1.23 SUBSYSTEM: Nucleotides Equation: [m] : dutp + h2o --> dump + h + ppi Confidence Level: 4 genes: LOCUS:_1854#TRANSCRIPT:1#ABBREVIATION:DUT#ECNUMBERS:3.6.1.23# proteins: NAME:dUTP pyrophosphatase #ABBREVIATION:Dut.1-m# citations: AUTHORS:Tinkelenberg BA, Fazzone W, Lynch FJ, Ladner RD. #TITLE:Identification of sequence determinants of human nuclear dUTPase isoform localization.#JOURNAL:Exp Cell Res#YEAR:2003#PUBMED:12799180# AUTHORS:Cohen D, Heng HH, Shi XM, McIntosh EM, Tsui LC, Pearlman RE.#TITLE:Assignment of the human dUTPase gene (DUT) to chromosome 15q15-q21. 1 by fluorescence in situ hybridization. #JOURNAL:Genomics#YEAR:1997#PUBMED:9070952# AUTHORS:Ladner RD, McNulty DE, Carr SA, Roberts GD, Caradonna SJ.#TITLE:Characterization of distinct nuclear and mitochondrial forms of human deoxyuridine triphosphate nucleotidohydrolase.#JOURNAL:J Biol Chem. #YEAR:1996#PUBMED:8631816# GENE ASSOCIATION: (1854.1)
Abbreviation: R_TAURt4_LPAREN_2_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) na1[e] + taur[e] <==> (2) na1[c] + taur[c] Confidence Level: 4 SAB genes: LOCUS:_6533#TRANSCRIPT:1#ABBREVIATION:SLC6A6# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, taurine), member 6#ABBREVIATION:Slc6a6.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (6533.1)
taurine transport (sodium symport) (2:1)
Abbreviation: R_FUT32g Synonyms: _5 EC Number: 2.4.1.65 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acngalacglcgalgluside_hs + gdpfuc --> fucacngalacglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2525#TRANSCRIPT:1#ABBREVIATION:FUT3#ECNUMBERS:2.4.1.65# proteins: NAME:fucosyltransferase 3 (galactoside 3(4)-L-fucosyltransferase, Lewis blood group)#ABBREVIATION:Fut3.1# citations: AUTHORS:Cakir B, Pankow JS, Salomaa V, Couper D, Morris TL, Brantley KR, Hiller KM, Heiss G, Weston BW.#TITLE:Distribution of Lewis (FUT3)genotype and allele: frequencies in a biethnic United States population.#JOURNAL:Ann Hematol#YEAR:2002#PUBMED:12424536# AUTHORS:Sousa VL, Brito C, Costa T, Lanoix J, Nilsson T, Costa J.#TITLE:Importance of Cys, Gln, and Tyr from the transmembrane domain of human alpha 3/4 fucosyltransferase III for its localization and sorting in the Golgi of baby hamster kidney cells.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12493760# AUTHORS:Kukowska-Latallo JF, Larsen RD, Nair RP, Lowe JB.#TITLE:A cloned human cDNA determines expression of a mouse stage-specific embryonic antigen and the Lewis blood group alpha(1,3/1,4)fucosyltransferase.#JOURNAL:Genes Dev#YEAR:1990#PUBMED:1977660# GENE ASSOCIATION: (2525.1)
Abbreviation: R_UMPK5 Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : datp + ump <==> dadp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_EX_gthox_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gthox <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MM7B2g Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m7masnB --> m6masnB2 + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
Abbreviation: R_CHOLK Synonyms: _0 EC Number: 2.7.1.32 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : atp + chol --> adp + cholp + h Confidence Level: 4 NCD genes: LOCUS:_1119#TRANSCRIPT:1#ABBREVIATION:CHK#ECNUMBERS:2.7.1.32# LOCUS:_1120#TRANSCRIPT:1#ABBREVIATION:CHKL#ECNUMBERS:2.7.1.32,2.7.1.82# LOCUS:_1119#TRANSCRIPT:2#ABBREVIATION:CHK# LOCUS:_1120#TRANSCRIPT:2#ABBREVIATION:CHKL# proteins: NAME:choline kinase#ABBREVIATION:Chk.1# NAME:choline kinase#ABBREVIATION:Chk.2# NAME:choline/ethanolamine kinase#ABBREVIATION:Chkl.1# NAME:choline/ethanolamine kinase#ABBREVIATION:Chkl.2# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Activity and tissue distribution of splice variants of alpha6-fucosyltransferase in human embryogenesis.#TITLE:Martinez-Duncker I, Michalski JC, Bauvy C, Candelier JJ, Mennesson B, Codogno P, Oriol R, Mollicone R.#JOURNAL:Glycobiology#YEAR:2004#PUBMED:14514715# AUTHORS:Kent C#TITLE:Eukaryotic phospholipid biosynthesis#JOURNAL:Annu Rev Biochem#YEAR:1995# GENE ASSOCIATION: (1120.2) or (1119.1) or (1120.1) or (1119.2)
chondroitin sulfate E (GalNAc4,6diS-GlcA), degradation product 6_C31H48NO32S2
Abbreviation: R_PAIL4P_HStn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: pail4p_hs[c] <==> pail4p_hs[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
phosphatidylinositol 4-phosphate nuclear transport (diffusion)
Abbreviation: R_AKGMALtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: akg[m] + mal-L[c] <==> akg[c] + mal-L[m] Confidence Level: 4 TV genes: LOCUS:_8402#TRANSCRIPT:1#ABBREVIATION:SLC25A11# proteins: NAME:Solute carrier family 25 (oxoglutarate carrier) member 11, mitochondrial #ABBREVIATION:Slc25a11.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (8402.1)
Abbreviation: R_DPHMBDCm Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 3dpdhb_me + h --> 2dp6mep + co2 Confidence Level: 2 enzyme has not been identified yet IT genes: none proteins: none citations: AUTHORS:Nambudiri AM, Ranganathan S, Rudney H#TITLE:The role of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in the regulation of ubiquinone synthesis in human fibroblasts. #JOURNAL:J Biol Chem.#YEAR:1980#PUBMED:7380842# AUTHORS:Szkopinska A.#TITLE:Ubiquinone. Biosynthesis of quinone ring and its isoprenoid side chain. Intracellular localization.#JOURNAL:Acta Biochim Pol. #YEAR:2000#PUBMED:11051212# GENE ASSOCIATION:
_3-Decaprenyl-4-hydroxy-5-methoxybenzoate decarboxylation
Abbreviation: R_RTOT5 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : R5coa_hs --> Rtotalcoa Confidence Level: 0 User defined, tissue specific fluxes that can be customized w/ various ratios of R1 through R5 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_C2M26DCOAHLm Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: Limonene and pinene degradation Equation: [m] : c2m26dcoa + h2o <==> 3h26dm5coa Confidence Level: 1 SAB genes: LOCUS:_3030#TRANSCRIPT:1#ABBREVIATION:HADHA#ECNUMBERS:4.2.1.17,1.1.1.35# LOCUS:_3032#TRANSCRIPT:1#ABBREVIATION:HADHB# LOCUS:_549#TRANSCRIPT:1#ABBREVIATION:AUH# proteins: NAME:AU RNA binding protein/enoyl-Coenzyme A hydratase (mito)#ABBREVIATION:Auh.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit #ABBREVIATION:Hadha.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit #ABBREVIATION:Hadhb.1-m# citations: none GENE ASSOCIATION: (3030.1) and (3032.1) or (549.1)
cis-2-Methyl-5-isopropylhexa-2,5-dienoyl-CoA hydro-lyase (m)
Abbreviation: R_CSPG_Dt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cspg_d[g] --> cspg_d[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate D transport, golgi to extracellular
Abbreviation: R_MCD Synonyms: _0 EC Number: 4.1.1.9 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h + malcoa --> accoa + co2 Confidence Level: 4 TDV (9/14/2004) NJ added cytoplasmic reaction removed "c" -- former "MCD" has been changed to "MCDm" to denote mitochondrial localization - NCD genes: LOCUS:_23417#TRANSCRIPT:1#ABBREVIATION:MLYCD#ECNUMBERS:4.1.1.9# proteins: NAME:malonyl-CoA decarboxylase#ABBREVIATION:Mlycd.1# citations: AUTHORS:Kerner J, Hoppel CL.#TITLE:Radiochemical malonyl-CoA decarboxylase assay: activity and subcellular distribution in heart and skeletal muscle.#JOURNAL:Anal Biochem#YEAR:2002#PUBMED:12123667# AUTHORS:Wightman PJ, Santer R, Ribes A, Dougherty F, McGill N, Thorburn DR, FitzPatrick DR#TITLE:MLYCD mutation analysis: evidence for protein mistargeting as a cause of MLYCD deficiency#JOURNAL:Hum Mutat#YEAR:2003#PUBMED:12955715# GENE ASSOCIATION: (23417.1)
Abbreviation: R_RDH3a Synonyms: _0 EC Number: 1.1.1.105 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : nadp + retinol-cis-11 <==> h + nadph + retinal-11-cis Confidence Level: 4 IT genes: LOCUS:_145226#TRANSCRIPT:1#ABBREVIATION:RDH12# LOCUS:_57665#TRANSCRIPT:1#ABBREVIATION:RDH14# LOCUS:_112724#TRANSCRIPT:1#ABBREVIATION:RDH13# LOCUS:_51109#TRANSCRIPT:1#ABBREVIATION:RDH11#ECNUMBERS:1.1.1.-# LOCUS:_195814#TRANSCRIPT:1#ABBREVIATION:RDHE2# proteins: NAME:retinol dehydrogenase 12 (all-trans and 9-cis) #ABBREVIATION:Rdh12.1# NAME:retinol dehydrogenase 14 (all-trans and 9-cis) #ABBREVIATION:Rdh14.1# NAME:retinol dehydrogenase 11 (all-trans and 9-cis) #ABBREVIATION:Rdh11.1# NAME:retinol dehydrogenase 13 (all-trans and 9-cis) #ABBREVIATION:Rdh13.1# NAME:epidermal retinal dehydrogenase 2 #ABBREVIATION:Rdhe2.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Haeseleer F, Jang GF, Imanishi Y, Driessen CA, Matsumura M, Nelson PS, Palczewski K.#TITLE:Dual-substrate specificity short chain retinol dehydrogenases from the vertebrate retina.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12226107# AUTHORS:Janecke AR, Thompson DA, Utermann G, Becker C, Hubner CA, Schmid E, McHenry CL, Nair AR, Ruschendorf F, Heckenlively J, Wissinger B, Nurnberg P, Gal #TITLE:Mutations in RDH12 encoding a photoreceptor cell retinol dehydrogenase cause childhood-onset severe retinal dystrophy. #JOURNAL:Nat Genet. #YEAR:2004#PUBMED:15258582# AUTHORS:Perrault I, Hanein S, Gerber S, Barbet F, Ducroq D, Dollfus H, Hamel C, Dufier JL, Munnich A, Kaplan J, Rozet JM. #TITLE:Retinal dehydrogenase 12 (RDH12) mutations in leber congenital amaurosis. #JOURNAL:Am J Hum Genet#YEAR:2004#PUBMED:15322982# AUTHORS:Matsuzaka Y, Okamoto K, Tsuji H, Mabuchi T, Ozawa A, Tamiya G, Inoko H.#TITLE:Identification of the hRDH-E2 gene, a novel member of the SDR family, and its increased expression in psoriatic lesion. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2002#PUBMED:12372410# GENE ASSOCIATION: (112724.1) or (145226.1) or (51109.1) or (57665.1) or (195814.1)
Abbreviation: R_DNDPt59m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[m] + dgtp[c] --> dadp[c] + dgtp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_AG13T5g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre3 + uacgam --> h + ksi_pre4 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
Abbreviation: R_C2M26DCOAHLx Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: Limonene and pinene degradation Equation: [x] : c2m26dcoa + h2o <==> 3h26dm5coa Confidence Level: 1 SAB genes: LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_1891#TRANSCRIPT:1#ABBREVIATION:ECH1# proteins: NAME:enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase (x)#ABBREVIATION:Ehhadh.1-x# NAME:enoyl Coenzyme A hydratase 1, peroxisomal #ABBREVIATION:Ech1.1-x# citations: none GENE ASSOCIATION: (1891.1) or (1962.1)
cis-2-Methyl-5-isopropylhexa-2,5-dienoyl-CoA hydro-lyase (x)
_1-alkyl 2-acylglycerol 3-phosphocholine_C10H20O5NPFULLRFULLR2CO
Abbreviation: R_ST3GAL23g Synonyms: _7 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gd1b_hs --> cmp + gt1b_hs + h Confidence Level: 4 NJ genes: LOCUS:_6483#TRANSCRIPT:1#ABBREVIATION:SIAT4B#ECNUMBERS:2.4.99.4# proteins: NAME:sialyltransferase 4B, Golgi apparatus#ABBREVIATION:Siat4b.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Giordanengo V, Bannwarth S, Laffont C, Van Miegem V, Harduin-Lepers A, Delannoy P, Lefebvre JC. #TITLE:Cloning and expression of cDNA for a human Gal(beta1-3)GalNAc alpha2,3-sialyltransferase from the CEM T-cell line. #JOURNAL:Eur J Biochem#YEAR:1997#PUBMED:9266697# GENE ASSOCIATION: (6483.1)
Abbreviation: R_DNADtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: dnad[c] <==> dnad[n] Confidence Level: 2 it genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DHCR241r Synonyms: _3 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : fadh2 + zymst --> fad + zymstnl Confidence Level: 4 lanosterol -> cholesterol pathway FAD known to be cofactor, other details missing NJ genes: LOCUS:_1718#TRANSCRIPT:1#ABBREVIATION:DHCR24# proteins: NAME:_24-dehydrocholesterol reductase#ABBREVIATION:Dhcr24.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (1718.1)
Abbreviation: R_EX_leuktrC4_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : leuktrC4 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_hxan_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hxan <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FPGS Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [c] : (4) atp + (4) glu-L + thf --> 5thf + (4) adp + (4) h + (4) pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_SRTNt6_LPAREN_2_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) k[c] + (2) na1[e] + srtn[e] <==> (2) k[e] + (2) na1[c] + srtn[c] Confidence Level: 4 SAB genes: LOCUS:_6532#TRANSCRIPT:1#ABBREVIATION:SLC6A4# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, serotonin), member 4#ABBREVIATION:Slc6a4.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (6532.1)
Seratonin reversible transport in via sodium symport/potassium antiport (1:2)
Abbreviation: R_LPASE Synonyms: _0 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : h2o + lpchol_hs --> Rtotal + g3pc + h Confidence Level: 4 phosphatidyl choline degradation NJ genes: LOCUS:_5321#TRANSCRIPT:1#ABBREVIATION:PLA2G4A# LOCUS:_1178#TRANSCRIPT:1#ABBREVIATION:CLC# LOCUS:_29124#TRANSCRIPT:1#ABBREVIATION:LGALS13# proteins: NAME:phospholipase A2, group IVA#ABBREVIATION:Pla2g4a.1# NAME:lectin, galactoside-binding, soluble, 13 (galectin 13)#ABBREVIATION:Lgals13# NAME:Charcot-Leyden crystal protein#ABBREVIATION:Clc.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Perisic O, Fong S, Lynch DE, Bycroft M, Williams RL.#TITLE:Crystal structure of a calcium-phospholipid binding domain from cytosolic phospholipase A2.#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9430701# GENE ASSOCIATION: (5321.1) or (1178.1) or (29124.1)
Abbreviation: R_THMTP Synonyms: _0 EC Number: 3.6.1.28 SUBSYSTEM: Thiamine Metabolism Equation: [c] : h2o + thmtp --> h + pi + thmpp Confidence Level: 4 _01-25-05 IT genes: LOCUS:_79178#TRANSCRIPT:1#ABBREVIATION:THTPA# proteins: NAME:thiamine triphosphatase #ABBREVIATION:ThmtP.1# citations: AUTHORS:Bettendorff L, Mastrogiacomo F, Kish SJ, Grisar T.#TITLE:Thiamine, thiamine phosphates, and their metabolizing enzymes in human brain. #JOURNAL:J Neurochem. #YEAR:1996#PUBMED:8522961# AUTHORS:Lakaye B, Makarchikov AF, Wins P, Margineanu I, Roland S, Lins L, Aichour R, Lebeau L, El Moualij B, Zorzi W, Coumans B, Grisar T, Bettendorff L.#TITLE:Human recombinant thiamine triphosphatase: purification, secondary structure and catalytic properties.#JOURNAL:Int J Biochem Cell Biol.#YEAR:2004#PUBMED:15109578# GENE ASSOCIATION: (79178.1)
Abbreviation: R_EX_lac_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lac-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CORE5GTg Synonyms: _7 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : Tn_antigen + udpacgal --> core5 + h + udp Confidence Level: 0 EC 2.4.1.- NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Core 5 alpha-GalNAc-transferase, Golgi apparatus
Abbreviation: R_PHYTt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: phyt[e] <==> phyt[c] Confidence Level: 2 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ECOAH1x Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: Tryptophan metabolism Equation: [x] : 3hbcoa <==> b2coa + h2o Confidence Level: 2 SAB for x genes: LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_1891#TRANSCRIPT:1#ABBREVIATION:ECH1# proteins: NAME:enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase (x)#ABBREVIATION:Ehhadh.1-x# NAME:enoyl Coenzyme A hydratase 1, peroxisomal #ABBREVIATION:Ech1.1-x# citations: AUTHORS:FitzPatrick DR, Germain-Lee E, Valle D.#TITLE:Isolation and characterization of rat and human cDNAs encoding a novel putative peroxisomal enoyl-CoA hydratase. #YEAR:1995#PUBMED:7558027# GENE ASSOCIATION: (1891.1) or (1962.1)
_3-hydroxyacyl-CoA dehydratase (3-hydroxybutanoyl-CoA) (perioxisome)
Abbreviation: R_EX_6htststerone_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 6htststerone <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ARTPLM1 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : Rtotalcoa <==> pmtcoa Confidence Level: 0 cytosolic conversion of pooled FA into palmitate (so subsequent transport into mit w/ FA oxidation can occur). Since Carbon stoichiometry normalized wrt C16 ratio between Rgroupcoa and pmtcoa is 1:1 HR fatty acid metabolism - R groups = acyl groups, Rtotal, Rtotal2, Rtotal3 correspond to three possible acyl groups added to glycerol R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_acetone_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acetone <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
L-serine/L-asparagine Na-dependent exchange (Asn-L in)
Abbreviation: R_ASNSERNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[e] + na1[e] + ser-L[c] --> asn-L[c] + na1[c] + ser-L[e] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
Abbreviation: R_ARACHCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : arachcrn + coa --> arachcoa + crn Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_ARABR Synonyms: _0 EC Number: 1.1.1.21 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : arab-L + h + nadph --> abt + nadp Confidence Level: 4 NCD genes: LOCUS:_10327#TRANSCRIPT:1#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# LOCUS:_231#TRANSCRIPT:1#ABBREVIATION:AKR1B1#ECNUMBERS:1.1.1.21# LOCUS:_8574#TRANSCRIPT:1#ABBREVIATION:AKR7A2# LOCUS:_10327#TRANSCRIPT:2#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# proteins: NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.1# NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.2# NAME:aldo-keto reductase family 1, member B1 (aldose reductase)#ABBREVIATION:Akr1b1.1# NAME:aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)#ABBREVIATION:Akr7a2.1# citations: AUTHORS:Onkenhout W, Groener JE, Verhoeven NM, Yin C, Laan LA#TITLE:L-Arabinosuria: a new defect in human pentose metabolism#JOURNAL:Mol Genet Metab#YEAR:2002#PUBMED:12359133# AUTHORS:Huck JH, Roos B, Jakobs C, van der Knaap MS, Verhoeven NM#TITLE:Evaluation of pentitol metabolism in mammalian tissues provides new insight into disorders of human sugar metabolism#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15234337# AUTHORS:Kelly VP, Sherratt PJ, Crouch DH, Hayes JD#TITLE:Novel homodimeric and heterodimeric rat gamma-hydroxybutyrate synthases that associate with the Golgi apparatus define a distinct subclass of aldo-keto reductase 7 family proteins.#JOURNAL:Biochem J#YEAR:2002#PUBMED:12071861# AUTHORS:Praml C, Savelyeva L, Perri P, Schwab M.#TITLE:Cloning of the human aflatoxin B1-aldehyde reductase gene at 1p35-1p36.1 in a region frequently altered in human tumor cells.#JOURNAL:Cancer Res#YEAR:1998#PUBMED:9823300# GENE ASSOCIATION: (8574.1) or (231.1) or (10327.2) or (10327.1)
Abbreviation: R_MCDp Synonyms: _0 EC Number: 4.1.1.9 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : h + malcoa --> accoa + co2 Confidence Level: 0 TDV (9/14/2004) NJ added peroxismal and cytoplasmic reactions genes: LOCUS:_23417#TRANSCRIPT:1#ABBREVIATION:MLYCD#ECNUMBERS:4.1.1.9# proteins: NAME:malonyl-CoA decarboxylase, peroxisomal#ABBREVIATION:Mlycd.1-p# citations: AUTHORS:Kerner J, Hoppel CL.#TITLE:Radiochemical malonyl-CoA decarboxylase assay: activity and subcellular distribution in heart and skeletal muscle.#JOURNAL:Anal Biochem#YEAR:2002#PUBMED:12123667# AUTHORS:Wightman PJ, Santer R, Ribes A, Dougherty F, McGill N, Thorburn DR, FitzPatrick DR#TITLE:MLYCD mutation analysis: evidence for protein mistargeting as a cause of MLYCD deficiency#JOURNAL:Hum Mutat#YEAR:2003#PUBMED:12955715# GENE ASSOCIATION: (23417.1)
Abbreviation: R_PCLAD Synonyms: _4 EC Number: 4.1.1.45 SUBSYSTEM: Tryptophan metabolism Equation: [c] : cmusa + h --> am6sa + co2 Confidence Level: 4 NCD genes: LOCUS:_130013#TRANSCRIPT:1#ABBREVIATION:ACMSD#ECNUMBERS:4.1.1.45# proteins: NAME:aminocarboxymuconate semialdehyde decarboxylase#ABBREVIATION:Acmsd.1# citations: AUTHORS:Fukuoka S, Ishiguro K, Yanagihara K, Tanabe A, Egashira Y, Sanada H, Shibata K#TITLE:Identification and expression of a cDNA encoding human alpha-amino-beta-carboxymuconate-epsilon-semialdehyde decarboxylase (ACMSD). A key enzyme for the tryptophan-niacine pathway and "quinolinate hypothesis"#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12140278# GENE ASSOCIATION: (130013.1)
S-Adenosyl-L-methionine:histamine N-tele-methyltransferase
Abbreviation: R_SAMHISTA Synonyms: _0 EC Number: 2.1.1.8 SUBSYSTEM: Histidine Metabolism Equation: [c] : amet + hista --> ahcys + h + mhista Confidence Level: 4 SAB R02155 genes: LOCUS:_3176#TRANSCRIPT:1#ABBREVIATION:HNMT#ECNUMBERS:2.1.1.8# proteins: NAME:histamine N-methyltransferase#ABBREVIATION:Hnmt.1# citations: AUTHORS:Yamauchi K, Sekizawa K, Suzuki H, Nakazawa H, Ohkawara Y, Katayose D, Ohtsu H, Tamura G, Shibahara S, Takemura M, et al#TITLE:Structure and function of human histamine N-methyltransferase: critical enzyme in histamine metabolism in airway#JOURNAL:Am J Physiol#YEAR:1994#PUBMED:7943261# GENE ASSOCIATION: (3176.1)
Abbreviation: R_XAO2x Synonyms: _7 SUBSYSTEM: Purine Catabolism Equation: [x] : h2o + hxan + o2 --> h2o2 + xan Confidence Level: 4 IT EC 1.17.3.2 genes: LOCUS:_7498#TRANSCRIPT:1#ABBREVIATION:XDH#ECNUMBERS:1.1.1.204# proteins: NAME:xanthine dehydrogenase #ABBREVIATION:Xdh.1-x# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (7498.1)
keratan sulfate I, degradation product 40_C40H68N2O31
Abbreviation: R_BILDGLCURt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: bildglcur[e] + hco3[c] <==> bildglcur[c] + hco3[e] Confidence Level: 4 NJ genes: LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# citations: AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# AUTHORS:Abe T, Kakyo M, Tokui T, Nakagomi R, Nishio T, Nakai D, Nomura H, Unno M, Suzuki M, Naitoh T, Matsuno S, Yawo H.#TITLE:Identification of a novel gene family encoding human liver-specific organic anion transporter LST-1.#JOURNAL:J Biol Chem.#YEAR:1999#PUBMED:10358072# GENE ASSOCIATION: (10599.1)
bilirubin beta-diglucuronide transport via bicarbonate countertransport
Abbreviation: R_URATEtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: urate[x] --> urate[c] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RNMK Synonyms: _0 EC Number: 2.7.1.22 SUBSYSTEM: NAD Metabolism Equation: [c] : atp + rnam --> adp + h + nmn Confidence Level: 4 genes: LOCUS:_54981#TRANSCRIPT:1#ABBREVIATION:C9orf95# proteins: NAME:Nicotinamide riboside kinase 1#ABBREVIATION:Nrk1.1# citations: AUTHORS:Bieganowski P, Brenner C.#TITLE:Discoveries of nicotinamide riboside as a nutrient and conserved NRK genes establish a Preiss-Handler independent route to NAD+ in fungi and humans.#JOURNAL:Cell#YEAR:2004#PUBMED:15137942# GENE ASSOCIATION: (54981.1)
Abbreviation: R_6THFtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _6thf[c] --> 6thf[l] Confidence Level: 2 carrier-mediated transport - but which and how is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
Abbreviation: R_NDPK7n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + dcdp <==> adp + dctp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:dCDP), nuclear
Abbreviation: R_DCK1n Synonyms: _0 EC Number: 2.7.1.74 SUBSYSTEM: Nucleotides Equation: [n] : atp + dcyt <==> adp + dcmp + h Confidence Level: 4 IT (2.7.1.74) genes: LOCUS:_1633#TRANSCRIPT:1#ABBREVIATION:DCK#ECNUMBERS:2.7.1.74# proteins: NAME:deoxycytidine kinase #ABBREVIATION:Dck.1# citations: AUTHORS:Turk B, Awad R, Usova EV, Bjork I, Eriksson S. #TITLE:A pre-steady-state kinetic analysis of substrate binding to human recombinant deoxycytidine kinase: a model for nucleoside kinase action. #JOURNAL:Biochemistry#YEAR:1999#PUBMED:10387103# AUTHORS:Johansson M, Brismar S, Karlsson A.#TITLE:Human deoxycytidine kinase is located in the cell nucleus.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9342341# AUTHORS:Krawiec K, Kierdaszuk B, Shugar D.#TITLE:Inorganic tripolyphosphate (PPP(i)) as a phosphate donor for human deoxyribonucleoside kinases. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12535661# GENE ASSOCIATION: (1633.1)
Abbreviation: R_GHMT3m Synonyms: _5 EC Number: 2.1.2.1 SUBSYSTEM: Lysine Metabolism Equation: [m] : 3htmelys + h --> 4tmeabut + gly Confidence Level: 2 MM-according to Hulse et. al (1978) genes: LOCUS:_6472#TRANSCRIPT:1#ABBREVIATION:SHMT2#ECNUMBERS:2.1.2.1# proteins: NAME:serine hydroxymethyltransferase 2 (mitochondrial) #ABBREVIATION:Shmt2.1-m# citations: AUTHORS:Hulse JD, Ellis SR, Henderson LM.#TITLE:Carnitine biosynthesis. beta-Hydroxylation of trimethyllysine by an alpha-ketoglutarate-dependent mitochondrial dioxygenase. #YEAR:1978#PUBMED:627563# GENE ASSOCIATION: (6472.1)
glycine hydroxymethyltransferase, mitochondrial
Abbreviation: R_GLYOp Synonyms: _0 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [x] : gly + h2o + o2 --> glx + h2o2 + nh4 Confidence Level: 4 genes: none proteins: none citations: AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# GENE ASSOCIATION:
Abbreviation: R_FE2tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: fe2[c] + h[c] --> fe2[m] + h[m] Confidence Level: 2 TDV Lange H, 1999. genes: none proteins: none citations: AUTHORS:Lange H, Kispal G, Lill R.#TITLE:Mechanism of iron transport to the site of heme synthesis inside yeast mitochondria.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10383398# AUTHORS:Chung J, Wessling-Resnick M#TITLE:Molecular mechanisms and regulation of iron transport.#JOURNAL:Crit Rev Clin Lab Sci#YEAR:2003#PUBMED:12755454# GENE ASSOCIATION:
keratan sulfate I biosynthesis, precursor 32_C227H362N16O192S10X
Abbreviation: R_FPGS4m Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [m] : (4) atp + dhf + (4) glu-L --> 5dhf + (4) adp + (4) h + (4) pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
folylpolyglutamate synthetase (DHF), mitochondrial
Abbreviation: R_ALAGLYexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + gly[c] <==> ala-L[c] + gly[e] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_FACOAL1832 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + lnlnca <==> amp + lnlncacoa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_TDCHOLAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: tdchola[x] <==> tdchola[c] Confidence Level: 2 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GP1Ctg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gp1c_hs[c] <==> gp1c_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_NH4tp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: nh4[c] <==> nh4[x] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FACOAL1822 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + lneldc <==> amp + lneldccoa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_GBSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gbside_hs[c] <==> gbside_hs[e] Confidence Level: 1 unknown mechanism for transport - not known if it is energy dependent or not, recycling and IC transport is important to get it to other areas of the cell (e.g. plasma membrane) - The goal of this transporter is to get globoside to the EC plasma membrane NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LINKDEG3ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_c_deg5 + (4) h2o --> (2) gal + glcur + h + so4 + xyl-D Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
Abbreviation: R_34DHXMANDACOX Synonyms: _0 EC Number: 1.2.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 34dhmald + h2o + nad --> 34dhoxmand + (2) h + nadh Confidence Level: 4 SAB genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# citations: AUTHORS:Sladek NE#TITLE:Human aldehyde dehydrogenases: potential pathological, pharmacological, and toxicological impact#JOURNAL:J Biochem Mol Toxicol#YEAR:2003#PUBMED:12616643# AUTHORS:Kawamura M, Eisenhofer G, Kopin IJ, Kador PF, Lee YS, Tsai JY, Fujisawa S, Lizak MJ, Sinz A, Sato S#TITLE:Aldose reductase, a key enzyme in the oxidative deamination of norepinephrine in rats#JOURNAL:Biochem Pharmacol#YEAR:1999#PUBMED:10424772# GENE ASSOCIATION: (221.1) or (218.1) or (220.1)
_3,4-Dihydroxymandelaldehyde:NAD+ oxidoreductase
Abbreviation: R_OBDHc Synonyms: _0 EC Number: 1.2.7.2 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : 2obut + coa + nad --> co2 + nadh + ppcoa Confidence Level: 2 as shown in Lehninger (4th ed.) for threonine degradation MM genes: none proteins: none citations: none GENE ASSOCIATION:
_2-Oxobutanoate dehydrogenase, cytosolic
Abbreviation: R_H2CO3D2 Synonyms: _0 SUBSYSTEM: Miscellaneous Equation: [c] : h + hco3 <==> h2co3 Confidence Level: 2 IT (pka (H2CO3) = 6.4) genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_FATP3t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[c] + ocdca[c] <==> na1[e] + ocdca[e] Confidence Level: 4 electroneutral transport NJ genes: LOCUS:_376497#TRANSCRIPT:1#ABBREVIATION:SLC27A1# LOCUS:_376497#TRANSCRIPT:2#ABBREVIATION:SLC27A1# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 1#ABBREVIATION:Slc27a1hs.1# NAME:solute carrier family 27 (fatty acid transporter), member 1#ABBREVIATION:Slc27a1hs.2# citations: AUTHORS:Stahl A.#TITLE:A current review of fatty acid transport proteins (SLC27).#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12856180# AUTHORS:Pohl J, Ring A, Hermann T, Stremmel W.#TITLE:Role of FATP in parenchymal cell fatty acid uptake.#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15522816# GENE ASSOCIATION: (376497.1) or (376497.2)
Abbreviation: R_NTPP10 Synonyms: _0 EC Number: 3.6.1.19 SUBSYSTEM: Nucleotides Equation: [c] : ditp + h2o --> dimp + h + ppi Confidence Level: 4 genes: LOCUS:_3704#TRANSCRIPT:1#ABBREVIATION:ITPA#ECNUMBERS:3.6.1.19# LOCUS:_3704#TRANSCRIPT:2#ABBREVIATION:ITPA#ECNUMBERS:3.6.1.19# proteins: NAME:inosine triphosphatase (nucleoside triphosphate pyrophosphatase) #ABBREVIATION:ItpA.1# NAME:inosine triphosphatase (nucleoside triphosphate pyrophosphatase) #ABBREVIATION:ItpA.2# citations: AUTHORS:Lin S, McLennan AG, Ying K, Wang Z, Gu S, Jin H, Wu C, Liu W, Yuan Y, Tang R, Xie Y, Mao Y. #TITLE:Cloning, expression, and characterization of a human inosine triphosphate pyrophosphatase encoded by the itpa gene. #JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11278832# GENE ASSOCIATION: (3704.1) or (3704.2)
Nucleoside triphosphate pyrophosphorylase (ditp)
Abbreviation: R_FALDtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: fald[l] <==> fald[c] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
formaldehyde transport via diffusion (lysosomall)
Abbreviation: R_CATp Synonyms: _0 EC Number: 1.11.1.6 SUBSYSTEM: Miscellaneous Equation: [x] : (2) h2o2 --> (2) h2o + o2 Confidence Level: 0 NCD genes: LOCUS:_847#TRANSCRIPT:1#ABBREVIATION:CAT#ECNUMBERS:1.11.1.6# proteins: NAME:catalase#ABBREVIATION:Cat.1-x# citations: none GENE ASSOCIATION: (847.1)
Abbreviation: R_KYN Synonyms: _0 EC Number: 3.7.1.3 SUBSYSTEM: Tryptophan metabolism Equation: [c] : Lkynr + h2o --> ala-L + anth + h Confidence Level: 4 NCD genes: LOCUS:_8942#TRANSCRIPT:1#ABBREVIATION:KYNU#ECNUMBERS:3.7.1.3# proteins: NAME:kynureninase (L-kynurenine hydrolase)#ABBREVIATION:Kynu.1# citations: AUTHORS:Toma S, Nakamura M, Tone S, Okuno E, Kido R, Breton J, Avanzi N, Cozzi L, Speciale C, Mostardini M, Gatti S, Benatti L#TITLE:Cloning and recombinant expression of rat and human kynureninase#JOURNAL:FEBS Lett#YEAR:1997#PUBMED:9180257# GENE ASSOCIATION: (8942.1)
Abbreviation: R_PDHm Synonyms: _0 EC Number: 1.2.4.1 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [m] : coa + nad + pyr --> accoa + co2 + nadh Confidence Level: 0 Lumped reaction: EC 1.2.4.1., EC 2.3.1.12 and EC 1.8.1.4 IF genes: LOCUS:_8050#TRANSCRIPT:1#ABBREVIATION:PDHX#ECNUMBERS:2.3.1.12# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_5160#TRANSCRIPT:1#ABBREVIATION:PDHA1#ECNUMBERS:1.2.4.1# LOCUS:_1737#TRANSCRIPT:1#ABBREVIATION:DLAT#ECNUMBERS:2.3.1.12# LOCUS:_5161#TRANSCRIPT:1#ABBREVIATION:PDHA2#ECNUMBERS:1.2.4.1# LOCUS:_5162#TRANSCRIPT:1#ABBREVIATION:PDHB#ECNUMBERS:1.2.4.1# proteins: NAME:pyruvate dehydrogenase (lipoamide), mitochondrial (testis)#ABBREVIATION:Pdha2b.1-m# NAME:pyruvate dehydrogenase (lipoamide), mitochondrial#ABBREVIATION:Pdha1b.1-m# NAME:dihydrolipoamide S-acetyltransferase (pyruvate dehydrogenase, E2 component), mitochondrial#ABBREVIATION:Dlat.1-m# NAME:dihydrolipoamide dehydrogenase (pyruvate dehydrogenase, E3 component), mitochondrial#ABBREVIATION:Dld/Pdhx.1-m# citations: none GENE ASSOCIATION: (1738.1 and 8050.1) and (5161.1 and 5162.1) and (1737.1) or (1738.1 and 8050.1) and (5160.1 and 5162.1) and (1737.1)
Abbreviation: R_DM_Ser_DASH_Gly_FSLASH_Ala_DASH_X_DASH_Gly_LPAREN_ly_RPAREN_ Synonyms: _0 Equation: [l] : Ser-Gly/Ala-X-Gly --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RETNtr2 Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: _13-cis-retn[c] --> 13-cis-retn[r] Confidence Level: 1 IT genes: none proteins: none citations: AUTHORS:Barua AB, Sidell N.#TITLE:Retinoyl beta-glucuronide: a biologically active interesting retinoid.#JOURNAL:J Nutr.#YEAR:2004#PUBMED:14704335# GENE ASSOCIATION:
Abbreviation: R_3SALAASPm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _3sala[m] + asp-L[c] <==> 3sala[c] + asp-L[m] Confidence Level: 2 PMID:486467 MM genes: LOCUS:_8604#TRANSCRIPT:1#ABBREVIATION:SLC25A12# LOCUS:_10165#TRANSCRIPT:1#ABBREVIATION:SLC25A13# proteins: NAME:Solute carrier family 25 member 12, mitochondrial #ABBREVIATION:Slc25a12.1-m# NAME: solute carrier family 25 member 13, mitochondrial #ABBREVIATION:Slc25a13.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (10165.1) or (8604.1)
cysteinesulfinate-aspartate mitochondrial shuttle
Nitric Oxide Synthase (intermediate forming)
Abbreviation: R_NOS1 Synonyms: _0 EC Number: 1.14.13.39 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : arg-L + h + nadph + o2 --> h2o + nadp + nwharg Confidence Level: 4 SAB genes: LOCUS:_4842#TRANSCRIPT:1#ABBREVIATION:NOS1#ECNUMBERS:1.14.13.39# LOCUS:_4843#TRANSCRIPT:1#ABBREVIATION:NOS2A#ECNUMBERS:1.14.13.39# LOCUS:_4846#TRANSCRIPT:1#ABBREVIATION:NOS3#ECNUMBERS:1.14.13.39# LOCUS:_4845#TRANSCRIPT:1#ABBREVIATION:NOS2C# LOCUS:_4844#TRANSCRIPT:1#ABBREVIATION:NOS2B# LOCUS:_4843#TRANSCRIPT:2#ABBREVIATION:NOS2A#ECNUMBERS:1.14.13.39# proteins: NAME:nitric oxide synthase 1 (neuronal)#ABBREVIATION:Nos1.1# NAME:nitric oxide synthase 2A (inducible, hepatocytes)#ABBREVIATION:Nos2a.1# NAME:nitric oxide synthase 2A (inducible, hepatocytes)#ABBREVIATION:Nos2a.2# NAME:nitric oxide synthase 2B#ABBREVIATION:Nos2b.1# NAME:nitric oxide synthase 2C#ABBREVIATION:Nos2c.1# NAME:nitric oxide synthase 3 (endothelial cell)#ABBREVIATION:Nos3.1# citations: AUTHORS:Brenman JE, Chao DS, Xia H, Aldape K, Bredt DS#TITLE:Nitric oxide synthase complexed with dystrophin and absent from skeletal muscle sarcolemma in Duchenne muscular dystrophy#JOURNAL:Cell#YEAR:1995#PUBMED:7545544# AUTHORS:Geoffrey M. Cooper#TITLE:The Cell A Molecular Approach#YEAR:2000# AUTHORS:Bloch KD, Wolfram JR, Brown DM, Roberts JD Jr, Zapol DG, Lepore JJ, Filippov G, Thomas JE, Jacob HJ, Bloch DB. #TITLE:Three members of the nitric oxide synthase II gene family (NOS2A, NOS2B, and NOS2C) colocalize to human chromosome 17. #JOURNAL:Genomics#YEAR:1995#PUBMED:7558036# AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# GENE ASSOCIATION: (4846.1) or (4844.1) or (4842.1) or (4843.2) or (4843.1) or (4845.1)
Abbreviation: R_PCLYSOX Synonyms: _0 EC Number: 1.14.11.4 SUBSYSTEM: Lysine Metabolism Equation: [c] : akg + o2 + pcollglys --> co2 + pcollg5hlys + succ Confidence Level: 4 MM unbalanced reaction genes: LOCUS:_5352#TRANSCRIPT:1#ABBREVIATION:PLOD2# LOCUS:_5351#TRANSCRIPT:1#ABBREVIATION:PLOD#ECNUMBERS:1.14.11.4# LOCUS:_8985#TRANSCRIPT:1#ABBREVIATION:PLOD3#ECNUMBERS:1.14.11.4# LOCUS:_5352#TRANSCRIPT:2#ABBREVIATION:PLOD2# proteins: NAME:procollagen-lysine, 2-oxoglutarate 5-dioxygenase (lysine hydroxylase, Ehlers-Danlos syndrome type VI) #ABBREVIATION:Plod.1# NAME:procollagen-lysine, 2-oxoglutarate 5-dioxygenase (lysine hydroxylase, Ehlers-Danlos syndrome type VI) #ABBREVIATION:Plod2.1# NAME:procollagen-lysine, 2-oxoglutarate 5-dioxygenase (lysine hydroxylase, Ehlers-Danlos syndrome type VI) #ABBREVIATION:Plod2.2# NAME:procollagen-lysine, 2-oxoglutarate 5-dioxygenase 3 #ABBREVIATION:Plod3.1# citations: none GENE ASSOCIATION: (8985.1) or (5352.2) or (5352.1) or (5351.1)
Procollagen-lysine 1, 2-oxoglutarate 5-dioxygenase
Abbreviation: R_EX_galgalgalthcrm_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : galgalgalthcrm_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Gal-Gal-Gal-Gal-Gal-Glc-Cer (homo sapiens) exchange
Abbreviation: R_AMCOXO Synonyms: _0 SUBSYSTEM: Tryptophan metabolism Equation: [c] : amuco + h + h2o + nadph --> 2oxoadp + nadp + nh4 Confidence Level: 4 not sure about name NCD genes: none proteins: none citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_DESAT18_5 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + nadh + o2 + stcoa --> (2) h2o + nad + od2coa Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_6319#TRANSCRIPT:1#ABBREVIATION:SCD# proteins: NAME:stearoyl-CoA desaturase#ABBREVIATION:Scd.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Zhang L, Ge L, Parimoo S, Stenn K, Prouty SM#TITLE:Human stearoyl-CoA desaturase: alternative transcripts generated from a single gene by usage of tandem polyadenylation sites#JOURNAL:Biochem J#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# AUTHORS:Wang Y, Kurdi-Haidar B, Oram JF.#TITLE:LXR-mediated activation of macrophage stearoyl-CoA desaturase generates unsaturated fatty acids that destabilize ABCA1.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:14967823# GENE ASSOCIATION: (6319.1)
Abbreviation: R_EX_Tyr_DASH_ggn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : Tyr-ggn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Tyr-194 of apo-glycogenin protein (primer for glycogen synthesis) exchange
Abbreviation: R_LEUKTRA4tr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: leuktrA4[c] <==> leuktrA4[r] Confidence Level: 1 unknown mechanism, presumed diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_pyr_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pyr <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DNDPt24m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[m] + dtdp[c] --> dadp[c] + dtdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_PMI1346PH Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + ppmi1346p --> h + mi13456p + pi Confidence Level: 4 NCD genes: LOCUS:_9807#TRANSCRIPT:1#ABBREVIATION:IHPK1# LOCUS:_117283#TRANSCRIPT:1#ABBREVIATION:IHPK3# LOCUS:_9807#TRANSCRIPT:2#ABBREVIATION:IHPK1# proteins: NAME:inositol hexaphosphate kinase 1#ABBREVIATION:Ihpk1.1# NAME:inositol hexaphosphate kinase 1#ABBREVIATION:Ihpk1.2# NAME:inositol hexaphosphate kinase 3#ABBREVIATION:Ihpk3.1# citations: AUTHORS:Saiardi A, Nagata E, Luo HR, Snowman AM, Snyder SH#TITLE:Identification and characterization of a novel inositol hexakisphosphate kinase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11502751# GENE ASSOCIATION: (117283.1) or (9807.2) or (9807.1)
diphosphoinositol-1,3,4,6-tetrakisphosphate diphosphohydrolase
Abbreviation: R_DNDPt43m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + dutp[c] --> atp[c] + dutp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_INSKm Synonyms: _0 EC Number: 2.7.1.73 SUBSYSTEM: Nucleotides Equation: [m] : atp + ins --> adp + h + imp Confidence Level: 1 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LNELDCCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + lneldccrn --> crn + lneldccoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_P45027A1m Synonyms: _4 SUBSYSTEM: Bile Acid Biosynthesis Equation: [m] : chsterol + h + nadph + o2 --> h2o + nadp + xol27oh Confidence Level: 4 genes: LOCUS:_1593#TRANSCRIPT:1#ABBREVIATION:CYP27A1# proteins: NAME:cytochrome P450, family 27, subfamily A, polypeptide 1#ABBREVIATION:Cyp27a1.1-m# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1593.1)
Abbreviation: R_DITPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: ditp[c] <==> ditp[n] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NaKt Synonyms: _7 EC Number: 3.6.3.9 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + (2) k[e] + (3) na1[c] --> adp[c] + h[c] + (2) k[c] + (3) na1[e] + pi[c] Confidence Level: 4 AMF TDV (8/25/2005, stoichiometry changed) genes: LOCUS:_23439#TRANSCRIPT:1#ABBREVIATION:ATP1B4# LOCUS:_481#TRANSCRIPT:1#ABBREVIATION:ATP1B1#ECNUMBERS:3.6.1.37# LOCUS:_483#TRANSCRIPT:1#ABBREVIATION:ATP1B3#ECNUMBERS:3.6.1.37# LOCUS:_476#TRANSCRIPT:1#ABBREVIATION:ATP1A1#ECNUMBERS:3.6.1.37# LOCUS:_482#TRANSCRIPT:1#ABBREVIATION:ATP1B2#ECNUMBERS:3.6.1.37# LOCUS:_477#TRANSCRIPT:1#ABBREVIATION:ATP1A2#ECNUMBERS:3.6.1.37# LOCUS:_478#TRANSCRIPT:1#ABBREVIATION:ATP1A3#ECNUMBERS:3.6.1.37# LOCUS:_480#TRANSCRIPT:1#ABBREVIATION:ATP1A4#ECNUMBERS:3.6.3.9# proteins: NAME:ATPase, (Na+)/K+ transporting (alpha1, beta1)#ABBREVIATION:Atp1a1b1# NAME:ATPase, (Na+)/K+ transporting (alpha1, beta2)#ABBREVIATION:Atp1a1b2# NAME:ATPase, (Na+)/K+ transporting (alpha1, beta3)#ABBREVIATION:Atp1a1b3# NAME:ATPase, (Na+)/K+ transporting (alpha1, beta4)#ABBREVIATION:Atp1a1b4# NAME:ATPase, (Na+)/K+ transporting (alpha2, beta1)#ABBREVIATION:Atp1a2b1# NAME:ATPase, (Na+)/K+ transporting (alpha3, beta1)#ABBREVIATION:Atp1a3b1# NAME:ATPase, Na+/K+ transporting (alpha4, beta1)#ABBREVIATION:Atp1a4b1# NAME:ATPase, Na+/K+ transporting (alpha2, beta2)#ABBREVIATION:Atp1a2b2# NAME:ATPase, Na+/K+ transporting (alpha3, beta2)#ABBREVIATION:Atp1a3b2# NAME:ATPase, Na+/K+ transporting (alpha4, beta4)#ABBREVIATION:Atp1a4b4# NAME:ATPase, Na+/K+ transporting (alpha2, beta4)#ABBREVIATION:Atp1a2b4# citations: AUTHORS:Stengelin MK, Hoffman JF.#TITLE:Na,K-ATPase subunit isoforms in human reticulocytes: evidence from reverse transcription-PCR for the presence of alpha1, alpha3, beta2, beta3, and gamma. #JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9159180# AUTHORS:Pestov NB, Adams G, Shakhparonov MI, Modyanov NN.#TITLE:Identification of a novel gene of the X,K-ATPase beta-subunit family that is predominantly expressed in skeletal and heart muscles#JOURNAL:FEBS Lett#YEAR:1999#PUBMED:10456317# AUTHORS:Wang J, Schwinger RH, Frank K, Muller-Ehmsen J, Martin-Vasallo P, Pressley TA, Xiang A, Erdmann E, McDonough AA.#TITLE:Regional expression of sodium pump subunits isoforms and Na+-Ca++ exchanger in the human heart.#JOURNAL:J Clin Invest#YEAR:1996#PUBMED:8833915# GENE ASSOCIATION: (481.1 and 478.1) or (477.1 and 23439.1) or (483.1 and 476.1) or (481.1 and 480.1) or (482.1 and 478.1) or (482.1 and 476.1) or (482.1 and 477.1) or (481.1 and 476.1) or (23439.1 and 476.1) or (23439.1 and 480.1) or (481.1 and 477.1)
Abbreviation: R_DNDPt5m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: datp[c] + dtdp[m] --> datp[m] + dtdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Deoxycytidine kinase, mitochondrial (ATP)
Abbreviation: R_DCK1m Synonyms: _0 EC Number: 2.7.1.74 SUBSYSTEM: Nucleotides Equation: [m] : atp + dcyt <==> adp + dcmp + h Confidence Level: 4 IT (2.7.1.74) genes: none proteins: none citations: AUTHORS:Cheng YC, Domin B, Lee LS.#TITLE:Human deoxycytidine kinase. Purification and characterization of the cytoplasmic and mitochondrial isozymes derived from blast cells of acute myelocytic leukemia patients.#JOURNAL:Biochim Biophys Acta.#YEAR:1977#PUBMED:265735# GENE ASSOCIATION:
Abbreviation: R_GCCcm Synonyms: _0 EC Number: 1.8.1.4 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : dhlpro + nad <==> h + lpro + nadh Confidence Level: 4 NCD genes: LOCUS:_2731#TRANSCRIPT:1#ABBREVIATION:GLDC#ECNUMBERS:1.4.4.2# LOCUS:_275#TRANSCRIPT:1#ABBREVIATION:AMT#ECNUMBERS:2.1.2.10# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_2653#TRANSCRIPT:1#ABBREVIATION:GCSH# proteins: NAME:glycine dehydrogenase (decarboxylating; glycine decarboxylase, glycine cleavage system protein P), mitochondrial #ABBREVIATION:Gldc.1-m# NAME:aminomethyltransferase (glycine cleavage system protein T), mitochondrial #ABBREVIATION:Amt.1-m# NAME:glycine cleavage system protein H (aminomethyl carrier) #ABBREVIATION:Gcsh.1-m# NAME:dihydrolipoamide dehydrogenase, mitochondrial#ABBREVIATION:Dld.1-m# citations: AUTHORS:Lee HH, Kim do J, Ahn HJ, Ha JY, Suh SW.#TITLE:Crystal structure of T-protein of the glycine cleavage system. Cofactor binding, insights into H-protein recognition, and molecular basis for understanding nonketotic hyperglycinemia.#YEAR:2004#PUBMED:15355973# GENE ASSOCIATION: (275.1) and (1738.1) and (2731.1) and (2653.1)
Abbreviation: R_EX_glygn4_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : glygn4 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
glycogen, structure 4 (glycogenin-1,6-{2[1,4-Glc], [1,4-Glc]}) exchange
Abbreviation: R_THYOXt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: thyox-L[e] <==> thyox-L[c] Confidence Level: 4 NJ genes: LOCUS:_6567#TRANSCRIPT:1#ABBREVIATION:SLC16A2# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 2#ABBREVIATION:Slc16a2.1-e# citations: AUTHORS:Friesema EC, Ganguly S, Abdalla A, Manning Fox JE, Halestrap AP, Visser TJ#TITLE:Identification of monocarboxylate transporter 8 as a specific thyroid hormone transporter#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12871948# AUTHORS:Lafreniere RG, Carrel L, Willard HF.#TITLE:A novel transmembrane transporter encoded by the XPCT gene in Xq13.2.#JOURNAL:Hum Mol Genet#YEAR:1994#PUBMED:7981683# GENE ASSOCIATION: (6567.1)
Abbreviation: R_EX_h2o_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : h2o <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_B3GALT41g Synonyms: _7 EC Number: 2.4.1.62 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : ga2_hs + udpgal --> ga1_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_8705#TRANSCRIPT:1#ABBREVIATION:B3GALT4# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 4#ABBREVIATION:B3galt4.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Amado M, Almeida R, Carneiro F, Levery ST, Holmes EH, Nomoto M, Hollingsworth MA, Hassan H, Schwientek T, Nielsen PA, Bennett EP, Clausen H#TITLE:A family of human beta3-galactosyltransferases#JOURNAL:The Journal of Biological Chemistry#YEAR:1998# GENE ASSOCIATION: (8705.1)
Abbreviation: R_FACOAL184 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + strdnc <==> amp + ppi + strdnccoa Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_EX_2425dhvitd3_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 2425dhvitd3 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DALAxt Synonyms: _0 SUBSYSTEM: D-alanine metabolism Equation: ala-D[c] --> ala-D[x] Confidence Level: 1 SAB genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DHPR2 Synonyms: _0 SUBSYSTEM: Tetrahydrobiopterin Equation: [c] : 5mthf + dhbpt --> h + mlthf + thbpt Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_DLNLCGt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dlnlcg[e] <==> dlnlcg[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_CRNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: crn[e] <==> crn[c] Confidence Level: 4 NCD genes: LOCUS:_6584#TRANSCRIPT:1#ABBREVIATION:SLC22A5# proteins: NAME:solute carrier family 22 (organic cation transporter), member 5#ABBREVIATION:Slc22a5.1# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# AUTHORS:Verhaagh S, Schweifer N, Barlow DP, Zwart R.#TITLE:Cloning of the mouse and human solute carrier 22a3 (Slc22a3/SLC22A3) identifies a conserved cluster of three organic cation transporters on mouse chromosome 17 and human 6q26-q27.#JOURNAL:Genomics#YEAR:1999#PUBMED:9933568# AUTHORS:Koepsell H, Schmitt BM, Gorboulev V.#TITLE:Organic cation transporters.#JOURNAL:Rev Physiol Biochem Pharmacol#YEAR:2003#PUBMED:12827517# GENE ASSOCIATION: (6584.1)
Abbreviation: R_GHMT2r Synonyms: _5 EC Number: 2.1.2.1 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : ser-L + thf <==> gly + h2o + mlthf Confidence Level: 4 NCD genes: LOCUS:_6470#TRANSCRIPT:1#ABBREVIATION:SHMT1# LOCUS:_6470#TRANSCRIPT:2#ABBREVIATION:SHMT1# proteins: NAME:serine hydroxymethyltransferase 1 (soluble) #ABBREVIATION:Shmt1.1# NAME:serine hydroxymethyltransferase 1 (soluble)#ABBREVIATION:Shmt1.2# citations: AUTHORS:Garrow TA, Brenner AA, Whitehead VM, Chen XN, Duncan RG, Korenberg JR, Shane B. #TITLE:Cloning of human cDNAs encoding mitochondrial and cytosolic serine hydroxymethyltransferases and chromosomal localization.#YEAR:1993#PUBMED:8505317# AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# AUTHORS:Narkewicz MR, Sauls SD, Tjoa SS, Teng C, Fennessey PV#TITLE:Evidence for intracellular partitioning of serine and glycine metabolism in Chinese hamster ovary cells#JOURNAL:Biochem J#YEAR:1996#PUBMED:8611185# GENE ASSOCIATION: (6470.1) or (6470.2)
glycine hydroxymethyltransferase, reversible
Abbreviation: R_ASNCYSNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[e] + cys-L[c] + na1[e] --> asn-L[c] + cys-L[e] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-cysteine/L-asparagine Na-dependent exchange (Asn-L in)
Abbreviation: R_GTHS Synonyms: _0 EC Number: 6.3.2.3 SUBSYSTEM: Glutathione Metabolism Equation: [c] : atp + glucys + gly --> adp + gthrd + h + pi Confidence Level: 4 NCD genes: LOCUS:_2937#TRANSCRIPT:1#ABBREVIATION:GSS#ECNUMBERS:6.3.2.3# proteins: NAME:Glutathione synthetase#ABBREVIATION:Gss.1# citations: AUTHORS:Dinescu A, Cundari TR, Bhansali VS, Luo JL, Anderson ME.#TITLE:Function of conserved residues of human glutathione synthetase: implications for the ATP-grasp enzymes. #JOURNAL:J Biol Chem.#YEAR:2004#PUBMED:14990577# GENE ASSOCIATION: (2937.1)
Abbreviation: R_ALAt2rL Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: ala-L[l] + h[l] <==> ala-L[c] + h[c] Confidence Level: 4 NCD SAB lyso genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
L-alanine reversible transport via proton symport (lysosome)
Abbreviation: R_AMPTASECG Synonyms: _0 EC Number: 3.4.11.2 SUBSYSTEM: Glutathione Metabolism Equation: [c] : cgly + h2o --> cys-L + gly Confidence Level: 1 SAB genes: LOCUS:_290#TRANSCRIPT:1#ABBREVIATION:ANPEP# proteins: NAME:alanyl aminopeptidase#ABBREVIATION:Anpep.1# citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# GENE ASSOCIATION: (290.1)
Abbreviation: R_XAOx Synonyms: _0 SUBSYSTEM: Purine Catabolism Equation: [x] : h2o + o2 + xan --> h2o2 + urate Confidence Level: 4 IT (1.17.3.2) genes: LOCUS:_7498#TRANSCRIPT:1#ABBREVIATION:XDH#ECNUMBERS:1.1.1.204# proteins: NAME:xanthine dehydrogenase #ABBREVIATION:Xdh.1-x# citations: AUTHORS:Saksela M, Raivio KO.#TITLE:Cloning and expression in vitro of human xanthine dehydrogenase/oxidase. #JOURNAL:Biochem J#YEAR:1996#PUBMED:8670112# AUTHORS:Linder N, Martelin E, Lapatto R, Raivio KO.#TITLE:Posttranslational inactivation of human xanthine oxidoreductase by oxygen under standard cell culture conditions.#JOURNAL:Am J Physiol Cell Physiol.#YEAR:2003#PUBMED:12637268# GENE ASSOCIATION: (7498.1)
Abbreviation: R_H2Otn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: h2o[n] <==> h2o[c] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FPGS5 Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [c] : 5dhf + atp + glu-L --> 6dhf + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_MI14Ptn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: mi14p[c] <==> mi14p[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
inositol 1,4-bisphosphate nuclear transport (diffusion)
_3 beta-hydroxysteroid dehydrogenase/delta 5-->4-isomerase type I
Abbreviation: R_HSD3B11 Synonyms: _7 SUBSYSTEM: Steroid Metabolism Equation: [c] : nad + prgnlone --> h + nadh + prgstrn Confidence Level: 4 Microsomal rxn This is a conglomerated reaction - in actuality there is an intermediate: progen-5-ene-3,20,dione (C21H30O2) for pregnenolone 5-androstene-3,17-dione for dhea genes: LOCUS:_3283#TRANSCRIPT:1#ABBREVIATION:HSD3B1#ECNUMBERS:1.1.1.145,,5.3.# proteins: NAME:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 1#ABBREVIATION:Hsd3b1.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (3283.1)
Abbreviation: R_ARGtiDF Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: arg-L[e] --> arg-L[c] Confidence Level: 4 SAB genes: LOCUS:_84889#TRANSCRIPT:1#ABBREVIATION:SLC7A3# LOCUS:_6542#TRANSCRIPT:1#ABBREVIATION:SLC7A2# LOCUS:_6541#TRANSCRIPT:1#ABBREVIATION:SLC7A1# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 1#ABBREVIATION:Slc7a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 2#ABBREVIATION:Slc7a2.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 3#ABBREVIATION:Slc7a3.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Albritton LM, Bowcock AM, Eddy RL, Morton CC, Tseng L, Farrer LA, Cavalli-Sforza LL, Shows TB, Cunningham JM#TITLE:The human cationic amino acid transporter (ATRC1): physical and genetic mapping to 13q12-q14#JOURNAL:Genomics#YEAR:1992#PUBMED:1348489# AUTHORS:Cariappa R, Heath-Monnig E, Furesz TC, Kamath SG, Smith CH#TITLE:Stable polarized expression of hCAT-1 in an epithelial cell line#JOURNAL:J Membr Biol#YEAR:2002#PUBMED:11891586# AUTHORS:Hoshide R, Ikeda Y, Karashima S, Matsuura T, Komaki S, Kishino T, Niikawa N, Endo F, Matsuda I#TITLE:Molecular cloning, tissue distribution, and chromosomal localization of human cationic amino acid transporter 2 (HCAT2)#JOURNAL:Genomics#YEAR:1996#PUBMED:8954799# AUTHORS:Furesz TC, Moe AJ, Smith CH#TITLE:Lysine uptake by human placental microvillous membrane: comparison of system y+ with basal membrane#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7534987# AUTHORS:Wolf S, Janzen A, Vekony N, Martine U, Strand D, Closs EI#TITLE:Expression of solute carrier 7A4 (SLC7A4) in the plasma membrane is not sufficient to mediate amino acid transport activity#JOURNAL:Biochem J#YEAR:2002#PUBMED:12049641# AUTHORS:Vekony N, Wolf S, Boissel JP, Gnauert K, Closs EI#TITLE:Human cationic amino acid transporter hCAT-3 is preferentially expressed in peripheral tissues#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11591158# AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (84889.1) or (55089.1) or (6542.1) or (6541.1)
L-arginine transport via diffusion (extracellular to cytosol)
Abbreviation: R_ASNt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[e] + na1[e] --> asn-L[c] + na1[c] Confidence Level: 4 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# GENE ASSOCIATION: (81539.1) or (11254.1) or (54407.1) or (55089.1)
L-asparagine transport in via sodium symport
Dehydrodolichol diphosphate, human uterine homolog_C1025H1650O70P20
Abbreviation: R_LCYSTATm Synonyms: _0 SUBSYSTEM: Cysteine Metabolism Equation: [m] : Lcyst + akg <==> 3spyr + glu-L Confidence Level: 2 described in PMID: 3346220 and KEGG MM genes: LOCUS:_2806#TRANSCRIPT:1#ABBREVIATION:GOT2#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase, mitochondrial#ABBREVIATION:Got2.1-m# citations: AUTHORS:Weinstein CL, Griffith OW.#TITLE:Cysteinesulfonate and beta-sulfopyruvate metabolism. Partitioning between decarboxylation, transamination, and reduction pathways.#YEAR:1988#PUBMED:3346220# GENE ASSOCIATION: (2806.1)
L-Cysteate:2-oxoglutarate aminotransferase, mitochondrial
Abbreviation: R_EX_appnn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : appnn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_KHK2 Synonyms: _1 EC Number: 2.7.1.3 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : atp + xylu-D --> adp + h + xu1p-D Confidence Level: 4 NCD genes: LOCUS:_3795#TRANSCRIPT:1#ABBREVIATION:KHK#ECNUMBERS:2.7.1.3# LOCUS:_3795#TRANSCRIPT:2#ABBREVIATION:KHK#ECNUMBERS:2.7.1.3# proteins: NAME:ketohexokinase (fructokinase)#ABBREVIATION:Khk.1# NAME:ketohexokinase (fructokinase) (liver, kidney, sm intestine)#ABBREVIATION:Khk.2# citations: AUTHORS:Bais R, James HM, Rofe AM, Conyers RA.#TITLE:The purification and properties of human liver ketohexokinase. A role for ketohexokinase and fructose-bisphosphate aldolase in the metabolic production of oxalate from xylitol#JOURNAL:Biochem J#YEAR:1985#PUBMED:2996495# AUTHORS:Barngrover DA, Dills WL Jr.#TITLE:The involvement of liver fructokinase in the metabolism of D-xylulose and xylitol in isolated rat hepatocytes#JOURNAL:J Nutr#YEAR:1983#PUBMED:6298387# AUTHORS:James HM, Bais R, Edwards JB, Rofe AM, Conyers AJ.#TITLE:Models for the metabolic production of oxalate from xylitol in humans: a role for fructokinase and aldolase.#JOURNAL:Aust J Exp Biol Med Sci#YEAR:1982#PUBMED:6284103# GENE ASSOCIATION: (3795.1) or (3795.2)
Abbreviation: R_S6TASE17ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg24 <==> h + ksi_deg25 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_HXANtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: hxan[l] <==> hxan[c] Confidence Level: 4 IT genes: LOCUS:_55315#TRANSCRIPT:1#ABBREVIATION:SLC29A3# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 3 #ABBREVIATION:Slc29a3.1-l# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Pisoni RL, Thoene JG.#TITLE:Detection and characterization of a nucleoside transport system in human fibroblast lysosomes.#JOURNAL:J Biol Chem.#YEAR:1989#PUBMED:2925670# AUTHORS:Baldwin SA, Yao SY, Hyde RJ, Ng AM, Foppolo S, Barnes K, Ritzel MW, Cass CE, Young JD.#TITLE:Functional characterization of novel human and mouse equilibrative nucleoside transporters (hENT3 and mENT3) located in intracellular membranes.#JOURNAL:J Biol Chem. #YEAR:2005#PUBMED:15701636# GENE ASSOCIATION: (55315.1)
hypoxanthine facilated transport from lysosome
Abbreviation: R_3SALAOX Synonyms: _0 SUBSYSTEM: Taurine and hypotaurine metabolism Equation: [c] : (2) 3sala + (2) h + o2 --> (2) Lcyst Confidence Level: 2 as described in Bender (Nutritional biochmeistry of the vitamins, 2nd edition) MM genes: none proteins: none citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# GENE ASSOCIATION:
sialytransferase 7 ((alpha-N-acetylneuraminyl 2,3-betagalactosyl-1,3)-N-acetyl galactosaminide alpha-2,6-sialytransferase) F
Abbreviation: R_ST6GALNAC62 Synonyms: _6 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gt1b_hs --> cmp + gq1balpha_hs + h Confidence Level: 4 NJ genes: LOCUS:_30815#TRANSCRIPT:1#ABBREVIATION:SIAT7F# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 6 #ABBREVIATION:St6galnac6.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Harduin-Lepers A, Vallejo-Ruiz V, Krzewinski-Recchi MA, Smyn-Petit B, Julien S, Delannoy P#TITLE:The human sialyltransferase family#JOURNAL:Biochimie#YEAR:2001# GENE ASSOCIATION: (30815.1)
Abbreviation: R_GLCtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: glc-D[l] --> glc-D[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Pisoni RL, Thoene JG#TITLE:The transport systems of mammalian lysosomes#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1751541# AUTHORS:Lloyd JB#TITLE:Metabolite efflux and influx across the lysosome membrane#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993166# GENE ASSOCIATION:
(alpha-D-mannosyl)2-beta-D-mannosyl-diacetylchitobiosyldiphosphodolichol, human liver homolog_C1420H2328N20O320P20
Glycolipid 1,6-alpha-D-mannosyltransferase (liver)
Abbreviation: R_G16MT_L Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : gdpmann + (0.1) m1mpdol_L --> gdp + h + (0.1) m2mpdol_L Confidence Level: 0 EC 2.4.1.- NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_ETFQO Synonyms: _0 EC Number: 1.5.5.1 SUBSYSTEM: Fatty acid oxidation Equation: [m] : etfrd + q10 --> etfox + q10h2 Confidence Level: 4 TV Ruzicka F, JBC, 1977 genes: LOCUS:_2110#TRANSCRIPT:1#ABBREVIATION:ETFDH# proteins: NAME:Electron-transferring-flavoprotein dehydrogenase, mitochondrial#ABBREVIATION:Etfdh.1-m# citations: AUTHORS:Simkovic M, Degala GD, Eaton SS, Frerman FE.#TITLE:Expression of human electron transfer flavoprotein-ubiquinone oxidoreductase from a baculovirus vector: kinetic and spectral characterization of the human protein.#JOURNAL:Biochem J#YEAR:2002#PUBMED:12049629# AUTHORS:Spector EB, Seltzer WK, Goodman SI.#TITLE:Assignment of electron transfer flavoprotein-ubiquinone oxidoreductase (ETF-QO) to human chromosome 4q33 by fluorescence in situ hybridization and somatic cell hybridization.#JOURNAL:Mol Genet Metab#YEAR:1997#PUBMED:10444348# GENE ASSOCIATION: (2110.1)
Electron transfer flavoprotein-ubiquinone oxidoreductase
Abbreviation: R_C161CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + hdcoa --> coa + hdcecrn Confidence Level: 4 TDV genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
methylmalonate-semialdehyde dehydrogenase (malonic semialdehyde), mitochondrial
Abbreviation: R_MMSAD3m Synonyms: _0 EC Number: 1.2.1.27 SUBSYSTEM: Propanoate Metabolism Equation: [m] : coa + msa + nad --> accoa + co2 + nadh Confidence Level: 4 also EC 1.2.1.18 - NCD genes: LOCUS:_4329#TRANSCRIPT:1#ABBREVIATION:ALDH6A1#ECNUMBERS:1.2.1.27# proteins: NAME:Aldehyde dehydrogenase 6 family, member A1, mitochondrial#ABBREVIATION:Aldh6a1.1-m# citations: AUTHORS:Kedishvili NY, Popov KM, Rougraff PM, Zhao Y, Crabb DW, Harris RA.#TITLE:CoA-dependent methylmalonate-semialdehyde dehydrogenase, a unique member of the aldehyde dehydrogenase superfamily. cDNA cloning, evolutionary relationships, and tissue distribution. #YEAR:1992#PUBMED:1527093# AUTHORS:Chambliss,K.L. , Gray,R.G. , Rylance,G. , Pollitt,R.J. , Gibson,K.M. #TITLE:Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency.#YEAR:2000#PUBMED:10947204# AUTHORS:Scholem RD, Brown GK#TITLE:Metabolism of malonic semialdehyde in man#JOURNAL:Biochem J#YEAR:1983#PUBMED:6418146# GENE ASSOCIATION: (4329.1)
Abbreviation: R_DNDPt46m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dttp[c] + dudp[m] --> dttp[m] + dudp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
lactaldehyde dehydrogenase, mitochondrial
Abbreviation: R_LCADi_Dm Synonyms: _0 EC Number: 1.2.1.21 SUBSYSTEM: Pyruvate Metabolism Equation: [m] : h2o + lald-D + nad --> (2) h + lac-D + nadh Confidence Level: 2 JLR- added irreversible form of LCAD added rxn for D-lac - NCD genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_7915#TRANSCRIPT:1#ABBREVIATION:ALDH5A1#ECNUMBERS:1.2.1.24# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# LOCUS:_4329#TRANSCRIPT:1#ABBREVIATION:ALDH6A1#ECNUMBERS:1.2.1.27# LOCUS:_5832#TRANSCRIPT:1#ABBREVIATION:ALDH18A1# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_7915#TRANSCRIPT:2#ABBREVIATION:ALDH5A1# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# NAME:aldehyde dehydrogenase 5 family, member A1 (succinate-semialdehyde dehydrogenase), mitochondrial#ABBREVIATION:Aldh5a1.1-m# NAME:aldehyde dehydrogenase 5 family, member A1 (succinate-semialdehyde dehydrogenase), mitochondrial#ABBREVIATION:Aldh5a1.2-m# NAME:aldehyde dehydrogenase 18 family, member A1#ABBREVIATION:Aldh18a1.1-m# NAME:Aldehyde dehydrogenase 6 family, member A1, mitochondrial#ABBREVIATION:Aldh6a1.1-m# citations: AUTHORS:Ewaschuk JB, Naylor JM, Zello GA#TITLE:D-lactate in human and ruminant metabolism#JOURNAL:J Nutr#YEAR:2005#PUBMED:15987839# AUTHORS:Christopher MM, Eckfeldt JH, Eaton JW.#TITLE:Propylene glycol ingestion causes D-lactic acidosis#JOURNAL:Lab Invest.#YEAR:1990#PUBMED:2296157# AUTHORS:Ray S, Ray M.#TITLE:Oxidation of lactaldehyde by cytosolic aldehyde dehydrogenase and inhibition of cytosolic and mitochondrial aldehyde dehydrogenase by metabolites.#JOURNAL:Biochim Biophys Acta.#YEAR:1984#PUBMED:6487654# GENE ASSOCIATION: (4329.1) or (7915.1) or (8659.1) or (5832.1) or (217.1) or (7915.2) or (219.1) or (8659.2)
Abbreviation: R_SERALANaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[c] + na1[e] + ser-L[e] --> ala-L[e] + na1[c] + ser-L[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
L-alanine/L-serine Na-dependent exchange (Ser-L in)
Abbreviation: R_WHDDCAte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: whddca[e] <==> whddca[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
N-acetylglucosamine-6-phosphate synthase
Abbreviation: R_ACGAM6PSi Synonyms: _1 EC Number: 2.3.1.4 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : accoa + gam6p --> acgam6p + coa + h Confidence Level: 2 NCD genes: LOCUS:_64841#TRANSCRIPT:1#ABBREVIATION:GNPNAT1# proteins: NAME:glucosamine-phosphate N-acetyltransferase 1#ABBREVIATION:Gnpnat1.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (64841.1)
Abbreviation: R_HS2ly Synonyms: _4 EC Number: 3.10.1.1 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg6 --> h + hs_deg7 + so4 Confidence Level: 4 NCD genes: LOCUS:_6448#TRANSCRIPT:1#ABBREVIATION:SGSH# proteins: NAME:N-sulfoglucosamine sulfohydrolase (sulfamidase), lysosomal#ABBREVIATION:Sgsh.1-l# citations: AUTHORS:Scott HS, Blanch L, Guo XH, Freeman C, Orsborn A, Baker E, Sutherland GR, Morris CP, Hopwood JJ#TITLE:Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7493035# AUTHORS:Bielicki J, Hopwood JJ, Melville EL, Anson DS#TITLE:Recombinant human sulphamidase: expression, amplification, purification and characterization#JOURNAL:Biochem J#YEAR:1998#PUBMED:9405287# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION: (6448.1)
Abbreviation: R_DURAD Synonyms: _0 EC Number: 1.3.1.2 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : 56dura + nadp <==> h + nadph + ura Confidence Level: 4 _01-26-05 IT based on Lu et al., JBC,1992,24,17102-17109 genes: LOCUS:_1806#TRANSCRIPT:1#ABBREVIATION:DPYD#ECNUMBERS:1.3.1.2# proteins: NAME:dihydropyrimidine dehydrogenase #ABBREVIATION:DpyD.1# citations: AUTHORS:Lu Z, Zhang R, Diasio RB. #TITLE:Dihydropyrimidine dehydrogenase activity in human peripheral blood mononuclear cells and liver: population characteristics, newly identified deficient patients, and clinical implication in 5-fluorouracil chemotherapy. #JOURNAL:Cancer Res.#YEAR:1993#PUBMED:8221682# AUTHORS:Lu ZH, Zhang R, Diasio RB.#TITLE:Purification and characterization of dihydropyrimidine dehydrogenase from human liver.#JOURNAL:J Biol Chem. #YEAR:1992#PUBMED:1512248# GENE ASSOCIATION: (1806.1)
Core 7 alpha-GalNAc-transferase, Golgi apparatus
Abbreviation: R_CORE7GTg Synonyms: _7 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : Tn_antigen + udpacgal --> core7 + h + udp Confidence Level: 0 EC 2.4.1.- NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_fucacngalacglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucacngalacglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NTD4e Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [e] : cmp + h2o --> cytd + pi Confidence Level: 2 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_4907#TRANSCRIPT:1#ABBREVIATION:NT5E#ECNUMBERS:3.1.3.5# proteins: NAME:_5'-nucleotidase, ecto (CD73) #ABBREVIATION:Nt5E.1-e# citations: none GENE ASSOCIATION: (4907.1)
Abbreviation: R_MTRI Synonyms: _0 EC Number: 5.3.1.23 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : 5mdr1p <==> 5mdru1p Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Myers RW, Abeles RH#TITLE:Conversion of 5-S-ethyl-5-thio-D-ribose to ethionine in Klebsiella pneumoniae. Basis for the selective toxicity of 5-S-ethyl-5-thio-D-ribose#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2543672# GENE ASSOCIATION:
_5-methylthioribose-1-phosphate isomerase
Abbreviation: R_VACCCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + vacccrn --> crn + vacccoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_CRNCAR3tp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: crn[c] + pcrn[x] --> crn[x] + pcrn[c] Confidence Level: 0 copied from NCD's CRNCARtp NJ genes: none proteins: none citations: none GENE ASSOCIATION:
carnitine-propcarnitine carrier, peroxisomal
Abbreviation: R_PEFLIP Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + pe_hs[e] --> adp[c] + h[c] + pe_hs[c] + pi[c] Confidence Level: 4 See PMID: 10856717 - flippase activity NJ genes: LOCUS:_10396#TRANSCRIPT:1#ABBREVIATION:ATP8A1#ECNUMBERS:3.6.3.1# LOCUS:_57194#TRANSCRIPT:1#ABBREVIATION:ATP10A#ECNUMBERS:3.6.3.1# proteins: NAME:ATPase, aminophospholipid transporter (APLT), Class I, type 8A, member 1#ABBREVIATION:Atp8a1.1# NAME:ATPase, Class V, type 10A#ABBREVIATION:Atp10a.1# citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# AUTHORS:Herzing LB, Kim SJ, Cook EH Jr, Ledbetter DH.#TITLE:The human aminophospholipid-transporting ATPase gene ATP10C maps adjacent to UBE3A and exhibits similar imprinted expression.#JOURNAL:Am J Hum Genet#YEAR:2001#PUBMED:11353404# AUTHORS:Mouro I, Halleck MS, Schlegel RA, Mattei MG, Williamson P, Zachowski A, Devaux P, Cartron JP, Colin Y.#TITLE:Cloning, expression, and chromosomal mapping of a human ATPase II gene, member of the third subfamily of P-type ATPases and orthologous to the presumed bovine and murine aminophospholipid translocase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10198212# GENE ASSOCIATION: (10396.1) or (57194.1)
Abbreviation: R_S6T13g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre31 + paps --> h + ksi_pre32 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (4166.1) or (10164.1) or (23563.2) or (23563.1) or (9435.1)
Abbreviation: R_HPYRR2x Synonyms: _0 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : h + hpyr + nadh --> glyc-S + nad Confidence Level: 2 NCD genes: LOCUS:_3945#TRANSCRIPT:1#ABBREVIATION:LDHB#ECNUMBERS:1.1.1.27# LOCUS:_3948#TRANSCRIPT:1#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_3939#TRANSCRIPT:1#ABBREVIATION:LDHA#ECNUMBERS:1.1.1.27# LOCUS:_160287#TRANSCRIPT:1#ABBREVIATION:LDHAL6A# LOCUS:_92483#TRANSCRIPT:1#ABBREVIATION:LDHAL6B# LOCUS:_197257#TRANSCRIPT:1#ABBREVIATION:LDHD# LOCUS:_3948#TRANSCRIPT:2#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_197257#TRANSCRIPT:2#ABBREVIATION:LDHD# proteins: NAME:lactate dehydrogenase A (liver, sk muscle)#ABBREVIATION:Ldha.1# NAME:lactate dehydrogenase B (heart, RBC)#ABBREVIATION:Ldhb.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.2# NAME:lactate dehydrogenase A-like 6B (testis)#ABBREVIATION:Ldhal6b.1# NAME:lactate dehydrogenase A-like 6A#ABBREVIATION:Ldhal6a.1# NAME:lactate dehydrogenase D#ABBREVIATION:Ldhd.1# NAME:lactate dehydrogenase D#ABBREVIATION:Ldhd.2# NAME:lactate dehydrogenase (heart, RBC, brain, kidney, liver, sk muscle)#ABBREVIATION:LdhAB# citations: AUTHORS:Cregeen DP, Williams EL, Hulton S, Rumsby G#TITLE:Molecular analysis of the glyoxylate reductase (GRHPR) gene and description of mutations underlying primary hyperoxaluria type 2#JOURNAL:Hum Mutat#YEAR:2003#PUBMED:14635115# AUTHORS:Williams HE, Smith LH Jr.#TITLE:L-glyceric aciduria. A new genetic variant of primary hyperoxaluria#JOURNAL:N Eng J Med#YEAR:1968#PUBMED:5635456# GENE ASSOCIATION: (92483.1) or (3948.2) or (160287.1) or (3945.1 and 3939.1) or (197257.2) or (3939.1) or (197257.1) or (3948.1) or (3945.1)
Abbreviation: R_D_DASH_3AIBt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _3aib-D[c] --> 3aib-D[e] Confidence Level: 2 MM genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S26Tg Synonyms: _0 EC Number: 2.4.99.1 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : (2) cmpacna + l2fn2m2masn --> (2) cmp + (2) h + s2l2fn2m2masn Confidence Level: 0 NCD genes: LOCUS:_6480#TRANSCRIPT:1#ABBREVIATION:SIAT1#ECNUMBERS:2.4.99.1# LOCUS:_6480#TRANSCRIPT:2#ABBREVIATION:SIAT1#ECNUMBERS:2.4.99.1# LOCUS:_6480#TRANSCRIPT:3#ABBREVIATION:SIAT1#ECNUMBERS:2.4.99.1# proteins: NAME:sialyltransferase 1 (beta-galactoside alpha-2,6-sialyltransferase), Golgi apparatus (sk muscle, liver, placenta)#ABBREVIATION:Siat1.1-g# NAME:sialyltransferase 1 (beta-galactoside alpha-2,6-sialyltransferase), Golgi apparatus (sk muscle, liver, placenta)#ABBREVIATION:Siat1.2-g# NAME:sialyltransferase 1 (beta-galactoside alpha-2,6-sialyltransferase), Golgi apparatus (sk muscle, liver, placenta)#ABBREVIATION:Siat1.3-g# citations: AUTHORS:Kitagawa H, Paulson JC#TITLE:Differential expression of five sialyltransferase genes in human tissues#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8027041# GENE ASSOCIATION: (6480.1) or (6480.3) or (6480.2)
beta-galactoside alpha-2,6-sialyltransferase
Abbreviation: R_LEUTAm Synonyms: _0 EC Number: 2.6.1.6 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : akg + leu-L <==> 4mop + glu-L Confidence Level: 4 NCD genes: LOCUS:_587#TRANSCRIPT:1#ABBREVIATION:BCAT2#ECNUMBERS:2.6.1.26# proteins: NAME:branched chain aminotransferase 2, mitochondrial #ABBREVIATION:Bcat2.1-m# citations: AUTHORS:Bledsoe RK, Dawson PA, Hutson SM.#TITLE:Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm). #YEAR:1997#PUBMED:9165094# AUTHORS:Naylor SL, Shows TB.#TITLE:Branched-chain aminotransferase deficiency in Chinese hamster cells complemented by two independent genes on human chromosomes 12 and 19. #YEAR:1980#PUBMED:6933702# GENE ASSOCIATION: (587.1)
Abbreviation: R_CSPG_Atly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: cspg_a[e] --> cspg_a[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate A transport, extracellular to lysosome
Abbreviation: R_GLUCYS Synonyms: _0 EC Number: 6.3.2.2 SUBSYSTEM: Glutathione Metabolism Equation: [c] : atp + cys-L + glu-L --> adp + glucys + h + pi Confidence Level: 4 NCD genes: LOCUS:_2730#TRANSCRIPT:1#ABBREVIATION:GCLM#ECNUMBERS:6.3.2.2# LOCUS:_2729#TRANSCRIPT:1#ABBREVIATION:GCLC#ECNUMBERS:6.3.2.2# proteins: NAME:Glutamate-cysteine ligase, modifier subunit #ABBREVIATION:Gclm.1# NAME:Glutamate-cysteine ligase, catalytic subunit #ABBREVIATION:Gclc.1# citations: AUTHORS:Gipp JJ, Bailey HH, Mulcahy RT.#TITLE:Cloning and sequencing of the cDNA for the light subunit of human liver gamma-glutamylcysteine synthetase and relative mRNA levels for heavy and light subunits in human normal tissues.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1995#PUBMED:7826375# GENE ASSOCIATION: (2729.1) and (2730.1)
Abbreviation: R_CHLP Synonyms: _0 EC Number: 2.7.7.15 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : cholp + h2o --> chol + pi Confidence Level: 0 no high energy phosphate compounds found associated w/ rxn NJ genes: LOCUS:_162466#TRANSCRIPT:1#ABBREVIATION:PHOSPHO1# proteins: NAME:phosphatase, orphan 1#ABBREVIATION:Phospho1.1# citations: AUTHORS:Roberts SJ, Stewart AJ, Sadler PJ, Farquharson C.#TITLE:Human PHOSPHO1 exhibits high specific phosphoethanolamine and phosphocholine phosphatase activities.#JOURNAL:Biochem J#YEAR:2004#PUBMED:15175005# GENE ASSOCIATION: (162466.1)
Abbreviation: R_SARCStm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: sarcs[c] --> sarcs[m] Confidence Level: 1 MM genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NTD12 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [c] : dimp + h2o --> din + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_30833#TRANSCRIPT:1#ABBREVIATION:NT5C#ECNUMBERS:3.1.3.5# proteins: NAME:_5', 3'-nucleotidase, cytosolic #ABBREVIATION:Nt5C.1# citations: AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION: (30833.1)
Abbreviation: R_RETFAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: retfa[c] --> retfa[e] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_HSD3B13r Synonyms: _7 SUBSYSTEM: Steroid Metabolism Equation: [r] : 17ahprgnlone + nad --> 17ahprgstrn + h + nadh Confidence Level: 4 Microsomal rxn This is a conglomerated reaction - in actuality there is an intermediate: progen-5-ene-3,20,dione (C21H30O2) for pregnenolone 5-androstene-3,17-dione for dhea HSDB13 added by inference from Payne + Hales Endocrine Reviews (2004) 25(6):947-970 17ahprgnlone -> 17ahprgstrn: the catalysis of this reaction (3rd in HSD3 family that was added) was inferred from above paper NJ genes: LOCUS:_3284#TRANSCRIPT:1#ABBREVIATION:HSD3B2#ECNUMBERS:1.1.1.145,,5.3.# proteins: NAME:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 2#ABBREVIATION:Hsd3b2.1# citations: AUTHORS:Leenders F, Dolez V, Begue A, Moller G, Gloeckner JC, deLaunoit Y, Adamski J#TITLE:Structure of the gene for the human 17-beta-hydroxysteroid dehydrogenase type IV#JOURNAL:Mammalian Genome#YEAR:1998# AUTHORS:Adamski J, Leenders F Carstensen JF, Kaumann M, Markus MM, Husen B, Tesdorpf JG, Seedor U, deLaunoit Y, Jakob F#TITLE:Steroids, fatty acyl-CoA, and sterols are substrates of 80-kDa multifunctional protein#JOURNAL:Steroids#YEAR:1997# AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (3284.1)
Abbreviation: R_ACALDt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acald[e] <==> acald[c] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RADH2 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : h2o + nadp + retinal <==> (2) h + nadph + retn Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_NTD1m Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [m] : dump + h2o --> duri + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_56953#TRANSCRIPT:1#ABBREVIATION:NT5M#ECNUMBERS:3.1.3.5# proteins: NAME:_5',3'-nucleotidase, mitochondrial #ABBREVIATION:Nt5M.1-m# citations: AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION: (56953.1)
Abbreviation: R_SERGLNexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gln-L[c] + ser-L[e] <==> gln-L[e] + ser-L[c] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_CITMCOALm Synonyms: _0 EC Number: 4.1.3.25 SUBSYSTEM: C5-Branched dibasic acid metabolism Equation: [m] : citmcoa-L --> accoa + pyr Confidence Level: 2 also EC 4.1.3.22 NCD genes: none proteins: none citations: AUTHORS:WANG SF, ADLER J, LARDY HA#TITLE:The pathway of itaconate metabolism by liver mitochondria#JOURNAL:J Biol Chem#YEAR:1961#PUBMED:13783048# GENE ASSOCIATION:
Abbreviation: R_ADHAPtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: adhap_hs[c] <==> adhap_hs[x] Confidence Level: 1 unknown mechanism, presumptive "diffusion" - based on simulation/gap filling NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CYSTGL Synonyms: _0 EC Number: 4.4.1.1 SUBSYSTEM: Cysteine Metabolism Equation: [c] : cyst-L + h2o --> 2obut + cys-L + nh4 Confidence Level: 4 NCD genes: LOCUS:_1491#TRANSCRIPT:1#ABBREVIATION:CTH#ECNUMBERS:4.4.1.1# LOCUS:_1491#TRANSCRIPT:2#ABBREVIATION:CTH#ECNUMBERS:4.4.1.1# proteins: NAME:cystathionase (cystathionine gamma-lyase) #ABBREVIATION:Cth.1# NAME:cystathionase (cystathionine gamma-lyase) #ABBREVIATION:Cth.2# citations: AUTHORS:Stipanuk MH#TITLE:Sulfur amino acid metabolism: pathways for production and removal of homocysteine and cysteine#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189131# AUTHORS:Levonen AL, Lapatto R, Saksela M, Raivio KO.#TITLE:Human cystathionine gamma-lyase: developmental and in vitro expression of two isoforms.#YEAR:2000#PUBMED:10727430# GENE ASSOCIATION: (1491.1) or (1491.2)
Abbreviation: R_G12MT1_L Synonyms: _0 EC Number: 2.4.1.131 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : gdpmann + (0.1) m2mpdol_L --> gdp + h + (0.1) m3mpdol_L Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Glycolipid 1,2-alpha-D-mannosyltransferase (liver)
Abbreviation: R_RAI3 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : retn <==> 13-cis-retn Confidence Level: 2 IT genes: LOCUS:_2944#TRANSCRIPT:1#ABBREVIATION:GSTM1#ECNUMBERS:2.5.1.18# LOCUS:_2944#TRANSCRIPT:2#ABBREVIATION:GSTM1#ECNUMBERS:2.5.1.18# proteins: NAME:glutathione S-transferase M1#ABBREVIATION:Gstm1.1# NAME:glutathione S-transferase M2#ABBREVIATION:Gstm1.2# citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Chen H, Juchau MR.#TITLE:Recombinant human glutathione S-transferases catalyse enzymic isomerization of 13-cis-retinoic acid to all-trans-retinoic acid in vitro.#JOURNAL:Biochem J. #YEAR:1998#PUBMED:9806904# AUTHORS:Gough WH, VanOoteghem S, Sint T, Kedishvili NY.#TITLE:cDNA cloning and characterization of a new human microsomal NAD+-dependent dehydrogenase that oxidizes all-trans-retinol and 3alpha-hydroxysteroids.#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9677409# GENE ASSOCIATION: (2944.1) or (2944.2)
Abbreviation: R_CYTK3 Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [c] : cmp + gtp <==> cdp + gdp Confidence Level: 4 genes: LOCUS:_26289#TRANSCRIPT:1#ABBREVIATION:AK5# LOCUS:_26289#TRANSCRIPT:2#ABBREVIATION:AK5# proteins: NAME:adenylate kinase 5 #ABBREVIATION:Ak5.1# NAME:adenylate kinase 5 #ABBREVIATION:Ak5.2# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Identification of a novel human adenylate kinase. cDNA cloning, expression analysis, chromosome localization and characterization of the recombinant protein. #JOURNAL:Eur J Biochem. #YEAR:1999#PUBMED:10215863# GENE ASSOCIATION: (26289.1) or (26289.2)
N-acetylgalactosamine 3-beta-galactosyltransferase, Golgi apparatus
Abbreviation: R_N3Tg Synonyms: _2 EC Number: 2.4.1.122 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : Tn_antigen + udpgal --> T_antigen + h + udp Confidence Level: 0 NCD genes: LOCUS:_56913#TRANSCRIPT:1#ABBREVIATION:C1GALT1#ECNUMBERS:2.4.1.122# proteins: NAME:core 1 UDP-galactose:N-acetylgalactosamine-alpha-R beta 1,3-galactosyltransferase, Golgi apparatus (kidney, heart, pancreas, liver, brain.skeletal muscle)#ABBREVIATION:C1galt1.1-g# citations: AUTHORS:Ju T, Brewer K, D'Souza A, Cummings RD, Canfield WM.#TITLE:Cloning and expression of human core 1 beta1,3-galactosyltransferase.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11677243# GENE ASSOCIATION: (56913.1)
Abbreviation: R_FUCFUCFUCGALACGLCGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucfucfucgalacglcgal14acglcgalgluside_hs[g] <==> fucfucfucgalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_IDOAASE2ly Synonyms: _1 EC Number: 3.2.1.76 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg16 --> hs_deg17 + idour Confidence Level: 4 NCD genes: LOCUS:_3425#TRANSCRIPT:1#ABBREVIATION:IDUA#ECNUMBERS:3.2.1.76# proteins: NAME:iduronidase, alpha-L-, lysosomal#ABBREVIATION:Idua.1-l# citations: AUTHORS:Scott HS, Anson DS, Orsborn AM, Nelson PV, Clements PR, Morris CP, Hopwood JJ#TITLE:Human alpha-L-iduronidase: cDNA isolation and expression#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1946389# AUTHORS:Clements PR, Brooks DA, McCourt PA, Hopwood JJ#TITLE:Immunopurification and characterization of human alpha-L-iduronidase with the use of monoclonal antibodies#JOURNAL:Biochem J#YEAR:1989#PUBMED:2470345# GENE ASSOCIATION: (3425.1)
Abbreviation: R_FAEL184 Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : (5) h + malcoa + (4) nadph + o2 + strdnccoa --> co2 + coa + eicostetcoa + (3) h2o + (4) nadp Confidence Level: 4 fatty acid elongation step NJ genes: LOCUS:_6785#TRANSCRIPT:1#ABBREVIATION:ELOVL4# proteins: NAME:elongation of very long chain fatty acids - like 4#ABBREVIATION:Elovl4.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Zhang K, Kniazeva M, Han M, Li W, Yu Z, Yang Z, Li Y, Metzker ML, Allikmets R, Zack DJ, Kakuk LE, Lagali PS, Wong PW, MacDonald IM, Sieving PA, Figueroa DJ, Austin CP, Gould RJ, Ayyagari R, Petrukhin K.#TITLE:A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy.#JOURNAL:Nat Genet#YEAR:2001#PUBMED:11138005# AUTHORS:Nakamura MT, Nara TY.#TITLE:Structure, function, and dietary regulation of delta6, delta5, and delta9 desaturases.#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189125# GENE ASSOCIATION: (6785.1)
Abbreviation: R_34DHPLACOX_LPAREN_NADP_RPAREN_ Synonyms: _0 EC Number: 1.2.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 34dhpac + h2o + nadp <==> 34dhpha + (2) h + nadph Confidence Level: 4 SAB genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Ambroziak W, Pietruszko R#TITLE:Human aldehyde dehydrogenase. Activity with aldehyde metabolites of monoamines, diamines, and polyamines#JOURNAL:J Biol Chem#YEAR:1991#PUBMED:2071588# AUTHORS:Keung WM#TITLE:Biogenic aldehyde(s) derived from the action of monoamine oxidase may mediate the antidipsotropic effect of daidzin#JOURNAL:Chem Biol Interact#YEAR:2001#PUBMED:11306106# GENE ASSOCIATION: (222.1) or (221.1) or (218.1)
_3,4-Dihydroxyphenylacetaldehyde:NADP+ oxidoreductase
Abbreviation: R_PVD3 Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [c] : 7dhchsterol <==> pd3 Confidence Level: 4 This reaction takes place in skin and needs UV 290-305. based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.194 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_DHAPA Synonyms: _4 EC Number: 2.3.1.42 SUBSYSTEM: Triacylglycerol Synthesis Equation: [c] : Rtotalcoa + dhap --> adhap_hs + coa Confidence Level: 4 NJ genes: LOCUS:_8443#TRANSCRIPT:1#ABBREVIATION:GNPAT#ECNUMBERS:2.3.1.15# proteins: NAME:glyceronephosphate O-acyltransferase#ABBREVIATION:Gnpat.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Coleman RA, Lee DP#TITLE:Enzymes of triacylglycerol synthesis and their regulation#JOURNAL:Prog in Lipid Research#YEAR:2004# AUTHORS:Thai TP, Heid H, Rackwitz HR, Hunziker A, Gorgas K, Just WW.#TITLE:Ether lipid biosynthesis: isolation and molecular characterization of human dihydroxyacetonephosphate acyltransferase.#JOURNAL:FEBS Lett.#YEAR:1997#PUBMED:9459311# GENE ASSOCIATION: (8443.1)
Abbreviation: R_UDPG1P Synonyms: _0 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : h2o + udpglcur --> glcur1p + (2) h + ump Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# GENE ASSOCIATION:
UDPglucuronate uridine-monophosphohydrolase
Abbreviation: R_AVITE1t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: avite1[e] --> avite1[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_LNLNCGCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: lnlncgcrn[c] --> lnlncgcrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
keratan sulfate II biosynthesis, precursor 7_C75H121N6O56SX
Abbreviation: R_EX_oagt3_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : oagt3_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_9-O-Acetylated GT3 (homo sapiens) exchange
Abbreviation: R_DNDPt25m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[m] + dtdp[c] --> dcdp[c] + dtdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_CLFORtex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[e] + (2) for[c] --> cl[c] + (2) for[e] Confidence Level: 2 SAB genes: LOCUS:_5172#TRANSCRIPT:1#ABBREVIATION:SLC26A4# proteins: NAME:solute carrier family 26, member 4#ABBREVIATION:Slc26a4.1# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (5172.1)
chloride transport via formate countertransport
keratan sulfate II biosynthesis, precursor 8_C83H134N7O61SX
Abbreviation: R_EX_dheas_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dheas <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
alkyl glycerol phosphate acyltransferase
Abbreviation: R_AGLPT Synonyms: _0 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : Rtotal2 + akgp_hs + h --> ak2gp_hs + h2o Confidence Level: 0 NJ genes: none proteins: none citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Brites P, Waterham HR, Wander RJA#TITLE:Functions and biosynthesis of plasmalogens in health and disease#JOURNAL:Biochimica et Biophysica Acta#YEAR:2004# AUTHORS:Nagan N, Zoeller RA#TITLE:Plasmalogens: biosynthesis and functions#JOURNAL:Progress in Lipid Research#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_AICART Synonyms: _0 EC Number: 2.1.2.3 SUBSYSTEM: IMP Biosynthesis Equation: [c] : 10fthf + aicar <==> fprica + thf Confidence Level: 4 genes: LOCUS:_471#TRANSCRIPT:1#ABBREVIATION:ATIC# proteins: NAME:_5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase #ABBREVIATION:AtiC.1# citations: AUTHORS:Cheong CG, Wolan DW, Greasley SE, Horton PA, Beardsley GP, Wilson IA.#TITLE:Crystal structures of human bifunctional enzyme aminoimidazole-4-carboxamide ribonucleotide transformylase/IMP cyclohydrolase in complex with potent sulfonyl-containing antifolates.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14966129# AUTHORS:Bulock KG, Beardsley GP, Anderson KS.#TITLE:The kinetic mechanism of the human bifunctional enzyme ATIC (5-amino-4-imidazolecarboxamide ribonucleotide transformylase/inosine 5'-monophosphate cyclohydrolase). A surprising lack of substrate channeling.#JOURNAL:J Biol Chem.#YEAR:2002#PUBMED:11948179# AUTHORS:Sugita T, Aya H, Ueno M, Ishizuka T, Kawashima K.#TITLE:Characterization of molecularly cloned human 5-aminoimidazole-4-carboxamide ribonucleotide transformylase.#JOURNAL:J Biochem (Tokyo). #YEAR:1997#PUBMED:9378707# GENE ASSOCIATION: (471.1)
phosphoribosylaminoimidazolecarboxamide formyltransferase
Abbreviation: R_EX_ksi_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ksi <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_C181CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: odecrn[c] --> odecrn[m] Confidence Level: 4 TDV genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (788.1)
Abbreviation: R_GLCAT3g Synonyms: _1 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_c,d,e_pre1 + udpglcur --> cs_c_pre2 + h + udp Confidence Level: 4 EC 2.4.1.226 NCD genes: LOCUS:_22856#TRANSCRIPT:1#ABBREVIATION:CHSY1# LOCUS:_54480#TRANSCRIPT:1#ABBREVIATION:CSGlcA-T# LOCUS:_79586#TRANSCRIPT:1#ABBREVIATION:CHPF# LOCUS:_337876#TRANSCRIPT:1#ABBREVIATION:CSS3# proteins: NAME:chondroitin sulfate glucuronyltransferase, Golgi apparatus#ABBREVIATION:Csglca-t.1-g# NAME:carbohydrate (chondroitin) synthase 1, Golgi apparatus#ABBREVIATION:Chsy1.1-g# NAME:chondroitin polymerizing factor, Golgi apparatus#ABBREVIATION:Chpf.1-g# NAME:chondroitin sulfate synthase 3, Golgi apparatus#ABBREVIATION:Css3.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Gotoh M, Yada T, Sato T, Akashima T, Iwasaki H, Mochizuki H, Inaba N, Togayachi A, Kudo T, Watanabe H, Kimata K, Narimatsu H#TITLE:Molecular cloning and characterization of a novel chondroitin sulfate glucuronyltransferase that transfers glucuronic acid to N-acetylgalactosamine#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12145278# AUTHORS:Kitagawa H, Uyama T, Sugahara K#TITLE:Molecular cloning and expression of a human chondroitin synthase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11514575# AUTHORS:Kitagawa H, Izumikawa T, Uyama T, Sugahara K#TITLE:Molecular cloning of a chondroitin polymerizing factor that cooperates with chondroitin synthase for chondroitin polymerization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12716890# AUTHORS:Yada T, Sato T, Kaseyama H, Gotoh M, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-3. Molecular cloning and characterization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12907687# AUTHORS:Yada T, Gotoh M, Sato T, Shionyu M, Go M, Kaseyama H, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12761225# GENE ASSOCIATION: (54480.1) or (337876.1) or (79586.1) and (22856.1)
Abbreviation: R_P4502C9 Synonyms: _5 SUBSYSTEM: CYP Metabolism Equation: [c] : h + nadph + o2 + tolbutamide --> 4mtolbutamide + h2o + nadp Confidence Level: 4 tolbutamide 4 methyl hydroxylation assumed to take place on outer membrane - xenobiotic metabolism NJ genes: LOCUS:_1559#TRANSCRIPT:1#ABBREVIATION:CYP2C9#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 2, subfamily C, polypeptide 9#ABBREVIATION:Cyp2c9.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Umbenhauer DR, Martin MV, Lloyd RS, Guengerich FP.#TITLE:Abstract Cloning and sequence determination of a complementary DNA related to human liver microsomal cytochrome P-450 S-mephenytoin 4-hydroxylase.#JOURNAL:Biochemistry#YEAR:1987#PUBMED:3032244# AUTHORS:Meehan RR, Gosden JR, Rout D, Hastie ND, Friedberg T, Adesnik M, Buckland R, van Heyningen V, Fletcher J, Spurr NK, et al.#TITLE:Human cytochrome P-450 PB-1: a multigene family involved in mephenytoin and steroid oxidations that maps to chromosome 10.#JOURNAL:Am J Hum Genet#YEAR:1988#PUBMED:2827463# GENE ASSOCIATION: (1559.1)
Abbreviation: R_CYTK9 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : cmp + dctp <==> cdp + dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
keratan sulfate I biosynthesis, precursor 11_C129H208N9O101S3X
Abbreviation: R_P4502C8 Synonyms: _5 SUBSYSTEM: CYP Metabolism Equation: [c] : h + nadph + o2 + taxol --> h2o + htaxol + nadp Confidence Level: 2 paclitaxol 6 alpha hydroxylation assumed to take place on outer membrane - xenobiotic metabolism NJ genes: LOCUS:_1558#TRANSCRIPT:1#ABBREVIATION:CYP2C8# LOCUS:_1558#TRANSCRIPT:2#ABBREVIATION:CYP2C8# proteins: NAME:cytochrome P450, family 2, subfamily C, polypeptide 8#ABBREVIATION:Cyp2c8.2# NAME:cytochrome P450, family 2, subfamily C, polypeptide 8#ABBREVIATION:Cyp2c8.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# GENE ASSOCIATION: (1558.1) or (1558.2)
Abbreviation: R_S4T2g Synonyms: _1 EC Number: 2.8.2.5 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_a,b_pre3 + paps --> cspg_a + h + pap Confidence Level: 4 NCD genes: LOCUS:_50515#TRANSCRIPT:1#ABBREVIATION:CHST11# LOCUS:_55501#TRANSCRIPT:1#ABBREVIATION:CHST12# LOCUS:_166012#TRANSCRIPT:1#ABBREVIATION:CHST13# LOCUS:_113189#TRANSCRIPT:1#ABBREVIATION:D4ST1# proteins: NAME:carbohydrate (chondroitin 4) sulfotransferase 11, Golgi#ABBREVIATION:Chst11.1-g# NAME:carbohydrate (chondroitin 4) sulfotransferase 12, Golgi apparatus#ABBREVIATION:Chst12.1-g# NAME:carbohydrate (chondroitin 4) sulfotransferase 13, Golgi apparatus#ABBREVIATION:Chst13.1-g# NAME:dermatan 4 sulfotransferase 1, Golgi apparatus#ABBREVIATION:D4st.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Yamauchi S, Mita S, Matsubara T, Fukuta M, Habuchi H, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of chondroitin 4-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10722746# AUTHORS:Okuda T, Mita S, Yamauchi S, Matsubara T, Yagi F, Yamamori D, Fukuta M, Kuroiwa A, Matsuda Y, Habuchi O#TITLE:Molecular cloning, expression, and chromosomal mapping of human chondroitin 4-sulfotransferase, whose expression pattern in human tissues is different from that of chondroitin 6-sulfotransferase#JOURNAL:J Biochem (Tokyo)#YEAR:2000#PUBMED:11056388# AUTHORS:Hiraoka N, Nakagawa H, Ong E, Akama TO, Fukuda MN, Fukuda M#TITLE:Molecular cloning and expression of two distinct human chondroitin 4-O-sulfotransferases that belong to the HNK-1 sulfotransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781601# AUTHORS:Kang HG, Evers MR, Xia G, Baenziger JU, Schachner M#TITLE:Molecular cloning and characterization of chondroitin-4-O-sulfotransferase-3. A novel member of the HNK-1 family of sulfotransferases#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12080076# AUTHORS:Evers MR, Xia G, Kang HG, Schachner M, Baenziger JU#TITLE:Molecular cloning and characterization of a dermatan-specific N-acetylgalactosamine 4-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:1147079# AUTHORS:Mikami T, Mizumoto S, Kago N, Kitagawa H, Sugahara K.#TITLE:Specificities of three distinct human chondroitin/dermatan N-acetylgalactosamine 4-O-sulfotransferases demonstrated using partially desulfated dermatan sulfate as an acceptor: implication of differential roles in dermatan sulfate biosynthesis.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12847091# GENE ASSOCIATION: (55501.1) or (166012.1) or (50515.1) or (113189.1)
Abbreviation: R_FUCACNGALACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucacngalacglcgalgluside_hs[e] <==> fucacngalacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_10FTHF7GLUtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _10fthf7glu[c] --> 10fthf7glu[l] Confidence Level: 2 carrier-mediated transport - but which and how is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
Abbreviation: R_PPAm Synonyms: _0 EC Number: 3.6.1.1 SUBSYSTEM: Oxidative Phosphorylation Equation: [m] : h2o + ppi --> h + (2) pi Confidence Level: 4 IF genes: LOCUS:_27068#TRANSCRIPT:2#ABBREVIATION:PPA2#ECNUMBERS:3.6.1.1# LOCUS:_27068#TRANSCRIPT:1#ABBREVIATION:PPA2#ECNUMBERS:3.6.1.1# LOCUS:_27068#TRANSCRIPT:3#ABBREVIATION:PPA2#ECNUMBERS:3.6.1.1# LOCUS:_27068#TRANSCRIPT:4#ABBREVIATION:PPA2#ECNUMBERS:3.6.1.1# proteins: NAME:Inorganic pyrophosphatase 2, mitochondrial#ABBREVIATION:Ppa2.1-m# NAME:Inorganic pyrophosphatase 2, mitochondrial#ABBREVIATION:Ppa2.2-m# NAME:Inorganic pyrophosphatase 2, mitochondrial#ABBREVIATION:Ppa2.3-m# NAME:Inorganic pyrophosphatase 2, mitochondrial#ABBREVIATION:Ppa2.4-m# citations: AUTHORS:Fairchild TA, Patejunas G.#TITLE:Cloning and expression profile of human inorganic pyrophosphatase.#JOURNAL:Biochim Biophys Acta#YEAR:1999#PUBMED:10542310# AUTHORS:Fabrichniy IP, Lehtio L, Salminen A, Zyryanov AB, Baykov AA, Lahti R, Goldman A.#TITLE:Structural studies of metal ions in family II pyrophosphatases: the requirement for a Janus ion.#JOURNAL:Biochemistry#YEAR:2004#PUBMED:15533045# GENE ASSOCIATION: (27068.4) or (27068.1) or (27068.3) or (27068.2)
Abbreviation: R_GLCURtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: glcur[c] + h[c] <==> glcur[l] + h[l] Confidence Level: 4 H symport into lysososome. See PMID: 2768261 and PMID: 10581036 ( for human evidence). Sialic acid storage disorders (due to transporter mutations) require import and export e.g. PMID: 2768266 NJ renamed from GLCURlt to GLCURtly - NCD genes: LOCUS:_26503#TRANSCRIPT:1#ABBREVIATION:SLC17A5# proteins: NAME:solute carrier family 17 (anion/sugar transporter), member 5#ABBREVIATION:Slc17a5.1# citations: AUTHORS:Mancini GM, de Jonge HR, Galjaard H, Verheijen FW.#TITLE:Characterization of a proton-driven carrier for sialic acid in the lysosomal membrane. Evidence for a group-specific transport system for acidic monosaccharides.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2768261# AUTHORS:Verheijen FW, Verbeek E, Aula N, Beerens CE, Havelaar AC, Joosse M, Peltonen L, Aula P, Galjaard H, van der Spek PJ, Mancini GM.#TITLE:A new gene, encoding an anion transporter, is mutated in sialic acid storage diseases.#JOURNAL:Nat Genet#YEAR:1999#PUBMED:10581036# GENE ASSOCIATION: (26503.1)
N4-Acetylaminobutanal:NAD+ oxidoreductase (m)
Abbreviation: R_NABTNOm Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : h2o + n4abutn + nad --> 4aabutn + (2) h + nadh Confidence Level: 1 NCD SAB m genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# citations: none GENE ASSOCIATION: (217.1) or (8659.1) or (219.1)
Abbreviation: R_CERK Synonyms: _4 EC Number: 2.7.1.138 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : atp + crm_hs --> adp + crmp_hs + h Confidence Level: 4 NJ genes: LOCUS:_64781#TRANSCRIPT:1#ABBREVIATION:CERK# LOCUS:_64781#TRANSCRIPT:2#ABBREVIATION:CERK# proteins: NAME:ceramide kinase#ABBREVIATION:Cerk.1# NAME:ceramide kinase#ABBREVIATION:Cerk.2# citations: AUTHORS:Sugiura M, Kono K, Liu H, Shimizugawa T, Minekura H, Spiegel S, Kohama T. #TITLE:Ceramide kinase, a novel lipid kinase. Molecular cloning and functional characterization.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11956206# GENE ASSOCIATION: (64781.1) or (64781.2)
Abbreviation: R_DESAT18_4 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + nadh + o2 + stcoa --> (2) h2o + nad + vacccoa Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_6319#TRANSCRIPT:1#ABBREVIATION:SCD# proteins: NAME:stearoyl-CoA desaturase#ABBREVIATION:Scd.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Zhang L, Ge L, Parimoo S, Stenn K, Prouty SM#TITLE:Human stearoyl-CoA desaturase: alternative transcripts generated from a single gene by usage of tandem polyadenylation sites#JOURNAL:Biochem J#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# AUTHORS:Wang Y, Kurdi-Haidar B, Oram JF.#TITLE:LXR-mediated activation of macrophage stearoyl-CoA desaturase generates unsaturated fatty acids that destabilize ABCA1.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:14967823# GENE ASSOCIATION: (6319.1)
Abbreviation: R_PCRNtm Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: pcrn[c] --> pcrn[m] Confidence Level: 4 NJ genes: none proteins: none citations: AUTHORS:Jakobs BS, Wanders RJ.#TITLE:Fatty acid beta-oxidation in peroxisomes and mitochondria: the first, unequivocal evidence for the involvement of carnitine in shuttling propionyl-CoA from peroxisomes to mitochondria.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1995#PUBMED:7654220# GENE ASSOCIATION:
transport into the mitochondria from cytosol (carnitine)
Abbreviation: R_THRSERNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + ser-L[c] + thr-L[e] --> na1[c] + ser-L[e] + thr-L[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
L-serine/L-threonine Na-dependent exchange (Thr-L in)
Abbreviation: R_CLPNDt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: clpnd[e] <==> clpnd[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_NACHEX1ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_a_deg1 + h2o --> acgal + cs_a_deg2 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3073.1) or (3074.1)
Abbreviation: R_6THFtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _6thf[m] --> 6thf[c] Confidence Level: 2 unknown mechanism. IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_6-glutamyl-THF transport,m mitochondrial
Abbreviation: R_UDPGLCAtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: udpglcur[g] + ump[c] <==> udpglcur[c] + ump[g] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
UDPGlcA Golgi transport via UMP antiport
Abbreviation: R_RTOTALCRNCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : Rtotalcrn + coa --> Rtotalcoa + crn Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# AUTHORS:Fraser F, Padovese R, Zammit VA.#TITLE:Distinct kinetics of carnitine palmitoyltransferase i in contact sites and outer membranes of rat liver mitochondria.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11274214# GENE ASSOCIATION: (1376.1)
nucleoside-diphosphatase (GDP), extracellular
Abbreviation: R_NDP3ex Synonyms: _0 EC Number: 3.6.1.6 SUBSYSTEM: Nucleotides Equation: [e] : gdp + h2o --> gmp + h + pi Confidence Level: 4 EC is 3.6.1.42? NCD genes: LOCUS:_124583#TRANSCRIPT:1#ABBREVIATION:CANT1# proteins: NAME:calcium activated nucleotidase 1 #ABBREVIATION:Cant1.1-e# citations: AUTHORS:Smith TM, Hicks-Berger CA, Kim S, Kirley TL.#TITLE:Cloning, expression, and characterization of a soluble calcium-activated nucleotidase, a human enzyme belonging to a new family of extracellular nucleotidases.#JOURNAL:Arch Biochem Biophys. #YEAR:2002#PUBMED:12234496# AUTHORS:Murphy DM, Ivanenkov VV, Kirley TL. #TITLE:Bacterial expression and characterization of a novel, soluble, calcium-binding, and calcium-activated human nucleotidase. #JOURNAL:Biochemistry#YEAR:2003#PUBMED:12600208# GENE ASSOCIATION: (124583.1)
Abbreviation: R_BILIRUBtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: bilirub[c] <==> bilirub[r] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_k_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : k <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DNDPt51m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[m] + dctp[c] --> dcdp[c] + dctp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_UGLT Synonyms: _0 EC Number: 2.7.7.12 SUBSYSTEM: Galactose metabolism Equation: [c] : gal1p + udpg <==> g1p + udpgal Confidence Level: 0 NCD genes: LOCUS:_2592#TRANSCRIPT:1#ABBREVIATION:GALT#ECNUMBERS:2.7.7.12# LOCUS:_2592#TRANSCRIPT:3#ABBREVIATION:GALT#ECNUMBERS:2.7.7.12# LOCUS:_2592#TRANSCRIPT:2#ABBREVIATION:GALT#ECNUMBERS:2.7.7.12# proteins: NAME:galactose-1-phosphate uridylyltransferase#ABBREVIATION:Galt.1# NAME:galactose-1-phosphate uridylyltransferase#ABBREVIATION:Galt.2# NAME:galactose-1-phosphate uridylyltransferase#ABBREVIATION:Galt.3# citations: AUTHORS:Holden HM, Rayment I, Thoden JB#TITLE:Structure and function of enzymes of the Leloir pathway for galactose metabolism#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12923184# GENE ASSOCIATION: (2592.1) or (2592.3) or (2592.2)
UDPglucose--hexose-1-phosphate uridylyltransferase
Abbreviation: R_XOL7AH2tr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: xol7ah2[c] <==> xol7ah2[r] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_FUT18g Synonyms: _4 EC Number: 2.4.1.69 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacglcgalacglcgal14acglcgalgluside_hs + (2) gdpfuc --> fucfucgalacglcgalacglcgal14acglcgalgluside_hs + (2) gdp + (2) h Confidence Level: 4 NJ genes: LOCUS:_2523#TRANSCRIPT:1#ABBREVIATION:FUT1#ECNUMBERS:2.4.1.69# proteins: NAME:fucosyltransferase 1#ABBREVIATION:Fut1.1# citations: AUTHORS:Larsen RD, Ernst LK, Nair RP, Lowe JB.#TITLE:Molecular cloning, sequence, and expression of a human GDP-L-fucose:beta-D-galactoside 2-alpha-L-fucosyltransferase cDNA that can form the H blood group antigen.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990#PUBMED:2118655# GENE ASSOCIATION: (2523.1)
Abbreviation: R_FACOAL1821 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + lnlc <==> amp + lnlccoa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_EX_drib_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : drib <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SPMDOX Synonyms: _0 EC Number: 1.5.99.6 SUBSYSTEM: beta-Alanine metabolism Equation: [c] : h2o + o2 + spmd --> 13dampp + 4abutn + h2o2 Confidence Level: 2 SAB genes: none proteins: none citations: AUTHORS:Salim EI, Wanibuchi H, Morimura K, Kim S, Yano Y, Yamamoto S, Fukushima S#TITLE:Inhibitory effects of 1,3-diaminopropane, an ornithine decarboxylase inhibitor, on rat two-stage urinary bladder carcinogenesis initiated by N-butyl-N-(4-hydroxybutyl)nitrosamine#JOURNAL:Carcinogenesis#YEAR:2000#PUBMED:10657958# GENE ASSOCIATION:
Abbreviation: R_RETH Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : h2o + retfa --> Rtotal2 + h + retinol Confidence Level: 4 IT genes: LOCUS:_8228#TRANSCRIPT:1#ABBREVIATION:PNPLA4# proteins: NAME:keratinocyte retinyl ester hydrolase #ABBREVIATION:Reh.1# citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Gao J, Simon M.#TITLE:Identification of a novel keratinocyte retinyl ester hydrolase as a transacylase and lipase.#JOURNAL:J Invest Dermatol. #YEAR:2005#PUBMED:15955102# GENE ASSOCIATION: (8228.1)
_1-alpha,24R,25-Vitamin D-hydroxylase (D2)
Abbreviation: R_1a_COMMA_25VITD2Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : 1a25dhvitd2 + h + nadph + o2 --> 1a2425thvitd2 + h2o + nadp Confidence Level: 4 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.198 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_PTE5x Synonyms: _1 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : adrncoa + h2o --> adrn + coa + h Confidence Level: 4 NJ genes: LOCUS:_10965#TRANSCRIPT:1#ABBREVIATION:ZAP128# proteins: NAME:peroxisomal long-chain acyl-CoA thioesterase#ABBREVIATION:Zap128.1# citations: AUTHORS:Jones JM, Gould SJ.#TITLE:Identification of PTE2, a human peroxisomal long-chain acyl-CoA thioesterase.#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10944470# GENE ASSOCIATION: (10965.1)
Abbreviation: R_THRGLNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gln-L[c] + na1[e] + thr-L[e] --> gln-L[e] + na1[c] + thr-L[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-threonine/L-glutamine Na-dependent exchange (Thr-L in)
Abbreviation: R_TDPDRE Synonyms: _5 EC Number: 5.1.3.13 SUBSYSTEM: Nucleotide Sugar Metabolism Equation: [c] : dtdp4d6dg --> dtdp4d6dm Confidence Level: 1 JLR genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Tonetti M, Sturla L, Bisso A, Zanardi D, Benatti U, De Flora A#TITLE:The metabolism of 6-deoxyhexoses in bacterial and animal cells#JOURNAL:Biochimie#YEAR:1998#PUBMED:9893952# GENE ASSOCIATION:
Abbreviation: R_GGH_DASH_10FTHF6GLUe Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [e] : 10fthf6glu + h2o --> 10fthf5glu + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-e# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (10FTHF6GLU), extracellular
Abbreviation: R_THYOXt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hco3[c] + thyox-L[e] <==> hco3[e] + thyox-L[c] Confidence Level: 4 NJ genes: LOCUS:_53919#TRANSCRIPT:1#ABBREVIATION:SLCO1C1# LOCUS:_6579#TRANSCRIPT:1#ABBREVIATION:SLCO1A2# LOCUS:_28231#TRANSCRIPT:1#ABBREVIATION:SLCO4A1# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1A2#ABBREVIATION:Slco1a2.1# NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# NAME:solute carrier organic anion transporter family, member 4A1#ABBREVIATION:Slco4a1.1# NAME:solute carrier organic anion transporter family, member 1C1#ABBREVIATION:Slco1c1.1# citations: AUTHORS:Kullak-Ublick GA, Beuers U, Meier PJ, Domdey H, Paumgartner G.#TITLE:Assignment of the human organic anion transporting polypeptide (OATP) gene to chromosome 12p12 by fluorescence in situ hybridization.#JOURNAL:J Hepatol.#YEAR:1996#PUBMED:9007731# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (53919.1) or (6579.1) or (28231.1) or (10599.1)
T4 transport via bicarbonate countertransport
Abbreviation: R_G16MT_U Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : gdpmann + (0.1) m1mpdol_U --> gdp + h + (0.1) m2mpdol_U Confidence Level: 0 EC 2.4.1.- NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Glycolipid 1,6-alpha-D-mannosyltransferase (uterus)
Abbreviation: R_GALASE6ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg14 --> gal + ksi_deg15 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_HPDCAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hpdca[e] <==> hpdca[c] Confidence Level: 2 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MMMm Synonyms: _0 EC Number: 5.4.99.2 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : mmcoa-R <==> succoa Confidence Level: 4 TDV genes: LOCUS:_4594#TRANSCRIPT:1#ABBREVIATION:MUT#ECNUMBERS:5.4.99.2# proteins: NAME:methylmalonyl Coenzyme A mutase, mitochondrial #ABBREVIATION:Mut.1-m# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Ledley FD, Rosenblatt DS. #TITLE:Mutations in mut methylmalonic acidemia: clinical and enzymatic correlations.#YEAR:1997#PUBMED:8990001# GENE ASSOCIATION: (4594.1)
Abbreviation: R_GLCAT2g Synonyms: _1 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_a,b,e_pre1 + udpglcur --> cs_a,b_pre2 + h + udp Confidence Level: 4 EC 2.4.1.226 NCD genes: LOCUS:_22856#TRANSCRIPT:1#ABBREVIATION:CHSY1# LOCUS:_54480#TRANSCRIPT:1#ABBREVIATION:CSGlcA-T# LOCUS:_79586#TRANSCRIPT:1#ABBREVIATION:CHPF# LOCUS:_337876#TRANSCRIPT:1#ABBREVIATION:CSS3# proteins: NAME:chondroitin sulfate glucuronyltransferase, Golgi apparatus#ABBREVIATION:Csglca-t.1-g# NAME:carbohydrate (chondroitin) synthase 1, Golgi apparatus#ABBREVIATION:Chsy1.1-g# NAME:chondroitin polymerizing factor, Golgi apparatus#ABBREVIATION:Chpf.1-g# NAME:chondroitin sulfate synthase 3, Golgi apparatus#ABBREVIATION:Css3.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Gotoh M, Yada T, Sato T, Akashima T, Iwasaki H, Mochizuki H, Inaba N, Togayachi A, Kudo T, Watanabe H, Kimata K, Narimatsu H#TITLE:Molecular cloning and characterization of a novel chondroitin sulfate glucuronyltransferase that transfers glucuronic acid to N-acetylgalactosamine#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12145278# AUTHORS:Kitagawa H, Uyama T, Sugahara K#TITLE:Molecular cloning and expression of a human chondroitin synthase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11514575# AUTHORS:Kitagawa H, Izumikawa T, Uyama T, Sugahara K#TITLE:Molecular cloning of a chondroitin polymerizing factor that cooperates with chondroitin synthase for chondroitin polymerization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12716890# AUTHORS:Yada T, Sato T, Kaseyama H, Gotoh M, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-3. Molecular cloning and characterization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12907687# AUTHORS:Yada T, Gotoh M, Sato T, Shionyu M, Go M, Kaseyama H, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12761225# GENE ASSOCIATION: (54480.1) or (337876.1) or (79586.1) and (22856.1)
Abbreviation: R_MI145PK Synonyms: _1 EC Number: 2.7.1.127 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + mi145p --> adp + h + mi1345p Confidence Level: 4 NCD genes: LOCUS:_3706#TRANSCRIPT:1#ABBREVIATION:ITPKA#ECNUMBERS:2.7.1.*# LOCUS:_3707#TRANSCRIPT:1#ABBREVIATION:ITPKB#ECNUMBERS:2.7.1.127# LOCUS:_80271#TRANSCRIPT:1#ABBREVIATION:ITPKC# proteins: NAME:inositol 1,4,5-trisphosphate 3-kinase A#ABBREVIATION:Itpka.1# NAME:inositol 1,4,5-trisphosphate 3-kinase B#ABBREVIATION:Itpkb.1# NAME:inositol 1,4,5-trisphosphate 3-kinase C#ABBREVIATION:Itpkc.1# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:Takazawa K, Perret J, Dumont JE, Erneux C#TITLE:Molecular cloning and expression of a new putative inositol 1,4,5-trisphosphate 3-kinase isoenzyme#JOURNAL:Biochem J#YEAR:1991#PUBMED:1654894# AUTHORS:Dewaste V, Pouillon V, Moreau C, Shears S, Takazawa K, Erneux C#TITLE:Cloning and expression of a cDNA encoding human inositol 1,4,5-trisphosphate 3-kinase C#JOURNAL:Biochem J#YEAR:2000#PUBMED:11085927# GENE ASSOCIATION: (3706.1) or (80271.1) or (3707.1)
Abbreviation: R_AKR1C42 Synonyms: _7 EC Number: 1.1.1.50 SUBSYSTEM: Bile Acid Biosynthesis Equation: [c] : h + nadh + xoldioloneh --> nad + xoltriol Confidence Level: 4 NJ genes: LOCUS:_1109#TRANSCRIPT:1#ABBREVIATION:AKR1C4#ECNUMBERS:1.1.1.225# proteins: NAME:aldo-keto reductase family 1, member C4#ABBREVIATION:Akr1c4.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1109.1)
Abbreviation: R_NACHEX14ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg19 --> acgam + ksi_deg20 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3074.1) or (3073.1)
Abbreviation: R_MAN6PI Synonyms: _0 EC Number: 5.3.1.8 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : man6p <==> f6p Confidence Level: 2 genes: LOCUS:_4351#TRANSCRIPT:1#ABBREVIATION:MPI#ECNUMBERS:5.3.1.8# proteins: NAME:mannose phosphate isomerase #ABBREVIATION:Mpi.1# citations: AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# GENE ASSOCIATION: (4351.1)
Abbreviation: R_XOLEST2te Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: xolest2_hs[e] <==> xolest2_hs[c] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_5HOMEPRAZOLEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _5homeprazole[e] <==> 5homeprazole[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_xoltri27_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : xoltri27 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_7-alpha,27-Dihydroxycholesterol exchange
Abbreviation: R_P4504B1r Synonyms: _2 EC Number: 1.14.14.1 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : arachd + h + nadph + o2 --> 12harachd + h2o + nadp Confidence Level: 4 _12 hydroxylation of arachidonic acid NJ genes: LOCUS:_1572#TRANSCRIPT:1#ABBREVIATION:CYP2F1# proteins: NAME:cytochrome P450, family 2, subfamily F, polypeptide 1#ABBREVIATION:Cyp2f1.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Nhamburo PT, Gonzalez FJ, McBride OW, Gelboin HV, Kimura S.#TITLE:Abstract Identification of a new P450 expressed in human lung: complete cDNA sequence, cDNA-directed expression, and chromosome mapping.#JOURNAL:Biochemistry#YEAR:1989#PUBMED:2574990# AUTHORS:Rendic S.#TITLE:Summary of information on human CYP enzymes: human P450 metabolism data.#JOURNAL:Drug Metab Rev#YEAR:2002#PUBMED:11996015# GENE ASSOCIATION: (1572.1)
Abbreviation: R_RAHY Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : h + nadph + o2 + retn --> h2o + hretn + nadp Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_UMPK4 Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : gtp + ump <==> gdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
holocarboxylase synthestase (biotin protein ligase)
Abbreviation: R_BTNPL Synonyms: _0 SUBSYSTEM: Biotin Metabolism Equation: [c] : apoC-Lys + btamp --> amp + apoC-Lys_btn + h Confidence Level: 4 IT for human biotin metabolism genes: LOCUS:_3141#TRANSCRIPT:1#ABBREVIATION:HLCS#ECNUMBERS:6.3.4.10# proteins: NAME:holocarboxylase synthetase (biotin-[proprionyl-Coenzyme A-carboxylase (ATP-hydrolysing)] ligase) #ABBREVIATION:Hcls.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (3141.1)
Abbreviation: R_MTHFD Synonyms: _0 EC Number: 1.5.1.5 SUBSYSTEM: Folate Metabolism Equation: [c] : mlthf + nadp <==> methf + nadph Confidence Level: 4 genes: LOCUS:_4522#TRANSCRIPT:1#ABBREVIATION:MTHFD1#ECNUMBERS:1.5.1.5,3.5.4.9,6.3.4.3# proteins: NAME:methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1, methenyltetrahydrofolate cyclohydrolase, formyltetrahydrofolate synthetase#ABBREVIATION:Mthfd1.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (4522.1)
methylenetetrahydrofolate dehydrogenase (NADP)
Abbreviation: R_EX_7thf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 7thf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ALAGLNexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + gln-L[c] <==> ala-L[c] + gln-L[e] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (6520.1) and (56301.1)
Abbreviation: R_DOLPMT1_Ler Synonyms: _2 EC Number: 2.4.1.130 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolmanp_L + (0.1) m4mpdol_L --> (0.1) dolp_L + h + (0.1) m5mpdol_L Confidence Level: 0 NCD genes: LOCUS:_10195#TRANSCRIPT:1#ABBREVIATION:ALG3#ECNUMBERS:2.4.1.130# LOCUS:_10195#TRANSCRIPT:4#ABBREVIATION:ALG3#ECNUMBERS:2.4.1.130# LOCUS:_10195#TRANSCRIPT:3#ABBREVIATION:ALG3#ECNUMBERS:2.4.1.130# LOCUS:_10195#TRANSCRIPT:2#ABBREVIATION:ALG3#ECNUMBERS:2.4.1.130# proteins: NAME:alpha-1,3-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg3.1-er# NAME:alpha-1,3-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg3.2-er# NAME:alpha-1,3-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg3.3-er# NAME:alpha-1,3-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg3.4-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (10195.4) or (10195.1) or (10195.3) or (10195.2)
Abbreviation: R_CSDPASEly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cspg_d + h2o --> Ser-Gly/Ala-X-Gly + cs_d Confidence Level: 1 Note that this reaction occurs in the endosome during endocytosis, but has been compartmentalized as lysosomal. NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
chondroitin sulfate D proteoglycan protease, lysosome (endosome)
Abbreviation: R_GALASE17ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core2_deg5 --> gal + ksii_core2_deg6 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_5THFtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _5thf[m] --> 5thf[c] Confidence Level: 2 unknown mechanism. IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_5-glutamyl-THF transport,m mitochondrial
Abbreviation: R_FUCGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucgal14acglcgalgluside_hs[e] <==> fucgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CHSTEROLtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: atp[c] + chsterol[g] + h2o[c] --> adp[c] + chsterol[c] + h[c] + pi[c] Confidence Level: 4 IT genes: LOCUS:_19#TRANSCRIPT:1#ABBREVIATION:ABCA1# proteins: NAME:ATP-binding cassette, sub-family A (ABC1), member 1 #ABBREVIATION:Abca1.1-g# citations: AUTHORS:Wollmer MA, Streffer JR, Lutjohann D, Tsolaki M, Iakovidou V, Hegi T, Pasch T, Jung HH, Bergmann K, Nitsch RM, Hock C, Papassotiropoulos A. #TITLE:ABCA1 modulates CSF cholesterol levels and influences the age at onset of Alzheimer's disease.#JOURNAL:Neurobiol Aging. #YEAR:2003#PUBMED:12600718# AUTHORS:Orso E, Broccardo C, Kaminski WE, Bottcher A, Liebisch G, Drobnik W, Gotz A, Chambenoit O, Diederich W, Langmann T, Spruss T, Luciani MF, Rothe G, Lackner KJ, Chimini G, Schmitz G. #TITLE:Transport of lipids from golgi to plasma membrane is defective in tangier disease patients and Abc1-deficient mice.#JOURNAL:Nat Genet. #YEAR:2000#PUBMED:10655069# GENE ASSOCIATION: (19.1)
cholesterol efflux (ATP depedent), golgi
Abbreviation: R_NAt3_1 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[e] + na1[c] <==> h[c] + na1[e] Confidence Level: 4 JLR genes: LOCUS:_6550#TRANSCRIPT:1#ABBREVIATION:SLC9A3# LOCUS:_6549#TRANSCRIPT:1#ABBREVIATION:SLC9A2# LOCUS:_6548#TRANSCRIPT:1#ABBREVIATION:SLC9A1# LOCUS:_389015#TRANSCRIPT:1#ABBREVIATION:SLC9A4# LOCUS:_6553#TRANSCRIPT:1#ABBREVIATION:SLC9A5# proteins: NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 1 (antiporter, Na+/H+, amiloride sensitive)#ABBREVIATION:Slc9a1.1-e# NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 2#ABBREVIATION:Slc9a2.1-e# NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 3#ABBREVIATION:Slc9a3.1-e# NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 4#ABBREVIATION:Slc9a4.1-e# NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 5#ABBREVIATION:Slc9a5.1-e# citations: AUTHORS:Aronson PS#TITLE:Kinetic properties of the plasma membrane Na+-H+ exchanger#JOURNAL:Annu Rev Physiol#YEAR:1985#PUBMED:2581505# AUTHORS:Sardet C, Franchi A, Pouyssegur J#TITLE:Molecular cloning, primary structure, and expression of the human growth factor-activatable Na+/H+ antiporter.#JOURNAL:Cell#YEAR:1989#PUBMED:2536298# AUTHORS:Orlowski J, Grinstein S#TITLE:Diversity of the mammalian sodium/proton exchanger SLC9 gene family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12845533# AUTHORS:Malakooti J, Dahdal RY, Schmidt L, Layden TJ, Dudeja PK, Ramaswamy K#TITLE:Molecular cloning, tissue distribution, and functional expression of the human Na(+)/H(+) exchanger NHE2#JOURNAL:Am J Physiol#YEAR:1999#PUBMED:10444453# AUTHORS:Ghishan FK, Knobel SM, Summar M#TITLE:Molecular cloning, sequencing, chromosomal localization, and tissue distribution of the human Na+/H+ exchanger (SLC9A2)#JOURNAL:Genomics#YEAR:1995#PUBMED:8595899# AUTHORS:Brant SR, Yun CH, Donowitz M, Tse CM#TITLE:Cloning, tissue distribution, and functional analysis of the human Na+/N+ exchanger isoform, NHE3#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7631746# AUTHORS:Szpirer C, Szpirer J, Riviere M, Levan G, Orlowski J#TITLE:Chromosomal assignment of four genes encoding Na/H exchanger isoforms in human and rat.#JOURNAL:Mamm Genome#YEAR:1994#PUBMED:8199403# AUTHORS:Klanke CA, Su YR, Callen DF, Wang Z, Meneton P, Baird N, Kandasamy RA, Orlowski J, Otterud BE, Leppert M, et al#TITLE:Molecular cloning and physical and genetic mapping of a novel human Na+/H+ exchanger (NHE5/SLC9A5) to chromosome 16q22.1#JOURNAL:Genomics#YEAR:1995#PUBMED:7759094# AUTHORS:Baird NR, Orlowski J, Szabo EZ, Zaun HC, Schultheis PJ, Menon AG, Shull GE#TITLE:Molecular cloning, genomic organization, and functional expression of Na+/H+ exchanger isoform 5 (NHE5) from human brain#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9933641# GENE ASSOCIATION: (6553.1) or (6549.1) or (389015.1) or (6550.1) or (6548.1)
Abbreviation: R_P4502E1 Synonyms: _2 SUBSYSTEM: CYP Metabolism Equation: [c] : 4nph + h + nadph + o2 --> 24nph + h2o + nadp Confidence Level: 4 _4 nitrophenol 2-hydroxylation assumed to take place on outer membrane (xenobiotic metabolism) NJ genes: LOCUS:_1571#TRANSCRIPT:1#ABBREVIATION:CYP2E1# proteins: NAME:cytochrome P450, family 2, subfamily E, polypeptide 1#ABBREVIATION:Cyp2e1.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Umeno M, McBride OW, Yang CS, Gelboin HV, Gonzalez FJ.#TITLE:Human ethanol-inducible P450IIE1: complete gene sequence, promoter characterization, chromosome mapping, and cDNA-directed expression.#JOURNAL:Biochemistry#YEAR:1988#PUBMED:3233219# GENE ASSOCIATION: (1571.1)
Abbreviation: R_DNDPt7m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[m] + datp[c] --> dadp[c] + datp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_ALDSTRNtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: aldstrn[c] <==> aldstrn[m] Confidence Level: 2 specific mechanism not known NJ genes: none proteins: none citations: none GENE ASSOCIATION:
pantetheine-phosphate adenylyltransferase
Abbreviation: R_PTPAT Synonyms: _0 EC Number: 2.7.7.3 SUBSYSTEM: CoA Biosynthesis Equation: [c] : atp + h + pan4p <==> dpcoa + ppi Confidence Level: 4 genes: LOCUS:_80347#TRANSCRIPT:1#ABBREVIATION:KDTB/COAE#ECNUMBERS:2.7.7.3/2.7.1.24# proteins: NAME:pantetheine-phosphate adenyltransferase#ABBREVIATION:KdtB.1# citations: AUTHORS:Daugherty M, Polanuyer B, Farrell M, Scholle M, Lykidis A, de Crecy-Lagard V, Osterman A. #TITLE:Complete reconstitution of the human coenzyme A biosynthetic pathway via comparative genomics.#JOURNAL:J Biol Chem.#YEAR:2002#PUBMED:11923312# AUTHORS:Aghajanian S, Worrall DM.#TITLE:Identification and characterization of the gene encoding the human phosphopantetheine adenylyltransferase and dephospho-CoA kinase bifunctional enzyme (CoA synthase).#JOURNAL:Biochem J.#YEAR:2002#PUBMED:11994049# GENE ASSOCIATION: (80347.1)
Abbreviation: R_KSIt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ksi[g] --> ksi[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
keratan sulfate I transport, golgi to extracellular
Abbreviation: R_RADH Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : h2o + nad + retinal <==> (2) h + nadh + retn Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_PERILLYLte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: perillyl[e] <==> perillyl[c] Confidence Level: 1 unknown mechanism, xenobiotic transport (original and metabolized) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ST8SIA51g Synonyms: _13 EC Number: 2.4.99.8 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gd3_hs --> cmp + gt3_hs + h Confidence Level: 4 NJ genes: LOCUS:_29906#TRANSCRIPT:1#ABBREVIATION:SIAT8E# proteins: NAME:ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 5 #ABBREVIATION:St8sia5.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Harduin-Lepers A, Vallejo-Ruiz V, Krzewinski-Recchi MA, Smyn-Petit B, Julien S, Delannoy P#TITLE:The human sialyltransferase family#JOURNAL:Biochimie#YEAR:2001# AUTHORS:Kim YJ, Kim KS, Do S, Kim CH, Kim SK, Lee YC.#TITLE:Molecular cloning and expression of human alpha2,8-sialyltransferase (hST8Sia V).#JOURNAL:Biochem Biophys Res Commun.#YEAR:1997#PUBMED:9199191# GENE ASSOCIATION: (29906.1)
Abbreviation: R_S6TASE22ly Synonyms: _7 EC Number: 3.1.6.4 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core2_deg1 <==> h + ksii_core2_deg2 + so4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_HSD17B42x Synonyms: _3 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : h + nadph + o2 + thcholoylcoa --> dhocholoylcoa + (2) h2o + nadp Confidence Level: 4 see Russell bile acid synthesis review paper in human recon citation db adamanski et al Steroids (1997) 62:159 notes cofactor NADP/NADPH use genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# proteins: NAME:hydroxysteroid (17-beta) dehydrogenase 4#ABBREVIATION:Hsd17b4.1-x# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Adamski J, Leenders F Carstensen JF, Kaumann M, Markus MM, Husen B, Tesdorpf JG, Seedor U, deLaunoit Y, Jakob F#TITLE:Steroids, fatty acyl-CoA, and sterols are substrates of 80-kDa multifunctional protein#JOURNAL:Steroids#YEAR:1997# AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (3295.1)
Abbreviation: R_FCLTm Synonyms: _0 EC Number: 4.99.1.1 SUBSYSTEM: Heme Biosynthesis Equation: [m] : fe2 + ppp9 --> (2) h + pheme Confidence Level: 2 NCD genes: LOCUS:_2235#TRANSCRIPT:1#ABBREVIATION:FECH#ECNUMBERS:4.99.1.1# LOCUS:_2235#TRANSCRIPT:2#ABBREVIATION:FECH#ECNUMBERS:4.99.1.1# proteins: NAME:ferrochelatase#ABBREVIATION:Fech.1-m# NAME:Ferrochelatase, mitochondrial#ABBREVIATION:Fech.2-m# citations: AUTHORS:Tugores A, Magness ST, Brenner DA.#TITLE:A single promoter directs both housekeeping and erythroid preferential expression of the human ferrochelatase gene. #JOURNAL:J Biol Chem#YEAR:1994#PUBMED:7983009# GENE ASSOCIATION: (2235.1) or (2235.2)
Abbreviation: R_ST6GALNAC24 Synonyms: _5 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gm1a_hs --> cmp + gd1b2_hs + h Confidence Level: 2 NJ genes: LOCUS:_10610#TRANSCRIPT:1#ABBREVIATION:SIAT7B#ECNUMBERS:2.4.99.7# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 2#ABBREVIATION:St6galnac2.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10610.1)
Abbreviation: R_HSAT1ly Synonyms: _1 EC Number: 2.3.1.78 SUBSYSTEM: Heparan sulfate degradation Equation: accoa[c] + hs_deg2[l] --> coa[c] + h[l] + hs_deg3[l] Confidence Level: 4 The proton is located in the lysosome since it comes from oxidation of the NH3+ group in glucosamine. This reaction assumes that the other proton liberated from glucosamine reduces coa in the cytosol. NCD genes: none proteins: none citations: AUTHORS:Ausseil J, Loredo-Osti JC, Verner A, Darmond-Zwaig C, Maire I, Poorthuis B, van Diggelen OP, Hudson TJ, Fujiwara TM, Morgan K, Pshezhetsky AV#TITLE:Localisation of a gene for mucopolysaccharidosis IIIC to the pericentromeric region of chromosome 8#JOURNAL:J Med Genet#YEAR:2004#PUBMED:15591281# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
Abbreviation: R_11DOCRTSTRNtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _11docrtstrn[c] <==> 11docrtstrn[m] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_bz_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : bz <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HSD3B11r Synonyms: _7 SUBSYSTEM: Steroid Metabolism Equation: [r] : nad + prgnlone --> h + nadh + prgstrn Confidence Level: 4 Microsomal rxn This is a conglomerated reaction - in actuality there is an intermediate: progen-5-ene-3,20,dione (C21H30O2) for pregnenolone 5-androstene-3,17-dione for dhea genes: LOCUS:_3284#TRANSCRIPT:1#ABBREVIATION:HSD3B2#ECNUMBERS:1.1.1.145,,5.3.# proteins: NAME:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 2#ABBREVIATION:Hsd3b2.1# citations: AUTHORS:Leenders F, Dolez V, Begue A, Moller G, Gloeckner JC, deLaunoit Y, Adamski J#TITLE:Structure of the gene for the human 17-beta-hydroxysteroid dehydrogenase type IV#JOURNAL:Mammalian Genome#YEAR:1998# AUTHORS:Adamski J, Leenders F Carstensen JF, Kaumann M, Markus MM, Husen B, Tesdorpf JG, Seedor U, deLaunoit Y, Jakob F#TITLE:Steroids, fatty acyl-CoA, and sterols are substrates of 80-kDa multifunctional protein#JOURNAL:Steroids#YEAR:1997# AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (3284.1)
Abbreviation: R_GMPS2 Synonyms: _0 EC Number: 6.3.5.2 SUBSYSTEM: Nucleotides Equation: [c] : atp + gln-L + h2o + xmp --> amp + glu-L + gmp + (2) h + ppi Confidence Level: 4 JLR genes: LOCUS:_8833#TRANSCRIPT:1#ABBREVIATION:GMPS#ECNUMBERS:6.3.5.2# proteins: NAME:guanine monophosphate synthetase#ABBREVIATION:Gmps.1# citations: AUTHORS:Nakamura J, Lou L. #TITLE:Biochemical characterization of human GMP synthetase.#JOURNAL: J Biol Chem. #YEAR:1995#PUBMED:7706277# GENE ASSOCIATION: (8833.1)
Abbreviation: R_RETNGLCt2r Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: _13-cis-retnglc[r] --> 13-cis-retnglc[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Barua AB, Sidell N.#TITLE:Retinoyl beta-glucuronide: a biologically active interesting retinoid.#JOURNAL:J Nutr.#YEAR:2004#PUBMED:14704335# AUTHORS:Tang GW, Russell RM.#TITLE:_13-cis-retinoic acid is an endogenous compound in human serum.#JOURNAL:J Lipid Res.#YEAR:1990#PUBMED:2324641# GENE ASSOCIATION:
retinoyl glucuronide efflux (13-cis) from ER
Abbreviation: R_AMPtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: amp[c] <==> amp[r] Confidence Level: 1 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SPHMYLNtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: sphmyln_hs[c] <==> sphmyln_hs[l] Confidence Level: 2 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# GENE ASSOCIATION:
Abbreviation: R_TREH Synonyms: _0 EC Number: 3.2.1.28 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : h2o + tre --> (2) glc-D Confidence Level: 2 IF genes: LOCUS:_80162#TRANSCRIPT:1#ABBREVIATION:FLJ22635# proteins: NAME:hypothetical protein FLJ22635#ABBREVIATION:Flj22635.1# citations: none GENE ASSOCIATION: (80162.1)
Abbreviation: R_INSTt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: inost[e] + na1[e] <==> inost[c] + na1[c] Confidence Level: 4 NCD genes: LOCUS:_6526#TRANSCRIPT:1#ABBREVIATION:SLC5A3# proteins: NAME:solute carrier family 5 (inositol transporters), member 3, extracellular#ABBREVIATION:Slc5a3.1-e# citations: AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# AUTHORS:Kwon HM, Yamauchi A, Uchida S, Preston AS, Garcia-Perez A, Burg MB, Handler JS#TITLE:Cloning of the cDNa for a Na+/myo-inositol cotransporter, a hypertonicity stress protein#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1372904# AUTHORS:Berry GT, Mallee JJ, Kwon HM, Rim JS, Mulla WR, Muenke M, Spinner NB#TITLE:The human osmoregulatory Na+/myo-inositol cotransporter gene (SLC5A3): molecular cloning and localization to chromosome 21#JOURNAL:Genomics#YEAR:1995#PUBMED:7789985# AUTHORS:Hager K, Hazama A, Kwon HM, Loo DD, Handler JS, Wright EM#TITLE:Kinetics and specificity of the renal Na+/myo-inositol cotransporter expressed in Xenopus oocytes#JOURNAL:J Membr Biol#YEAR:1995#PUBMED:7537337# AUTHORS:Wright EM, Loo DD, Hirayama BA, Turk E#TITLE:Surprising versatility of Na+-glucose cotransporters: SLC5#JOURNAL:Physiology (Bethesda)#YEAR:2004#PUBMED:15546855# GENE ASSOCIATION: (6526.1)
Abbreviation: R_EX_4mtolbutamide_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 4mtolbutamide <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_C226COAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: c226coa[c] <==> c226coa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: AUTHORS:Sprecher H#TITLE:An update on the pathways of polyunsaturated fatty acid metabolism#JOURNAL:Curr Opn in Clinical Nutrition and Metabolic Care#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_P45039A1r Synonyms: _1 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : h + nadph + o2 + xol24oh --> h2o + nadp + xoltri24 Confidence Level: 4 NJ - by analogy/similarity to xol hydrolase reactions (NADPH w/ O2 - NADH also noted to be cofactor if needed) genes: LOCUS:_51302#TRANSCRIPT:1#ABBREVIATION:CYP39A1# proteins: NAME:cytochrome P450, family 39, subfamily A, polypeptide 1#ABBREVIATION:Cyp39a1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (51302.1)
Abbreviation: R_HEX4 Synonyms: _0 EC Number: 2.7.1.7 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : atp + man --> adp + h + man6p Confidence Level: 2 D-Glucose, D-mannose, D-fructose, sorbitol and D-glucosamine can act as acceptors; ITP and dATP can act as donors. The liver isoenzyme has sometimes been called glucokinase. also active in ecoli PMID: 4557975 genes: LOCUS:_3098#TRANSCRIPT:1#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3101#TRANSCRIPT:1#ABBREVIATION:HK3#ECNUMBERS:2.7.1.1# LOCUS:_3099#TRANSCRIPT:1#ABBREVIATION:HK2#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:1#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# LOCUS:_80201#TRANSCRIPT:1#ABBREVIATION:FLJ22761# LOCUS:_3098#TRANSCRIPT:4#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:3#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:5#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:2#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:2#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:3#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# proteins: NAME:glucokinase (hexokinase 4) (pancreatic beta islet)#ABBREVIATION:Gck.1# NAME:glucokinase (hexokinase 4) (major liver isoform)#ABBREVIATION:Gck.2# NAME:glucokinase (hexokinase 4) (minor liver isoform)#ABBREVIATION:Gck.3# NAME:hexokinase 1 (ubiquitous)#ABBREVIATION:Hk1.1# NAME:hexokinase 1 (erythrocyte)#ABBREVIATION:Hk1.2# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.3# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.4# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.5# NAME:hexokinase 2 (sk muscle)#ABBREVIATION:Hk2.1# NAME:hexokinase 3 (liver, lung, placenta)#ABBREVIATION:Hk3.1# NAME:hypothetical protein FLJ22761#ABBREVIATION:Flj22761.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (80201.1) or (3098.1) or (2645.3) or (2645.2) or (3098.3) or (3098.2) or (3099.1) or (2645.1) or (3098.5) or (3101.1) or (3098.4)
Abbreviation: R_DOPABMO Synonyms: _12 EC Number: 1.14.17.1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : ascb-L + dopa + o2 --> dhdascb + h2o + nrpphr Confidence Level: 4 SAB genes: LOCUS:_1621#TRANSCRIPT:1#ABBREVIATION:DBH#ECNUMBERS:1.14.17.1# LOCUS:_26002#TRANSCRIPT:1#ABBREVIATION:MOXD1# proteins: NAME:dopamine beta-hydroxylase (dopamine beta-monooxygenase)#ABBREVIATION:Dbh.1# NAME:monooxygenase, DBH-like 1#ABBREVIATION:Moxd1.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (26002.1) or (1621.1)
Abbreviation: R_SRTN23OX Synonyms: _0 EC Number: 1.13.11.42 SUBSYSTEM: Tryptophan metabolism Equation: [c] : o2 + srtn --> f5hoxkyn Confidence Level: 4 SAB genes: LOCUS:_3620#TRANSCRIPT:1#ABBREVIATION:INDO#ECNUMBERS:1.13.11.42# proteins: NAME:indoleamine-pyrrole 2,3 dioxygenase#ABBREVIATION:Indo.1# citations: AUTHORS:Okuma M, Tokuyama T, Senoh S, Hirata F, Hayaishi O#TITLE:Antagonism of 5-hydroxykynurenamine against serotonin action on platelet aggregation#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1976#PUBMED:1061163# GENE ASSOCIATION: (3620.1)
_5-Hydroxytryptamine:oxygen 2,3-dioxygenase (indole-decyclizing)
Abbreviation: R_GLCAE1g Synonyms: _4 EC Number: 5.1.3.19 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_a,b_pre3 --> cs_b_pre4 Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# GENE ASSOCIATION:
Abbreviation: R_S6TASE4ly Synonyms: _7 EC Number: 3.1.6.4 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_c + h2o <==> cs_c_deg1 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_FUCFUC132GALACGLCGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucfuc132galacglcgal14acglcgalgluside_hs[g] <==> fucfuc132galacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S3TASE3ly Synonyms: _1 EC Number: 3.1.6.15 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg23 <==> h + hs_deg24 + so4 Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Leder IG#TITLE:A novel 3-O sulfatase from human urine acting on methyl-2-deoxy-2-sulfamino-alphs-D-glucopyranoside 3-sulfate#JOURNAL:Biochem Biophys Res Commun#YEAR:1980#PUBMED:7396957# GENE ASSOCIATION:
N-acetylglucosamine-3-sulfatase, lysosomal
Abbreviation: R_NTD11 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Purine Catabolism Equation: [c] : h2o + imp --> ins + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_93034#TRANSCRIPT:1#ABBREVIATION:NT5C1B#ECNUMBERS:3.1.3.5# LOCUS:_22978#TRANSCRIPT:1#ABBREVIATION:NT5C2# LOCUS:_93034#TRANSCRIPT:2#ABBREVIATION:NT5C1B#ECNUMBERS:3.1.3.5# proteins: NAME:_5'-nucleotidase, cytosolic II #ABBREVIATION:Nt5C2.1# NAME:_5'-nucleotidase, cytosolic IB #ABBREVIATION:Nt5c1B.1# NAME:_5'-nucleotidase, cytosolic IB #ABBREVIATION:Nt5c1B.2# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# AUTHORS:Hunsucker SA, Spychala J, Mitchell BS.#TITLE:Human cytosolic 5'-nucleotidase I: characterization and role in nucleoside analog resistance. #JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11133996# GENE ASSOCIATION: (22978.1) or (93034.2) or (93034.1)
Abbreviation: R_HYPTROX Synonyms: _0 SUBSYSTEM: Taurine and hypotaurine metabolism Equation: [c] : (2) h + (2) hyptaur + o2 --> (2) taur Confidence Level: 2 SAB mechanism follows uncertain information in PMID 15189131 genes: none proteins: none citations: AUTHORS:Stipanuk MH#TITLE:Sulfur amino acid metabolism: pathways for production and removal of homocysteine and cysteine#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189131# GENE ASSOCIATION:
keratan sulfate II biosynthesis, precursor 9_C83H133N7O64S2X
Abbreviation: R_G14T21g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core4_pre9 + udpgal --> h + ksii_core4_pre10 + udp Confidence Level: 4 Keratan sulfate II biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
Abbreviation: R_GP1CALPHAte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gp1calpha_hs[c] <==> gp1calpha_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_ACNACNGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acnacngal14acglcgalgluside_hs[e] <==> acnacngal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NNATr Synonyms: _0 EC Number: 2.7.7.18 SUBSYSTEM: NAD Metabolism Equation: [c] : atp + h + nicrnt <==> dnad + ppi Confidence Level: 4 JLR genes: LOCUS:_349565#TRANSCRIPT:1#ABBREVIATION:NMNAT3# LOCUS:_23057#TRANSCRIPT:1#ABBREVIATION:NMNAT2#ECNUMBERS:2.7.7.1# LOCUS:_23057#TRANSCRIPT:2#ABBREVIATION:NMNAT2#ECNUMBERS:2.7.7.1# proteins: NAME:Nicotinamide nucleotide adenylyltransferase 2 #ABBREVIATION:Nmnat2.1# NAME:Nicotinamide nucleotide adenylyltransferase 2 #ABBREVIATION:Nmnat2.2# NAME:nicotinamide nucleotide adenylyltransferase 3 #ABBREVIATION:Nmnat3.1# citations: AUTHORS:Zhou T, Kurnasov O, Tomchick DR, Binns DD, Grishin NV, Marquez VE, Osterman AL, Zhang H.#TITLE:Structure of human nicotinamide/nicotinic acid mononucleotide adenylyltransferase. Basis for the dual substrate specificity and activation of the oncolytic agent tiazofurin#JOURNAL: J Biol Chem#YEAR:2002# AUTHORS:Yalowitz JA, Xiao S, Biju MP, Antony AC, Cummings OW, Deeg MA, Jayaram HN.#TITLE:Characterization of human brain nicotinamide 5'-mononucleotide adenylyltransferase-2 and expression in human pancreas. #JOURNAL:Biochem J.#YEAR:2004#PUBMED:14516279# AUTHORS:Zhang X, Kurnasov OV, Karthikeyan S, Grishin NV, Osterman AL, Zhang H. #TITLE:Structural characterization of a human cytosolic NMN/NaMN adenylyltransferase and implication in human NAD biosynthesis.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:12574164# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION: (349565.1) or (23057.2) or (23057.1)
nicotinate-nucleotide adenylyltransferase
Abbreviation: R_G14T16g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre32 + udpgal --> h + ksi_pre33 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (9334.1) or (8704.1) or (8703.1) or (8704.2) or (2683.1)
keratan sulfate I biosynthesis, precursor 33_C233H372N16O197S10X
Abbreviation: R_PE_HStm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: pe_hs[c] <==> pe_hs[m] Confidence Level: 1 for assumed reversible, non-energy dep scramblase mechanism (PMID: 10856717). NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_htaxol_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : htaxol <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RETH1 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : 9-cis-retfa + h2o --> Rtotal2 + h + retinol-9-cis Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_CSPG_Bt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cspg_b[g] --> cspg_b[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate B transport, golgi to extracellular
Abbreviation: R_EX_tststerones_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tststerones <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S3TASE2ly Synonyms: _1 EC Number: 3.1.6.15 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg17 <==> h + hs_deg18 + so4 Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Leder IG#TITLE:A novel 3-O sulfatase from human urine acting on methyl-2-deoxy-2-sulfamino-alphs-D-glucopyranoside 3-sulfate#JOURNAL:Biochem Biophys Res Commun#YEAR:1980#PUBMED:7396957# GENE ASSOCIATION:
Abbreviation: R_GF6PTA Synonyms: _0 EC Number: 2.6.1.16 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : f6p + gln-L --> gam6p + glu-L Confidence Level: 4 genes: LOCUS:_9945#TRANSCRIPT:1#ABBREVIATION:GFPT2# LOCUS:_2673#TRANSCRIPT:1#ABBREVIATION:GFPT1#ECNUMBERS:2.6.1.16# proteins: NAME:glutamine-fructose-6-phosphate transaminase 1#ABBREVIATION:Gfpt1.1# NAME:glutamine-fructose-6-phosphate transaminase 2#ABBREVIATION:Gfpt2.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:McKnight GL, Mudri SL, Mathewes SL, Traxinger RR, Marshall S, Sheppard PO, O'Hara PJ. #TITLE:Molecular cloning, cDNA sequence, and bacterial expression of human glutamine:fructose-6-phosphate amidotransferase.#YEAR:1992#PUBMED:1460020# AUTHORS:Oki T, Yamazaki K, Kuromitsu J, Okada M, Tanaka I. #TITLE:cDNA cloning and mapping of a novel subtype of glutamine:fructose-6-phosphate amidotransferase (GFAT2) in human and mouse. #YEAR:1999#PUBMED:10198162# AUTHORS:Broschat KO, Gorka C, Page JD, Martin-Berger CL, Davies MS, Huang Hc HC, Gulve EA, Salsgiver WJ, Kasten TP. #TITLE:Kinetic characterization of human glutamine-fructose-6-phosphate amidotransferase I: potent feedback inhibition by glucosamine 6-phosphate.#YEAR:2002#PUBMED:11842094# GENE ASSOCIATION: (2673.1) or (9945.1)
glutamine-fructose-6-phosphate transaminase
Abbreviation: R_UMPKn Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [n] : atp + ump <==> adp + udp Confidence Level: 4 NCD genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_EX_retnglc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : retnglc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_tag_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tag_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
glycine hydroxymethyltransferase, reversible, mitochondrial
Abbreviation: R_GHMT2rm Synonyms: _0 EC Number: 2.1.2.1 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : ser-L + thf <==> gly + h2o + mlthf Confidence Level: 4 NCD genes: LOCUS:_6472#TRANSCRIPT:1#ABBREVIATION:SHMT2#ECNUMBERS:2.1.2.1# proteins: NAME:serine hydroxymethyltransferase 2 (mitochondrial) #ABBREVIATION:Shmt2.1-m# citations: AUTHORS:Garrow TA, Brenner AA, Whitehead VM, Chen XN, Duncan RG, Korenberg JR, Shane B. #TITLE:Cloning of human cDNAs encoding mitochondrial and cytosolic serine hydroxymethyltransferases and chromosomal localization.#YEAR:1993#PUBMED:8505317# AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# AUTHORS:Narkewicz MR, Sauls SD, Tjoa SS, Teng C, Fennessey PV#TITLE:Evidence for intracellular partitioning of serine and glycine metabolism in Chinese hamster ovary cells#JOURNAL:Biochem J#YEAR:1996#PUBMED:8611185# GENE ASSOCIATION: (6472.1)
Abbreviation: R_EX_prostge2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : prostge2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DGSNtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgsn[c] <==> dgsn[m] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Watkins LF, Lewis RA.#TITLE:The metabolism of deoxyguanosine in mitochondria. Characterization of the uptake process.#JOURNAL:Mol Cell Biochem.#YEAR:1987#PUBMED:3696164# GENE ASSOCIATION:
Abbreviation: R_FUT94g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucgalacglcgal14acglcgalgluside_hs + gdpfuc --> fucfucgalacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
Abbreviation: R_TYRTAm Synonyms: _0 EC Number: 2.6.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [m] : akg + tyr-L <==> 34hpp + glu-L Confidence Level: 4 NCD genes: LOCUS:_6898#TRANSCRIPT:1#ABBREVIATION:TAT#ECNUMBERS:2.6.1.5# LOCUS:_2806#TRANSCRIPT:1#ABBREVIATION:GOT2#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase, mitochondrial#ABBREVIATION:Got2.1-m# NAME:tyrosine aminotransferase#ABBREVIATION:Tat.1-m# citations: AUTHORS:Rettenmeier R, Natt E, Zentgraf H, Scherer G.#TITLE:Isolation and characterization of the human tyrosine aminotransferase gene.#JOURNAL:Nucleic Acids Res. #YEAR:1990#PUBMED:1973834# GENE ASSOCIATION: (6898.1) or (2806.1)
Abbreviation: R_DHFtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: dhf[c] <==> dhf[l] Confidence Level: 2 NCD,IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
dihydrofolate reversible lysosomal transport
dehydrodolichol diphosphate phosphatase (uterus)
Abbreviation: R_DEDOLP1_U Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dedoldp_U + h2o --> (0.1) dedolp_U + h + pi Confidence Level: 0 EC-3.6.1.- NCD genes: none proteins: none citations: AUTHORS:Schenk B, Fernandez F, Waechter CJ.#TITLE:The ins(ide) and out(side) of dolichyl phosphate biosynthesis and recycling in the endoplasmic reticulum.#JOURNAL:Glycobiology#YEAR:2001#PUBMED:11425794# GENE ASSOCIATION:
Abbreviation: R_THRt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + thr-L[e] --> na1[c] + thr-L[c] Confidence Level: 4 JLR genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# proteins: NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# GENE ASSOCIATION: (11254.1) or (81539.1) or (54407.1)
Abbreviation: R_25VITD2Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : 25hvitd2 + h + nadph + o2 --> 1a25dhvitd2 + h2o + nadp Confidence Level: 4 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.196 IT genes: LOCUS:_1594#TRANSCRIPT:1#ABBREVIATION:CYP27B1#ECNUMBERS:1.14.-.-# proteins: NAME:cytochrome P450, family 27, subfamily B, polypeptide 1 #ABBREVIATION:Cyp27b1.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Yokomura K, Suda T, Sasaki S, Inui N, Chida K, Nakamura H. #TITLE:Increased expression of the 25-hydroxyvitamin D(3)-1alpha-hydroxylase gene in alveolar macrophages of patients with lung cancer.#JOURNAL:J Clin Endocrinol Metab. #YEAR:2003#PUBMED:14671156# AUTHORS:St-Arnaud R, Messerlian S, Moir JM, Omdahl JL, Glorieux FH.#TITLE:The 25-hydroxyvitamin D 1-alpha-hydroxylase gene maps to the pseudovitamin D-deficiency rickets (PDDR) disease locus.#JOURNAL: J Bone Miner Res. #YEAR:1997#PUBMED:9333115# GENE ASSOCIATION: (1594.1)
Abbreviation: R_DHAAt1r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dhdascb[e] <==> dhdascb[c] Confidence Level: 4 NCD genes: LOCUS:_6515#TRANSCRIPT:1#ABBREVIATION:SLC2A3# LOCUS:_6513#TRANSCRIPT:1#ABBREVIATION:SLC2A1# LOCUS:_6517#TRANSCRIPT:1#ABBREVIATION:SLC2A4# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 1, plasma membrane#ABBREVIATION:Slc2a1.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 3, extracellular#ABBREVIATION:Slc2a3.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 4, extracellular#ABBREVIATION:Slc2a4.1-e# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Uldry M, Thorens B#TITLE:The SLC2 family of facilitated hexose and polyol transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750891# AUTHORS:Haber RS, Weinstein SP, O'Boyle E, Morgello S#TITLE:Tissue distribution of the human GLUT3 glucose transporte#JOURNAL:Endocrinology#YEAR:1993#PUBMED:8504756# AUTHORS:Heijnen HF, Oorschot V, Sixma JJ, Slot JW, James DE#TITLE:Thrombin stimulates glucose transport in human platelets via the translocation of the glucose transporter GLUT-3 from alpha-granules to the cell surface#JOURNAL:J Cell Biol#YEAR:1997#PUBMED:9230074# AUTHORS:Kayano T, Fukumoto H, Eddy RL, Fan YS, Byers MG, Shows TB, Bell GI#TITLE:Evidence for a family of human glucose transporter-like proteins. Sequence and gene localization of a protein expressed in fetal skeletal muscle and other tissues#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3170580# AUTHORS:Stuart CA, Wen G, Jiang J#TITLE:GLUT3 protein and mRNA in autopsy muscle specimens#JOURNAL:Metabolism#YEAR:1999#PUBMED:10421229# AUTHORS:Fukumoto H, Kayano T, Buse JB, Edwards Y, Pilch PF, Bell GI, Seino S#TITLE:Cloning and characterization of the major insulin-responsive glucose transporter expressed in human skeletal muscle and other insulin-responsive tissues#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2656669# AUTHORS:Wilson JX#TITLE:Regulation of Vitamin C Transport#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15705056# GENE ASSOCIATION: (6515.1) or (6513.1) or (6517.1)
Abbreviation: R_PPCDC Synonyms: _0 EC Number: 4.1.1.36 SUBSYSTEM: CoA Biosynthesis Equation: [c] : 4ppcys + h --> co2 + pan4p Confidence Level: 4 genes: LOCUS:_60490#TRANSCRIPT:1#ABBREVIATION:PPCDC# proteins: NAME:DNA/pantothenate metabolism flavoprotein#ABBREVIATION:Dfp.1# citations: AUTHORS:Daugherty M, Polanuyer B, Farrell M, Scholle M, Lykidis A, de Crecy-Lagard V, Osterman A. #TITLE:Complete reconstitution of the human coenzyme A biosynthetic pathway via comparative genomics.#JOURNAL:J Biol Chem.#YEAR:2002#PUBMED:11923312# GENE ASSOCIATION: (60490.1)
phosphopantothenoylcysteine decarboxylase
Abbreviation: R_DCSPTN1CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + dcsptn1coa --> coa + dcsptn1crn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_GLU5Km Synonyms: _0 EC Number: 2.7.2.11 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : atp + glu-L --> adp + glu5p Confidence Level: 2 SAB m genes: LOCUS:_5832#TRANSCRIPT:1#ABBREVIATION:ALDH18A1# proteins: NAME:aldehyde dehydrogenase 18 family, member A1#ABBREVIATION:Aldh18a1.1-m# citations: AUTHORS:Hu CA, Lin WW, Obie C, Valle D#TITLE:Molecular enzymology of mammalian Delta1-pyrroline-5-carboxylate synthase. Alternative splice donor utilization generates isoforms with different sensitivity to ornithine inhibition#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10037775# GENE ASSOCIATION: (5832.1)
Abbreviation: R_GALASE4ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg8 --> gal + ksi_deg9 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_MESCOALm Synonyms: _0 EC Number: 4.1.3.25 SUBSYSTEM: C5-Branched dibasic acid metabolism Equation: [m] : h2o + mescoa --> accoa + pyr Confidence Level: 2 also EC 4.1.3.22 NCD genes: none proteins: none citations: AUTHORS:WANG SF, ADLER J, LARDY HA#TITLE:The pathway of itaconate metabolism by liver mitochondria#JOURNAL:J Biol Chem#YEAR:1961#PUBMED:13783048# GENE ASSOCIATION:
Abbreviation: R_ARTCOAL3 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : Rtotal3 + coa <==> Rtotal3coa Confidence Level: 0 HR fatty acid metabolism - R groups = acyl groups, Rtotal, Rtotal2, Rtotal3 correspond to three possible acyl groups added to glycerol Carbon stoichiometry normalized wrt C16 R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GP1Cte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gp1c_hs[c] <==> gp1c_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_TETPENT3CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: tetpent3crn[c] --> tetpent3crn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_NACHEX20ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg37 --> acgam + ksi_deg38 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3074.1) or (3073.1)
Abbreviation: R_TETHEX3COAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: tethex3coa[c] <==> tethex3coa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SULFOX Synonyms: _1 EC Number: 1.8.3.1 SUBSYSTEM: Cysteine Metabolism Equation: (2) ficytC[m] + h2o[c] + so3[c] --> (2) focytC[m] + (2) h[c] + so4[c] Confidence Level: 4 MM/TDV genes: LOCUS:_6821#TRANSCRIPT:1#ABBREVIATION:SUOX# proteins: NAME:Sulfite oxidase #ABBREVIATION:Suox.1# citations: none GENE ASSOCIATION: (6821.1)
Abbreviation: R_ARTFR56 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (2) fadh2[m] + (2) h[m] + (2) nadph[m] + tettet6coa[c] --> (1.5) R5coa_hs[c] + (2) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACNACNGALGBSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acnacngalgbside_hs[c] <==> acnacngalgbside_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_H3ETer Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : m2gacpail_hs + pe_hs --> dag_hs + memgacpail_hs Confidence Level: 2 NCD genes: LOCUS:_23556#TRANSCRIPT:1#ABBREVIATION:PIGN# LOCUS:_23556#TRANSCRIPT:2#ABBREVIATION:PIGN# proteins: NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.1-r# NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.2-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Hong Y, Maeda Y, Watanabe R, Ohishi K, Mishkind M, Riezman H, Kinoshita T#TITLE:Pig-n, a mammalian homologue of yeast Mcd4p, is involved in transferring phosphoethanolamine to the first mannose of the glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10574991# GENE ASSOCIATION: (23556.1) or (23556.2)
H3 phosphoethanolaminyl transferase, endoplasmic reticulum
Abbreviation: R_MI134PP Synonyms: _1 EC Number: 3.1.3.57 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi134p --> mi34p + pi Confidence Level: 4 NCD genes: LOCUS:_3628#TRANSCRIPT:1#ABBREVIATION:INPP1#ECNUMBERS:3.1.3.61# proteins: NAME:inositol polyphosphate-1-phosphatase#ABBREVIATION:Inpp1.1# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:York JD, Veile RA, Donis-Keller H, Majerus PW#TITLE:Cloning, heterologous expression, and chromosomal localization of human inositol polyphosphate 1-phosphatase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1993#PUBMED:8390685# GENE ASSOCIATION: (3628.1)
inositol-1,3,4-trisphosphate 1-phosphatase
Abbreviation: R_MINOHPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: minohp[c] <==> minohp[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
inositol hexakisphosphate nuclear transport (diffusion)
Abbreviation: R_EX_glygn2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : glygn2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
glycogen, structure 2 (glycogenin-1,6-{7[1,4-Glc], 4[1,4-Glc]}) exchange
Abbreviation: R_FADtru Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: fad[r] --> fad[c] Confidence Level: 1 diffusion - but unidirectional output from ER (to avoid loops w/ NAD/NADP) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UGT1A6r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Steroid Metabolism Equation: [r] : estradiol + udpglcur --> estradiolglc + udp Confidence Level: 4 NJ genes: LOCUS:_54575#TRANSCRIPT:1#ABBREVIATION:UGT1A10# LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A10#ABBREVIATION:Ugt1a10.1# NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# citations: AUTHORS:Basu NK, Kubota S, Meselhy MR, Ciotti M, Chowdhury B, Hartori M, Owens IS.#TITLE:Free Full Text Gastrointestinally distributed UDP-glucuronosyltransferase 1A10, which metabolizes estrogens and nonsteroidal anti-inflammatory drugs, depends upon phosphorylation.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117964# AUTHORS:Mackenzie PI, Owens IS, Burchell B, Bock KW, Bairoch A, Belanger A, Fournel-Gigleux S, Green M, Hum DW, Iyanagi T, Lancet D, Louisot P, Magdalou J, Chowdhury JR, Ritter JK, Schachter H, Tephly TR, Tipton KF, Nebert DW.#TITLE:The UDP glycosyltransferase gene superfamily: recommended nomenclature update based on evolutionary divergence.#JOURNAL:Pharmacogenetics#YEAR:1997#PUBMED:9295054# GENE ASSOCIATION: (54575.1) or (54576.1)
Abbreviation: R_DRPA Synonyms: _0 EC Number: 4.1.2.4 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : 2dr5p --> acald + g3p Confidence Level: 2 genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_B3GNT38g Synonyms: _9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : galside_hs + udpgal --> digalside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10331.1)
ribonucleoside-diphosphate reductase (ADP)
Abbreviation: R_RNDR1 Synonyms: _0 EC Number: 1.17.4.1 SUBSYSTEM: Nucleotides Equation: [c] : adp + trdrd --> dadp + h2o + trdox Confidence Level: 2 genes: LOCUS:_6241#TRANSCRIPT:1#ABBREVIATION:RRM2#ECNUMBERS:1.17.4.1# LOCUS:_6240#TRANSCRIPT:1#ABBREVIATION:RRM1#ECNUMBERS:1.7.14.1# proteins: NAME:ribonucleotide reductase #ABBREVIATION:Rrm12.1# citations: AUTHORS:Shao J, Zhou B, Zhu L, Qiu W, Yuan YC, Xi B, Yen Y.#TITLE: In vitro characterization of enzymatic properties and inhibition of the p53R2 subunit of human ribonucleotide reductase. #JOURNAL:Cancer Res. #YEAR:2004#PUBMED:14729598# GENE ASSOCIATION: (6240.1 and 6241.1)
Abbreviation: R_EX_dhf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dhf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6TASE10ly Synonyms: _7 EC Number: 3.1.6.4 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg4 <==> h + ksi_deg5 + so4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_ECOAH12m Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 2mp2coa + h2o <==> 3hibutcoa Confidence Level: 2 MM genes: LOCUS:_1892#TRANSCRIPT:1#ABBREVIATION:ECHS1# LOCUS:_3030#TRANSCRIPT:1#ABBREVIATION:HADHA#ECNUMBERS:4.2.1.17,1.1.1.35# LOCUS:_3032#TRANSCRIPT:1#ABBREVIATION:HADHB# proteins: NAME:enoyl Coenzyme A hydratase, short chain, 1, mitochondrial#ABBREVIATION:Echs1.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit #ABBREVIATION:Hadha.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit #ABBREVIATION:Hadhb.1-m# citations: AUTHORS:Sweetman, L, Williams, JC#TITLE:Branched chain organic acidurias#JOURNAL:The Metabolic and Molecular Bases of Inherited Disease, 8th ed (Scriver CR, et al., editors)#YEAR:2001# GENE ASSOCIATION: (1892.1) or (3030.1) and (3032.1)
_3-hydroxyacyl-CoA dehydratase (3-hydroxyisobutyryl-CoA) (mitochondria)
Abbreviation: R_GTPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: gtp[c] <==> gtp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GALFUCGALACGLCGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: galfucgalacglcgal14acglcgalgluside_hs[g] <==> galfucgalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
heparan sulfate, precursor 11_C79H121N5O77S4X
Abbreviation: R_GLCAE2g Synonyms: _5 EC Number: 5.1.3.17 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre10 --> hs_pre11 Confidence Level: 4 NCD genes: LOCUS:_26035#TRANSCRIPT:1#ABBREVIATION:GLCE# proteins: NAME:glucuronyl C5-epimerase, Golgi apparatus#ABBREVIATION:Glce.1-g# citations: AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Li JP, Gong F, El Darwish K, Jalkanen M, Lindahl U#TITLE:Characterization of the D-glucuronyl C5-epimerase involved in the biosynthesis of heparin and heparan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11274177# AUTHORS:Crawford BE, Olson SK, Esko JD, Pinhal MA#TITLE:Cloning, Golgi localization, and enzyme activity of the full-length heparin/heparan sulfate-glucuronic acid C5-epimerase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11279150# AUTHORS:Hagner-McWhirter A, Li JP, Oscarson S, Lindahl U#TITLE:Irreversible glucuronyl C5-epimerization in the biosynthesis of heparan sulfate.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14718527# GENE ASSOCIATION: (26035.1)
heparosan-N-sulfate-glucuronate 5-epimerase
Abbreviation: R_DNDPt42m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[m] + dutp[c] --> adp[c] + dutp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_HCO3_NAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hco3[e] + na1[e] <==> hco3[c] + na1[c] Confidence Level: 4 MM genes: LOCUS:_9497#TRANSCRIPT:1#ABBREVIATION:SLC4A7# proteins: NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 7, extracellular #ABBREVIATION:Slc4a7.1-e# citations: AUTHORS:Romero MF, Fulton CM, Boron WF.#TITLE:The SLC4 family of HCO 3 - transporters.#YEAR:2004#PUBMED:14722772# AUTHORS: Wang CZ, Yano H, Nagashima K, Seino S.#TITLE:The Na+-driven Cl-/HCO3- exchanger. Cloning, tissue distribution, and functional characterization. #YEAR:2000#PUBMED:10993873# GENE ASSOCIATION: (9497.1)
bicarbonate transport (Na/HCO3 cotransport)
Abbreviation: R_DM_bvite_LPAREN_c_RPAREN_ Synonyms: _0 Equation: [c] : bvite --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Dodecanoyl-ACP (n-C12:0ACP)_C23H43N2O8PRS
Abbreviation: R_NADPN Synonyms: _0 EC Number: 3.2.2.6 SUBSYSTEM: NAD Metabolism Equation: [c] : h2o + nadp --> adprbp + h + ncam Confidence Level: 2 See EC 3.2.2.5 for NAD nucleosidase genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Boyer CS, Moore GA, Moldeus P.#TITLE:Submitochondrial localization of the NAD+ glycohydrolase. Implications for the role of pyridine nucleotide hydrolysis in mitochondrial calcium fluxes.#JOURNAL:J Biol Chem. #YEAR:1993#PUBMED:8382685# GENE ASSOCIATION:
Abbreviation: R_LTDCL Synonyms: _0 EC Number: 4.1.1.28 SUBSYSTEM: Tryptophan metabolism Equation: [c] : h + trp-L --> co2 + trypta Confidence Level: 4 SAB genes: LOCUS:_1644#TRANSCRIPT:1#ABBREVIATION:DDC#ECNUMBERS:4.1.1.28# proteins: NAME:aromatic-L-amino-acid decarboxylase#ABBREVIATION:Ddc.1# citations: AUTHORS:Kitahama K, Ikemoto K, Jouvet A, Nagatsu I, Sakamoto N, Pearson J#TITLE:Aromatic L-amino acid decarboxylase- and tyrosine hydroxylase-immunohistochemistry in the adult human hypothalamus#JOURNAL:J Chem Neuroanat#YEAR:1998#PUBMED:9924972# GENE ASSOCIATION: (1644.1)
Abbreviation: R_NACHEX17ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg28 --> acgam + ksi_deg29 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3074.1) or (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_CAt7r Synonyms: _1 SUBSYSTEM: Transport, Extracellular Equation: ca2[c] + (3) na1[e] <==> ca2[e] + (3) na1[c] Confidence Level: 4 MM genes: LOCUS:_6546#TRANSCRIPT:1#ABBREVIATION:SLC8A1# LOCUS:_6543#TRANSCRIPT:1#ABBREVIATION:SLC8A2# LOCUS:_6547#TRANSCRIPT:1#ABBREVIATION:SLC8A3# LOCUS:_6547#TRANSCRIPT:5#ABBREVIATION:SLC8A3# LOCUS:_6547#TRANSCRIPT:4#ABBREVIATION:SLC8A3# LOCUS:_6547#TRANSCRIPT:3#ABBREVIATION:SLC8A3# LOCUS:_6547#TRANSCRIPT:2#ABBREVIATION:SLC8A3# LOCUS:_6547#TRANSCRIPT:6#ABBREVIATION:SLC8A3# proteins: NAME:solute carrier family 8 (sodium/calcium exchanger), member 1, extracellular #ABBREVIATION:Slc8a1.1-e# NAME:solute carrier family 8 (sodium-calcium exchanger), member 2, extracellular #ABBREVIATION:Slc8a2.1-e# NAME:solute carrier family 8 (sodium-calcium exchanger), member 3, extracellular #ABBREVIATION:Slc8a3.1-e# NAME:solute carrier family 8 (sodium-calcium exchanger), member 3, extracellular #ABBREVIATION:Slc8a3.2-e# NAME:solute carrier family 8 (sodium-calcium exchanger), member 3, extracellular #ABBREVIATION:Slc8a3.3-e# NAME:solute carrier family 8 (sodium-calcium exchanger), member 3, extracellular #ABBREVIATION:Slc8a3.4-e# NAME:solute carrier family 8 (sodium-calcium exchanger), member 3, extracellular #ABBREVIATION:Slc8a3.5-e# NAME:solute carrier family 8 (sodium-calcium exchanger), member 3, extracellular #ABBREVIATION:Slc8a3.6-e# citations: AUTHORS:Gabellini,N., Bortoluzzi,S., Danieli,G.A., Carafoli,E., #TITLE:The human SLC8A3 gene and the tissue-specific Na+/Ca2+ exchanger 3 isoforms. #YEAR:2002#PUBMED:12406570# AUTHORS:Schnetkamp PP.#TITLE:Na-Ca or Na-Ca-K exchange in rod photoreceptors.#YEAR:1989#PUBMED:2484986# GENE ASSOCIATION: (6547.6) or (6547.4) or (6547.2) or (6546.1) or (6547.1) or (6547.5) or (6543.1) or (6547.3)
calcium / sodium antiporter (1:3), reversible
Abbreviation: R_ADSS Synonyms: _0 EC Number: 6.3.4.4 SUBSYSTEM: Nucleotides Equation: [c] : asp-L + gtp + imp --> dcamp + gdp + (2) h + pi Confidence Level: 2 genes: LOCUS:_159#TRANSCRIPT:1#ABBREVIATION:ADSS#ECNUMBERS:6.3.4.4# LOCUS:_122622#TRANSCRIPT:1#ABBREVIATION:ADSSL1# LOCUS:_122622#TRANSCRIPT:2#ABBREVIATION:ADSSL1# proteins: NAME:adenylosuccinate synthase #ABBREVIATION:Adss.1# NAME:adenylosuccinate synthase like 1#ABBREVIATION:Adssl1.2# NAME:adenylosuccinate synthase like 1#ABBREVIATION:Adssl1.1# citations: none GENE ASSOCIATION: (159.1) or (122622.1) or (122622.2)
Abbreviation: R_PCFLOPm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[c] + h2o[c] + pchol_hs[m] --> adp[c] + h[c] + pchol_hs[c] + pi[c] Confidence Level: 4 See PMID: 10856717 - FLOPPASE activity no detailed data on which compartment ATP hydrolysis occurs, so for the time being assumed to be cytosolic NJ genes: LOCUS:_57194#TRANSCRIPT:1#ABBREVIATION:ATP10A#ECNUMBERS:3.6.3.1# proteins: NAME:ATPase, Class V, type 10A#ABBREVIATION:Atp10a.1# citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# AUTHORS:Herzing LB, Kim SJ, Cook EH Jr, Ledbetter DH.#TITLE:The human aminophospholipid-transporting ATPase gene ATP10C maps adjacent to UBE3A and exhibits similar imprinted expression.#JOURNAL:Am J Hum Genet#YEAR:2001#PUBMED:11353404# GENE ASSOCIATION: (57194.1)
Abbreviation: R_O2Stx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: o2s[c] <==> o2s[x] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Archibald F.#TITLE:Oxygen toxicity and the health and survival of eukaryote cells: a new piece is added to the puzzle. #JOURNAL:Proc Natl Acad Sci U S A#YEAR:2003#PUBMED:12939409# GENE ASSOCIATION:
superoxide anion transport via diffusion (peroxisome)
Abbreviation: R_EAFLATOXINte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: eaflatoxin[e] <==> eaflatoxin[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_tethex3_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tethex3 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DNDPt14m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dtdp[m] + dudp[c] --> dtdp[c] + dudp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_G14T10g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre14 + udpgal --> h + ksi_pre15 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (9334.1) or (2683.1) or (8703.1) or (8704.2) or (8704.1)
Abbreviation: R_PGSr Synonyms: _9 EC Number: 1.14.99.1 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : arachd + h + nadph + (2) o2 --> h2o + nadp + prostgh2 Confidence Level: 4 NJ genes: LOCUS:_5742#TRANSCRIPT:1#ABBREVIATION:PTGS1#ECNUMBERS:1.14.99.1# LOCUS:_5743#TRANSCRIPT:1#ABBREVIATION:PTGS2#ECNUMBERS:1.14.99.1# proteins: NAME:prostaglandin-endoperoxide synthase 2#ABBREVIATION:Ptgs2.1# NAME:prostaglandin-endoperoxide synthase 1#ABBREVIATION:Ptgs1.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Maihofner C, Probst-Cousin S, Bergmann M, Neuhuber W, Neundorfer B, Heuss D.#TITLE:Expression and localization of cyclooxygenase-1 and -2 in human sporadic amyotrophic lateral sclerosis.#JOURNAL:Eur J Neurosci#YEAR:2003#PUBMED:14511332# AUTHORS:Funk CD, Funk LB, Kennedy ME, Pong AS, Fitzgerald GA.#TITLE:Human platelet/erythroleukemia cell prostaglandin G/H synthase: cDNA cloning, expression, and gene chromosomal assignment.#JOURNAL:FASEB J#YEAR:1991#PUBMED:1907252# GENE ASSOCIATION: (5743.1) or (5742.1)
Abbreviation: R_FUT99g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacglc13galacglcgal14acglcgalgluside_hs + gdpfuc --> fucgalacglc13galacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
keratan sulfate I, degradation product 10_C188H302N13O163S9
Abbreviation: R_NACHEX11ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg10 --> acgam + ksi_deg11 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1) or (3074.1)
Abbreviation: R_RIBt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[e] + rib-D[e] --> h[c] + rib-D[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Huck JH, Roos B, Jakobs C, van der Knaap MS, Verhoeven NM#TITLE:Evaluation of pentitol metabolism in mammalian tissues provides new insight into disorders of human sugar metabolism#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15234337# GENE ASSOCIATION:
ribose transport in via proton symporter
Abbreviation: R_NACHEXA7ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e + (3) h2o --> acgal + cs_e_deg3 + (2) h + (2) so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_APRGSTRNte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: aprgstrn[e] <==> aprgstrn[c] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_P4502C19 Synonyms: _5 SUBSYSTEM: CYP Metabolism Equation: [c] : h + nadph + o2 + omeprazole --> 5homeprazole + h2o + nadp Confidence Level: 4 omeprazole 5 methyl hydroxylation assumed to take place on outer membrane - since a xenobiotic NJ genes: LOCUS:_1557#TRANSCRIPT:1#ABBREVIATION:CYP2C19#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 2, subfamily C, polypeptide 19#ABBREVIATION:Cyp2c19.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Meier UT, Meyer UA.#TITLE:Genetic polymorphism of human cytochrome P-450 (S)-mephenytoin 4-hydroxylase. Studies with human autoantibodies suggest a functionally altered cytochrome P-450 isozyme as cause of the genetic deficiency.#JOURNAL:Biochemistry#YEAR:1987#PUBMED:3442670# GENE ASSOCIATION: (1557.1)
Abbreviation: R_MM5ag Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m5masnC --> m4masn + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
Abbreviation: R_10FTHF5GLUtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _10fthf5glu[c] --> 10fthf5glu[l] Confidence Level: 2 carrier-mediated transport - but which and how is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
trihexosyl ceramide (homo sapiens)_C36H66NO17FULLRCO
Abbreviation: R_DHCHOLESTANATEtm Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: dhcholestanate[m] <==> dhcholestanate[x] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_ORETNF Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : o2 + (2) retn --> (2) oretn Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
_4-oxo-retinoic acid formation (oxidation)
UDP-D-glucuronate:galactosylgalactosylxylose glucuronosyltransferase, Golgi apparatus
Abbreviation: R_GLCATg Synonyms: _3 EC Number: 2.4.1.135 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : l2xser + udpglcur --> cs,hs_linkage + h + udp Confidence Level: 4 NCD genes: LOCUS:_26229#TRANSCRIPT:1#ABBREVIATION:B3GAT3# LOCUS:_27087#TRANSCRIPT:1#ABBREVIATION:B3GAT1# LOCUS:_135152#TRANSCRIPT:1#ABBREVIATION:B3GAT2# LOCUS:_27087#TRANSCRIPT:2#ABBREVIATION:B3GAT1# proteins: NAME:beta-1,3-glucuronyltransferase 2 (glucuronosyltransferase S), Golgi apparatus#ABBREVIATION:B3gat2.1-g# NAME: beta-1,3-glucuronyltransferase 3 (glucuronosyltransferase I), Golgi apparatus#ABBREVIATION:B3gat3.1-g# NAME:beta-1,3-glucuronyltransferase 1 (glucuronosyltransferase P), Golgi apparatus#ABBREVIATION:B3gat.1-g# NAME:beta-1,3-glucuronyltransferase 1 (glucuronosyltransferase P), Golgi apparatus#ABBREVIATION:B3gat1.2-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Marcos I, Galan JJ, Borrego S, Antinolo G#TITLE:Cloning, characterization, and chromosome mapping of the human GlcAT-S gene#JOURNAL:J Hum Genet#YEAR:2002#PUBMED:12522689# AUTHORS:Kitagawa H, Tone Y, Tamura J, Neumann KW, Ogawa T, Oka S, Kawasaki T, Sugahara K#TITLE:Molecular cloning and expression of glucuronyltransferase I involved in the biosynthesis of the glycosaminoglycan-protein linkage region of proteoglycans#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9506957# AUTHORS:Mitsumoto Y, Oka S, Sakuma H, Inazawa J, Kawasaki T#TITLE:Cloning and chromosomal mapping of human glucuronyltransferase involved in biosynthesis of the HNK-1 carbohydrate epitope#JOURNAL:Genomics#YEAR:2000#PUBMED:10783264# GENE ASSOCIATION: (27087.2) or (135152.1) or (27087.1) or (26229.1)
mesaconate--CoA ligase (ADP-forming), mitochondrial
Abbreviation: R_MECOALm Synonyms: _1 EC Number: 6.2.1.5 SUBSYSTEM: C5-Branched dibasic acid metabolism Equation: [m] : atp + coa + mescon <==> adp + mescoa + pi Confidence Level: 1 NCD genes: LOCUS:_8802#TRANSCRIPT:1#ABBREVIATION:SUCLG1#ECNUMBERS:6.2.1.4# LOCUS:_8803#TRANSCRIPT:1#ABBREVIATION:SUCLA2#ECNUMBERS:6.2.1.5# proteins: NAME:succinate--CoA ligase, ADP forming, mitochondrial#ABBREVIATION:Sucla-m# citations: AUTHORS:WANG SF, ADLER J, LARDY HA#TITLE:The pathway of itaconate metabolism by liver mitochondria#JOURNAL:J Biol Chem#YEAR:1961#PUBMED:13783048# GENE ASSOCIATION: (8802.1 and 8803.1)
Abbreviation: R_NACHEX21ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg39 --> acgam + ksi_deg40 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3074.1) or (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_ARGSL Synonyms: _0 EC Number: 4.3.2.1 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [c] : argsuc <==> arg-L + fum Confidence Level: 4 genes: LOCUS:_435#TRANSCRIPT:1#ABBREVIATION:ASL#ECNUMBERS:4.3.2.1# proteins: NAME:argininosuccinate lyase#ABBREVIATION:Asl.1# citations: AUTHORS:O'Brien WE, McInnes R, Kalumuck K, Adcock M.#TITLE:Cloning and sequence analysis of cDNA for human argininosuccinate lyase.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1986#PUBMED:3463959# AUTHORS:Kleijer WJ et al.#TITLE:Clinical, enzymatic, and molecular genetic characterization of a biochemical variant type of argininosuccinic aciduria: prenatal and postnatal diagnosis in five unrelated families.#YEAR:2002#PUBMED:12408190# AUTHORS:Sampaleanu LM, Vallee F, Thompson GD, Howell PL.#TITLE:Three-dimensional structure of the argininosuccinate lyase frequently complementing allele Q286R.#YEAR:2001#PUBMED:11747432# GENE ASSOCIATION: (435.1)
Abbreviation: R_SPODMn Synonyms: _1 EC Number: 1.15.1.1 SUBSYSTEM: ROS Detoxification Equation: [n] : (2) h + (2) o2s --> h2o2 + o2 Confidence Level: 4 JLR genes: LOCUS:_6647#TRANSCRIPT:1#ABBREVIATION:SOD1#ECNUMBERS:1.15.1.1# proteins: NAME:superoxide dismutase 1, soluble (amyotrophic lateral sclerosis 1 (adult))#ABBREVIATION:Sod1.1-n# citations: AUTHORS:Crapo JD, Oury T, Rabouille C, Slot JW, Chang LY#TITLE:Copper,zinc superoxide dismutase is primarily a cytosolic protein in human cells#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1992#PUBMED:1332049# AUTHORS:Hallewell RA, Masiarz FR, Najarian RC, Puma JP, Quiroga MR, Randolph A, Sanchez-Pescador R, Scandella CJ, Smith B, Steimer KS, et al#TITLE:Human Cu/Zn superoxide dismutase cDNA: isolation of clones synthesising high levels of active or inactive enzyme from an expression library#JOURNAL:Nucleic Acids Res#YEAR:1985#PUBMED:3889846# GENE ASSOCIATION: (6647.1)
Abbreviation: R_VITD2tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: vitd2[m] <==> vitd2[c] Confidence Level: 2 I am not sure how vitamin D3 is transported in cytosol based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_CTPS1 Synonyms: _0 EC Number: 6.3.4.2 SUBSYSTEM: Nucleotides Equation: [c] : atp + nh4 + utp --> adp + ctp + (2) h + pi Confidence Level: 2 genes: LOCUS:_1503#TRANSCRIPT:1#ABBREVIATION:CTPS#ECNUMBERS:6.3.4.2# proteins: NAME:CTP synthase #ABBREVIATION:Ctps.1# citations: AUTHORS:Takahashi E, Yamauchi M, Tsuji H, Hitomi A, Meuth M, Hori T.#TITLE:Chromosome mapping of the human cytidine-5'-triphosphate synthetase (CTPS) gene to band 1p34.1-p34.3 by fluorescence in situ hybridization.#JOURNAL:Hum Genet.#YEAR:1991#PUBMED:1959918# GENE ASSOCIATION: (1503.1)
Abbreviation: R_PYDXNK Synonyms: _0 EC Number: 2.7.1.35 SUBSYSTEM: Vitamin B6 Metabolism Equation: [c] : atp + pydxn --> adp + h + pdx5p Confidence Level: 4 NCD JLR-Pyridoxine, pyridoxamine and various derivatives can also act as acceptors genes: LOCUS:_8566#TRANSCRIPT:1#ABBREVIATION:PDXK#ECNUMBERS:2.7.1.35# proteins: NAME:pyridoxal (pyridoxine, vitamin B6) kinase #ABBREVIATION:PdxK.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# AUTHORS:Chern CJ, Beutler E. #TITLE:Biochemical and electrophoretic studies of erythrocyte pyridoxine kinase in white and black Americans.#JOURNAL:Am J Hum Genet#YEAR:1976#PUBMED:2009# GENE ASSOCIATION: (8566.1)
Abbreviation: R_CHOLt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: chol[e] + na1[e] <==> chol[c] + na1[c] Confidence Level: 4 NCD genes: LOCUS:_60482#TRANSCRIPT:1#ABBREVIATION:SLC5A7# proteins: NAME:solute carrier family 5 (choline transporter), member 7#ABBREVIATION:Slc5a7.1-e# citations: AUTHORS:Okuda T, Haga T#TITLE:High-affinity choline transporter#JOURNAL:Neurochem Res#YEAR:2003#PUBMED:12675135# AUTHORS:Okuda T, Haga T.#TITLE:Functional characterization of the human high-affinity choline transporter#JOURNAL:FEBS Lett#YEAR:2000#PUBMED:11068039# AUTHORS:Apparsundaram S, Ferguson SM, George AL Jr, Blakely RD#TITLE:Molecular cloning of a human, hemicholinium-3-sensitive choline transporter#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:11027560# GENE ASSOCIATION: (60482.1)
Abbreviation: R_DESAT20_2 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : eicostetcoa + h + nadh + o2 --> (2) h2o + nad + tmndnccoa Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_3992#TRANSCRIPT:1#ABBREVIATION:FADS1#ECNUMBERS:1.14.99.25# proteins: NAME:fatty acid desaturase 1#ABBREVIATION:Fads1.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Cho HP, Nakamura M, Clarke SD#TITLE:Cloning, expression, and fatty acid regulation of the human delta-5 desaturase#JOURNAL:The Journal of Biological Chemistry#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION: (3992.1)
fatty acyl-CoA desaturase (n-C20:4CoA -> n-C20:5CoA)
Abbreviation: R_FPGS7m Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [m] : 10fthf + (4) atp + (4) glu-L --> 10fthf5glu + (4) adp + (4) h + (4) pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_DUDPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: dudp[c] <==> dudp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GNMT Synonyms: _0 EC Number: 2.1.1.20 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : amet + gly --> ahcys + h + sarcs Confidence Level: 4 genes: LOCUS:_27232#TRANSCRIPT:1#ABBREVIATION:GNMT#ECNUMBERS:2.1.1.20# proteins: NAME:glycine N-methyltransferase #ABBREVIATION:Gnmt.1# citations: AUTHORS:Chen YM, Chen LY, Wong FH, Lee CM, Chang TJ, Yang-Feng TL.#TITLE:Genomic structure, expression, and chromosomal localization of the human glycine N-methyltransferase gene.#YEAR:2000#PUBMED:10843803# AUTHORS:Luka Z, Cerone R, Phillips JA 3rd, Mudd HS, Wagner C.#TITLE:Mutations in human glycine N-methyltransferase give insights into its role in methionine metabolism. #YEAR:2002#PUBMED:11810299# GENE ASSOCIATION: (27232.1)
Abbreviation: R_DASPO1p Synonyms: _0 EC Number: 1.4.3.1 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [x] : asp-D + h2o + o2 --> h2o2 + nh4 + oaa Confidence Level: 4 MM genes: LOCUS:_8528#TRANSCRIPT:1#ABBREVIATION:DDO#ECNUMBERS:1.4.3.1# LOCUS:_8528#TRANSCRIPT:2#ABBREVIATION:DDO#ECNUMBERS:1.4.3.1# proteins: NAME:D-aspartate oxidase, perixosomal#ABBREVIATION:Ddo.1-x# NAME:D-aspartate oxidase, perixosomal#ABBREVIATION:Ddo.2-x# citations: AUTHORS:Zaar K, Kost HP, Schad A, Volkl A, Baumgart E, Fahimi HD.#TITLE:Cellular and subcellular distribution of D-aspartate oxidase in human and rat brain. #YEAR:2002#PUBMED:12209855# AUTHORS:Amery L, Brees C, Baes M, Setoyama C, Miura R, Mannaerts GP, Van Veldhoven PP. #TITLE:C-terminal tripeptide Ser-Asn-Leu (SNL) of human D-aspartate oxidase is a functional peroxisome-targeting signal.#YEAR:1998#PUBMED:9820813# GENE ASSOCIATION: (8528.2) or (8528.1)
Abbreviation: R_HISDC Synonyms: _0 EC Number: 4.1.1.22 SUBSYSTEM: Histidine Metabolism Equation: [c] : h + his-L --> co2 + hista Confidence Level: 4 IF genes: LOCUS:_3067#TRANSCRIPT:1#ABBREVIATION:HDC#ECNUMBERS:4.1.1.22# LOCUS:_1644#TRANSCRIPT:1#ABBREVIATION:DDC#ECNUMBERS:4.1.1.28# proteins: NAME:histidine decarboxylase#ABBREVIATION:Hdc.1# NAME:aromatic-L-amino-acid decarboxylase#ABBREVIATION:Ddc.1# citations: AUTHORS:LOVENBERG W, WEISSBACH H, UDENFRIEND S. #TITLE:Aromatic L-amino acid decarboxylase.#JOURNAL: J Biol Chem#YEAR:1962#PUBMED:14466899# AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (1644.1) or (3067.1)
Abbreviation: R_ENO Synonyms: _0 EC Number: 4.2.1.11 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : 2pg <==> h2o + pep Confidence Level: 0 SMP genes: LOCUS:_2026#TRANSCRIPT:1#ABBREVIATION:ENO2#ECNUMBERS:4.2.1.11# LOCUS:_2023#TRANSCRIPT:1#ABBREVIATION:ENO1#ECNUMBERS:4.2.1.11# LOCUS:_26237#TRANSCRIPT:1#ABBREVIATION:ENO1B#ECNUMBERS:4.2.1.11# LOCUS:_2027#TRANSCRIPT:1#ABBREVIATION:ENO3#ECNUMBERS:4.2.1.11# LOCUS:_2027#TRANSCRIPT:2#ABBREVIATION:ENO3#ECNUMBERS:4.2.1.11# proteins: NAME:alpha enolase 1#ABBREVIATION:Eno1.1# NAME:neuronal gamma enolase 2 (neurons)#ABBREVIATION:Eno2.1# NAME:muscle beta enolase 3 (sk muscle)#ABBREVIATION:Eno3.1# NAME:muscle beta enolase 3 (sk muscle)#ABBREVIATION:Eno3.2# NAME:lung-specific alpha enolase#ABBREVIATION:Eno1b.1# citations: none GENE ASSOCIATION: (2023.1) or (2027.1) or (26237.1) or (2027.2) or (2026.1)
Abbreviation: R_EX_galgalfucfucgalacglcgalacglcgal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : galgalfucfucgalacglcgalacglcgal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(Gal)6 (Glc)1 (GlcNAc)3 (LFuc)2 (Cer)1 exchange
Abbreviation: R_DOLPGT3_Uer Synonyms: _2 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolglcp_U + (0.1) g2m8mpdol_U --> (0.1) dolp_U + (0.1) g3m8mpdol_U + h Confidence Level: 0 EC 2.4.1.- NCD genes: LOCUS:_84920#TRANSCRIPT:1#ABBREVIATION:ALG10# proteins: NAME:alpha-1,2-glucosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg10.1-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (84920.1)
Abbreviation: R_NACHEXA9ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (3) h2o + ksi_deg6 --> (2) acgam + h + ksi_deg8 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_GGH_DASH_7THFe Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [e] : 7thf + h2o --> 6thf + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-e# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (7THF), extracellular
Abbreviation: R_PDXPP Synonyms: _0 SUBSYSTEM: Vitamin B6 Metabolism Equation: [c] : h2o + pdx5p --> pi + pydxn Confidence Level: 2 JLR genes: LOCUS:_57026#TRANSCRIPT:1#ABBREVIATION:PDXP#ECNUMBERS:3.1.3.-# proteins: NAME:Pyridoxal Phosphatase #ABBREVIATION:PdxP.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# AUTHORS:Jang YM, Kim DW, Kang TC, Won MH, Baek NI, Moon BJ, Choi SY, Kwon OS.#TITLE:Human pyridoxal phosphatase. Molecular cloning, functional expression, and tissue distribution.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:14522954# GENE ASSOCIATION: (57026.1)
Abbreviation: R_GSNt5 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gsn[e] + (2) na1[e] --> gsn[c] + (2) na1[c] Confidence Level: 4 genes: LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (64078.1)
guanosine transport in via sodium (1:2) symport
Abbreviation: R_EX_gq1b_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gq1b_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ADRNCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: adrncrn[c] --> adrncrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1) or (788.1)
Abbreviation: R_GLGNS1 Synonyms: _0 EC Number: 2.4.1.11 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : ggn + (3) udpg --> glygn1 + (3) h + (3) udp Confidence Level: 2 NCD genes: LOCUS:_2998#TRANSCRIPT:1#ABBREVIATION:GYS2#ECNUMBERS:2.4.1.11# LOCUS:_2997#TRANSCRIPT:1#ABBREVIATION:GYS1#ECNUMBERS:2.4.1.11# LOCUS:_2992#TRANSCRIPT:1#ABBREVIATION:GYG# LOCUS:_8908#TRANSCRIPT:1#ABBREVIATION:GYG2# proteins: NAME:glycogenin#ABBREVIATION:Gyg.1# NAME:glycogenin 2#ABBREVIATION:Gyg2.1# NAME:glycogen synthase 1 (muscle)#ABBREVIATION:Gys1.1# NAME:glycogen synthase 2 (liver)#ABBREVIATION:Gys2.1# citations: AUTHORS:Lomako J, Lomako WM, Whelan WJ#TITLE:Glycogenin: the primer for mammalian and yeast glycogen synthesis#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15238248# GENE ASSOCIATION: (8908.1) and (2997.1) or (2998.1) and (2992.1) or (2997.1) and (2992.1) or (8908.1) and (2998.1)
Abbreviation: R_GGH_DASH_5THFe Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [e] : 5thf + (4) h2o --> (4) glu-L + thf Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) - last step from 5-glutamyl to monoglutamyl is in one step IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-e# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (5THF), extracellular
Abbreviation: R_ADSK Synonyms: _0 EC Number: 2.7.1.25 SUBSYSTEM: Nucleotides Equation: [c] : aps + atp --> adp + h + paps Confidence Level: 4 genes: LOCUS:_9061#TRANSCRIPT:1#ABBREVIATION:PAPSS1#ECNUMBERS:2.7.1.25,2.7.7.4# LOCUS:_9060#TRANSCRIPT:1#ABBREVIATION:PAPSS2#ECNUMBERS:2.7.7.4,&,2.7.1# proteins: NAME:_3'-phosphoadenosine 5'-phosphosulfate synthase 2 #ABBREVIATION:Papss2.1# NAME:_3'-phosphoadenosine 5'-phosphosulfate synthase 1 #ABBREVIATION:Papss1.1# citations: AUTHORS:Venkatachalam KV.#TITLE:Human 3'-phosphoadenosine 5'-phosphosulfate (PAPS) synthase: biochemistry, molecular biology and genetic deficiency.#JOURNAL:IUBMB Life. #YEAR:2003#PUBMED:12716056# AUTHORS:Fuda H, Shimizu C, Lee YC, Akita H, Strott CA.#TITLE:Characterization and expression of human bifunctional 3'-phosphoadenosine 5'-phosphosulphate synthase isoforms. #JOURNAL:Biochem J.#YEAR:2002#PUBMED:11931637# GENE ASSOCIATION: (9060.1) or (9061.1)
Abbreviation: R_FAS120COA Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : dcacoa + (3) h + malcoa + (2) nadph --> co2 + coa + ddcacoa + h2o + (2) nadp Confidence Level: 4 NCD genes: LOCUS:_2194#TRANSCRIPT:1#ABBREVIATION:FASN#ECNUMBERS:2.3.1.85# proteins: NAME:fatty acid synthase#ABBREVIATION:Fasn.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Chakravarty B, Gu Z, Chirala SS, Wakil SJ, Quiocho FA.#TITLE:Human fatty acid synthase: structure and substrate selectivity of the thioesterase domain.#YEAR:2004#PUBMED:15507492# AUTHORS:Brink J, Ludtke SJ, Yang CY, Gu ZW, Wakil SJ, Chiu W.#TITLE:Quaternary structure of human fatty acid synthase by electron cryomicroscopy.#JOURNAL:Proc Natl Acad Sci#YEAR:2002#PUBMED:11756679# GENE ASSOCIATION: (2194.1)
Abbreviation: R_DM_13_DASH_cis_DASH_oretn_LPAREN_n_RPAREN_ Synonyms: _0 Equation: [n] : 13-cis-oretn --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACACT4p Synonyms: _0 EC Number: 2.3.1.16 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : 3odcoa + coa --> accoa + occoa Confidence Level: 4 NCD genes: LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# proteins: NAME:acetyl-Coenzyme A acyltransferase 1#ABBREVIATION:Acaa1.1# citations: AUTHORS:Bout A, Franse MM, Collins J, Blonden L, Tager JM, Benne R.#TITLE:Characterization of the gene encoding human peroxisomal 3-oxoacyl-CoA thiolase (ACAA). No large DNA rearrangement in a thiolase-deficient patient.#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1679347# GENE ASSOCIATION: (30.1)
acetyl-CoA C-acetyltransferase (octanoyl-CoA), peroxisomal
Abbreviation: R_PSP_L Synonyms: _0 EC Number: 3.1.3.3 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : h2o + pser-L --> pi + ser-L Confidence Level: 4 genes: LOCUS:_5723#TRANSCRIPT:1#ABBREVIATION:PSPH#ECNUMBERS:3.1.3.3# LOCUS:_8781#TRANSCRIPT:1#ABBREVIATION:PSPHL# proteins: NAME:phosphoserine phosphatase #ABBREVIATION:Psph.1# NAME:phosphoserine phosphatase-like #ABBREVIATION:Psphl.1# citations: AUTHORS:Planitzer SA, Machl AW, Rueckels M, Kubbies M.#TITLE:Identification of a novel c-DNA overexpressed in Fanconi's anemia fibroblasts partially homologous to a putative L-3-phosphoserine-phosphatase.#YEAR:1998#PUBMED:9573387# AUTHORS:Collet JF, Gerin I, Rider MH, Veiga-da-Cunha M, Van Schaftingen E. #TITLE:Human L-3-phosphoserine phosphatase: sequence, expression and evidence for a phosphoenzyme intermediate.#YEAR:1997#PUBMED:9188776# GENE ASSOCIATION: (5723.1) or (8781.1)
Abbreviation: R_GGT_U Synonyms: _1 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (16.5) ipdp + ttc-ggdp --> (0.1) dedoldp_U + (16.5) ppi Confidence Level: 0 EC 2.5.1.- NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_VLCSp Synonyms: _5 EC Number: 6.2.1.7 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : atp + coa + thcholstoic --> amp + cholcoar + ppi Confidence Level: 4 from uniprot and lit - see human recon lit refs divalent cation requirement - unclear which one, reaction inhibited in the presence of EDTA (may just be MgATP or MgATP) - see Kelley and Vessey Biochimica et Biophysica Acta (1994) 1209: 51-55 temporarily will have H+ balance reaction NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# proteins: NAME:bile acid CoA ligase#ABBREVIATION:Slc27a1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Steinberg SJ, Wang SJ, Kim DG, Mihalik J, Watkins PA#TITLE:Human very-long-chain-acyl-CoA synthetase#JOURNAL:Biochemical and biophysical research communications#YEAR:1999# AUTHORS:Steinberg SJ, Mihalik SJ, Kim DG, Cuebas DA, Watkins PA#TITLE:The human liver-specific homolog of very long-chain acyl-CoA synthetase is cholate: CoA ligase#JOURNAL:Journal of Biological Chemistry#YEAR:2000# AUTHORS:Mihalik SJ, Steinberg SJ, Pei Z, Park J, Kim DG, Heinzer AK, Dacremont G, Wanders JAR, Cuebas DA, Smith KD, Watkins PA#TITLE:Participation of two members of the veryl long-chain acyl-CoA synthetase family in bile acid synthesis and recycling#JOURNAL:Journal of Biological Chemistry#YEAR:2002# AUTHORS:Kelley M, Vessey DA#TITLE:Dual role of diavlent cations in the bile acid: CoA ligase catalyzed reaction#JOURNAL:Biochimica et Biophysica Acta#YEAR:1994# GENE ASSOCIATION: (11001.1)
Abbreviation: R_EX_cmp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cmp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ANDRSTRNte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: andrstrn[e] <==> andrstrn[c] Confidence Level: 1 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DADNK Synonyms: _0 EC Number: 2.7.1.76 SUBSYSTEM: Nucleotides Equation: [c] : atp + dad-2 --> adp + damp + h Confidence Level: 4 Deoxyguanosine can also act as acceptor. Possibly identical with EC 2.7.1.74 deoxycytidine kinase. genes: none proteins: none citations: AUTHORS:Hurley MC, Palella TD, Fox IH.#TITLE:Human placental deoxyadenosine and deoxyguanosine phosphorylating activity.#JOURNAL: J Biol Chem.#YEAR:1983#PUBMED:6317685# GENE ASSOCIATION:
Abbreviation: R_NACHEX7ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_d_deg5 + h2o --> acgal + cs_d_deg6 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3074.1) or (3073.1)
Serine/Lysine Na-dependent exchange (Ser in)
Abbreviation: R_SERLYSNaex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lys-L[c] + na1[e] + ser-L[e] --> lys-L[e] + na1[c] + ser-L[c] Confidence Level: 1 SAB see PMID 14770310 genes: LOCUS:_9056#TRANSCRIPT:1#ABBREVIATION:SLC7A7# LOCUS:_6519#TRANSCRIPT:1#ABBREVIATION:SLC3A1# proteins: NAME:solute carrier family 3 member 1/family 7 member 7#ABBREVIATION:Slc3a1/7a7# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (9056.1 and 6519.1)
Abbreviation: R_H2MTer_L Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_L + mgacpail_hs --> (0.1) dolp_L + h + m2gacpail_hs Confidence Level: 4 NCD genes: LOCUS:_55650#TRANSCRIPT:1#ABBREVIATION:PIGV# proteins: NAME:phosphatidylinositol glycan, class V, endoplasmic reticulum#ABBREVIATION:Pigv.1-r# citations: AUTHORS:Kang JY, Hong Y, Ashida H, Shishioh N, Murakami Y, Morita YS, Maeda Y, Kinoshita T#TITLE:IG-V involved in transferring the second mannose in glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15623507# GENE ASSOCIATION: (55650.1)
Abbreviation: R_ACN23ACNGALGBSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acn23acngalgbside_hs[c] <==> acn23acngalgbside_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
acn23acngalgbside intracellular transport
Abbreviation: R_EX_inost_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : inost <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ARGN Synonyms: _0 EC Number: 3.5.3.1 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : arg-L + h2o --> orn + urea Confidence Level: 4 genes: LOCUS:_383#TRANSCRIPT:1#ABBREVIATION:ARG1#ECNUMBERS:3.5.3.1# proteins: NAME:arginase, liver#ABBREVIATION:Arg1.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# GENE ASSOCIATION: (383.1)
Abbreviation: R_EX_cspg_e_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cspg_e <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate E (GalNAc4,6diS-GlcA) proteoglycan exchange
cardiolipin (homo sapiens)_C9H16O9P2FULLRCO2FULLR2CO2FULLRCO2FULLR2CO2
Abbreviation: R_G2M8MASNterg Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: g2m8masn[r] --> g2m8masn[g] Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
g2m8masn transport from endoplasmic reticulum to Golgi apparatus
Abbreviation: R_GQ1Bte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gq1b_hs[c] <==> gq1b_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_ILETA Synonyms: _0 EC Number: 2.6.1.42 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [c] : akg + ile-L <==> 3mop + glu-L Confidence Level: 4 genes: LOCUS:_586#TRANSCRIPT:1#ABBREVIATION:BCAT1#ECNUMBERS:2.6.1.26# proteins: NAME:branched chain aminotransferase 1, cytosolic #ABBREVIATION:Bcat1.1# citations: AUTHORS:Bledsoe RK, Dawson PA, Hutson SM.#TITLE:Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm). #YEAR:1997#PUBMED:9165094# AUTHORS:Naylor SL, Shows TB.#TITLE:Branched-chain aminotransferase deficiency in Chinese hamster cells complemented by two independent genes on human chromosomes 12 and 19. #YEAR:1980#PUBMED:6933702# GENE ASSOCIATION: (586.1)
Abbreviation: R_GPAMm_hs Synonyms: _1 EC Number: 2.3.1.15 SUBSYSTEM: Triacylglycerol Synthesis Equation: [m] : Rtotalcoa + glyc3p --> alpa_hs + coa Confidence Level: 4 NJ genes: LOCUS:_57678#TRANSCRIPT:1#ABBREVIATION:KIAA1560# proteins: NAME:glycerol 3-phosphate acyltransferase, mitochondrial#ABBREVIATION:Gpam.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Coleman RA, Lee DP#TITLE:Enzymes of triacylglycerol synthesis and their regulation#JOURNAL:Prog in Lipid Research#YEAR:2004# GENE ASSOCIATION: (57678.1)
Abbreviation: R_DNAMTn Synonyms: _30 EC Number: 2.1.1.37 SUBSYSTEM: Methionine Metabolism Equation: [n] : amet + dna --> ahcys + dna5mtc + h Confidence Level: 4 genes: LOCUS:_1786#TRANSCRIPT:1#ABBREVIATION:DNMT1#ECNUMBERS:2.1.1.37# LOCUS:_1789#TRANSCRIPT:1#ABBREVIATION:DNMT3B#ECNUMBERS:2.1.1.37# LOCUS:_1789#TRANSCRIPT:2#ABBREVIATION:DNMT3B#ECNUMBERS:2.1.1.37# LOCUS:_1789#TRANSCRIPT:3#ABBREVIATION:DNMT3B#ECNUMBERS:2.1.1.37# LOCUS:_1789#TRANSCRIPT:4#ABBREVIATION:DNMT3B#ECNUMBERS:2.1.1.37# LOCUS:_1788#TRANSCRIPT:1#ABBREVIATION:DNMT3A#ECNUMBERS:2.1.1.37# LOCUS:_1788#TRANSCRIPT:2#ABBREVIATION:DNMT3A#ECNUMBERS:2.1.1.37# LOCUS:_1788#TRANSCRIPT:3#ABBREVIATION:DNMT3A#ECNUMBERS:2.1.1.37# LOCUS:_1788#TRANSCRIPT:4#ABBREVIATION:DNMT3A#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:1#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:2#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:3#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:4#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:5#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:6#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# proteins: NAME:DNA (cytosine-5-)-methyltransferase 1, nuclear #ABBREVIATION:Dnmt1.1-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.1-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.2-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.3-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.4-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.5-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.6-n# NAME:DNA (cytosine-5-)-methyltransferase 3, nuclear #ABBREVIATION:Dnmt3a.1-n# NAME:DNA (cytosine-5-)-methyltransferase 3, nuclear #ABBREVIATION:Dnmt3a.2-n# NAME:DNA (cytosine-5-)-methyltransferase 3, nuclear #ABBREVIATION:Dnmt3a.3-n# NAME:DNA (cytosine-5-)-methyltransferase 3, nuclear #ABBREVIATION:Dnmt3a.4-n# NAME:DNA (cytosine-5-)-methyltransferase 3 beta, nuclear #ABBREVIATION:Dnmt3b.1-n# NAME:DNA (cytosine-5-)-methyltransferase 3 beta, nuclear #ABBREVIATION:Dnmt3b.2-n# NAME:DNA (cytosine-5-)-methyltransferase 3 beta, nuclear #ABBREVIATION:Dnmt3b.3-n# NAME:DNA (cytosine-5-)-methyltransferase 3 beta, nuclear #ABBREVIATION:Dnmt3b.4-n# citations: AUTHORS:Yen RW, Vertino PM, Nelkin BD, Yu JJ, el-Deiry W, Cumaraswamy A, Lennon GG, Trask BJ, Celano P, Baylin SB.#TITLE:Isolation and characterization of the cDNA encoding human DNA methyltransferase.#YEAR:1992#PUBMED:1594447# AUTHORS:Deloukas,P. , Matthews,L.H. , Ashurst,J.#TITLE:The DNA sequence and comparative analysis of human chromosome 20.#YEAR:2001# GENE ASSOCIATION: (1787.5) or (1788.4) or (1787.2) or (1787.6) or (1788.2) or (1789.1) or (1787.1) or (1789.4) or (1789.3) or (1788.1) or (1786.1) or (1787.3) or (1789.2) or (1788.3) or (1787.4)
DNA (cytosine-5-)-methyltransferase, nucleus
Abbreviation: R_GLYC_DASH_St Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glyc-S[c] --> glyc-S[e] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Petrarulo M, Vitale C, Facchini P, Marangella M#TITLE:Biochemical approach to diagnosis and differentiation of primary hyperoxalurias: an update#JOURNAL:J Nephrol#YEAR:1998#PUBMED:9604805# GENE ASSOCIATION:
Abbreviation: R_GLRASE Synonyms: _2 EC Number: 1.1.1.20 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : gullac + nadp <==> glac + h + nadph Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_O2Stm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: o2s[c] <==> o2s[m] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Archibald F.#TITLE:Oxygen toxicity and the health and survival of eukaryote cells: a new piece is added to the puzzle. #JOURNAL:Proc Natl Acad Sci U S A#YEAR:2003#PUBMED:12939409# GENE ASSOCIATION:
superoxide anion transport via diffusion (mitochondria)
Abbreviation: R_CBR2 Synonyms: _4 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : h + nadph + prostge2 --> nadp + prostgf2 Confidence Level: 4 multiple EC EC 1.1.1.189 EC 1.1.1.197 NJ genes: LOCUS:_873#TRANSCRIPT:1#ABBREVIATION:CBR1#ECNUMBERS:1.1.1.184# proteins: NAME:carbonyl reductase 1#ABBREVIATION:Cbr.1# citations: AUTHORS:Watanabe K, Sugawara C, Ono A, Fukuzumi Y, Itakura S, Yamazaki M, Tashiro H, Osoegawa K, Soeda E, Nomura T.#TITLE:Mapping of a novel human carbonyl reductase, CBR3, and ribosomal pseudogenes to human chromosome 21q22.2.#JOURNAL:Genomics#YEAR:1998#PUBMED:9740676# AUTHORS:Okita RT, Okita JR.#TITLE:Prostaglandin-metabolizing enzymes during pregnancy: characterization of NAD(+)-dependent prostaglandin dehydrogenase, carbonyl reductase, and cytochrome P450-dependent prostaglandin omega-hydroxylase.#JOURNAL:Crit Rev Biochem Mol Biol#YEAR:1996#PUBMED:8740524# GENE ASSOCIATION: (873.1)
de-Fuc form of PA6 (w/o peptide linkage)_C84H136N6O62
Abbreviation: R_ENGASE Synonyms: _15 EC Number: 3.2.1.96 SUBSYSTEM: N-Glycan Degradation Equation: [c] : h2o + s2l2n2m2m --> acgam + s2l2n2m2mn Confidence Level: 0 NCD genes: LOCUS:_64772#TRANSCRIPT:1#ABBREVIATION:FLJ21865#ECNUMBERS:3.2.1.96# proteins: NAME:endo-beta-N-acetylglucosaminidase (thymus, spleen, pancreas, kidney, heart, sk muscle, placenta, brain)#ABBREVIATION:Flj21865.1# citations: AUTHORS:Suzuki T, Yano K, Sugimoto S, Kitajima K, Lennarz WJ, Inoue S, Inoue Y, Emori Y#TITLE:Endo-beta-N-acetylglucosaminidase, an enzyme involved in processing of free oligosaccharides in the cytosol#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2002#PUBMED:12114544# GENE ASSOCIATION: (64772.1)
Abbreviation: R_EX_fuc13galacglcgal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fuc13galacglcgal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_G14T6g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre2 + udpgal --> h + ksi_pre3 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
Abbreviation: R_EX_amp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : amp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6T22g Synonyms: _3 EC Number: 2.8.2.17 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_a,b,e_pre1 + (2) paps --> cs_e_pre2 + (2) h + (2) pap Confidence Level: 4 NCD genes: LOCUS:_56548#TRANSCRIPT:1#ABBREVIATION:CHST7# proteins: NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 7, Golgi apparatus#ABBREVIATION:Chst7.1-g# citations: AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Tsutsumi K, Shimakawa H, Kitagawa H, Sugahara K#TITLE:Functional expression and genomic structure of human chondroitin 6-sulfotransferase#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9883891# AUTHORS:Kitagawa H, Fujita M, Ito N, Sugahara K#TITLE:Molecular cloning and expression of a novel chondroitin 6-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781596# AUTHORS:Bhakta S, Bartes A, Bowman KG, Kao WM, Polsky I, Lee JK, Cook BN, Bruehl RE, Rosen SD, Bertozzi CR, Hemmerich S#TITLE:Sulfation of N-acetylglucosamine by chondroitin 6-sulfotransferase 2 (GST-5)#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10956661# GENE ASSOCIATION: (56548.1)
Abbreviation: R_PIt9 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) na1[e] + pi[e] <==> (2) na1[c] + pi[c] Confidence Level: 4 MM genes: LOCUS:_142680#TRANSCRIPT:1#ABBREVIATION:SLC34A3# proteins: NAME:solute carrier family 34 (sodium phosphate), member 3, extracellular #ABBREVIATION:Slc34a3.1-e# citations: AUTHORS:Murer H, Forster I, Biber J.#TITLE:The sodium phosphate cotransporter family SLC34. #YEAR:2004#PUBMED:12750889# GENE ASSOCIATION: (142680.1)
phosphate transport in/out via two Na+ symporter
Abbreviation: R_UDPGLCAter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: udpglcur[r] + ump[c] <==> udpglcur[c] + ump[r] Confidence Level: 4 NCD genes: LOCUS:_23169#TRANSCRIPT:1#ABBREVIATION:SLC35D1# proteins: NAME:solute carrier family 35 (UDP-glucuronic acid/UDP-N-acetylgalactosamine dual transporter), member D1#ABBREVIATION:Slc35d1.1-r# citations: AUTHORS:Muraoka M, Kawakita M, Ishida N#TITLE:Molecular characterization of human UDP-glucuronic acid/UDP-N-acetylgalactosamine transporter, a novel nucleotide sugar transporter with dual substrate specificity#JOURNAL:FEBS Lett.#YEAR:2001#PUBMED:11322953# GENE ASSOCIATION: (23169.1)
UDPGlcA endoplasmic reticulum transport via UMP antiport
Abbreviation: R_SPRn Synonyms: _0 EC Number: 1.1.1.153 SUBSYSTEM: Tetrahydrobiopterin Equation: [n] : 6pthp + (2) h + (2) nadph --> (2) nadp + thbpt Confidence Level: 4 IT genes: LOCUS:_6697#TRANSCRIPT:1#ABBREVIATION:SPR#ECNUMBERS:1.1.1.153# proteins: NAME:sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase) #ABBREVIATION:Spr1.1# citations: AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# AUTHORS:Elzaouk L, Laufs S, Heerklotz D, Leimbacher W, Blau N, Resibois A, Thony B.#TITLE:Nuclear localization of tetrahydrobiopterin biosynthetic enzymes.#JOURNAL:Biochim Biophys Acta.#YEAR:2004#PUBMED:14729142# GENE ASSOCIATION: (6697.1)
Abbreviation: R_EX_andrstrnglc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : andrstrnglc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RETFA Synonyms: _3 EC Number: 2.3.1.76 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : Rtotal2coa + retinol --> coa + retfa Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
L-alanine/L-asparagine Na-dependent exchange (Asn-L in)
Abbreviation: R_ASNALANaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[c] + asn-L[e] + na1[e] --> ala-L[e] + asn-L[c] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
Abbreviation: R_S23T4g Synonyms: _0 EC Number: 2.4.99.4 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : cmpacna + ksii_core4_pre1 --> cmp + h + ksii_core4_pre2 Confidence Level: 4 NCD genes: LOCUS:_6483#TRANSCRIPT:1#ABBREVIATION:SIAT4B#ECNUMBERS:2.4.99.4# LOCUS:_6482#TRANSCRIPT:1#ABBREVIATION:SIAT4A#ECNUMBERS:2.4.99.4# LOCUS:_6484#TRANSCRIPT:1#ABBREVIATION:SIAT4C#ECNUMBERS:2.4.99.4# LOCUS:_6482#TRANSCRIPT:2#ABBREVIATION:SIAT4A#ECNUMBERS:2.4.99.4# proteins: NAME:sialyltransferase 4A (beta-galactoside alpha-2,3-sialyltransferase), Golgi apparatus (placenta, liver, sk muscle)#ABBREVIATION:Siat4a.1-g# NAME:_3-sialyltransferase), Golgi apparatus (placenta, liver, sk muscle)#ABBREVIATION:Siat4a.2-g# NAME:sialyltransferase 4B, Golgi apparatus#ABBREVIATION:Siat4b.1-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase, Golgi#ABBREVIATION:Siat4c.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Kitagawa H, Paulson JC#TITLE:Differential expression of five sialyltransferase genes in human tissues#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8027041# AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Shang J, Qiu R, Wang J, Liu J, Zhou R, Ding H, Yang S, Zhang S, Jin C#TITLE:Molecular cloning and expression of Galbeta1,3GalNAc alpha2, 3-sialyltransferase from human fetal liver#JOURNAL:Eur J Biochem#YEAR:1999#PUBMED:10504389# AUTHORS:Kim YJ, Kim KS, Kim SH, Kim CH, Ko JH, Choe IS, Tsuji S, Lee YC#TITLE:Molecular cloning and expression of human Gal beta 1,3GalNAc alpha 2,3-sialytransferase (hST3Gal II)#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8920913# AUTHORS:Kitagawa H, Paulson JC#TITLE:Cloning of a novel alpha 2,3-sialyltransferase that sialylates glycoprotein and glycolipid carbohydrate groups#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8288606# AUTHORS:Sasaki K, Watanabe E, Kawashima K, Sekine S, Dohi T, Oshima M, Hanai N, Nishi T, Hasegawa M#TITLE:Expression cloning of a novel Gal beta (1-3/1-4) GlcNAc alpha 2,3-sialyltransferase using lectin resistance selection#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:7901202# GENE ASSOCIATION: (6484.1) or (6482.1) or (6483.1) or (6482.2)
Abbreviation: R_FAOXC200180x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : arachcoa + coa + h2o + nad + o2 --> accoa + h + h2o2 + nadh + stcoa Confidence Level: 4 peroxisomal (very long chain FA) - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) c20:0 -> c18:0 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (51.1 and 30.1 and 1962.1) or (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1)
Abbreviation: R_AG13T18g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core4_pre7 + uacgam --> h + ksii_core4_pre8 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (10678.2) or (11041.1) or (10678.1) or (374907.2) or (93010.1) or (374907.1) or (284208.1) or (79369.1) or (10331.1)
Abbreviation: R_DOLPGT2_Uer Synonyms: _2 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolglcp_U + (0.1) g1m8mpdol_U --> (0.1) dolp_U + (0.1) g2m8mpdol_U + h Confidence Level: 0 EC 2.4.1.- NCD genes: LOCUS:_79053#TRANSCRIPT:1#ABBREVIATION:ALG8# LOCUS:_79053#TRANSCRIPT:2#ABBREVIATION:ALG8# LOCUS:_79053#TRANSCRIPT:3#ABBREVIATION:ALG8# proteins: NAME:alpha-1,3-glucosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg8.1-er# NAME:alpha-1,3-glucosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg8.2-er# NAME:alpha-1,3-glucosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg8.3-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (79053.1) or (79053.3) or (79053.2)
Abbreviation: R_HEXCCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: hexccoa[c] <==> hexccoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NADHtru Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: nadh[c] --> nadh[r] Confidence Level: 1 diffusion - but unidirectional uptake into ER (to avoid loops w/ NAD/NADP) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAS160COA Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : (3) h + malcoa + (2) nadph + tdcoa --> co2 + coa + h2o + (2) nadp + pmtcoa Confidence Level: 4 NCD genes: LOCUS:_2194#TRANSCRIPT:1#ABBREVIATION:FASN#ECNUMBERS:2.3.1.85# proteins: NAME:fatty acid synthase#ABBREVIATION:Fasn.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Chakravarty B, Gu Z, Chirala SS, Wakil SJ, Quiocho FA.#TITLE:Human fatty acid synthase: structure and substrate selectivity of the thioesterase domain.#YEAR:2004#PUBMED:15507492# AUTHORS:Brink J, Ludtke SJ, Yang CY, Gu ZW, Wakil SJ, Chiu W.#TITLE:Quaternary structure of human fatty acid synthase by electron cryomicroscopy.#JOURNAL:Proc Natl Acad Sci#YEAR:2002#PUBMED:11756679# AUTHORS:Wakil SJ.#TITLE:Fatty acid synthase, a proficient multifunctional enzyme.#JOURNAL:Biochemistry#YEAR:1989#PUBMED:2669958# GENE ASSOCIATION: (2194.1)
Abbreviation: R_ATPtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: atp[c] <==> atp[x] Confidence Level: 1 most likely NOT physiological - detailed information about the peroxisomal transporters has not yet been achieved - this transport is most likely dependent on a concomitant energy consuming reaction (human ABC peroxisomal transporters: PEX family - check in future for new research articles describing biochemistry and stoichiometry) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_gln_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gln-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FA182ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + ocdcyaACP <==> ACP + h + ocdcya Confidence Level: 4 NCD genes: LOCUS:_55301#TRANSCRIPT:1#ABBREVIATION:THEDC1# proteins: NAME:s-acyl fatty acid synthase thioesterase#ABBREVIATION:Thedc1.1# citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION: (55301.1)
Abbreviation: R_UMPK7 Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : dgtp + ump <==> dgdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_GGH_DASH_6THFe Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [e] : 6thf + h2o --> 5thf + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-e# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (6THF), extracellular
Abbreviation: R_PRPNCOAHYDm Synonyms: _0 EC Number: 4.2.1.17 SUBSYSTEM: beta-Alanine metabolism Equation: [m] : h2o + prpncoa --> 3hpcoa Confidence Level: 1 SAB made up name because name in KEGG seemed wrong genes: LOCUS:_1892#TRANSCRIPT:1#ABBREVIATION:ECHS1# LOCUS:_3030#TRANSCRIPT:1#ABBREVIATION:HADHA#ECNUMBERS:4.2.1.17,1.1.1.35# LOCUS:_3032#TRANSCRIPT:1#ABBREVIATION:HADHB# LOCUS:_549#TRANSCRIPT:1#ABBREVIATION:AUH# proteins: NAME:AU RNA binding protein/enoyl-Coenzyme A hydratase (mito)#ABBREVIATION:Auh.1-m# NAME:enoyl Coenzyme A hydratase, short chain, 1, mitochondrial#ABBREVIATION:Echs1.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit #ABBREVIATION:Hadha.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit #ABBREVIATION:Hadhb.1-m# citations: none GENE ASSOCIATION: (1892.1) or (3030.1) and (3032.1) or (549.1)
Abbreviation: R_25HVITD2tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _25hvitd2[m] --> 25hvitd2[c] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_25-hydroxyvitamin D2 transport from mitochondria
Abbreviation: R_EX_xolest2_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : xolest2_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
cholesterol ester (from FULLR2) exchange
Abbreviation: R_EX_abt_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : abt <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FPGS3 Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [c] : 6thf + atp + glu-L --> 7thf + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_FPGS6 Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [c] : 6dhf + atp + glu-L --> 7dhf + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_GLCt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glc-D[e] + h[e] <==> glc-D[c] + h[c] Confidence Level: 4 genes: LOCUS:_6524#TRANSCRIPT:1#ABBREVIATION:SLC5A2# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 2, extracellular#ABBREVIATION:Slc5a2.1-e# citations: AUTHORS:Kanai Y, Lee WS, You G, Brown D, Hediger MA#TITLE:The human kidney low affinity Na+/glucose cotransporter SGLT2. Delineation of the major renal reabsorptive mechanism for D-glucose#JOURNAL:J Clin Invest#YEAR:1994#PUBMED:8282810# AUTHORS:Wells RG, Pajor AM, Kanai Y, Turk E, Wright EM, Hediger MA#TITLE:Cloning of a human kidney cDNA with similarity to the sodium-glucose cotransporter#JOURNAL:Am J Physiol#YEAR:1992#PUBMED:1415574# AUTHORS:Wright EM#TITLE:Renal Na(+)-glucose cotransporters#JOURNAL:Am J Physiol Renal Physiol#YEAR:2001#PUBMED:11133510# GENE ASSOCIATION: (6524.1)
D-glucose transport in via proton symport
Abbreviation: R_MCITS Synonyms: _2 EC Number: 4.1.3.31 SUBSYSTEM: Propanoate Metabolism Equation: [c] : h2o + oaa + ppcoa --> 2mcit + coa + h Confidence Level: 2 NCD new EC is 2.3.3.5 - NCD genes: none proteins: none citations: AUTHORS:Ando T, Rasmussen K, Nyhan WL, Hull D. #TITLE:_3-hydroxypropionate: significance of -oxidation of propionate in patients with propionic acidemia and methylmalonic acidemia#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1972#PUBMED:4507604# AUTHORS:Weidman SW, Drysdale GR.#TITLE:he biosynthesis of methylcitrate.#JOURNAL:Biochem J#YEAR:1979#PUBMED:426765# GENE ASSOCIATION:
Abbreviation: R_URATEt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: urate[c] --> urate[e] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
_17-beta-D-glucuronide transport (ATP-dependent)
Abbreviation: R_ESTRADIOLGLCt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + estradiolglc[c] + h2o[c] --> adp[c] + estradiolglc[e] + h[c] + pi[c] Confidence Level: 4 IT genes: LOCUS:_10257#TRANSCRIPT:1#ABBREVIATION:ABCC4# proteins: NAME:ATP-binding cassette, sub-family C (CFTR/MRP), member 4 #ABBREVIATION:Abcc4.1-e# citations: AUTHORS:Chen ZS, Lee K, Kruh GD.#TITLE:Transport of cyclic nucleotides and estradiol 17-beta-D-glucuronide by multidrug resistance protein 4. Resistance to 6-mercaptopurine and 6-thioguanine.#JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11447229# AUTHORS:Reid G, Wielinga P, Zelcer N, van der Heijden I, Kuil A, de Haas M, Wijnholds J, Borst P. #TITLE:The human multidrug resistance protein MRP4 functions as a prostaglandin efflux transporter and is inhibited by nonsteroidal antiinflammatory drugs.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2003#PUBMED:12835412# AUTHORS:Wielinga PR, van der Heijden I, Reid G, Beijnen JH, Wijnholds J, Borst P. #TITLE:Characterization of the MRP4- and MRP5-mediated transport of cyclic nucleotides from intact cells. #JOURNAL: J Biol Chem. #YEAR:2003#PUBMED:12637526# GENE ASSOCIATION: (10257.1)
Abbreviation: R_P45027A13m Synonyms: _4 EC Number: 1.14.13.15 SUBSYSTEM: Bile Acid Biosynthesis Equation: [m] : nadph + o2 + thcholst --> h2o + nadp + thcholstoic Confidence Level: 4 from uniprot NJ genes: LOCUS:_1593#TRANSCRIPT:1#ABBREVIATION:CYP27A1# proteins: NAME:cytochrome P450, family 27, subfamily A, polypeptide 1#ABBREVIATION:Cyp27a1.1-m# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1593.1)
Abbreviation: R_KYNATESYN Synonyms: _0 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 4aphdob --> h2o + kynate Confidence Level: 4 SAB name is a guess genes: none proteins: none citations: AUTHORS: Cooper AJ#TITLE:The role of glutamine transaminase K (GTK) in sulfur and alpha-keto acid metabolism in the brain, and in the possible bioactivation of neurotoxicants#JOURNAL:Neurochem Int#YEAR:2004#PUBMED:15016471# AUTHORS:Hartai Z, Klivenyi P, Janaky T, Penke B, Dux L, Vecsei L#TITLE:Kynurenine metabolism in multiple sclerosis#JOURNAL:Acta Neurol Scand#YEAR:2005#PUBMED:16008534# GENE ASSOCIATION:
_4-(2-Aminophenyl)-2,4-dioxobutanoate dehydratase
Abbreviation: R_DNDPt60m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgtp[c] + dudp[m] --> dgtp[m] + dudp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_P45027A11m Synonyms: _4 EC Number: 1.14.13.15 SUBSYSTEM: Bile Acid Biosynthesis Equation: [m] : h + nadph + o2 + xoltriol --> h2o + nadp + xoltetrol Confidence Level: 4 from uniprot NJ genes: LOCUS:_1593#TRANSCRIPT:1#ABBREVIATION:CYP27A1# proteins: NAME:cytochrome P450, family 27, subfamily A, polypeptide 1#ABBREVIATION:Cyp27a1.1-m# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1593.1)
Abbreviation: R_ACETONEt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acetone[e] + h[e] <==> acetone[c] + h[c] Confidence Level: 4 NCD genes: LOCUS:_9194#TRANSCRIPT:1#ABBREVIATION:SLC16A7# LOCUS:_9123#TRANSCRIPT:1#ABBREVIATION:SLC16A3# LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 7#ABBREVIATION:Slc16a7.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1#ABBREVIATION:Slc16a1.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 3#ABBREVIATION:Slc16a3.1-e# citations: AUTHORS:Lin RY, Vera JC, Chaganti RS, Golde DW#TITLE:Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9786900# AUTHORS:Halestrap AP, Price NT#TITLE:The proton-linked monocarboxylate transporter (MCT) family: structure, function and regulation#JOURNAL:Biochem J#YEAR:1999#PUBMED:10510291# AUTHORS:Garcia CK, Li X, Luna J, Francke U#TITLE:cDNA cloning of the human monocarboxylate transporter 1 and chromosomal localization of the SLC16A1 locus to 1p13.2-p12#JOURNAL:Genomics#YEAR:1994#PUBMED:7835905# AUTHORS:Poole RC, Halestrap AP#TITLE:Transport of lactate and other monocarboxylates across mammalian plasma membranes#JOURNAL:Am J Physiol#YEAR:1993#PUBMED:8476015# AUTHORS:Ritzhaupt A, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:The characterization of butyrate transport across pig and human colonic luminal membrane#JOURNAL:J Physiol#YEAR:1998#PUBMED:9508842# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification of a monocarboxylate transporter isoform type 1 (MCT1) on the luminal membrane of human and pig colon#JOURNAL:Biochem Soc Trans#YEAR:1998#PUBMED:9649795# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification and characterization of a monocarboxylate transporter (MCT1) in pig and human colon: its potential to transport L-lactate as well as butyrate#JOURNAL:J Physiol#YEAR:1998#PUBMED:9824713# AUTHORS:Halestrap AP, Meredith D#TITLE:The SLC16 gene family-from monocarboxylate transporters (MCTs) to aromatic amino acid transporters and beyond#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739169# AUTHORS:Manning Fox JE, Meredith D, Halestrap AP#TITLE:Characterisation of human monocarboxylate transporter 4 substantiates its role in lactic acid efflux from skeletal muscle#JOURNAL:J Physiol#YEAR:2000#PUBMED:11101640# AUTHORS:Price NT, Jackson VN, Halestrap AP#TITLE:Cloning and sequencing of four new mammalian monocarboxylate transporter (MCT) homologues confirms the existence of a transporter family with an ancient past#JOURNAL:Biochem J#YEAR:1998#PUBMED:9425115# GENE ASSOCIATION: (6566.1) or (9123.1) or (9194.1)
Abbreviation: R_B3GALT3g Synonyms: _8 EC Number: 2.4.1.79 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : thcrm_hs + udpacgal --> gbside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_8706#TRANSCRIPT:1#ABBREVIATION:B3GALT3# LOCUS:_8706#TRANSCRIPT:4#ABBREVIATION:B3GALT3# LOCUS:_8706#TRANSCRIPT:2#ABBREVIATION:B3GALT3# LOCUS:_8706#TRANSCRIPT:3#ABBREVIATION:B3GALT3# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 3#ABBREVIATION:B3galt3.1# NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 3#ABBREVIATION:B3galt3.2# NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 3#ABBREVIATION:B3galt3.3# NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 3#ABBREVIATION:B3galt3.4# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Amado M, Almeida R, Carneiro F, Levery ST, Holmes EH, Nomoto M, Hollingsworth MA, Hassan H, Schwientek T, Nielsen PA, Bennett EP, Clausen H#TITLE:A family of human beta3-galactosyltransferases#JOURNAL:The Journal of Biological Chemistry#YEAR:1998# GENE ASSOCIATION: (8706.4) or (8706.1) or (8706.3) or (8706.2)
Abbreviation: R_TCHOLAt3 Synonyms: _10 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + tchola[c] --> adp[c] + h[c] + pi[c] + tchola[e] Confidence Level: 4 for bile acid secretion into bile NJ genes: LOCUS:_8647#TRANSCRIPT:1#ABBREVIATION:ABCB11# LOCUS:_8714#TRANSCRIPT:1#ABBREVIATION:ABCC3# LOCUS:_8714#TRANSCRIPT:2#ABBREVIATION:ABCC3# LOCUS:_8714#TRANSCRIPT:3#ABBREVIATION:ABCC3# proteins: NAME:ABC, sub-family B (MDR/TAP), member 11#ABBREVIATION:Abcb11.1# NAME:ABC, sub-family C (CFTR/MRP), member 3#ABBREVIATION:Abcc3.1# NAME:ABC, sub-family C, (CFTR/MRP), member 3#ABBREVIATION:Abcc3.2# NAME:ABC, sub-family C, (CFTR/MRP), member 3#ABBREVIATION:Abcc3.3# citations: AUTHORS:Strautnieks SS, Bull LN, Knisely AS, Kocoshis SA, Dahl N, Arnell H, Sokal E, Dahan K, Childs S, Ling V, Tanner MS, Kagalwalla AF, Nemeth A, Pawlowska J, Baker A, Mieli-Vergani G, Freimer NB, Gardiner RM, Thompson RJ.#TITLE:A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis.#JOURNAL:Nat Genet.#YEAR:1998#PUBMED:9806540# AUTHORS:Kiuchi Y, Suzuki H, Hirohashi T, Tyson CA, Sugiyama Y.#TITLE:cDNA cloning and inducible expression of human multidrug resistance associated protein 3 (MRP3).#JOURNAL:FEBS Lett.#YEAR:1998#PUBMED:9738950# AUTHORS:Uchiumi T, Hinoshita E, Haga S, Nakamura T, Tanaka T, Toh S, Furukawa M, Kawabe T, Wada M, Kagotani K, Okumura K, Kohno K, Akiyama S, Kuwano M.#TITLE:Isolation of a novel human canalicular multispecific organic anion transporter, cMOAT2/MRP3, and its expression in cisplatin-resistant cancer cells with decreased ATP-dependent drug transport.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1998#PUBMED:9813153# GENE ASSOCIATION: (8714.3) or (8647.1) or (8714.2) or (8714.1)
Abbreviation: R_UMPK6 Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : dctp + ump <==> dcdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_KHK3 Synonyms: _1 EC Number: 2.7.1.3 SUBSYSTEM: Galactose metabolism Equation: [c] : atp + tagat-D --> adp + h + tag1p-D Confidence Level: 4 NCD genes: LOCUS:_3795#TRANSCRIPT:1#ABBREVIATION:KHK#ECNUMBERS:2.7.1.3# LOCUS:_3795#TRANSCRIPT:2#ABBREVIATION:KHK#ECNUMBERS:2.7.1.3# proteins: NAME:ketohexokinase (fructokinase)#ABBREVIATION:Khk.1# NAME:ketohexokinase (fructokinase) (liver, kidney, sm intestine)#ABBREVIATION:Khk.2# citations: AUTHORS:Bais R, James HM, Rofe AM, Conyers RA.#TITLE:The purification and properties of human liver ketohexokinase. A role for ketohexokinase and fructose-bisphosphate aldolase in the metabolic production of oxalate from xylitol#JOURNAL:Biochem J#YEAR:1985#PUBMED:2996495# AUTHORS:Barngrover DA, Dills WL Jr.#TITLE:The involvement of liver fructokinase in the metabolism of D-xylulose and xylitol in isolated rat hepatocytes#JOURNAL:J Nutr#YEAR:1983#PUBMED:6298387# AUTHORS:James HM, Bais R, Edwards JB, Rofe AM, Conyers AJ.#TITLE:Models for the metabolic production of oxalate from xylitol in humans: a role for fructokinase and aldolase.#JOURNAL:Aust J Exp Biol Med Sci#YEAR:1982#PUBMED:6284103# GENE ASSOCIATION: (3795.1) or (3795.2)
Abbreviation: R_C160CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: pmtcrn[c] --> pmtcrn[m] Confidence Level: 4 TV genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (788.1)
Abbreviation: R_PGESr Synonyms: _3 EC Number: 5.3.99.3 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : prostgh2 <==> prostge2 Confidence Level: 4 NJ genes: LOCUS:_80142#TRANSCRIPT:1#ABBREVIATION:PTGES2# LOCUS:_80142#TRANSCRIPT:3#ABBREVIATION:PTGES2# LOCUS:_80142#TRANSCRIPT:2#ABBREVIATION:PTGES2# LOCUS:_80142#TRANSCRIPT:4#ABBREVIATION:PTGES2# LOCUS:_9536#TRANSCRIPT:1#ABBREVIATION:PTGES# LOCUS:_9536#TRANSCRIPT:2#ABBREVIATION:PTGES# proteins: NAME:prostaglandin E synthase isoform 1#ABBREVIATION:Ptges.1# NAME:prostaglandin E synthase isoform 2#ABBREVIATION:Ptges.2# NAME:prostaglandin E synthase 2#ABBREVIATION:Ptges2.2# NAME:prostaglandin E synthase 2#ABBREVIATION:Ptges2.1# NAME:prostaglandin E synthase 2#ABBREVIATION:Ptges2.3# NAME:prostaglandin E synthase 2#ABBREVIATION:Ptges2.4# citations: TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Han R, Tsui S, Smith TJ.#TITLE:Up-regulation of prostaglandin E2 synthesis by interleukin-1beta in human orbital fibroblasts involves coordinate induction of prostaglandin-endoperoxide H synthase-2 and glutathione-dependent prostaglandin E2 synthase expression.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11847219# GENE ASSOCIATION: (80142.4) or (80142.2) or (9536.1) or (80142.1) or (9536.2) or (80142.3)
Abbreviation: R_C4STMO2Pr Synonyms: _2 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : 4mzym_int2 + nadp + o2 --> co2 + h + nadph + zym_int2 Confidence Level: 4 double check rxn specifics - see lit ref: Marijanovic, Molecular Endocrinology 2003 17:1715-1725 ER version NJ genes: LOCUS:_50814#TRANSCRIPT:1#ABBREVIATION:NSDHL# proteins: NAME:NAD(P) dependent steroid dehydrogenase#ABBREVIATION:Nsdhl.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# AUTHORS:Caldas H, Herman GE#TITLE:NSDHL, an eznyme invovled in cholesterol biosynthesis, traffics through the Golgi and accumulates on ER membranes on the surface of lipid droplets#JOURNAL:Human Molecular Genetics#YEAR:2003# AUTHORS:Konig A, Happle R, Bornholdt D, Engel H, Grzeschik KH#TITLE:Mutations in the NSDHL gene, encoding a 3-beta-hydroxysteroid dehydrogenase, cause CHILD syndrome#JOURNAL:Am J Med Genet#YEAR:2000# GENE ASSOCIATION: (50814.1)
Abbreviation: R_CHSTEROLt3 Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: chsterol[c] <==> chsterol[m] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: AUTHORS:Jefcoate C.#TITLE:High-flux mitochondrial cholesterol trafficking, a specialized function of the adrenal cortex.#JOURNAL:J Clin Invest#YEAR:2002#PUBMED:12370263# AUTHORS:Strauss JF 3rd, Kishida T, Christenson LK, Fujimoto T, Hiroi H.#TITLE:START domain proteins and the intracellular trafficking of cholesterol in steroidogenic cells.#JOURNAL:Mol Cell Endocrinol#YEAR:2003#PUBMED:12770731# GENE ASSOCIATION:
Abbreviation: R_PUNP3 Synonyms: _0 EC Number: 2.4.2.1 SUBSYSTEM: Purine Catabolism Equation: [c] : gsn + pi <==> gua + r1p Confidence Level: 4 Specificity not completely determined. Can also catalyse ribosyltransferase reactions of the type catalysed by EC 2.4.2.5, nucleoside ribosyltransferase genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# citations: AUTHORS:Erion MD, Stoeckler JD, Guida WC, Walter RL, Ealick SE.#TITLE:Purine nucleoside phosphorylase. 2. Catalytic mechanism. #JOURNAL:Biochemistry#YEAR:1997#PUBMED:9305963# AUTHORS:Williams SR, Goddard JM, Martin DW Jr. #TITLE:Human purine nucleoside phosphorylase cDNA sequence and genomic clone characterization.#JOURNAL:Nucleic Acids Res.#YEAR:1984#PUBMED:6087295# GENE ASSOCIATION: (4860.1)
purine-nucleoside phosphorylase (Guanosine)
keratan sulfate II biosynthesis, precursor 9_C75H120N6O59S2X
Abbreviation: R_CMPACNAtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: cmpacna[n] --> cmpacna[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_THRALANaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[c] + na1[e] + thr-L[e] --> ala-L[e] + na1[c] + thr-L[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
L-alanine/L-threonine Na-dependent exchange (Thr-L in)
Abbreviation: R_ADPRIBt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: adprib[e] --> adprib[c] Confidence Level: 4 IT genes: LOCUS:_952#TRANSCRIPT:1#ABBREVIATION:CD38# proteins: NAME:cyclic ADP-ribose hydrolase#ABBREVIATION:Cadph.1# citations: AUTHORS:Kim UH, Han MK, Park BH, Kim HR, An NH.#TITLE:Function of NAD glycohydrolase in ADP-ribose uptake from NAD by human erythrocytes.#JOURNAL:Biochim Biophys Acta.#YEAR:1993#PUBMED:8394137# GENE ASSOCIATION: (952.1)
Abbreviation: R_PYDXtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: pydx[e] <==> pydx[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# AUTHORS:Mehansho H, Henderson LM.#TITLE:Transport and accumulation of pyridoxine and pyridoxal by erythrocytes.#JOURNAL:J Biol Chem. #YEAR:1980#PUBMED:J Biol Chem.# GENE ASSOCIATION:
Abbreviation: R_THFt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: oh1[c] + thf[e] <==> oh1[e] + thf[c] Confidence Level: 4 IT genes: LOCUS:_6573#TRANSCRIPT:1#ABBREVIATION:SLC19A1# LOCUS:_6573#TRANSCRIPT:2#ABBREVIATION:SLC19A1# proteins: NAME:solute carrier family 19 (folate transporter), member 1 #ABBREVIATION:Slc19a1.1# NAME:solute carrier family 19 (folate transporter), member 1#ABBREVIATION:Slc19a1.2# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Ganapathy V, Smith SB, Prasad PD.#TITLE:SLC19: the folate/thiamine transporter family.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:14770311# AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION: (6573.1) or (6573.2)
tetrahydrofolate transport via anion exchange
Abbreviation: R_APNNOXte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: apnnox[e] <==> apnnox[c] Confidence Level: 1 unknown mechanism, xenobiotic transport (original and metabolized) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ANDRSTRNtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: andrstrn[r] <==> andrstrn[c] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_srtn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : srtn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_Kt3g Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: h[g] + k[c] <==> h[c] + k[g] Confidence Level: 4 genes: LOCUS:_23315#TRANSCRIPT:1#ABBREVIATION:SLC9A8# LOCUS:_84679#TRANSCRIPT:1#ABBREVIATION:SLC9A7# proteins: NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 7#ABBREVIATION:Slc9a7.1-g# NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 8#ABBREVIATION:Slc9a8.1-g# citations: AUTHORS:Numata M, Orlowski J#TITLE:Molecular cloning and characterization of a novel (Na+,K+)/H+ exchanger localized to the trans-Golgi network#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11279194# AUTHORS:Nakamura N, Tanaka S, Teko Y, Mitsui K, Kanazawa H.#TITLE:Four Na+/H+ exchanger isoforms are distributed to Golgi and post-Golgi compartments and are involved in organelle pH regulation.#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15522866# GENE ASSOCIATION: (84679.1) or (23315.1)
potassium transport via proton antiport
Abbreviation: R_AMPTASECGe Synonyms: _0 EC Number: 3.4.11.2 SUBSYSTEM: Glutathione Metabolism Equation: [e] : cgly + h2o --> cys-L + gly Confidence Level: 4 SAB genes: LOCUS:_290#TRANSCRIPT:1#ABBREVIATION:ANPEP# proteins: NAME:alanyl (membrane) aminopeptidase#ABBREVIATION:Anpep.1-e# citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# GENE ASSOCIATION: (290.1)
Abbreviation: R_LSTO1r Synonyms: _4 EC Number: 1.3.3.2 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : chlstol + h + nadph + o2 --> ddsmsterol + (2) h2o + nadp Confidence Level: 4 lanosterol -> cholesterol pathway NJ genes: LOCUS:_6309#TRANSCRIPT:1#ABBREVIATION:SC5DL# proteins: NAME:sterol-C5-desaturase#ABBREVIATION:Sc5dl.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# AUTHORS:Taton M, Husselstein T, Benveniste P, Rahier A#TITLE:Role of highly conserved residues in the reaction catalyzed by recombinant delta-sterol-C5(6)-desaturase studied by site-directed mutagensis#JOURNAL:Biochemistry#YEAR:2000# GENE ASSOCIATION: (6309.1)
Abbreviation: R_DOLICHOL_Lter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: (0.1) dolichol_L[r] <==> (0.1) dolichol_L[c] Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PHCHGSm Synonyms: _0 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : 1p3h5c + h + h2o <==> 4hglusa Confidence Level: 2 taken from KEGG map. No enzyme assigned, so assumed this is a non-enzymatic reaction. NCD genes: none proteins: none citations: AUTHORS:Takayama T, Fujita K, Suzuki K, Sakaguchi M, Fujie M, Nagai E, Watanabe S, Ichiyama A, Ogawa Y#TITLE:Control of oxalate formation from L-hydroxyproline in liver mitochondria#JOURNAL:J Am Soc Nephrol#YEAR:2003#PUBMED:12660328# AUTHORS:Knight J, Holmes RP#TITLE:Mitochondrial hydroxyproline metabolism: implications for primary hyperoxaluria#JOURNAL:Am J Nephrol#YEAR:2005#PUBMED:15849464# GENE ASSOCIATION:
L-1-Pyrroline-3-hydroxy-5-carboxylate spontaneous conversion to L-4-Hydroxyglutamate semialdehyde, mitochondrial
Abbreviation: R_P45021A2r Synonyms: _2 EC Number: 1.14.99.10 SUBSYSTEM: Steroid Metabolism Equation: [r] : 17ahprgstrn + h + nadph + o2 --> 11docrtsl + h2o + nadp Confidence Level: 4 aka CYP21B NJ genes: LOCUS:_1589#TRANSCRIPT:1#ABBREVIATION:CYP21A2#ECNUMBERS:1.14.99.10# proteins: NAME:cytochrome P450, family 21, subfamily A, polypeptide 2#ABBREVIATION:Cyp21a2.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (1589.1)
Abbreviation: R_NACHEXA21ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksii_core2_deg6 --> acgam + h + ksii_core2_deg8 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_MANtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: man[l] --> man[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Pisoni RL, Thoene JG#TITLE:The transport systems of mammalian lysosomes#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1751541# AUTHORS:Lloyd JB#TITLE:Metabolite efflux and influx across the lysosome membrane#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993166# GENE ASSOCIATION:
Abbreviation: R_35CGMPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: _35cgmp[c] <==> 35cgmp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EPCTX Synonyms: _0 EC Number: 2.7.7.14 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : 2ameph + ctp + h --> cmp2amep + ppi Confidence Level: 4 SAB genes: LOCUS:_5833#TRANSCRIPT:1#ABBREVIATION:PCYT2# proteins: NAME:phosphate cytidylyltransferase 2, ethanolamine#ABBREVIATION:Pcyt2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Tan SA, Tan LG#TITLE:Distribution of ciliatine(2-aminoethylphosphonic acid) and phosphonalanine (2-amino-3-phosphonopropionic acid) in human tissues#JOURNAL:Clin Physiol Biochem#YEAR:1989# AUTHORS:Lykidis A, Murtis KG, Jackowski S#TITLE:Cloning and characterization of a second human CTP:Phosphocholine cytidylyltransferase#JOURNAL:Journal of Biological Chemistry#YEAR:1998# AUTHORS:Nakashima A, Hosaka K, Nikawa J#TITLE:Cloning of a human cDNA for CTP-Phosphoethanolamine cytidylyltransferase by complementation in vivo of a yeast mutant#JOURNAL:Journal of Biological Chemistry#YEAR:1997# GENE ASSOCIATION: (5833.1)
ethanolamine-phosphate cytidylyltransferase
Abbreviation: R_S4T5g Synonyms: _1 EC Number: 2.8.2.5 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_e_pre5b + paps --> cspg_e + h + pap Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Hiraoka N, Nakagawa H, Ong E, Akama TO, Fukuda MN, Fukuda M#TITLE:Molecular cloning and expression of two distinct human chondroitin 4-O-sulfotransferases that belong to the HNK-1 sulfotransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781601# GENE ASSOCIATION:
Abbreviation: R_CLPNDCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : clpndcoa + crn --> clpndcrn + coa Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_M8MASNterg Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: m8masn[r] --> m8masn[g] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# GENE ASSOCIATION:
m8masn transport from ER to Golgi apparatus
Abbreviation: R_EX_debrisoquine_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : debrisoquine <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACCOAtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: accoa[c] <==> accoa[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CYTK10n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : cmp + dgtp <==> cdp + dgdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_CORE4GTg Synonyms: _7 EC Number: 2.4.1.148 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : core3 + uacgam --> core4 + h + udp Confidence Level: 0 NCD genes: LOCUS:_9245#TRANSCRIPT:1#ABBREVIATION:GCNT3# proteins: NAME:glucosaminyl (N-acetyl) transferase 3, mucin type, Golgi apparatus (colon, sm intestine, stomach, thyroid, testis, prostate, kidney, pancreas)#ABBREVIATION:Gcnt3.1-g# citations: AUTHORS:Yeh JC, Ong E, Fukuda M#TITLE:Free Full Text Molecular cloning and expression of a novel beta-1, 6-N-acetylglucosaminyltransferase that forms core 2, core 4, and I branches#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9915862# GENE ASSOCIATION: (9245.1)
Core 4 beta6-GalNAc-transferase, Golgi apparatus
Abbreviation: R_CBR1 Synonyms: _4 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : h + nadph + prostg1 --> nadp + prostge1 Confidence Level: 4 multiple EC EC 1.1.1.189 EC 1.1.1.197 NJ genes: LOCUS:_873#TRANSCRIPT:1#ABBREVIATION:CBR1#ECNUMBERS:1.1.1.184# proteins: NAME:carbonyl reductase 1#ABBREVIATION:Cbr.1# citations: AUTHORS:Watanabe K, Sugawara C, Ono A, Fukuzumi Y, Itakura S, Yamazaki M, Tashiro H, Osoegawa K, Soeda E, Nomura T.#TITLE:Mapping of a novel human carbonyl reductase, CBR3, and ribosomal pseudogenes to human chromosome 21q22.2.#JOURNAL:Genomics#YEAR:1998#PUBMED:9740676# AUTHORS:Okita RT, Okita JR.#TITLE:Prostaglandin-metabolizing enzymes during pregnancy: characterization of NAD(+)-dependent prostaglandin dehydrogenase, carbonyl reductase, and cytochrome P450-dependent prostaglandin omega-hydroxylase.#JOURNAL:Crit Rev Biochem Mol Biol#YEAR:1996#PUBMED:8740524# GENE ASSOCIATION: (873.1)
Abbreviation: R_HEX7 Synonyms: _4 EC Number: 2.7.1.1 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : atp + fru --> adp + f6p + h Confidence Level: 2 D-Glucose, D-mannose, D-fructose, sorbitol and D-glucosamine can act as acceptors; ITP and dATP can act as donors. The liver isoenzyme has sometimes been called glucokinase. Same as 2.7.1.4 genes: LOCUS:_3098#TRANSCRIPT:1#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3101#TRANSCRIPT:1#ABBREVIATION:HK3#ECNUMBERS:2.7.1.1# LOCUS:_3099#TRANSCRIPT:1#ABBREVIATION:HK2#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:1#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# LOCUS:_80201#TRANSCRIPT:1#ABBREVIATION:FLJ22761# LOCUS:_3098#TRANSCRIPT:4#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:3#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:5#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:2#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:2#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:3#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# proteins: NAME:glucokinase (hexokinase 4) (pancreatic beta islet)#ABBREVIATION:Gck.1# NAME:glucokinase (hexokinase 4) (major liver isoform)#ABBREVIATION:Gck.2# NAME:glucokinase (hexokinase 4) (minor liver isoform)#ABBREVIATION:Gck.3# NAME:hexokinase 1 (ubiquitous)#ABBREVIATION:Hk1.1# NAME:hexokinase 1 (erythrocyte)#ABBREVIATION:Hk1.2# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.3# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.4# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.5# NAME:hexokinase 2 (sk muscle)#ABBREVIATION:Hk2.1# NAME:hexokinase 3 (liver, lung, placenta)#ABBREVIATION:Hk3.1# NAME:hypothetical protein FLJ22761#ABBREVIATION:Flj22761.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# GENE ASSOCIATION: (3098.4) or (2645.3) or (3098.1) or (3098.5) or (3101.1) or (2645.2) or (3099.1) or (2645.1) or (3098.2) or (80201.1) or (3098.3)
Abbreviation: R_LYSMTF1n Synonyms: _7 EC Number: 2.1.1.43 SUBSYSTEM: Lysine Metabolism Equation: [n] : amet + peplys --> Nmelys + ahcys Confidence Level: 4 MM genes: LOCUS:_79723#TRANSCRIPT:1#ABBREVIATION:SUV39H2# LOCUS:_10919#TRANSCRIPT:1#ABBREVIATION:BAT8# LOCUS:_9869#TRANSCRIPT:1#ABBREVIATION:SETDB1# LOCUS:_6839#TRANSCRIPT:1#ABBREVIATION:SUV39H1# LOCUS:_83852#TRANSCRIPT:1#ABBREVIATION:SETDB2# LOCUS:_58508#TRANSCRIPT:1#ABBREVIATION:MLL3# LOCUS:_58508#TRANSCRIPT:2#ABBREVIATION:MLL3# LOCUS:_10919#TRANSCRIPT:2#ABBREVIATION:BAT8# LOCUS:_84444#TRANSCRIPT:1#ABBREVIATION:DOT1L# LOCUS:_80854#TRANSCRIPT:1#ABBREVIATION:SET7# LOCUS:_79813#TRANSCRIPT:1#ABBREVIATION:EHMT1# LOCUS:_387893#TRANSCRIPT:1#ABBREVIATION:SET8# proteins: NAME:suppressor of variegation 3-9 homolog 1 (Drosophila), nuclear #ABBREVIATION:Suv39h1.1-n# NAME:SET domain, bifurcated 1, nuclear #ABBREVIATION:Setdb1.1-n# NAME:myeloid/lymphoid or mixed-lineage leukemia 3, nuclear#ABBREVIATION:Mll3.1-n# NAME:myeloid/lymphoid or mixed-lineage leukemia 3, nuclear#ABBREVIATION:Mll3.2-n# NAME:DOT1-like, histone H3 methyltransferase (S. cerevisiae), nuclear #ABBREVIATION:Dot1l.1-n# NAME:SET domain-containing protein 7, nuclear #ABBREVIATION:Set7.1-n# NAME:HLA-B associated transcript 8, nuclear#ABBREVIATION:Bat8.1-n# NAME:HLA-B associated transcript 8, nuclear#ABBREVIATION:Bat8.2-n# NAME:SET domain, bifurcated 2, nuclear #ABBREVIATION:Setdb2.1-n# NAME:suppressor of variegation 3-9 homolog 2 (Drosophila), nuclear #ABBREVIATION:Suv39h2.1-n# NAME:euchromatic histone methyltransferase 1, nuclear #ABBREVIATION:Ehmt1.1-n# NAME:PR/SET domain containing protein 8, nuclear #ABBREVIATION:Set8.1-n# citations: none GENE ASSOCIATION: (80854.1) or (58508.2) or (79723.1) or (10919.1) or (9869.1) or (84444.1) or (83852.1) or (6839.1) or (10919.2) or (79813.1) or (58508.1) or (387893.1)
Abbreviation: R_GALASE15ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg40 --> gal + ksi_deg41 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_G14T18g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : core4 + (2) udpgal --> (2) h + ksii_core4_pre1 + (2) udp Confidence Level: 4 Keratan sulfate II biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (8704.2) or (8704.1) or (2683.1) or (9334.1)
Abbreviation: R_NACHEX16ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg25 --> acgam + ksi_deg26 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3074.1) or (3073.1)
Abbreviation: R_CITt4_4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cit[e] + (4) na1[e] <==> cit[c] + (4) na1[c] Confidence Level: 4 NCD genes: LOCUS:_284111#TRANSCRIPT:1#ABBREVIATION:SLC13A5# proteins: NAME:solute carrier family 13 (sodium-dependent citrate transporter), member 5#ABBREVIATION:Slc13a5.1-e# citations: AUTHORS:Inoue K, Zhuang L, Ganapathy V#TITLE:Human Na+ -coupled citrate transporter: primary structure, genomic organization, and transport function#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12445824# AUTHORS:Inoue K, Zhuang L, Maddox DM, Smith SB, Ganapathy V#TITLE:Structure, function, and expression pattern of a novel sodium-coupled citrate transporter (NaCT) cloned from mammalian brain#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12177002# GENE ASSOCIATION: (284111.1)
Abbreviation: R_FAOXC2442246x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : coa + h2o + nad + o2 + tettet6coa --> accoa + adrncoa + h + h2o2 + nadh Confidence Level: 4 _1 round beta ox - - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) peroxisomal (very long chain FA) c24:4,n-6 -> c22:4, n-6 - final '6' in abbreviation indicates omega6 FA 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (51.1 and 3295.1 and 30.1) or (51.1 and 30.1 and 1962.1) or (30.1 and 51.2 and 1962.1) or (3295.1 and 30.1 and 51.2)
Abbreviation: R_ACGAMPM Synonyms: _0 EC Number: 5.4.2.3 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acgam6p <==> acgam1p Confidence Level: 4 NCD genes: LOCUS:_5238#TRANSCRIPT:1#ABBREVIATION:PGM3#ECNUMBERS:5.4.2.2# proteins: NAME:phosphoglucomutase 3#ABBREVIATION:Pgm3.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Mio T, Yamada-Okabe T, Arisawa M, Yamada-Okabe H#TITLE:Functional cloning and mutational analysis of the human cDNA for phosphoacetylglucosamine mutase: identification of the amino acid residues essential for the catalysis#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:11004509# GENE ASSOCIATION: (5238.1)
Abbreviation: R_P45017A4r Synonyms: _4 EC Number: 1.14.99.9 SUBSYSTEM: Steroid Metabolism Equation: [r] : 17ahprgstrn + h + nadph + (0.5) o2 --> acald + andrstndn + h2o + nadp Confidence Level: 4 ER noted in locuslink ref - RETURN AND CHECK AFTER DEEPER LIT SEARCH NJ genes: LOCUS:_1586#TRANSCRIPT:1#ABBREVIATION:CYP17A1#ECNUMBERS:1.14.99.9# proteins: NAME:cytochrome P450, family 17, subfamily A, polypeptide 1#ABBREVIATION:Cyp17a1.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# AUTHORS:Korzekwa KR, Trager WF, Mancewicz J, Osawa Y#TITLE:Studies on the mechanism of aromatase and other cytochrome P450 mediated deformylation reactions#JOURNAL:J Steroid Biochem Mol Biol#YEAR:1993# GENE ASSOCIATION: (1586.1)
Abbreviation: R_TMDK1 Synonyms: _0 EC Number: 2.7.1.21 SUBSYSTEM: Nucleotides Equation: [c] : atp + thymd --> adp + dtmp + h Confidence Level: 4 genes: LOCUS:_7083#TRANSCRIPT:1#ABBREVIATION:TK1#ECNUMBERS:2.7.1.21# LOCUS:_7084#TRANSCRIPT:1#ABBREVIATION:TK2#ECNUMBERS:2.7.1.21# proteins: NAME:thymidine kinase 1, soluble #ABBREVIATION:Tk1.1# NAME:thymidine kinase 2, mitochondrial #ABBREVIATION:Tk2.1# citations: AUTHORS:Johansson M, Brismar S, Karlsson A.#TITLE:Human deoxycytidine kinase is located in the cell nucleus.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9342341# AUTHORS:Krawiec K, Kierdaszuk B, Shugar D.#TITLE:Inorganic tripolyphosphate (PPP(i)) as a phosphate donor for human deoxyribonucleoside kinases. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12535661# GENE ASSOCIATION: (7084.1) or (7083.1)
Abbreviation: R_FACOAL140i Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + ttdca --> amp + ppi + tdcoa Confidence Level: 4 JLR genes: LOCUS:_2181#TRANSCRIPT:1#ABBREVIATION:ACSL3# proteins: NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.1# citations: none GENE ASSOCIATION: (2181.1)
Abbreviation: R_C110CPT2m Synonyms: _0 SUBSYSTEM: Fatty Acid Metabolism Equation: [m] : coa + dmnoncrn --> crn + dmnoncoa Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Verhoeven NM, Wanders RJ, Poll-The BT, Saudubray JM, Jakobs C.#TITLE:The metabolism of phytanic acid and pristanic acid in man: a review.#JOURNAL:J Inherit Metab Dis#YEAR:1998#PUBMED:9819701# GENE ASSOCIATION:
Abbreviation: R_ALADGLNexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-D[e] + gln-L[c] <==> ala-D[c] + gln-L[e] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_3DPHBH2 Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 3dphb + h + nadph + o2 --> 3dpdhb + h2o + nadp Confidence Level: 2 Used to make Ubiquinone-10 enzyme has not been identified yet. IT genes: none proteins: none citations: AUTHORS:Szkopinska A.#TITLE:Ubiquinone. Biosynthesis of quinone ring and its isoprenoid side chain. Intracellular localization.#JOURNAL:Acta Biochim Pol. #YEAR:2000#PUBMED:11051212# GENE ASSOCIATION:
Hydroxylation of 3-Decaprenyl-4-hydroxybenzoate (NADPH)
Abbreviation: R_BACCLm Synonyms: _7 EC Number: 6.3.4.15 SUBSYSTEM: Biotin Metabolism Equation: [m] : atp + btn + h --> btamp + ppi Confidence Level: 2 IT genes: LOCUS:_3141#TRANSCRIPT:1#ABBREVIATION:HLCS#ECNUMBERS:6.3.4.10# proteins: NAME:holocarboxylase synthetase (biotin-[proprionyl-Coenzyme A-carboxylase (ATP-hydrolysing)] ligase) #ABBREVIATION:Hcls.1-m# citations: AUTHORS:Suzuki Y, Aoki Y, Ishida Y, Chiba Y, Iwamatsu A, Kishino T, Niikawa N, Matsubara Y, Narisawa K.#TITLE:Isolation and characterization of mutations in the human holocarboxylase synthetase cDNA.#JOURNAL:Nat Genet. #YEAR:1994#PUBMED:7842009# AUTHORS:Pacheco-Alvarez D, Solorzano-Vargas RS, Del Rio AL.#TITLE:Biotin in metabolism and its relationship to human disease.#JOURNAL:Arch Med Res. #YEAR:2002#PUBMED:12459313# GENE ASSOCIATION: (3141.1)
biotin-[acetyl-CoA-carboxylase] ligase, mitochondrial
Abbreviation: R_AACTOOR Synonyms: _0 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : aact + h2o + o2 --> h2o2 + mthgxl + nh4 Confidence Level: 4 MM genes: LOCUS:_8639#TRANSCRIPT:1#ABBREVIATION:AOC3#ECNUMBERS:1.4.3.6# proteins: NAME:amine oxidase, copper containing 3#ABBREVIATION:Aoc3.1# citations: AUTHORS:Dalfo E, Hernandez M, Lizcano JM, Tipton KF, Unzeta M.#TITLE:Activation of human lung semicarbazide sensitive amine oxidase by a low molecular weight component present in human plasma. #YEAR:2003#PUBMED:12878330# AUTHORS:Deng Y, Yu PH.#TITLE:Assessment of the deamination of aminoacetone, an endogenous substrate for semicarbazide-sensitive amine oxidase.#YEAR:1999#PUBMED:10328770# GENE ASSOCIATION: (8639.1)
Aminoacetone:oxygen oxidoreductase(deaminating)(flavin-containing)
Abbreviation: R_PItn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: pi[n] <==> pi[c] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DM_datp_LPAREN_n_RPAREN_ Synonyms: _0 Equation: [n] : datp --> Confidence Level: IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_5fthf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 5fthf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_nrpphr_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : nrpphr <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACS2 Synonyms: _0 EC Number: 6.2.1.1 SUBSYSTEM: Propanoate Metabolism Equation: [c] : atp + coa + ppa --> amp + ppcoa + ppi Confidence Level: 4 AMF genes: LOCUS:_65985#TRANSCRIPT:1#ABBREVIATION:AACS#ECNUMBERS:6.2.1.16# LOCUS:_55902#TRANSCRIPT:1#ABBREVIATION:ACAS2# LOCUS:_55902#TRANSCRIPT:2#ABBREVIATION:ACAS2# proteins: NAME:acetly-CoA synthetase 2 (ADP forming)#ABBREVIATION:Acas2.1# NAME:acetyl-CoA synthetase 2 (ADP forming)#ABBREVIATION:Acas2.2# NAME:acetoacetyl-CoA synthetase#ABBREVIATION:Aacs.1# citations: AUTHORS:Ohgami M, Takahashi N, Yamasaki M, Fukui T#TITLE:Expression of acetoacetyl-CoA synthetase, a novel cytosolic ketone body-utilizing enzyme, in human brain#JOURNAL:Biochem Pharmacol#YEAR:2003#PUBMED:12623130# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Luong A, Hannah VC, Brown MS, Goldstein JL#TITLE:Molecular characterization of human acetyl-CoA synthetase, an enzyme regulated by sterol regulatory element-binding proteins#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10843999# GENE ASSOCIATION: (55902.1) or (65985.1) or (55902.2)
Abbreviation: R_A4GALTg Synonyms: _8 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : galgluside_hs + udpgal --> h + thcrm_hs + udp Confidence Level: 4 EC: 2.4.1.228 NJ genes: LOCUS:_53947#TRANSCRIPT:1#ABBREVIATION:A4GALT# proteins: NAME:alpha 1,4-galactosyltransferase#ABBREVIATION:A4galt.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Kojima Y, Fukumoto S, Furukawa K, Okajima T, Wiels J, Yokoyama K, Suzuki Y, Urano T, Ohta M, Furukawa K.#TITLE:Molecular cloning of globotriaosylceramide/CD77 synthase, a glycosyltransferase that initiates the synthesis of globo series glycosphingolipids.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10748143# AUTHORS:Steffensen R, Carlier K, Wiels J, Levery SB, Stroud M, Cedergren B, Nilsson Sojka B, Bennett EP, Jersild C, Clausen H.#TITLE:Cloning and expression of the histo-blood group Pk UDP-galactose: Ga1beta-4G1cbeta1-cer alpha1, 4-galactosyltransferase. Molecular genetic basis of the p phenotype.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747952# GENE ASSOCIATION: (53947.1)
Lactosylceramide 4-alpha-galactosyltransferase
Abbreviation: R_H2O2tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h2o2[c] <==> h2o2[m] Confidence Level: 2 it genes: none proteins: none citations: AUTHORS:Ohno Y, Gallin JI. #TITLE:Diffusion of extracellular hydrogen peroxide into intracellular compartments of human neutrophils. Studies utilizing the inactivation of myeloperoxidase by hydrogen peroxide and azide.#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2989289# GENE ASSOCIATION:
hydrogen peroxide mitochondrial transport
Abbreviation: R_DNDPt48m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgdp[m] + dttp[c] --> dgdp[c] + dttp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_24_COMMA_25DHVITD2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _2425dhvitd2[c] --> 2425dhvitd2[e] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_24,25-Dihydroxyvitamin D2 transport from cytoplasm
Squalene epoxidase, endoplasmic reticular (NADP)
Abbreviation: R_SQLEr Synonyms: _0 EC Number: 1.14.99.7 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : h + nadph + o2 + sql --> Ssq23epx + h2o + nadp Confidence Level: 4 NCD genes: LOCUS:_6713#TRANSCRIPT:1#ABBREVIATION:SQLE# proteins: NAME:squalene epoxidase#ABBREVIATION:Sqle.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (6713.1)
Abbreviation: R_FADH2tru Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: fadh2[c] --> fadh2[r] Confidence Level: 1 diffusion - but unidirectional uptake into ER (to avoid loops w/ NAD/NADP) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
FADH2 transporter, endoplasmic reticulum
Abbreviation: R_24_COMMA_25DHVITD3t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _2425dhvitd3[c] --> 2425dhvitd3[e] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_24,25-Dihydroxyvitamin D3 transport from cytoplasm
Abbreviation: R_ECGISOr Synonyms: _0 SUBSYSTEM: Alkaloid biosynthesis II Equation: [r] : ecgon <==> pecgon Confidence Level: 1 SAB reaction name uncertain genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_gq1balpha_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gq1balpha_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RADH4 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : h2o + nadp + retinal-cis-13 <==> 13-cis-retn + (2) h + nadph Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Blaner WS.#TITLE:Cellular metabolism and actions of 13-cis-retinoic acid. #JOURNAL:J Am Acad Dermatol. #YEAR:2001#PUBMED:11606944# GENE ASSOCIATION:
Abbreviation: R_3AIBt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _3aib[c] --> 3aib[e] Confidence Level: 2 HOW? IT genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION:
Abbreviation: R_TDPGDH Synonyms: _2 EC Number: 4.2.1.46 SUBSYSTEM: Nucleotide Sugar Metabolism Equation: [c] : dtdpglu --> dtdp4d6dg + h2o Confidence Level: 2 JLR genes: LOCUS:_23483#TRANSCRIPT:1#ABBREVIATION:TGDS#ECNUMBERS:4.2.1.46# proteins: NAME:TDP-glucose 4,6-dehydratase#ABBREVIATION:Tgds.1# citations: AUTHORS:Strausberg RL, Feingold EA, Grouse LH, Derge JG, Klausner RD, et al.#TITLE:Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2002#PUBMED:12477932# GENE ASSOCIATION: (23483.1)
Abbreviation: R_DOLDPP_Ler Synonyms: _0 EC Number: 3.6.1.43 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) doldp_L + h2o --> (0.1) dolp_L + h + pi Confidence Level: 0 NCD genes: LOCUS:_57171#TRANSCRIPT:1#ABBREVIATION:DOLPP1# proteins: NAME:Dolichol pyrophosphate phosphatase, endoplasmic reticulum#ABBREVIATION:Dolpp1.1-er# citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION: (57171.1)
Dolichyl-diphosphate phosphohydrolase, human (liver)
Abbreviation: R_PROSTGE2t2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: prostge2[e] <==> prostge2[c] Confidence Level: 4 electrogenic, Na indepdendent & rev NJ genes: LOCUS:_6580#TRANSCRIPT:1#ABBREVIATION:SLC22A1# LOCUS:_6582#TRANSCRIPT:1#ABBREVIATION:SLC22A2# LOCUS:_6582#TRANSCRIPT:2#ABBREVIATION:SLC22A2# proteins: NAME:solute carrier family 22 (organic cation transporter), member 1#ABBREVIATION:Slc22a1.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.2# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# AUTHORS:Koepsell H, Schmitt BM, Gorboulev V.#TITLE:Organic cation transporters.#JOURNAL:Rev Physiol Biochem Pharmacol#YEAR:2003#PUBMED:12827517# AUTHORS:Zhang L, Dresser MJ, Gray AT, Yost SC, Terashita S, Giacomini KM.#TITLE:Cloning and functional expression of a human liver organic cation transporter.#JOURNAL:Mol Pharmacol. #YEAR:1997#PUBMED:9187257# GENE ASSOCIATION: (6582.2) or (6582.1) or (6580.1)
Abbreviation: R_HIStN1 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[c] + his-L[e] + (2) na1[e] <==> h[e] + his-L[c] + (2) na1[c] Confidence Level: 4 SAB based om PMID 10823827 genes: LOCUS:_10991#TRANSCRIPT:1#ABBREVIATION:SLC38A3# proteins: NAME:solute carrier family 38, member 3#ABBREVIATION:Slc38a3.1# citations: AUTHORS:Fei YJ, Sugawara M, Nakanishi T, Huang W, Wang H, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, genomic organization, and functional and electrogenic characteristics of human system N 1, a Na+- and H+-coupled glutamine transporter#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10823827# AUTHORS:Mackenzie B, Erickson JD#TITLE:Sodium-coupled neutral amino acid (System N/A) transporters of the SLC38 gene family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12845534# GENE ASSOCIATION: (10991.1)
Abbreviation: R_PHYCBOXL Synonyms: _0 EC Number: 4.1.1.28 SUBSYSTEM: Phenylalanine metabolism Equation: [c] : h + phe-L --> co2 + peamn Confidence Level: 1 SAB genes: LOCUS:_1644#TRANSCRIPT:1#ABBREVIATION:DDC#ECNUMBERS:4.1.1.28# proteins: NAME:aromatic-L-amino-acid decarboxylase#ABBREVIATION:Ddc.1# citations: none GENE ASSOCIATION: (1644.1)
Abbreviation: R_ARTPLM1m Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [m] : Rtotalcoa <==> pmtcoa Confidence Level: 0 cytosolic conversion of pooled FA into palmitate (so subsequent transport into mit w/ FA oxidation can occur). Since Carbon stoichiometry normalized wrt C16 ratio between Rgroupcoa and pmtcoa is 1:1 HR fatty acid metabolism - R groups = acyl groups, Rtotal, Rtotal2, Rtotal3 correspond to three possible acyl groups added to glycerol R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DM_gpi_sig_LPAREN_er_RPAREN_ Synonyms: _0 Equation: [r] : gpi_sig --> Confidence Level: NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_nh4_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : nh4 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PLYSPSer Synonyms: _0 SUBSYSTEM: Lysine Metabolism Equation: [r] : Ntmelys + h2o --> pepslys + tmlys Confidence Level: 4 MM genes: LOCUS:_23478#TRANSCRIPT:1#ABBREVIATION:SPC18#ECNUMBERS:3.4.21.89# LOCUS:_60559#TRANSCRIPT:1#ABBREVIATION:SPCS3# LOCUS:_28972#TRANSCRIPT:1#ABBREVIATION:SPCS1# LOCUS:_9789#TRANSCRIPT:1#ABBREVIATION:SPCS2# LOCUS:_90701#TRANSCRIPT:1#ABBREVIATION:SEC11L3# proteins: NAME:signal peptidase complex subunit 3 homolog, endoplasmic reticulum#ABBREVIATION:Spcs3.1-r# NAME:signal peptidase complex (18kD), endoplasmic reticulum#ABBREVIATION:Spc18.1-r # NAME:signal peptidase complex subunit 1 homolog, endoplasmic reticulum #ABBREVIATION:Spcs1.1-r# NAME:signal peptidase complex subunit 2 homolog, endoplasmic reticulum #ABBREVIATION:Spcs2.1-r# NAME:SEC11-like 3, endoplasmic reticulum#ABBREVIATION:Sec11l3.1-r# citations: none GENE ASSOCIATION: (60559.1) and (28972.1) and (90701.1) and (9789.1) and (23478.1)
protein lysine peptidase (endoplasmic reticulum)
Abbreviation: R_PGLYCP Synonyms: _3 EC Number: 3.1.3.18 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : 2pglyc + h2o --> glyclt + pi Confidence Level: 4 JLR genes: LOCUS:_5240#TRANSCRIPT:1#ABBREVIATION:PGP#ECNUMBERS:3.1.3.18# proteins: NAME:phosphoglycolate phosphatase#ABBREVIATION:Pgp.1# citations: AUTHORS:Mulley JC, Barton N, Callen DF#TITLE:Localisation of human PGP and HAGH genes to 16p13.3#JOURNAL:Cytogenet Cell Genet.#YEAR:1990#PUBMED:2164460# AUTHORS:Zecher R, Schwulera U, Wolf HU.#TITLE:Purification, isolation and characterization of a phosphoglycolate phosphatase isoenzyme from human erythrocytes.#JOURNAL:Int J Biochem.#YEAR:1982#PUBMED:6290284# GENE ASSOCIATION: (5240.1)
Abbreviation: R_DGK1 Synonyms: _0 EC Number: 2.7.4.8 SUBSYSTEM: Nucleotides Equation: [c] : atp + dgmp <==> adp + dgdp Confidence Level: 4 Added EC- JLR genes: LOCUS:_2987#TRANSCRIPT:1#ABBREVIATION:GUK1#ECNUMBERS:2.7.4.8# proteins: NAME:guanylate kinase#ABBREVIATION:Guk1.1# citations: AUTHORS:Brady WA, Kokoris MS, Fitzgibbon M, Black ME. #TITLE:Cloning, characterization, and modeling of mouse and human guanylate kinases.#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8663313# GENE ASSOCIATION: (2987.1)
Abbreviation: R_CYTK8n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : cmp + datp <==> cdp + dadp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_AMPtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: amp[c] <==> amp[x] Confidence Level: 0 "diffusion" NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_so4_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : so4 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_P45027A15m Synonyms: _5 EC Number: 1.14.13.15 SUBSYSTEM: Bile Acid Biosynthesis Equation: [m] : h + nadph + o2 + xol7ah3 --> (2) h2o + nadp + xol7ah2al Confidence Level: 4 from uniprot NJ genes: LOCUS:_1593#TRANSCRIPT:1#ABBREVIATION:CYP27A1# proteins: NAME:cytochrome P450, family 27, subfamily A, polypeptide 1#ABBREVIATION:Cyp27a1.1-m# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1593.1)
_5-beta-cytochrome P450, family 27, subfamily A, polypeptide 1
Abbreviation: R_MTHFD2 Synonyms: _0 EC Number: 1.5.1.5 SUBSYSTEM: Folate Metabolism Equation: [c] : mlthf + nad <==> methf + nadh Confidence Level: 2 NCD genes: LOCUS:_80068#TRANSCRIPT:1#ABBREVIATION:FLJ13105# LOCUS:_285216#TRANSCRIPT:1#ABBREVIATION:LOC285216# proteins: NAME:hypothetical protein FLJ13105#ABBREVIATION:Flj13105.1# NAME:hypothetical protein LOC285216#ABBREVIATION:Loc285216.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (285216.1) or (80068.1)
methylenetetrahydrofolate dehydrogenase (NAD)
Abbreviation: R_TRIOK Synonyms: _3 EC Number: 2.7.1.28 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : atp + glyald --> adp + g3p + h Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# GENE ASSOCIATION:
Abbreviation: R_EX_bvite_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : bvite <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_KSII_CORE2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ksii_core2[g] --> ksii_core2[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
keratan sulfate II (core2) transport, golgi to extracellular
Abbreviation: R_LPS4e Synonyms: _0 SUBSYSTEM: Triacylglycerol Synthesis Equation: [e] : h2o + pglyc_hs --> 1glyc_hs + Rtotal2 + h Confidence Level: 4 FULLR corresponds to acyl/R groups in texts. NJ genes: LOCUS:_50487#TRANSCRIPT:1#ABBREVIATION:PLA2G3# proteins: NAME:phospholipase A2, group III#ABBREVIATION:Pla2g3.1# citations: AUTHORS:Valentin E, Ghomashchi F, Gelb MH, Lazdunski M, Lambeau G.#TITLE:Novel human secreted phospholipase A(2) with homology to the group III bee venom enzyme.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10713052# GENE ASSOCIATION: (50487.1)
Abbreviation: R_ACACT10m Synonyms: _0 EC Number: 2.3.1.9 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 2maacoa + coa <==> accoa + ppcoa Confidence Level: 4 MM genes: LOCUS:_38#TRANSCRIPT:1#ABBREVIATION:ACAT1#ECNUMBERS:2.3.1.9# proteins: NAME:acetyl-Coenzyme A acetyltransferase 1 (acetoacetyl Coenzyme A thiolase)#ABBREVIATION:Acat1.1-m# citations: none GENE ASSOCIATION: (38.1)
Abbreviation: R_NACHEX23ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core2_deg4 --> acgam + ksii_core2_deg5 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3073.1) or (3074.1)
Abbreviation: R_FACOAL224 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : adrn + atp + coa <==> adrncoa + amp + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_FACOAL203 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + dlnlcg <==> amp + dlnlcgcoa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_ICDHxm Synonyms: _0 EC Number: 1.1.1.41 SUBSYSTEM: Citric Acid Cycle Equation: [m] : icit + nad --> akg + co2 + nadh Confidence Level: 0 The reaction may be reversible. Strathern shows the reactions as irreversible. IF genes: LOCUS:_3421#TRANSCRIPT:1#ABBREVIATION:IDH3G#ECNUMBERS:1.1.1.41# LOCUS:_3420#TRANSCRIPT:1#ABBREVIATION:IDH3B#ECNUMBERS:1.1.1.41# LOCUS:_3419#TRANSCRIPT:1#ABBREVIATION:IDH3A# LOCUS:_3420#TRANSCRIPT:2#ABBREVIATION:IDH3B#ECNUMBERS:1.1.1.41# LOCUS:_3421#TRANSCRIPT:2#ABBREVIATION:IDH3G#ECNUMBERS:1.1.1.41# LOCUS:_3420#TRANSCRIPT:3#ABBREVIATION:IDH3B#ECNUMBERS:1.1.1.41# proteins: NAME:isocitrate dehydrogenase 3 (NAD+), mitochondrial#ABBREVIATION:Idhab1g1-m# NAME:isocitrate dehydrogenase 3 (NAD+), mitochondrial#ABBREVIATION:Idhab2g1-m# NAME:isocitrate dehydrogenase 3 (NAD+), mitochondrial#ABBREVIATION:Idhab3g1-m# NAME:isocitrate dehydrogenase 3 (NAD+), mitochondrial#ABBREVIATION:Idhab1g2-m# NAME:isocitrate dehydrogenase 3 (NAD+), mitochondrial#ABBREVIATION:Idhab2g2-m# NAME:isocitrate dehydrogenase 3 (NAD+), mitochondrial#ABBREVIATION:Idhab3g2-m# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (3421.2 and 3420.3 and 3419.1) or (3420.1 and 3421.1 and 3419.1) or (3420.1 and 3421.1 and 3419.1) or (3420.3 and 3421.1 and 3419.1) or (3421.1 and 3419.1 and 3420.2) or (3421.2 and 3419.1 and 3420.2)
Abbreviation: R_GDCHOLAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: gdchola[x] <==> gdchola[c] Confidence Level: 2 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TRPt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + trp-L[e] --> na1[c] + trp-L[c] Confidence Level: 2 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# proteins: NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# AUTHORS:Van Winkle LJ#TITLE:Amino acid transport regulation and early embryo development#JOURNAL:Biol Reprod#YEAR:2001#PUBMED:11133652# GENE ASSOCIATION: (11254.1)
L-tryptophan transport in via sodium symport
Abbreviation: R_MANt1r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: man[e] <==> man[c] Confidence Level: 4 NCD genes: LOCUS:_6515#TRANSCRIPT:1#ABBREVIATION:SLC2A3# LOCUS:_6514#TRANSCRIPT:1#ABBREVIATION:SLC2A2# LOCUS:_6513#TRANSCRIPT:1#ABBREVIATION:SLC2A1# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 1, plasma membrane#ABBREVIATION:Slc2a1.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 2, extracellular#ABBREVIATION:Slc2a2.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 3, extracellular#ABBREVIATION:Slc2a3.1-e# citations: AUTHORS:Hosokawa M, Thorens B#TITLE:Glucose release from GLUT2-null hepatocytes: characterization of a major and a minor pathway#JOURNAL:Am J Physiol Endocrinol Metab#YEAR:2002#PUBMED:11882499# AUTHORS:Guillam MT, Burcelin R, Thorens B#TITLE:Normal hepatic glucose production in the absence of GLUT2 reveals an alternative pathway for glucose release from hepatocytes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9770484# AUTHORS:Uldry M, Thorens B#TITLE:The SLC2 family of facilitated hexose and polyol transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750891# AUTHORS:Flier JS, Mueckler MM, Usher P, Lodish HF#TITLE:Elevated levels of glucose transport and transporter messenger RNA are induced by ras or src oncogenes#JOURNAL:Science#YEAR:1987#PUBMED:3103217# AUTHORS:Maher F, Vannucci SJ, Simpson IA#TITLE:Glucose transporter proteins in brain#JOURNAL:FASEB J#YEAR:1994#PUBMED:7926364# AUTHORS:Mueckler M, Caruso C, Baldwin SA, Panico M, Blench I, Morris HR, Allard WJ, Lienhard GE, Lodish HF#TITLE:Sequence and structure of a human glucose transporter#JOURNAL:Science#YEAR:1985#PUBMED:3839598# AUTHORS:Uldry M, Ibberson M, Hosokawa M, Thorens B#TITLE:GLUT2 is a high affinity glucosamine transporter#JOURNAL:FEBS Lett#YEAR:2002#PUBMED:12135767# AUTHORS:Fukumoto H, Seino S, Imura H, Seino Y, Eddy RL, Fukushima Y, Byers MG, Shows TB, Bell GI#TITLE:Sequence, tissue distribution, and chromosomal localization of mRNA encoding a human glucose transporter-like protein#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1988#PUBMED:3399500# AUTHORS:Johnson JH, Newgard CB, Milburn JL, Lodish HF, Thorens B#TITLE:The high Km glucose transporter of islets of Langerhans is functionally similar to the low affinity transporter of liver and has an identical primary sequence#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2182619# AUTHORS:Thorens B, Cheng ZQ, Brown D, Lodish HF#TITLE:Liver glucose transporter: a basolateral protein in hepatocytes and intestine and kidney cells#JOURNAL:Am J Physiol#YEAR:1990#PUBMED:1701966# GENE ASSOCIATION: (6513.1) or (6515.1) or (6514.1)
Abbreviation: R_GLCt2_2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glc-D[e] + (2) h[e] <==> glc-D[c] + (2) h[c] Confidence Level: 4 genes: LOCUS:_6523#TRANSCRIPT:1#ABBREVIATION:SLC5A1# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 1, extracellular#ABBREVIATION:Slc5a1.1-e# citations: AUTHORS:Hirayama BA, Loo DD, Wright EM.#TITLE:Protons drive sugar transport through the Na+/glucose cotransporter (SGLT1)#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8063771# AUTHORS:Hediger MA, Turk E, Wright EM#TITLE:Homology of the human intestinal Na+/glucose and Escherichia coli Na+/proline cotransporters#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1989#PUBMED:2490366# AUTHORS:Quick M, Loo DD, Wright EM#TITLE:Neutralization of a conserved amino acid residue in the human Na+/glucose transporter (hSGLT1) generates a glucose-gated H+ channel#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11024018# AUTHORS:Wright EM, Hirayama BA, Loo DDF, Turk E, Hager K#TITLE:Intestinal sugar transport#YEAR:1994# AUTHORS:Leung DW, Loo DD, Hirayama BA, Zeuthen T, Wright EM#TITLE:Urea transport by cotransporters#JOURNAL:J Physiol#YEAR:2000#PUBMED:11034615# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# GENE ASSOCIATION: (6523.1)
Abbreviation: R_DCMPDA Synonyms: _0 EC Number: 3.5.4.12 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : dcmp + h + h2o <==> dump + nh4 Confidence Level: 2 NCD genes: LOCUS:_1635#TRANSCRIPT:1#ABBREVIATION:DCTD# proteins: NAME:dCMP deaminase #ABBREVIATION:Dctd.1# citations: AUTHORS:Weiner KX, Ciesla J, Jaffe AB, Ketring R, Maley F, Maley GF.#TITLE:Chromosomal location and structural organization of the human deoxycytidylate deaminase gene.#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7642519# AUTHORS:Weiner KX, Weiner RS, Maley F, Maley GF.#TITLE:Primary structure of human deoxycytidylate deaminase and overexpression of its functional protein in Escherichia coli.#JOURNAL:J Biol Chem.#YEAR:1993#PUBMED:7685356# GENE ASSOCIATION: (1635.1)
Abbreviation: R_GD1B2te Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gd1b2_hs[c] <==> gd1b2_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_GLCNACASE2ly Synonyms: _4 EC Number: 3.2.1.50 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg8 --> acgam + hs_deg9 Confidence Level: 4 NCD genes: LOCUS:_4669#TRANSCRIPT:1#ABBREVIATION:NAGLU#ECNUMBERS:3.2.1.50# proteins: NAME:N-acetylglucosaminidase, alpha- (Sanfilippo disease IIIB), lysosome#ABBREVIATION:Naglu.1-l# citations: AUTHORS:Weber B, Blanch L, Clements PR, Scott HS, Hopwood JJ#TITLE:Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B)#JOURNAL:Hum Mol Genet#YEAR:1996#PUBMED:8776591# AUTHORS:Sasaki T, Sukegawa K, Masue M, Fukuda S, Tomatsu S, Orii T#TITLE:Purification and partial characterization of alpha-N-acetylglucosaminidase from human liver#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1783617# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION: (4669.1)
Abbreviation: R_NDPK1n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + gdp <==> adp + gtp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:GDP), nuclear
Abbreviation: R_RPI Synonyms: _0 EC Number: 5.3.1.6 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : r5p <==> ru5p-D Confidence Level: 0 SMP genes: LOCUS:_22934#TRANSCRIPT:1#ABBREVIATION:RPIA#ECNUMBERS:5.3.1.6# proteins: NAME:ribose 5-phosphate isomerase#ABBREVIATION:Rpia.1# citations: none GENE ASSOCIATION: (22934.1)
Abbreviation: R_SERDGLYexR Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gly[c] + ser-D[e] <==> gly[e] + ser-D[c] Confidence Level: 2 SAB many other exchanges possible genes: LOCUS:_6520#TRANSCRIPT:1#ABBREVIATION:SLC3A2# LOCUS:_56301#TRANSCRIPT:1#ABBREVIATION:SLC7A10# proteins: NAME: solute carrier family 3 (activators of dibasic and neutral amino acid transport), member 2, extracellular#ABBREVIATION:Slc3a2.1-e# NAME:solute carrier family 7, (neutral amino acid transporter, y+ system) member 10#ABBREVIATION:Slc7a10.1# citations: AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# AUTHORS:Fukasawa Y, Segawa H, Kim JY, Chairoungdua A, Kim DK, Matsuo H, Cha SH, Endou H, Kanai Y#TITLE:Identification and characterization of a Na(+)-independent neutral amino acid transporter that associates with the 4F2 heavy chain and exhibits substrate selectivity for small neutral D- and L-amino acids#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10734121# AUTHORS:Nakauchi J, Matsuo H, Kim DK, Goto A, Chairoungdua A, Cha SH, Inatomi J, Shiokawa Y, Yamaguchi K, Saito I, Endou H, Kanai Y#TITLE:Cloning and characterization of a human brain Na(+)-independent transporter for small neutral amino acids that transports D-serine with high affinity#JOURNAL:Neurosci Lett#YEAR:2000#PUBMED:10863037# GENE ASSOCIATION: (56301.1) and (6520.1)
Abbreviation: R_CYTDtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: cytd[c] <==> cytd[m] Confidence Level: 4 IT genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-m# citations: AUTHORS:Lai Y, Tse CM, Unadkat JD.#TITLE:Mitochondrial expression of the human equilibrative nucleoside transporter 1 (hENT1) results in enhanced mitochondrial toxicity of antiviral drugs.#JOURNAL:J Biol Chem. #YEAR:2004#PUBMED:14607828# AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (2030.1)
cytidine facilated transport in mitochondria
Abbreviation: R_TXASr Synonyms: _2 EC Number: 5.3.99.5 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : prostgh2 --> txa2 Confidence Level: 0 NJ genes: LOCUS:_6916#TRANSCRIPT:1#ABBREVIATION:TBXAS1#ECNUMBERS:5.3.99.5# proteins: NAME:thromboxane A synthase 1#ABBREVIATION:Tbxas1.1# citations: TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# GENE ASSOCIATION: (6916.1)
Abbreviation: R_MI13456Ptn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: mi13456p[c] <==> mi13456p[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
inositol 1,3,4,5,6-pentakisphosphate nuclear transport (diffusion)
Abbreviation: R_THCHOLSTOICtm Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: thcholstoic[m] <==> thcholstoic[x] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_LFORKYNHYD Synonyms: _0 EC Number: 3.7.1.3 SUBSYSTEM: Tryptophan metabolism Equation: [c] : Lfmkynr + h2o --> ala-L + h + nformanth Confidence Level: 1 SAB changed to L form genes: LOCUS:_8942#TRANSCRIPT:1#ABBREVIATION:KYNU#ECNUMBERS:3.7.1.3# proteins: NAME:kynureninase (L-kynurenine hydrolase)#ABBREVIATION:Kynu.1# citations: none GENE ASSOCIATION: (8942.1)
Abbreviation: R_GDPFUCtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gdpfuc[c] + gmp[g] <==> gdpfuc[g] + gmp[c] Confidence Level: 4 NCD genes: LOCUS:_55343#TRANSCRIPT:1#ABBREVIATION:SLC35C1# proteins: NAME:solute carrier family 35, member C1, Golgi apparatus#ABBREVIATION:Slc35c1.1-g# citations: AUTHORS:Luhn K, Wild MK, Eckhardt M, Gerardy-Schahn R, Vestweber D#TITLE:The gene defective in leukocyte adhesion deficiency II encodes a putative GDP-fucose transporter#JOURNAL:Nat Genet#YEAR:2001#PUBMED:11326279# AUTHORS:Lubke T, Marquardt T, Etzioni A, Hartmann E, von Figura K, Korner C#TITLE:Complementation cloning identifies CDG-IIc, a new type of congenital disorders of glycosylation, as a GDP-fucose transporter deficiency#JOURNAL:Nat Genet#YEAR:2001#PUBMED:11326280# GENE ASSOCIATION: (55343.1)
Abbreviation: R_STS2r Synonyms: _4 EC Number: 3.1.6.2 SUBSYSTEM: Steroid Metabolism Equation: [r] : estrones + h2o --> estrone + h + so4 Confidence Level: 4 NJ genes: LOCUS:_412#TRANSCRIPT:1#ABBREVIATION:STS#ECNUMBERS:3.1.6.2# proteins: NAME:steroid sulfatase (microsomal), arylsulfatase C#ABBREVIATION:Sts.1# citations: AUTHORS:Stein C, Hille A, Seidel J, Rijnbout S, Waheed A, Schmidt B, Geuze H, von Figura K.#TITLE:Cloning and expression of human steroid-sulfatase. Membrane topology, glycosylation, and subcellular distribution in BHK-21 cells.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2668275# AUTHORS:Migeon BR, Shapiro LJ, Norum RA, Mohandas T, Axelman J, Dabora RL.#TITLE:Abstract Differential expression of steroid sulphatase locus on active and inactive human X chromosome.#JOURNAL:Nature#YEAR:1982#PUBMED:6957717# GENE ASSOCIATION: (412.1)
Abbreviation: R_AGTix Synonyms: _0 EC Number: 2.6.1.44 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [x] : ala-L + glx --> gly + pyr Confidence Level: 4 NCD genes: LOCUS:_189#TRANSCRIPT:1#ABBREVIATION:AGXT#ECNUMBERS:2.6.1.44# proteins: NAME:alanine-glyoxylate aminotransferase (oxalosis I; hyperoxaluria I; glycolicaciduria; serine-pyruvate aminotransferase), peroxisomal #ABBREVIATION:Agxt.1-x# citations: AUTHORS:Holmes RP, Assimos DG#TITLE:Glyoxylate synthesis, and its modulation and influence on oxalate synthesis#JOURNAL:J Urol#YEAR:1998#PUBMED:9783918# AUTHORS:Purdue PE, Takada Y, Danpure CJ.#TITLE:Identification of mutations associated with peroxisome-to-mitochondrion mistargeting of alanine/glyoxylate aminotransferase in primary hyperoxaluria type 1.#YEAR:1990#PUBMED:1703535# AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# GENE ASSOCIATION: (189.1)
alanine-glyoxylate transaminase (irreversible), (peroxisomal)
Abbreviation: R_UPPDC1 Synonyms: _0 EC Number: 4.1.1.37 SUBSYSTEM: Heme Biosynthesis Equation: [c] : (4) h + uppg3 --> (4) co2 + cpppg3 Confidence Level: 4 Acts on a number of porphyrinogens genes: LOCUS:_7389#TRANSCRIPT:1#ABBREVIATION:UROD#ECNUMBERS:4.1.1.37# proteins: NAME:uroporphyrinogen decarboxylase#ABBREVIATION:Urod.1# citations: AUTHORS:Romeo PH, Raich N, Dubart A, Beaupain D, Pryor M, Kushner J, Cohen-Solal M, Goossens M.#TITLE:Molecular cloning and nucleotide sequence of a complete human uroporphyrinogen decarboxylase cDNA. #JOURNAL:J Biol Chem#YEAR:1986#PUBMED:3015909# GENE ASSOCIATION: (7389.1)
uroporphyrinogen decarboxylase (uroporphyrinogen III)
Abbreviation: R_DOLPMT1_Uer Synonyms: _2 EC Number: 2.4.1.130 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolmanp_U + (0.1) m4mpdol_U --> (0.1) dolp_U + h + (0.1) m5mpdol_U Confidence Level: 0 NCD genes: LOCUS:_10195#TRANSCRIPT:1#ABBREVIATION:ALG3#ECNUMBERS:2.4.1.130# LOCUS:_10195#TRANSCRIPT:4#ABBREVIATION:ALG3#ECNUMBERS:2.4.1.130# LOCUS:_10195#TRANSCRIPT:3#ABBREVIATION:ALG3#ECNUMBERS:2.4.1.130# LOCUS:_10195#TRANSCRIPT:2#ABBREVIATION:ALG3#ECNUMBERS:2.4.1.130# proteins: NAME:alpha-1,3-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg3.1-er# NAME:alpha-1,3-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg3.2-er# NAME:alpha-1,3-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg3.3-er# NAME:alpha-1,3-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg3.4-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (10195.4) or (10195.1) or (10195.3) or (10195.2)
Abbreviation: R_P45017A2r Synonyms: _4 EC Number: 1.14.99.9 SUBSYSTEM: Steroid Metabolism Equation: [r] : 17ahprgnlone + h + nadph + (0.5) o2 --> acald + dhea + h2o + nadp Confidence Level: 4 ER noted in locuslink ref - RETURN AND CHECK AFTER DEEPER LIT SEARCH NJ genes: LOCUS:_1586#TRANSCRIPT:1#ABBREVIATION:CYP17A1#ECNUMBERS:1.14.99.9# proteins: NAME:cytochrome P450, family 17, subfamily A, polypeptide 1#ABBREVIATION:Cyp17a1.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# AUTHORS:Korzekwa KR, Trager WF, Mancewicz J, Osawa Y#TITLE:Studies on the mechanism of aromatase and other cytochrome P450 mediated deformylation reactions#JOURNAL:J Steroid Biochem Mol Biol#YEAR:1993# GENE ASSOCIATION: (1586.1)
Abbreviation: R_FAEL183 Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : (5) h + lnlncgcoa + malcoa + (4) nadph + o2 --> co2 + coa + dlnlcgcoa + (3) h2o + (4) nadp Confidence Level: 4 fatty acid elongation step NJ genes: LOCUS:_6785#TRANSCRIPT:1#ABBREVIATION:ELOVL4# proteins: NAME:elongation of very long chain fatty acids - like 4#ABBREVIATION:Elovl4.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Zhang K, Kniazeva M, Han M, Li W, Yu Z, Yang Z, Li Y, Metzker ML, Allikmets R, Zack DJ, Kakuk LE, Lagali PS, Wong PW, MacDonald IM, Sieving PA, Figueroa DJ, Austin CP, Gould RJ, Ayyagari R, Petrukhin K.#TITLE:A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy.#JOURNAL:Nat Genet#YEAR:2001#PUBMED:11138005# AUTHORS:Nakamura MT, Nara TY.#TITLE:Structure, function, and dietary regulation of delta6, delta5, and delta9 desaturases.#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189125# GENE ASSOCIATION: (6785.1)
Abbreviation: R_CHOLPtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: cholp[c] <==> cholp[l] Confidence Level: 1 unknown mechanism for transport - no strong evidence to indicate further lysosomal or golgi metabolism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
choline phosphate intracellular transport
Abbreviation: R_TMDPK Synonyms: _0 EC Number: 2.7.6.2 SUBSYSTEM: Thiamine Metabolism Equation: [c] : atp + thm --> amp + h + thmpp Confidence Level: 4 genes: LOCUS:_27010#TRANSCRIPT:1#ABBREVIATION:TPK1# proteins: NAME:thiamin pyrophosphokinase 1 #ABBREVIATION:Tpk1.1# citations: AUTHORS:Bettendorff L, Mastrogiacomo F, Kish SJ, Grisar T.#TITLE:Thiamine, thiamine phosphates, and their metabolizing enzymes in human brain. #JOURNAL:J Neurochem. #YEAR:1996#PUBMED:8522961# AUTHORS:Zhao R, Gao F, Goldman ID.#TITLE:Molecular cloning of human thiamin pyrophosphokinase. #JOURNAL:Biochim Biophys Acta. #YEAR:2001#PUBMED:11342117# GENE ASSOCIATION: (27010.1)
Abbreviation: R_SARCStp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: sarcs[c] --> sarcs[x] Confidence Level: 1 MM genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ALCD21_L Synonyms: _0 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : 12ppd-S + nad --> h + lald-L + nadh Confidence Level: 2 NCD genes: LOCUS:_130#TRANSCRIPT:1#ABBREVIATION:ADH6#ECNUMBERS:1.1.1.1# LOCUS:_126#TRANSCRIPT:1#ABBREVIATION:ADH1C#ECNUMBERS:1.1.1.1# LOCUS:_125#TRANSCRIPT:1#ABBREVIATION:ADH1B#ECNUMBERS:1.1.1.1# LOCUS:_128#TRANSCRIPT:1#ABBREVIATION:ADH5#ECNUMBERS:1.1.1.1# LOCUS:_127#TRANSCRIPT:1#ABBREVIATION:ADH4#ECNUMBERS:1.1.1.1# LOCUS:_124#TRANSCRIPT:1#ABBREVIATION:ADH1A#ECNUMBERS:1.1.1.1# LOCUS:_131#TRANSCRIPT:1#ABBREVIATION:ADH7#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:1#ABBREVIATION:ZADH2# LOCUS:_137872#TRANSCRIPT:1#ABBREVIATION:ADHFE1#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:2#ABBREVIATION:ZADH2# proteins: NAME:alcohol dehydrogenase IV #ABBREVIATION:Adh4# NAME:alcohol dehydrogenase V#ABBREVIATION:Adh5# NAME:alcohol dehydrogenase 1A (class I), alpha polypeptide#ABBREVIATION:Adh1a.1# NAME:alcohol dehydrogenase IB (class I), beta polypeptide#ABBREVIATION:Adh1b.1# NAME:alcohol dehydrogenase 1C (class I), gamma polypeptide#ABBREVIATION:Adh1c.1# NAME:alcohol dehydrogenase 6 (class V)#ABBREVIATION:Adh6.1# NAME:alcohol dehydrogenase 7 (class IV), mu or sigma polypeptide#ABBREVIATION:Adh7.1# NAME:alcohol dehydrogenase, iron containing, 1 (liver)#ABBREVIATION:Adhfe1.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ab.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ac# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1bc# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.1# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.2# citations: AUTHORS:Ewaschuk JB, Naylor JM, Zello GA#TITLE:D-lactate in human and ruminant metabolism#JOURNAL:J Nutr#YEAR:2005#PUBMED:15987839# AUTHORS:Christopher MM, Eckfeldt JH, Eaton JW.#TITLE:Propylene glycol ingestion causes D-lactic acidosis#JOURNAL:Lab Invest.#YEAR:1990#PUBMED:2296157# GENE ASSOCIATION: (130.1) or (125.1) or (124.1) or (128.1) or (126.1) or (284273.2) or (126.1 and 124.1) or (127.1) or (125.1 and 124.1) or (137872.1) or (284273.1) or (125.1 and 126.1) or (131.1)
alcohol dehydrogenase (L-1,2-propanediol)
Abbreviation: R_ACGALK2 Synonyms: _0 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acgal + itp --> acgal1p + h + idp Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_PGK Synonyms: _0 EC Number: 2.7.2.3 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : 3pg + atp <==> 13dpg + adp Confidence Level: 0 SMP genes: LOCUS:_5230#TRANSCRIPT:1#ABBREVIATION:PGK1#ECNUMBERS:2.7.2.3# LOCUS:_5232#TRANSCRIPT:1#ABBREVIATION:PGK2# LOCUS:_348477#TRANSCRIPT:1#ABBREVIATION:FLJ39207# proteins: NAME:C219-reactive peptide#ABBREVIATION:Flj39207.1# NAME:phosphoglycerate kinase 1#ABBREVIATION:Pgk1.1# NAME:phosphoglycerate kinase 2 (testis)#ABBREVIATION:Pgk2.1# citations: none GENE ASSOCIATION: (5230.1) or (348477.1) or (5232.1)
Abbreviation: R_DNDPt30m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[m] + dcdp[c] --> adp[c] + dcdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_FACOAL160i Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + hdca --> amp + pmtcoa + ppi Confidence Level: 4 TV catalyzed by acyl-CoA synthetases genes: LOCUS:_51703#TRANSCRIPT:1#ABBREVIATION:ACSL5# LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# LOCUS:_2181#TRANSCRIPT:1#ABBREVIATION:ACSL3# LOCUS:_2181#TRANSCRIPT:2#ABBREVIATION:ACSL3# LOCUS:_22305#TRANSCRIPT:1#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# LOCUS:_22305#TRANSCRIPT:2#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.1# NAME:acyl-CoA synthetase long-chain family member 5#ABBREVIATION:Acsl5.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.2# NAME:acyl-CoA synthetase long-chain family member 6#ABBREVIATION:Acsl6.1# NAME:acyl-CoA synthetase long-chain family member 6 #ABBREVIATION:Acsl6.2# citations: AUTHORS:Stanczak H, Stanczak JJ, Singh I.#TITLE:Chromosomal localization of the human gene for palmitoyl-CoA ligase (FACL1).#JOURNAL:Cytogenet Cell Genet.#YEAR:1992#PUBMED:1531127# GENE ASSOCIATION: (2181.2) or (22305.2) or (51703.1) or (22305.1) or (2180.1) or (2181.1)
Abbreviation: R_G14T7g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre5 + udpgal --> h + ksi_pre6 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
Abbreviation: R_ACOAO7p Synonyms: _0 EC Number: 1.3.3.6 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : o2 + pmtcoa --> h2o2 + hdd2coa Confidence Level: 4 NCD genes: LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:acyl-Coenzyme A oxidase 1#ABBREVIATION:Acox.1# NAME:acyl-Coenzyme A oxidase 1#ABBREVIATION:Acox.2# citations: AUTHORS:Varanasi U, Chu R, Chu S, Espinosa R, LeBeau MM, Reddy JK.#TITLE:Isolation of the human peroxisomal acyl-CoA oxidase gene: organization, promoter analysis, and chromosomal localization.#JOURNAL:Proc Natl Acad Sci#YEAR:1994#PUBMED:8159712# AUTHORS:Chu R, Varanasi U, Chu S, Lin Y, Usuda N, Rao MS, Reddy JK.#TITLE:Overexpression and characterization of the human peroxisomal acyl-CoA oxidase in insect cells.#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7876265# GENE ASSOCIATION: (51.1) or (51.2)
acyl-CoA oxidase (hexadecanoyl-CoA), peroxisomal
Abbreviation: R_KYNAKGAT Synonyms: _0 EC Number: 2.6.1.7 SUBSYSTEM: Tryptophan metabolism Equation: [c] : Lkynr + akg --> 4aphdob + glu-L Confidence Level: 4 SAB genes: LOCUS:_883#TRANSCRIPT:1#ABBREVIATION:CCBL1#ECNUMBERS:2.6.1.64# LOCUS:_51166#TRANSCRIPT:1#ABBREVIATION:AADAT# LOCUS:_51166#TRANSCRIPT:2#ABBREVIATION:AADAT# proteins: NAME:cysteine conjugate-beta lyase; cytoplasmic (glutamine transaminase K, kyneurenine aminotransferase) #ABBREVIATION:Ccbl1.1# NAME:aminoadipate aminotransferase#ABBREVIATION:Aadat.1# NAME:aminoadipate aminotransferase#ABBREVIATION:Aadat.2# citations: AUTHORS: Cooper AJ#TITLE:The role of glutamine transaminase K (GTK) in sulfur and alpha-keto acid metabolism in the brain, and in the possible bioactivation of neurotoxicants#JOURNAL:Neurochem Int#YEAR:2004#PUBMED:15016471# GENE ASSOCIATION: (51166.1) or (883.1) or (51166.2)
L-Kynurenine:2-oxoglutarate aminotransferase
Abbreviation: R_L_DASH_LACtcm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: lac-L[c] --> lac-L[m] Confidence Level: 2 SAB genes: LOCUS:_366#TRANSCRIPT:1#ABBREVIATION:AQP9# proteins: NAME:aquaporin 9#ABBREVIATION:Aqp9.1# citations: AUTHORS:Amiry-Moghaddam M, Lindland H, Zelenin S, Roberg BA, Gundersen BB, Petersen P, Rinvik E, Torgner IA, Ottersen OP#TITLE:Brain mitochondria contain aquaporin water channels: evidence for the expression of a short AQP9 isoform in the inner mitochondrial membrane#JOURNAL:FASEB J#YEAR:2005#PUBMED:16126913# AUTHORS:Tsukaguchi H, Shayakul C, Berger UV, Mackenzie B, Devidas S, Guggino WB, van Hoek AN, Hediger MA#TITLE:Molecular characterization of a broad selectivity neutral solute channel#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9733774# AUTHORS:Agre P, King LS, Yasui M, Guggino WB, Ottersen OP, Fujiyoshi Y, Engel A, Nielsen S#TITLE:Aquaporin water channels--from atomic structure to clinical medicine#JOURNAL:J Physiol#YEAR:2002#PUBMED:12096044# GENE ASSOCIATION: (366.1)
L-lactate transport via diffusion (cytosol to mitochondria)
L-threonine/L-asparagine Na-dependent exchange (Asn-L in)
Abbreviation: R_ASNTHRNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[e] + na1[e] + thr-L[c] --> asn-L[c] + na1[c] + thr-L[e] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
Abbreviation: R_HSD3B12r Synonyms: _7 SUBSYSTEM: Steroid Metabolism Equation: [r] : dhea + nad --> andrstndn + h + nadh Confidence Level: 4 Microsomal rxn This is a conglomerated reaction - in actuality there is an intermediate: progen-5-ene-3,20,dione (C21H30O2) for pregnenolone 5-androstene-3,17-dione for dhea genes: LOCUS:_3284#TRANSCRIPT:1#ABBREVIATION:HSD3B2#ECNUMBERS:1.1.1.145,,5.3.# proteins: NAME:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 2#ABBREVIATION:Hsd3b2.1# citations: AUTHORS:Leenders F, Dolez V, Begue A, Moller G, Gloeckner JC, deLaunoit Y, Adamski J#TITLE:Structure of the gene for the human 17-beta-hydroxysteroid dehydrogenase type IV#JOURNAL:Mammalian Genome#YEAR:1998# AUTHORS:Adamski J, Leenders F Carstensen JF, Kaumann M, Markus MM, Husen B, Tesdorpf JG, Seedor U, deLaunoit Y, Jakob F#TITLE:Steroids, fatty acyl-CoA, and sterols are substrates of 80-kDa multifunctional protein#JOURNAL:Steroids#YEAR:1997# AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (3284.1)
Abbreviation: R_ARTFR51 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : lgnccoa --> (1.5) R5coa_hs Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CSNAT2x Synonyms: _0 EC Number: 2.3.1.7 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : crn + dmnoncoa <==> coa + dmnoncrn Confidence Level: 4 NJ genes: LOCUS:_54677#TRANSCRIPT:1#ABBREVIATION:CROT# LOCUS:_1384#TRANSCRIPT:2#ABBREVIATION:CRAT#ECNUMBERS:2.3.1.7# proteins: NAME:carnitine acetyltransferase, perixosomal#ABBREVIATION:Crat.2-x# NAME:carnitine O-octanoyltransferase#ABBREVIATION:Crot.1# citations: AUTHORS:Corti O, Finocchiaro G, Rossi E, Zuffardi O, DiDonato S.#TITLE:Molecular cloning of cDNAs encoding human carnitine acetyltransferase and mapping of the corresponding gene to chromosome 9q34.1.#JOURNAL:Genomics#YEAR:1994#PUBMED:7829107# AUTHORS:Wanders RJ.#TITLE:Peroxisomes, lipid metabolism, and peroxisomal disorders.#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15464416# GENE ASSOCIATION: (54677.1) or (1384.2)
carnitine dimethyl nonanoyl transferase, revsible, peroxisomal
Abbreviation: R_RDH2a Synonyms: _0 EC Number: 1.1.1.105 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : nadp + retinol-9-cis <==> h + nadph + retinal-cis-9 Confidence Level: 4 IT genes: LOCUS:_145226#TRANSCRIPT:1#ABBREVIATION:RDH12# LOCUS:_50700#TRANSCRIPT:1#ABBREVIATION:RDH8# LOCUS:_57665#TRANSCRIPT:1#ABBREVIATION:RDH14# LOCUS:_112724#TRANSCRIPT:1#ABBREVIATION:RDH13# LOCUS:_51109#TRANSCRIPT:1#ABBREVIATION:RDH11#ECNUMBERS:1.1.1.-# LOCUS:_195814#TRANSCRIPT:1#ABBREVIATION:RDHE2# proteins: NAME:retinol dehydrogenase 12 (all-trans and 9-cis) #ABBREVIATION:Rdh12.1# NAME:retinol dehydrogenase 14 (all-trans and 9-cis) #ABBREVIATION:Rdh14.1# NAME:retinol dehydrogenase 11 (all-trans and 9-cis) #ABBREVIATION:Rdh11.1# NAME:retinol dehydrogenase 13 (all-trans and 9-cis) #ABBREVIATION:Rdh13.1# NAME:retinol dehydrogenase 8 (all-trans) #ABBREVIATION:Rdh8.1# NAME:epidermal retinal dehydrogenase 2 #ABBREVIATION:Rdhe2.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# AUTHORS:Haeseleer F, Jang GF, Imanishi Y, Driessen CA, Matsumura M, Nelson PS, Palczewski K.#TITLE:Dual-substrate specificity short chain retinol dehydrogenases from the vertebrate retina.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12226107# AUTHORS:Janecke AR, Thompson DA, Utermann G, Becker C, Hubner CA, Schmid E, McHenry CL, Nair AR, Ruschendorf F, Heckenlively J, Wissinger B, Nurnberg P, Gal #TITLE:Mutations in RDH12 encoding a photoreceptor cell retinol dehydrogenase cause childhood-onset severe retinal dystrophy. #JOURNAL:Nat Genet. #YEAR:2004#PUBMED:15258582# AUTHORS:Perrault I, Hanein S, Gerber S, Barbet F, Ducroq D, Dollfus H, Hamel C, Dufier JL, Munnich A, Kaplan J, Rozet JM. #TITLE:Retinal dehydrogenase 12 (RDH12) mutations in leber congenital amaurosis. #JOURNAL:Am J Hum Genet#YEAR:2004#PUBMED:15322982# AUTHORS:Belyaeva OV, Kedishvili NY.#TITLE:Human pancreas protein 2 (PAN2) has a retinal reductase activity and is ubiquitously expressed in human tissues. #JOURNAL:FEBS Lett#YEAR:2002#PUBMED:12435598# AUTHORS:Matsuzaka Y, Okamoto K, Tsuji H, Mabuchi T, Ozawa A, Tamiya G, Inoko H.#TITLE:Identification of the hRDH-E2 gene, a novel member of the SDR family, and its increased expression in psoriatic lesion. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2002#PUBMED:12372410# GENE ASSOCIATION: (195814.1) or (112724.1) or (145226.1) or (51109.1) or (57665.1) or (50700.1)
Abbreviation: R_DM_kdn_LPAREN_c_RPAREN_ Synonyms: _0 Equation: [c] : kdn --> Confidence Level: NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DNDPt45m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[m] + dttp[c] --> adp[c] + dttp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_RTOTAL2CRNCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : Rtotal2crn + coa --> Rtotal2coa + crn Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# AUTHORS:Fraser F, Padovese R, Zammit VA.#TITLE:Distinct kinetics of carnitine palmitoyltransferase i in contact sites and outer membranes of rat liver mitochondria.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11274214# GENE ASSOCIATION: (1376.1)
Abbreviation: R_ST6GALNAC26 Synonyms: _5 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gt1alpha_hs --> cmp + gq1balpha_hs + h Confidence Level: 2 NJ genes: LOCUS:_10610#TRANSCRIPT:1#ABBREVIATION:SIAT7B#ECNUMBERS:2.4.99.7# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 2#ABBREVIATION:St6galnac2.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10610.1)
Abbreviation: R_HMGLm Synonyms: _0 EC Number: 4.1.3.4 SUBSYSTEM: Cholesterol Metabolism Equation: [m] : hmgcoa --> acac + accoa Confidence Level: 4 TV genes: LOCUS:_3155#TRANSCRIPT:1#ABBREVIATION:HMGCL#ECNUMBERS:4.1.3.4# LOCUS:_54511#TRANSCRIPT:1#ABBREVIATION:HMGCLL1# proteins: NAME:_3-hydroxylmethyl-3-methylglutaryl-Coenzyme A lyase#ABBREVIATION:Hmgcl.1# NAME:_3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase-like 1, mitochondrial #ABBREVIATION:Hmgcll1.1-m# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# GENE ASSOCIATION: (3155.1) or (54511.1)
Abbreviation: R_EX_trp_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : trp-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_OXAtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: oxa[x] --> oxa[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP.#TITLE:Glycolate and glyoxylate metabolism in HepG2 cells.#YEAR:2004#PUBMED:15240345# GENE ASSOCIATION:
Abbreviation: R_MI1346Ptn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: mi1346p[c] <==> mi1346p[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
_1D-myo-Inositol 1,3,4,6-tetrakisphosphate nuclear transport (diffusion)
Abbreviation: R_VITD3t2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: vitd3[e] --> vitd3[c] Confidence Level: 2 I am not sure how vitamin D3 is taken up from peripheral cells based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_CYTDn Synonyms: _0 EC Number: 3.5.4.5 SUBSYSTEM: Nucleotides Equation: [n] : cytd + h + h2o --> nh4 + uri Confidence Level: 2 genes: LOCUS:_57379#TRANSCRIPT:1#ABBREVIATION:AICDA# proteins: NAME:activation-induced cytidine deaminase #ABBREVIATION:Aicda.1-n# citations: AUTHORS:Brar SS, Watson M, Diaz M.#TITLE:Activation-induced cytosine deaminase (AID) is actively exported out of the nucleus but retained by the induction of DNA breaks.#JOURNAL:J Biol Chem.#YEAR:2004#PUBMED:15087440# GENE ASSOCIATION: (57379.1)
Abbreviation: R_G3PD2m Synonyms: _0 EC Number: 1.1.99.5 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: fad[m] + glyc3p[c] --> dhap[c] + fadh2[m] Confidence Level: 2 replaces old version which contains redundant metabolite (gly3p) NCD split compartmentation NJ genes: LOCUS:_2820#TRANSCRIPT:1#ABBREVIATION:GPD2#ECNUMBERS:1.1.1.8# proteins: NAME:glycerol 3-phosphate dehydrogenase#ABBREVIATION:Gpd2.1# citations: AUTHORS:Ferrer J, Aoki M, Behn P, Nestorowicz A, Riggs A, Permutt MA.#TITLE:Mitochondrial glycerol-3-phosphate dehydrogenase. Cloning of an alternatively spliced human islet-cell cDNA, tissue distribution, physical mapping, and identification of a polymorphic genetic marker.#JOURNAL:Diabetes#YEAR:1996#PUBMED:8549872# AUTHORS:Brisson D, Vohl MC, St-Pierre J, Hudson TJ, Gaudet D.#TITLE:Glycerol: a neglected variable in metabolic processes?#JOURNAL:Bioessays#YEAR:2001#PUBMED:11385633# GENE ASSOCIATION: (2820.1)
glycerol-3-phosphate dehydrogenase (FAD), mitochondrial
Abbreviation: R_A4GALTc Synonyms: _8 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : galgluside_hs + udpgal --> h + thcrm_hs + udp Confidence Level: 4 EC: 2.4.1.228 NJ genes: LOCUS:_53947#TRANSCRIPT:1#ABBREVIATION:A4GALT# proteins: NAME:alpha 1,4-galactosyltransferase#ABBREVIATION:A4galt.1# citations: AUTHORS:Kojima Y, Fukumoto S, Furukawa K, Okajima T, Wiels J, Yokoyama K, Suzuki Y, Urano T, Ohta M, Furukawa K.#TITLE:Molecular cloning of globotriaosylceramide/CD77 synthase, a glycosyltransferase that initiates the synthesis of globo series glycosphingolipids.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10748143# GENE ASSOCIATION: (53947.1)
Abbreviation: R_RTOTALCRNCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : Rtotalcoa + crn --> Rtotalcrn + coa Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (1375.1) or (1375.4) or (1375.3) or (1374.1) or (126129.1) or (1375.2)
Abbreviation: R_UGALGTg Synonyms: _0 EC Number: 2.4.1.22 SUBSYSTEM: Galactose metabolism Equation: [g] : glc-D + udpgal --> h + lcts + udp Confidence Level: 4 NCD genes: LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_3906#TRANSCRIPT:1#ABBREVIATION:LALBA# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# NAME:alpha-lactalbumin, Golgi apparatus (lactating mammary gland)#ABBREVIATION:Lalba.1-g# citations: AUTHORS:Amado M, Almeida R, Carneiro F, Levery ST, Holmes EH, Nomoto M, Hollingsworth MA, Hassan H, Schwientek T, Nielsen PA, Bennett EP, Clausen H#TITLE:A family of human beta3-galactosyltransferases#JOURNAL:The Journal of Biological Chemistry#YEAR:1998# GENE ASSOCIATION: (3906.1) and (2683.1) or (8704.2) or (8704.1)
UDPgalactose:D-glucose 4-beta-D-galactosyltransferase, Golgi apparatus
phosphatidylinositol-5-phosphate 3-kinase, endoplasmic reticulum
Abbreviation: R_PI5P3Ker Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [r] : atp + pail5p_hs --> adp + h + pail35p_hs Confidence Level: 1 NCD genes: LOCUS:_5287#TRANSCRIPT:1#ABBREVIATION:PIK3C2B#ECNUMBERS:2.7.1.137# proteins: NAME:phosphoinositide-3-kinase, class 2, beta polypeptide#ABBREVIATION:Pik3c2b.1-er# citations: AUTHORS:Tolias KF, Cantley LC#TITLE:Pathways for phosphoinositide synthesis#JOURNAL:Chem Phys Lipids#YEAR:1999#PUBMED:10358929# GENE ASSOCIATION: (5287.1)
Abbreviation: R_PMANM Synonyms: _0 EC Number: 5.4.2.8 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : man1p <==> man6p Confidence Level: 0 genes: LOCUS:_55276#TRANSCRIPT:1#ABBREVIATION:PGM2#ECNUMBERS:5.4.2.2# LOCUS:_5373#TRANSCRIPT:1#ABBREVIATION:PMM2# LOCUS:_5372#TRANSCRIPT:1#ABBREVIATION:PMM1# proteins: NAME:phosphomannomutase 1#ABBREVIATION:Pmm1.1# NAME:phosphomannomutase 2#ABBREVIATION:Pmm2.1# NAME:phosphoglucomutase 2#ABBREVIATION:Pgm2# citations: none GENE ASSOCIATION: (5372.1) or (55276.1) or (5373.1)
Abbreviation: R_GLYCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: glyc[c] <==> glyc[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# AUTHORS:Kuriyama H, Kawamoto S, Ishida N, Ohno I, Mita S, Matsuzawa Y, Matsubara K, Okubo K#TITLE:Molecular cloning and expression of a novel human aquaporin from adipose tissue with glycerol permeability#JOURNAL:Biochem Biophys Res Commun#YEAR:1997#PUBMED:9405233# GENE ASSOCIATION:
Abbreviation: R_NNAT Synonyms: _0 EC Number: 2.7.7.18 SUBSYSTEM: NAD Metabolism Equation: [c] : atp + h + nicrnt --> dnad + ppi Confidence Level: 4 genes: LOCUS:_5167#TRANSCRIPT:1#ABBREVIATION:ENPP1#ECNUMBERS:3.1.4.1,3.6.1.9# LOCUS:_5168#TRANSCRIPT:1#ABBREVIATION:ENPP2#ECNUMBERS:3.1.4.1,3.6.1.9# LOCUS:_5169#TRANSCRIPT:1#ABBREVIATION:ENPP3#ECNUMBERS:3.1.4.1,3.6.1.9# proteins: NAME:ectonucleotide pyrophosphatase/phosphodiesterase 1 #ABBREVIATION:EnpP1.1# NAME:ectonucleotide pyrophosphatase/phosphodiesterase 2 (autotaxin)#ABBREVIATION:Enpp2.1# NAME:ectonucleotide pyrophosphatase/phosphodiesterase 3#ABBREVIATION:Enpp3.1# citations: AUTHORS:Zhou T, Kurnasov O, Tomchick DR, Binns DD, Grishin NV, Marquez VE, Osterman AL, Zhang H.#TITLE:Structure of human nicotinamide/nicotinic acid mononucleotide adenylyltransferase. Basis for the dual substrate specificity and activation of the oncolytic agent tiazofurin#JOURNAL: J Biol Chem#YEAR:2002# AUTHORS:Yalowitz JA, Xiao S, Biju MP, Antony AC, Cummings OW, Deeg MA, Jayaram HN.#TITLE:Characterization of human brain nicotinamide 5'-mononucleotide adenylyltransferase-2 and expression in human pancreas. #JOURNAL:Biochem J.#YEAR:2004#PUBMED:14516279# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION: (5167.1) or (5169.1) or (5168.1)
Abbreviation: R_FE2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fe2[e] --> fe2[c] Confidence Level: 4 genes: none proteins: none citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Goswami T, Rolfs A, Hediger MA#TITLE:Iron transport: emerging roles in health and disease#JOURNAL:Biochem Cell Biol#YEAR:2002#PUBMED:12440707# GENE ASSOCIATION:
Abbreviation: R_MMCD Synonyms: _3 EC Number: 4.1.1.41 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [c] : h + mmcoa-S --> co2 + ppcoa Confidence Level: 1 JLR genes: LOCUS:_23417#TRANSCRIPT:1#ABBREVIATION:MLYCD#ECNUMBERS:4.1.1.9# proteins: NAME:malonyl-CoA decarboxylase#ABBREVIATION:Mlycd.1# citations: AUTHORS:Kerner J, Hoppel CL.#TITLE:Radiochemical malonyl-CoA decarboxylase assay: activity and subcellular distribution in heart and skeletal muscle.#JOURNAL:Anal Biochem#YEAR:2002#PUBMED:12123667# AUTHORS:Wightman PJ, Santer R, Ribes A, Dougherty F, McGill N, Thorburn DR, FitzPatrick DR#TITLE:MLYCD mutation analysis: evidence for protein mistargeting as a cause of MLYCD deficiency#JOURNAL:Hum Mutat#YEAR:2003#PUBMED:12955715# GENE ASSOCIATION: (23417.1)
Abbreviation: R_MMCDm Synonyms: _3 EC Number: 4.1.1.41 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : h + mmcoa-S --> co2 + ppcoa Confidence Level: 1 JLR mitochondrial isoform - NCD genes: LOCUS:_23417#TRANSCRIPT:1#ABBREVIATION:MLYCD#ECNUMBERS:4.1.1.9# proteins: NAME:malonyl-CoA decarboxylase#ABBREVIATION:Mlycd.1-m# citations: AUTHORS:Kerner J, Hoppel CL.#TITLE:Radiochemical malonyl-CoA decarboxylase assay: activity and subcellular distribution in heart and skeletal muscle.#JOURNAL:Anal Biochem#YEAR:2002#PUBMED:12123667# AUTHORS:Wightman PJ, Santer R, Ribes A, Dougherty F, McGill N, Thorburn DR, FitzPatrick DR#TITLE:MLYCD mutation analysis: evidence for protein mistargeting as a cause of MLYCD deficiency#JOURNAL:Hum Mutat#YEAR:2003#PUBMED:12955715# GENE ASSOCIATION: (23417.1)
Methylmalonyl-CoA decarboxylase, mitochondrial
Abbreviation: R_TMDPPK Synonyms: _0 EC Number: 2.7.4.15 SUBSYSTEM: Thiamine Metabolism Equation: [c] : atp + thmpp --> adp + thmtp Confidence Level: 2 also EC 2.7.6.2 - NCD genes: none proteins: none citations: AUTHORS:Bettendorff L, Mastrogiacomo F, Kish SJ, Grisar T.#TITLE:Thiamine, thiamine phosphates, and their metabolizing enzymes in human brain. #JOURNAL:J Neurochem. #YEAR:1996#PUBMED:8522961# GENE ASSOCIATION:
Abbreviation: R_VALTAm Synonyms: _0 EC Number: 2.6.1.42 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : akg + val-L <==> 3mob + glu-L Confidence Level: 4 NCD genes: LOCUS:_587#TRANSCRIPT:1#ABBREVIATION:BCAT2#ECNUMBERS:2.6.1.26# proteins: NAME:branched chain aminotransferase 2, mitochondrial #ABBREVIATION:Bcat2.1-m# citations: AUTHORS:Bledsoe RK, Dawson PA, Hutson SM.#TITLE:Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm). #YEAR:1997#PUBMED:9165094# AUTHORS:Naylor SL, Shows TB.#TITLE:Branched-chain aminotransferase deficiency in Chinese hamster cells complemented by two independent genes on human chromosomes 12 and 19. #YEAR:1980#PUBMED:6933702# GENE ASSOCIATION: (587.1)
Abbreviation: R_D3AIBTm Synonyms: _0 EC Number: 2.6.1.40 SUBSYSTEM: Pyrimidine Catabolism Equation: [m] : 3aib-D + pyr <==> 2mop + ala-L Confidence Level: 4 MM genes: none proteins: none citations: AUTHORS:Kakimoto Y, Taniguchi K, Sano I.#TITLE:D-beta-aminoisobutyrate:pyruvate aminotransferase in mammalian liver and excretion of beta-aminoisobutyrate by man.#YEAR:1969#PUBMED:5773299# GENE ASSOCIATION:
D-3-Amino-isobutanoate:pyruvate aminotransferase, mitochondrial
Abbreviation: R_OAGD3tg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: oagd3_hs[c] <==> oagd3_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_EX_cspg_b_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cspg_b <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate B / dermatan sulfate (IdoA2S-GalNAc4S) proteoglycan exchange
Abbreviation: R_FBA2 Synonyms: _0 EC Number: 4.1.2.13 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : f1p <==> dhap + glyald Confidence Level: 2 NCD genes: LOCUS:_226#TRANSCRIPT:1#ABBREVIATION:ALDOA#ECNUMBERS:4.1.2.13# LOCUS:_229#TRANSCRIPT:1#ABBREVIATION:ALDOB#ECNUMBERS:4.1.2.13# LOCUS:_230#TRANSCRIPT:1#ABBREVIATION:ALDOC#ECNUMBERS:4.1.2.13# LOCUS:_226#TRANSCRIPT:2#ABBREVIATION:ALDOA#ECNUMBERS:4.1.2.13# LOCUS:_226#TRANSCRIPT:3#ABBREVIATION:ALDOA#ECNUMBERS:4.1.2.13# proteins: NAME:aldolase A, fructose-bisphosphate (muscle)#ABBREVIATION:Aldoa.1# NAME:aldolase A, fructose-bisphosphate (muscle)#ABBREVIATION:Aldoa.2# NAME:aldolase A, fructose-bisphosphate (muscle)#ABBREVIATION:Aldoa.3# NAME:aldolase B, fructose-bisphosphate (liver)#ABBREVIATION:Aldob.1# NAME:aldolase C, fructose-bisphosphate (brain)#ABBREVIATION:Aldoc.1# citations: none GENE ASSOCIATION: (226.2) or (226.1) or (226.3) or (229.1) or (230.1)
Abbreviation: R_EX_ncam_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ncam <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
cytochrome P450, family 46, subfamily A, polypeptide 1
Abbreviation: R_P45046A1r Synonyms: _4 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : chsterol + h + nadph + o2 --> h2o + nadp + xol24oh Confidence Level: 4 genes: LOCUS:_10858#TRANSCRIPT:1#ABBREVIATION:CYP46A1# proteins: NAME:cytochrome P450, family 46, subfamily A, polypeptide 1#ABBREVIATION:Cyp46a1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (10858.1)
Abbreviation: R_APOCF Synonyms: _0 SUBSYSTEM: Biotin Metabolism Equation: [c] : apoC + lys-L --> apoC-Lys + h + h2o Confidence Level: 2 IT faked reaction to balance reaction in human biotin metabolism genes: none proteins: none citations: AUTHORS:Monti E, Preti A, Rossi E, Ballabio A, Borsani G.#TITLE:Cloning and characterization of NEU2, a human gene homologous to rodent soluble sialidases.#JOURNAL:Genomics#YEAR:1999#PUBMED:10191093# AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# GENE ASSOCIATION:
Abbreviation: R_APAT2rm Synonyms: _0 EC Number: 2.6.1.19 SUBSYSTEM: beta-Alanine metabolism Equation: [m] : akg + ala-B <==> glu-L + msa Confidence Level: 1 SAB genes: LOCUS:_18#TRANSCRIPT:1#ABBREVIATION:ABAT#ECNUMBERS:2.6.19.4# LOCUS:_18#TRANSCRIPT:2#ABBREVIATION:ABAT#ECNUMBERS:2.6.19.4# proteins: NAME:_4-aminobutyrate aminotransferase, mitochondrial#ABBREVIATION:Abat.1-m# NAME:_4-aminobutyrate aminotransferase, mitochondrial#ABBREVIATION:Abat.2-m# citations: AUTHORS:Ohyama T, Matsuda K, Tachibana H, Fujimoto Sakata S, Mori M, Horiuchi M, Tamaki N#TITLE:Purification and expression of a processing protease on beta-alanine-oxoglutarate aminotransferase from rat liver mitochondria#JOURNAL:FEBS Lett#YEAR:2004#PUBMED:15304357# GENE ASSOCIATION: (18.1) or (18.2)
_3-Aminopropanoate:2-oxoglutarate aminotransferase (m)
Abbreviation: R_NOt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: no[e] <==> no[c] Confidence Level: 1 IT (06-16-04) genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GCHOLAt3 Synonyms: _10 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + gchola[c] + h2o[c] --> adp[c] + gchola[e] + h[c] + pi[c] Confidence Level: 4 for bile acid secretion into bile NJ genes: LOCUS:_8647#TRANSCRIPT:1#ABBREVIATION:ABCB11# LOCUS:_8714#TRANSCRIPT:1#ABBREVIATION:ABCC3# LOCUS:_8714#TRANSCRIPT:2#ABBREVIATION:ABCC3# LOCUS:_8714#TRANSCRIPT:3#ABBREVIATION:ABCC3# proteins: NAME:ABC, sub-family B (MDR/TAP), member 11#ABBREVIATION:Abcb11.1# NAME:ABC, sub-family C (CFTR/MRP), member 3#ABBREVIATION:Abcc3.1# NAME:ABC, sub-family C, (CFTR/MRP), member 3#ABBREVIATION:Abcc3.2# NAME:ABC, sub-family C, (CFTR/MRP), member 3#ABBREVIATION:Abcc3.3# citations: AUTHORS:Strautnieks SS, Bull LN, Knisely AS, Kocoshis SA, Dahl N, Arnell H, Sokal E, Dahan K, Childs S, Ling V, Tanner MS, Kagalwalla AF, Nemeth A, Pawlowska J, Baker A, Mieli-Vergani G, Freimer NB, Gardiner RM, Thompson RJ.#TITLE:A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis.#JOURNAL:Nat Genet.#YEAR:1998#PUBMED:9806540# AUTHORS:Kiuchi Y, Suzuki H, Hirohashi T, Tyson CA, Sugiyama Y.#TITLE:cDNA cloning and inducible expression of human multidrug resistance associated protein 3 (MRP3).#JOURNAL:FEBS Lett.#YEAR:1998#PUBMED:9738950# AUTHORS:Uchiumi T, Hinoshita E, Haga S, Nakamura T, Tanaka T, Toh S, Furukawa M, Kawabe T, Wada M, Kagotani K, Okumura K, Kohno K, Akiyama S, Kuwano M.#TITLE:Isolation of a novel human canalicular multispecific organic anion transporter, cMOAT2/MRP3, and its expression in cisplatin-resistant cancer cells with decreased ATP-dependent drug transport.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1998#PUBMED:9813153# GENE ASSOCIATION: (8714.3) or (8647.1) or (8714.2) or (8714.1)
Abbreviation: R_GLNASNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[c] + gln-L[e] + na1[e] --> asn-L[e] + gln-L[c] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-glutamine/L-asparagine Na-dependent exchange (Gln-L in)
Abbreviation: R_EX_gbside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gbside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_THYMDtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: thymd[l] <==> thymd[c] Confidence Level: 4 IT genes: LOCUS:_55315#TRANSCRIPT:1#ABBREVIATION:SLC29A3# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 3 #ABBREVIATION:Slc29a3.1-l# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Pisoni RL, Thoene JG.#TITLE:Detection and characterization of a nucleoside transport system in human fibroblast lysosomes.#JOURNAL:J Biol Chem.#YEAR:1989#PUBMED:2925670# AUTHORS:Baldwin SA, Yao SY, Hyde RJ, Ng AM, Foppolo S, Barnes K, Ritzel MW, Cass CE, Young JD.#TITLE:Functional characterization of novel human and mouse equilibrative nucleoside transporters (hENT3 and mENT3) located in intracellular membranes.#JOURNAL:J Biol Chem. #YEAR:2005#PUBMED:15701636# GENE ASSOCIATION: (55315.1)
thymidine facilated transport from lysosome
Abbreviation: R_EX_hexc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hexc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PTE3x Synonyms: _1 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : h2o + pristcoa --> coa + h + prist Confidence Level: 4 NJ genes: LOCUS:_10965#TRANSCRIPT:1#ABBREVIATION:ZAP128# proteins: NAME:peroxisomal long-chain acyl-CoA thioesterase#ABBREVIATION:Zap128.1# citations: AUTHORS:Jones JM, Gould SJ.#TITLE:Identification of PTE2, a human peroxisomal long-chain acyl-CoA thioesterase.#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10944470# GENE ASSOCIATION: (10965.1)
Abbreviation: R_THRASNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[c] + na1[e] + thr-L[e] --> asn-L[e] + na1[c] + thr-L[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-threonine/L-asparagine Na-dependent exchange (Thr-L in)
Glycine secretion via secretory vesicle (ATP driven)
Abbreviation: R_GLYVESSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + (3) gly[c] + h2o[c] --> adp[c] + (3) gly[e] + h[c] + pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of glu from the cell. It leaves out the vesicle because a compartment is not available for that. ATP demand is not known for sure stoichiometrically, but 1:1 H coupling ratio is assumed, which could translate to 3 gly/ATP. genes: LOCUS:_140679#TRANSCRIPT:1#ABBREVIATION:SLC32A1# proteins: NAME:solute carrier family 32 (GABA vesicular transporter), member 1#ABBREVIATION:Slc32a1.1# citations: AUTHORS:Gasnier B#TITLE:The SLC32 transporter, a key protein for the synaptic release of inhibitory amino acids#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750892# AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# GENE ASSOCIATION: (140679.1)
Abbreviation: R_7THFtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _7thf[m] --> 7thf[c] Confidence Level: 2 unknown mechanism. IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_7-glutamyl-THF transport,m mitochondrial
Abbreviation: R_PI4P3Ker Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [r] : atp + pail4p_hs --> adp + h + pail34p_hs Confidence Level: 4 NCD Notice that contrary to some standards in texts, KEGG, etc, the Rtotal group accounts for the whole fatty acyl CoA (so the CHO only accounts for the core backbone), manner in which Rtotal was produced accounts for O and H balances. Formation of the Rtotal group (rxn flux)accounted for mass balance for CoA NJ changed EC from 2.7.1.149 (pail5p) to 2.7.1.68 (pail4p) - NCD genes: LOCUS:_5287#TRANSCRIPT:1#ABBREVIATION:PIK3C2B#ECNUMBERS:2.7.1.137# proteins: NAME:phosphoinositide-3-kinase, class 2, beta polypeptide#ABBREVIATION:Pik3c2b.1-er# citations: AUTHORS:Arcaro A, Volinia S, Zvelebil MJ, Stein R, Watton SJ, Layton MJ, Gout I, Ahmadi K, Downward J, Waterfield MD#TITLE:Human phosphoinositide 3-kinase C2beta, the role of calcium and the C2 domain in enzyme activity#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9830063# GENE ASSOCIATION: (5287.1)
phosphatidylinositol 4-phosphate 3-kinase, endoplasmic reticulum
Abbreviation: R_EX_phe_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : phe-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FADDP Synonyms: _0 EC Number: 3.6.1.9 SUBSYSTEM: Riboflavin Metabolism Equation: [c] : fad + h2o --> amp + fmn + (2) h Confidence Level: 2 genes: LOCUS:_5167#TRANSCRIPT:1#ABBREVIATION:ENPP1#ECNUMBERS:3.1.4.1,3.6.1.9# proteins: NAME:ectonucleotide pyrophosphatase/phosphodiesterase 1 #ABBREVIATION:EnpP1.1# citations: AUTHORS:Funakoshi I, Kato H, Horie K, Yano T, Hori Y, Kobayashi H, Inoue T, Suzuki H, Fukui S, Tsukahara M, et al.#TITLE:Molecular cloning of cDNAs for human fibroblast nucleotide pyrophosphatase.#JOURNAL:Arch Biochem Biophys.#YEAR:1992#PUBMED:1315502# GENE ASSOCIATION: (5167.1)
Abbreviation: R_CRTSTRNtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: crtstrn[c] <==> crtstrn[m] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NACHEXA15ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg24 --> acgam + h + ksi_deg26 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_NDPK1 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + gdp <==> adp + gtp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.1) or (29922.2) or (4831.1 and 4830.1) or (4832.1) or (4831.1 and 4830.2) or (10201.1)
Abbreviation: R_FAH2 Synonyms: _1 SUBSYSTEM: CYP Metabolism Equation: [c] : h + nadph + o2 + ttdca --> h2o + nadp + whttdca Confidence Level: 4 fatty acid omega hydroxylation NJ genes: LOCUS:_1579#TRANSCRIPT:1#ABBREVIATION:CYP4A11# proteins: NAME:cytochrome P450, family 4, subfamily A, polypeptide#ABBREVIATION:Cyp4a11.1# citations: AUTHORS:Rendic S.#TITLE:Summary of information on human CYP enzymes: human P450 metabolism data.#JOURNAL:Drug Metab Rev#YEAR:2002#PUBMED:11996015# AUTHORS:Palmer CN, Richardson TH, Griffin KJ, Hsu MH, Muerhoff AS, Clark JE, Johnson EF.#TITLE:Characterization of a cDNA encoding a human kidney, cytochrome P-450 4A fatty acid omega-hydroxylase and the cognate enzyme expressed in Escherichia coli.#JOURNAL:Biochim Biophys Acta#YEAR:1993#PUBMED:7679927# AUTHORS:Imaoka S, Ogawa H, Kimura S, Gonzalez FJ.#TITLE:Complete cDNA sequence and cDNA-directed expression of CYP4A11, a fatty acid omega-hydroxylase expressed in human kidney.#JOURNAL:DNA Cell Biol#YEAR:1993#PUBMED:8274222# GENE ASSOCIATION: (1579.1)
Abbreviation: R_CYSO Synonyms: _1 EC Number: 1.13.11.20 SUBSYSTEM: Taurine and hypotaurine metabolism Equation: [c] : cys-L + o2 --> 3sala + (2) h Confidence Level: 4 IT genes: LOCUS:_1036#TRANSCRIPT:1#ABBREVIATION:CDO1# proteins: NAME:cysteine dioxygenase, type I#ABBREVIATION:Cdo1.1# citations: AUTHORS:Millard J, Parsons RB, Waring RH, Williams AC, Ramsden DB#TITLE:Expression of cysteine dioxygenase (EC 1.13.11.20) and sulfite oxidase in the human lung: a potential role for sulfate production in the protection from airborne xenobiotica#JOURNAL:Mol Pathol#YEAR:2003#PUBMED:14514920# GENE ASSOCIATION: (1036.1)
Abbreviation: R_PE_HStg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: pe_hs[c] <==> pe_hs[g] Confidence Level: 2 NCD for assumed reversible, non-energy dep scramblase mechanism (PMID: 10856717). NJ genes: none proteins: none citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# GENE ASSOCIATION:
Abbreviation: R_EX_ca2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ca2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ABTD Synonyms: _2 EC Number: 1.1.1.12 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : abt + nad <==> h + nadh + xylu-L Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Onkenhout W, Groener JE, Verhoeven NM, Yin C, Laan LA#TITLE:L-Arabinosuria: a new defect in human pentose metabolism#JOURNAL:Mol Genet Metab#YEAR:2002#PUBMED:12359133# AUTHORS:Huck JH, Roos B, Jakobs C, van der Knaap MS, Verhoeven NM#TITLE:Evaluation of pentitol metabolism in mammalian tissues provides new insight into disorders of human sugar metabolism#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15234337# AUTHORS:Touster O, Shaw DR#TITLE:Biochemistry of the acyclic polyols#JOURNAL:Physiol Rev.#YEAR:1962#PUBMED:13922173# GENE ASSOCIATION:
Abbreviation: R_TMDS Synonyms: _4 EC Number: 2.1.1.45 SUBSYSTEM: Nucleotides Equation: [c] : dump + mlthf --> dhf + dtmp Confidence Level: 4 genes: LOCUS:_7298#TRANSCRIPT:1#ABBREVIATION:TYMS#ECNUMBERS:2.1.1.45# proteins: NAME:thymidylate synthetase #ABBREVIATION:Tyms.1# citations: AUTHORS:Forsthoefel AM, Pena MM, Xing YY, Rafique Z, Berger FG.#TITLE:Structural determinants for the intracellular degradation of human thymidylate synthase.#JOURNAL:Biochemistry#YEAR:2004#PUBMED:14967037# AUTHORS:Kaneda S, Nalbantoglu J, Takeishi K, Shimizu K, Gotoh O, Seno T, Ayusawa D.#TITLE:Structural and functional analysis of the human thymidylate synthase gene.#JOURNAL:J Biol Chem.#YEAR:1990#PUBMED:2243092# GENE ASSOCIATION: (7298.1)
Abbreviation: R_UDPXYLtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: udpxyl[c] + ump[g] <==> udpxyl[g] + ump[c] Confidence Level: 4 NCD genes: LOCUS:_84912#TRANSCRIPT:1#ABBREVIATION:SLC35B4# proteins: NAME:solute carrier family 35, member B4, Golgi apparatus#ABBREVIATION:Slc35b4.1-g# citations: AUTHORS:Ashikov A, Routier F, Fuhlrott J, Helmus Y, Wild M, Gerardy-Schahn R, Bakker H#TITLE:The Human Solute Carrier Gene SLC35B4 Encodes a Bifunctional Nucleotide Sugar Transporter with Specificity for UDP-Xylose and UDP-N-Acetylglucosamine#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15911612# AUTHORS:Nuwayhid N, Glaser JH, Johnson JC, Conrad HE, Hauser SC, Hirschberg CB#TITLE:Xylosylation and glucuronosylation reactions in rat liver Golgi apparatus and endoplasmic reticulum#JOURNAL:J Biol Chem#YEAR:1986#PUBMED:3093474# AUTHORS:Milla ME, Clairmont CA, Hirschberg CB#TITLE:Reconstitution into proteoliposomes and partial purification of the Golgi apparatus membrane UDP-galactose, UDP-xylose, and UDP-glucuronic acid transport activities#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1730575# GENE ASSOCIATION: (84912.1)
UDP-Xyl Golgi transport via CMP antiport
_2-oxoisovalerate dehydrogenase (acylating; 4-methyl-2-oxopentaoate), mitochondrial
Abbreviation: R_OIVD1m Synonyms: _0 EC Number: 1.2.1.25 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 4mop + coa + nad --> co2 + ivcoa + nadh Confidence Level: 4 genes: LOCUS:_593#TRANSCRIPT:1#ABBREVIATION:BCKDHA# LOCUS:_1629#TRANSCRIPT:1#ABBREVIATION:DBT#ECNUMBERS:2.3.1.-# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_594#TRANSCRIPT:1#ABBREVIATION:BCKDHB# LOCUS:_594#TRANSCRIPT:2#ABBREVIATION:BCKDHB# proteins: NAME:dihydrolipoamide dehydrogenase, mitochondrial#ABBREVIATION:Dld.1-m# NAME:branched chain keto acid dehydrogenase E1, mitochondrial#ABBREVIATION:Bckdhab1-m# NAME:dihydrolipoamide branched chain transacylase (E2 component of branched chain keto acid dehydrogenase complex#ABBREVIATION:Dbt.1-m# NAME:branched chain keto acid dehydrogenase E1#ABBREVIATION:Bckdhab2-m# citations: AUTHORS:Lau KS, Herring WJ, Chuang JL, McKean M, Danner DJ, Cox RP, Chuang DT.#TITLE:Structure of the gene encoding dihydrolipoyl transacylase (E2) component of human branched chain alpha-keto acid dehydrogenase complex and characterization of an E2 pseudogene. #YEAR:1992#PUBMED:1429740# AUTHORS:Fisher CW, Chuang JL, Griffin TA, Lau KS, Cox RP, Chuang DT. #TITLE:Molecular phenotypes in cultured maple syrup urine disease cells. Complete E1 alpha cDNA sequence and mRNA and subunit contents of the human branched chain alpha-keto acid dehydrogenase complex. #YEAR:1989#PUBMED:2914958# GENE ASSOCIATION: (1629.1) and (594.1 and 593.1) and (1738.1) or (1629.1) and (1738.1) and (594.2 and 593.1)
Abbreviation: R_NTD4 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : cmp + h2o --> cytd + pi Confidence Level: 2 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_51251#TRANSCRIPT:1#ABBREVIATION:NT5C3#ECNUMBERS:3.1.3.5# proteins: NAME:_5'-nucleotidase, cytosolic III #ABBREVIATION:Nt5C3.1# citations: AUTHORS:Amici A, Magni G. #TITLE:Human erythrocyte pyrimidine 5'-nucleotidase, PN-I.#JOURNAL:Arch Biochem Biophys#YEAR:2002#PUBMED:11795870# GENE ASSOCIATION: (51251.1)
Abbreviation: R_ADSL1 Synonyms: _0 EC Number: 4.3.2.2 SUBSYSTEM: Nucleotides Equation: [c] : dcamp --> amp + fum Confidence Level: 4 genes: LOCUS:_158#TRANSCRIPT:1#ABBREVIATION:ADSL#ECNUMBERS:4.3.2.2# LOCUS:_158#TRANSCRIPT:2#ABBREVIATION:ADSL# proteins: NAME:adenylosuccinate lyase#ABBREVIATION:Adsl.1# NAME:adenylosuccinate lyase#ABBREVIATION:Adsl.2# citations: AUTHORS:Barton JW, Hart IM, Patterson D.#TITLE:Mapping of a locus correcting lack of phosphoribosylaminoimidazole carboxylase activity in Chinese hamster ovary cell Ade-D mutants to human chromosome 4. #JOURNAL:Genomics#YEAR:1991#PUBMED:2004782# AUTHORS:Stone RL, Aimi J, Barshop BA, Jaeken J, Van den Berghe G, Zalkin H, Dixon JE.#TITLE:A mutation in adenylosuccinate lyase associated with mental retardation and autistic features.#JOURNAL:Nat Genet. #YEAR:1992#PUBMED:1302001# GENE ASSOCIATION: (158.1) or (158.2)
GlcNAc-Gal globoside (homo sapiens)_C58H102N3O32FULLRCO
Abbreviation: R_B3GNT35g Synonyms: _9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : acglcgalgbside_hs + udpgal --> galacglcgalgbside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10331.1)
Abbreviation: R_KSII_CORE4tly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: ksii_core4[e] --> ksii_core4[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
keratan sulfate II (core 4) transport, extracellular to lysosome
Abbreviation: R_ARTFR203 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (2) fadh2[m] + h[m] + lnlncgcoa[c] + nadph[m] --> (1.125) R2coa_hs[c] + (2) fad[m] + nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_OCCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: occoa[c] <==> occoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_eicostet_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : eicostet <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_camp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : camp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_hpdca_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hpdca <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_gdp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gdp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_tolbutamide_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tolbutamide <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PHEt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + phe-L[e] --> na1[c] + phe-L[c] Confidence Level: 4 SAB genes: LOCUS:_84889#TRANSCRIPT:1#ABBREVIATION:SLC7A3# LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_6542#TRANSCRIPT:1#ABBREVIATION:SLC7A2# LOCUS:_6541#TRANSCRIPT:1#ABBREVIATION:SLC7A1# LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 1#ABBREVIATION:Slc7a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 2#ABBREVIATION:Slc7a2.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 3#ABBREVIATION:Slc7a3.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# AUTHORS:Albritton LM, Bowcock AM, Eddy RL, Morton CC, Tseng L, Farrer LA, Cavalli-Sforza LL, Shows TB, Cunningham JM#TITLE:The human cationic amino acid transporter (ATRC1): physical and genetic mapping to 13q12-q14#JOURNAL:Genomics#YEAR:1992#PUBMED:1348489# AUTHORS:Cariappa R, Heath-Monnig E, Furesz TC, Kamath SG, Smith CH#TITLE:Stable polarized expression of hCAT-1 in an epithelial cell line#JOURNAL:J Membr Biol#YEAR:2002#PUBMED:11891586# AUTHORS:Hoshide R, Ikeda Y, Karashima S, Matsuura T, Komaki S, Kishino T, Niikawa N, Endo F, Matsuda I#TITLE:Molecular cloning, tissue distribution, and chromosomal localization of human cationic amino acid transporter 2 (HCAT2)#JOURNAL:Genomics#YEAR:1996#PUBMED:8954799# AUTHORS:Furesz TC, Moe AJ, Smith CH#TITLE:Lysine uptake by human placental microvillous membrane: comparison of system y+ with basal membrane#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7534987# AUTHORS:Wolf S, Janzen A, Vekony N, Martine U, Strand D, Closs EI#TITLE:Expression of solute carrier 7A4 (SLC7A4) in the plasma membrane is not sufficient to mediate amino acid transport activity#JOURNAL:Biochem J#YEAR:2002#PUBMED:12049641# AUTHORS:Vekony N, Wolf S, Boissel JP, Gnauert K, Closs EI#TITLE:Human cationic amino acid transporter hCAT-3 is preferentially expressed in peripheral tissues#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11591158# AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (6542.1) or (81539.1) or (11254.1) or (54407.1) or (84889.1) or (55089.1) or (6541.1)
L-phenylalanine transport in via sodium symport
Abbreviation: R_EX_acgam_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acgam <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MANt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: man[e] + na1[e] <==> man[c] + na1[c] Confidence Level: 4 NCD genes: LOCUS:_200010#TRANSCRIPT:1#ABBREVIATION:SLC5A9# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 9, extracellular#ABBREVIATION:Slc5a9.1-e# citations: AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# AUTHORS:Tazawa S, Yamato T, Fujikura H, Hiratochi M, Itoh F, Tomae M, Takemura Y, Maruyama H, Sugiyama T, Wakamatsu A, Isogai T, Isaji M#TITLE:SLC5A9/SGLT4, a new Na+-dependent glucose transporter, is an essential transporter for mannose, 1,5-anhydro-D-glucitol, and fructose#JOURNAL:Life Sci#YEAR:2005#PUBMED:15607332# GENE ASSOCIATION: (200010.1)
D-mannose transport via sodium cotransport
Abbreviation: R_ST3GAL21g Synonyms: _7 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + ga1_hs --> cmp + gm1b_hs + h Confidence Level: 4 NJ genes: LOCUS:_6483#TRANSCRIPT:1#ABBREVIATION:SIAT4B#ECNUMBERS:2.4.99.4# proteins: NAME:sialyltransferase 4B, Golgi apparatus#ABBREVIATION:Siat4b.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Giordanengo V, Bannwarth S, Laffont C, Van Miegem V, Harduin-Lepers A, Delannoy P, Lefebvre JC. #TITLE:Cloning and expression of cDNA for a human Gal(beta1-3)GalNAc alpha2,3-sialyltransferase from the CEM T-cell line. #JOURNAL:Eur J Biochem#YEAR:1997#PUBMED:9266697# GENE ASSOCIATION: (6483.1)
Abbreviation: R_G3PD1 Synonyms: _0 EC Number: 1.1.1.94 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : glyc3p + nad <==> dhap + h + nadh Confidence Level: 4 NAD-specific; see also G3PD2 genes: LOCUS:_2819#TRANSCRIPT:1#ABBREVIATION:GPD1#ECNUMBERS:1.1.1.8# proteins: NAME:glycerol 3-phosphate dehydrogenase#ABBREVIATION:Gpd1.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Menaya J, Gonzalez-Manchon C, Parrilla R, Ayuso MS.#TITLE:Molecular cloning, sequencing and expression of a cDNA encoding a human liver NAD-dependent alpha-glycerol-3-phosphate dehydrogenase.#JOURNAL:Biochim Biophys Acta#YEAR:1995#PUBMED:7772607# AUTHORS:Brisson D, Vohl MC, St-Pierre J, Hudson TJ, Gaudet D.#TITLE:Glycerol: a neglected variable in metabolic processes?#JOURNAL:Bioessays#YEAR:2001#PUBMED:11385633# GENE ASSOCIATION: (2819.1)
glycerol-3-phosphate dehydrogenase (NAD)
Abbreviation: R_ARTPLM2m Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [m] : Rtotal2coa <==> pmtcoa Confidence Level: 0 cytosolic conversion of pooled FA into palmitate (so subsequent transport into mit w/ FA oxidation can occur). Since Carbon stoichiometry normalized wrt C16 ratio between Rgroupcoa and pmtcoa is 1:1 HR fatty acid metabolism - R groups = acyl groups, Rtotal, Rtotal2, Rtotal3 correspond to three possible acyl groups added to glycerol R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FAOXC18480x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : (5) coa + (5) h2o + (5) nad + o2 + strdnccoa --> (5) accoa + (5) h + h2o2 + (5) nadh + occoa Confidence Level: 4 _5 rounds of beta ox, 4 degrees loss of saturation -> only 1 fad oxidized to fadh2 - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) peroxisomal (very long chain FA) c18:4,n-6 -> c8:0, 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (51.1 and 3295.1 and 30.1) or (30.1 and 51.2 and 1962.1) or (51.1 and 30.1 and 1962.1) or (3295.1 and 30.1 and 51.2)
Abbreviation: R_MI134P4P Synonyms: _1 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi134p --> mi13p + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# GENE ASSOCIATION:
inositol-1,3,4-trisphosphate 4-phosphatase
Abbreviation: R_HMGCOASim Synonyms: _0 EC Number: 4.1.3.5 SUBSYSTEM: Cholesterol Metabolism Equation: [m] : aacoa + accoa + h2o --> coa + h + hmgcoa Confidence Level: 4 NCD New assignment: EC-2.3.3.10 EC-4.1.3.5 (created 1961, deleted 2002). Enzyme Commission mitochondrial enzyme NJ Made irreversible to match KEGG data for S. aureus genes: LOCUS:_3158#TRANSCRIPT:1#ABBREVIATION:HMGCS2#ECNUMBERS:4.1.3.5# proteins: NAME:_3-hydroxy-3-methylglutaryl-Coenzyme A synthase 2 (mitochondrial)#ABBREVIATION:Hmgcs2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Mascaro C, Buesa C, Ortiz JA, Haro D, Hegardt FG.#TITLE:Molecular cloning and tissue expression of human mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase.#JOURNAL:Arch Biochem Biophys#YEAR:1995#PUBMED:7893153# GENE ASSOCIATION: (3158.1)
Abbreviation: R_GLYOXm Synonyms: _0 EC Number: 3.1.2.6 SUBSYSTEM: Pyruvate Metabolism Equation: [m] : h2o + lgt-S --> gthrd + h + lac-D Confidence Level: 4 IF genes: LOCUS:_3029#TRANSCRIPT:1#ABBREVIATION:HAGH#ECNUMBERS:3.1.2.6# proteins: NAME:hydroxyacylglutathione hydrolase, mitochondrial#ABBREVIATION:Hagh.1-m# citations: AUTHORS:Cordell PA, Futers TS, Grant PJ, Pease RJ#TITLE:The Human hydroxyacylglutathione hydrolase (HAGH) gene encodes both cytosolic and mitochondrial forms of glyoxalase II.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117945# AUTHORS:Ridderstrom M, Saccucci F, Hellman U, Bergman T, Principato G, Mannervik B#TITLE:Molecular cloning, heterologous expression, and characterization of human glyoxalase II#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8550579# GENE ASSOCIATION: (3029.1)
hydroxyacylglutathione hydrolase, mitochondrial
Abbreviation: R_5HTRPDOX Synonyms: _0 EC Number: 1.13.11.42 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 5htrp + o2 --> 5hoxnfkyn Confidence Level: 1 SAB genes: LOCUS:_3620#TRANSCRIPT:1#ABBREVIATION:INDO#ECNUMBERS:1.13.11.42# proteins: NAME:indoleamine-pyrrole 2,3 dioxygenase#ABBREVIATION:Indo.1# citations: none GENE ASSOCIATION: (3620.1)
_5-Hydroxy-L-tryptophan:oxygen 2,3-dioxygenase (indole-decyclizing)
Abbreviation: R_CYTK1 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : atp + cmp <==> adp + cdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_DEDOLR_U Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) dedol_U + h + nadph --> (0.1) dolichol_U + nadp Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Schenk B, Fernandez F, Waechter CJ.#TITLE:The ins(ide) and out(side) of dolichyl phosphate biosynthesis and recycling in the endoplasmic reticulum.#JOURNAL:Glycobiology#YEAR:2001#PUBMED:11425794# GENE ASSOCIATION:
Abbreviation: R_3HBCDm Synonyms: _3 EC Number: 4.2.1.55 SUBSYSTEM: Butanoate Metabolism Equation: [m] : b2coa + h2o <==> 3hbcoa-R Confidence Level: 2 NCD genes: LOCUS:_55268#TRANSCRIPT:1#ABBREVIATION:ECHDC2# proteins: NAME:enoyl Coenzyme A hydratase domain containing 2, mitochondrial#ABBREVIATION:Echdc2.1-m# citations: none GENE ASSOCIATION: (55268.1)
_3-hydroxybutyryl-CoA dehydratase, mitochondrial
Abbreviation: R_PPD2CSPp Synonyms: _0 SUBSYSTEM: Lysine Metabolism Equation: [x] : 6a2ohxnt --> 1pipdn2c + h2o Confidence Level: 2 spontaneous cyclization reaction MM genes: none proteins: none citations: AUTHORS:IJlst L, de Kromme I, Oostheim W, Wanders RJ.#TITLE:Molecular cloning and expression of human L-pipecolate oxidase.#YEAR:2000#PUBMED:10772957# AUTHORS:Murthy SN, Janardanasarma MK.#TITLE:Identification of L-amino acid/L-lysine alpha-amino oxidase in mouse brain.#YEAR:1999#PUBMED:10485319# GENE ASSOCIATION:
Abbreviation: R_NPTHLte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: npthl[e] <==> npthl[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DGULND Synonyms: _2 EC Number: 4.1.1.34 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : 3dhguln + h <==> co2 + xylu-L Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Wang YM, van Eys J#TITLE:Nutritional significance of fructose and sugar alcohols#JOURNAL:Annu Rev Nutr.#YEAR:1981#PUBMED:6821187# GENE ASSOCIATION:
Abbreviation: R_SELCYSTGL Synonyms: _0 EC Number: 4.4.1.1 SUBSYSTEM: Selenoamino acid metabolism Equation: [c] : h2o + selcyst --> 2obut + nh4 + selcys Confidence Level: 4 NCD SAB genes: LOCUS:_1491#TRANSCRIPT:1#ABBREVIATION:CTH#ECNUMBERS:4.4.1.1# LOCUS:_1491#TRANSCRIPT:2#ABBREVIATION:CTH#ECNUMBERS:4.4.1.1# proteins: NAME:cystathionase (cystathionine gamma-lyase) #ABBREVIATION:Cth.1# NAME:cystathionase (cystathionine gamma-lyase) #ABBREVIATION:Cth.2# citations: AUTHORS:Esaki N, Nakamura T, Tanaka H, Soda K#TITLE:Selenocysteine lyase, a novel enzyme that specifically acts on selenocysteine. Mammalian distribution and purification and properties of pig liver enzyme#JOURNAL:J Biol Chem#YEAR:1982#PUBMED:6461656# GENE ASSOCIATION: (1491.1) or (1491.2)
Abbreviation: R_SO4t4_2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) na1[e] + so4[e] <==> (2) na1[c] + so4[c] Confidence Level: 4 NCD genes: LOCUS:_26266#TRANSCRIPT:1#ABBREVIATION:SLC13A4# proteins: NAME:solute carrier family 13 (sodium/sulfate symporters), member 4#ABBREVIATION:Slc13a4.1-e# citations: AUTHORS:Girard JP, Baekkevold ES, Feliu J, Brandtzaeg P, Amalric F#TITLE:Molecular cloning and functional analysis of SUT-1, a sulfate transporter from human high endothelial venules#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1999#PUBMED:10535998# AUTHORS:Markovich D, Regeer RR, Kunzelmann K, Dawson PA#TITLE:Functional characterization and genomic organization of the human Na(+)-sulfate cotransporter hNaS2 gene (SLC13A4)#JOURNAL:Biochem Biophys Res Commun#YEAR:2005#PUBMED:15607730# GENE ASSOCIATION: (26266.1)
Abbreviation: R_TYROXDAc Synonyms: _0 EC Number: 1.4.3.6 SUBSYSTEM: Tyrosine metabolism Equation: [c] : h2o + o2 + tym --> 4hoxpacd + h2o2 + nh4 Confidence Level: 4 SAB also 1.4.3.4 genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# proteins: NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Anderson MC, Hasan F, McCrodden JM, Tipton KF#TITLE:Monoamine oxidase inhibitors and the cheese effect#JOURNAL:Neurochem Res#YEAR:1993#PUBMED:8255365# AUTHORS:Humphrey SJ, Curry JT, Turman CN, Stryd RP#TITLE:Cardiovascular sympathomimetic amine interactions in rats treated with monoamine oxidase inhibitors and the novel oxazolidinone antibiotic linezolid#JOURNAL:J Cardiovasc Pharmacol#YEAR:2001#PUBMED:11336106# AUTHORS:Youdim MB, Weinstock M#TITLE:Therapeutic applications of selective and non-selective inhibitors of monoamine oxidase A and B that do not cause significant tyramine potentiation#JOURNAL:Neurotoxicology#YEAR:2004#PUBMED:14697899# GENE ASSOCIATION: (4128.1) or (4129.1)
Tyramine:oxygen oxidoreductase(deaminating)(flavin-containing) (cytosol)
Abbreviation: R_PLA2 Synonyms: _0 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : ak2gchol_hs + h2o --> Rtotal2 + ak2lgchol_hs + h Confidence Level: 4 paf synthesis pathway NJ genes: none proteins: none citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_DHCRD1 Synonyms: _1 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : dhcrm_hs + nadp --> crm_hs + h + nadph Confidence Level: 4 NJ genes: LOCUS:_8560#TRANSCRIPT:1#ABBREVIATION:DEGS1# LOCUS:_8560#TRANSCRIPT:2#ABBREVIATION:DEGS1# proteins: NAME:degenerative spermatocyte homolog 1, lipid desaturase#ABBREVIATION:Degs1.1# NAME:degenerative spermatocyte homolog 1, lipid desaturase#ABBREVIATION:Degs1.2# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Cadena DL, Kurten RC, Gill GN.#TITLE:The product of the MLD gene is a member of the membrane fatty acid desaturase family: overexpression of MLD inhibits EGF receptor biosynthesis.#JOURNAL:Biochemistry#YEAR:1997#PUBMED:9188692# GENE ASSOCIATION: (8560.1) or (8560.2)
Abbreviation: R_FAOXC2242046x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : adrncoa + coa + h2o + nad + o2 --> accoa + arachdcoa + h + h2o2 + nadh Confidence Level: 4 _1 round beta ox - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) peroxisomal (very long chain FA) c22:4,n-6 -> c20:4, n-6 - final '6' in abbreviation indicates omega6 FA 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (51.1 and 30.1 and 1962.1) or (30.1 and 51.2 and 1962.1) or (51.1 and 3295.1 and 30.1)
Abbreviation: R_P45021A1r Synonyms: _2 EC Number: 1.14.99.10 SUBSYSTEM: Steroid Metabolism Equation: [r] : h + nadph + o2 + prgstrn --> 11docrtstrn + h2o + nadp Confidence Level: 4 aka CYP21B NJ genes: LOCUS:_1589#TRANSCRIPT:1#ABBREVIATION:CYP21A2#ECNUMBERS:1.14.99.10# proteins: NAME:cytochrome P450, family 21, subfamily A, polypeptide 2#ABBREVIATION:Cyp21a2.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (1589.1)
Abbreviation: R_NTD2 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : h2o + ump --> pi + uri Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_51251#TRANSCRIPT:1#ABBREVIATION:NT5C3#ECNUMBERS:3.1.3.5# proteins: NAME:_5'-nucleotidase, cytosolic III #ABBREVIATION:Nt5C3.1# citations: AUTHORS:Amici A, Magni G. #TITLE:Human erythrocyte pyrimidine 5'-nucleotidase, PN-I.#JOURNAL:Arch Biochem Biophys#YEAR:2002#PUBMED:11795870# GENE ASSOCIATION: (51251.1)
Abbreviation: R_FAH3 Synonyms: _1 SUBSYSTEM: CYP Metabolism Equation: [c] : h + hdca + nadph + o2 --> h2o + nadp + whhdca Confidence Level: 4 fatty acid omega hydroxylation NJ genes: LOCUS:_1579#TRANSCRIPT:1#ABBREVIATION:CYP4A11# proteins: NAME:cytochrome P450, family 4, subfamily A, polypeptide#ABBREVIATION:Cyp4a11.1# citations: AUTHORS:Rendic S.#TITLE:Summary of information on human CYP enzymes: human P450 metabolism data.#JOURNAL:Drug Metab Rev#YEAR:2002#PUBMED:11996015# AUTHORS:Palmer CN, Richardson TH, Griffin KJ, Hsu MH, Muerhoff AS, Clark JE, Johnson EF.#TITLE:Characterization of a cDNA encoding a human kidney, cytochrome P-450 4A fatty acid omega-hydroxylase and the cognate enzyme expressed in Escherichia coli.#JOURNAL:Biochim Biophys Acta#YEAR:1993#PUBMED:7679927# AUTHORS:Imaoka S, Ogawa H, Kimura S, Gonzalez FJ.#TITLE:Complete cDNA sequence and cDNA-directed expression of CYP4A11, a fatty acid omega-hydroxylase expressed in human kidney.#JOURNAL:DNA Cell Biol#YEAR:1993#PUBMED:8274222# GENE ASSOCIATION: (1579.1)
Abbreviation: R_PDE1g Synonyms: _0 EC Number: 3.1.4.17 SUBSYSTEM: Nucleotides Equation: [g] : camp + h2o --> amp + h Confidence Level: 4 NCD, IT genes: LOCUS:_10846#TRANSCRIPT:1#ABBREVIATION:PDE10A# proteins: NAME:phosphodiesterase 10A #ABBREVIATION:Pde10A# citations: none GENE ASSOCIATION: (10846.1)
_3',5'-cyclic-nucleotide phosphodiesterase,Golgi
Abbreviation: R_NTD5l Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleic acid degradation Equation: [l] : dtmp + h2o --> pi + thymd Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:R.L. Pisoni#TITLE:Lysosomal Nucleic Acid and Phosphate Metabolism and Related Metabolic Reactions, Chapter 9#YEAR:1996# GENE ASSOCIATION: (53.1)
Abbreviation: R_HACD1x Synonyms: _0 EC Number: 1.1.1.35 SUBSYSTEM: Tryptophan metabolism Equation: [x] : aacoa + h + nadh <==> 3hbcoa + nad Confidence Level: 0 Same as EC 1.1.1.157 SAB x genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# proteins: NAME:enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase (x)#ABBREVIATION:Ehhadh.1-x# NAME:hydroxysteroid (17-beta) dehydrogenase 4#ABBREVIATION:Hsd17b4.1-x# citations: none GENE ASSOCIATION: (1962.1) or (3295.1)
_3-hydroxyacyl-CoA dehydrogenase (acetoacetyl-CoA) (peroxisome)
Abbreviation: R_PSSA2_hs Synonyms: _0 EC Number: 2.7.8.8 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : pe_hs + ser-L <==> etha + ps_hs Confidence Level: 4 phosphatidylethanolamine version as substrate NJ genes: LOCUS:_81490#TRANSCRIPT:1#ABBREVIATION:PTDSS2# proteins: NAME:phosphatidylserine synthase 2#ABBREVIATION:PTDSS2# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION: (81490.1)
Abbreviation: R_GALASE9ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg23 --> gal + ksi_deg24 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_DM_oretn_LPAREN_n_RPAREN_ Synonyms: _0 Equation: [n] : oretn --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ARTFR34 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (2) fadh2[m] + lneldccoa[c] --> (1.125) R3coa_hs[c] + (2) fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DASCBH Synonyms: _0 EC Number: 3.1.1.17 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : dhdascb + h2o --> 23doguln + h Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Banhegyi G, Braun L, Csala M, Puskas F, Mandl J#TITLE:Ascorbate metabolism and its regulation in animals#JOURNAL:Free Radic Biol Med#YEAR:1997#PUBMED:9296457# AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# GENE ASSOCIATION:
Abbreviation: R_S6T1g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre5 + paps --> h + ksii_core2_pre6 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_PHEMEt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: pheme[e] --> pheme[c] Confidence Level: 4 NCD genes: LOCUS:_113235#TRANSCRIPT:1#ABBREVIATION:MGC9564# proteins: NAME:similar to RIKEN cDNA 1110002C08 gene#ABBREVIATION:Mgc9564.1# citations: AUTHORS:Shayeghi M, Latunde-Dada GO, Oakhill JS, Laftah AH, Takeuchi K, Halliday N, Khan Y, Warley A, McCann FE, Hider RC, Frazer DM, Anderson GJ, Vulpe CD, Simpson RJ, McKie AT#TITLE:Identification of an intestinal heme transporter#JOURNAL:Cell#YEAR:2005#PUBMED:16143108# GENE ASSOCIATION: (113235.1)
Abbreviation: R_OCOAT1m Synonyms: _0 EC Number: 2.8.3.5 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : acac + succoa <==> aacoa + succ Confidence Level: 4 TDV genes: LOCUS:_5019#TRANSCRIPT:1#ABBREVIATION:OXCT1# LOCUS:_64064#TRANSCRIPT:1#ABBREVIATION:OXCT2# proteins: NAME:_3-oxoacid CoA transferase 1, mitochondrial#ABBREVIATION:Oxct1.1-m# NAME:_3-oxoacid CoA transferase 2, mitochondrial#ABBREVIATION:Oxct2.1-m# citations: AUTHORS:Tanaka H, Kohroki J, Iguchi N, Onishi M, Nishimune Y. #TITLE:Cloning and characterization of a human orthologue of testis-specific succinyl CoA: 3-oxo acid CoA transferase (Scot-t) cDNA.#YEAR:2002#PUBMED:11756565# AUTHORS:Fukao T, Mitchell GA, Song XQ, Nakamura H, Kassovska-Bratinova S, Orii KE, Wraith JE, Besley G, Wanders RJ, Niezen-Koning KE, Berry GT, Palmieri M, Kondo N.#TITLE:Succinyl-CoA:3-ketoacid CoA transferase (SCOT): cloning of the human SCOT gene, tertiary structural modeling of the human SCOT monomer, and characterization of three pathogenic mutations. #YEAR:2000#PUBMED:10964512# GENE ASSOCIATION: (5019.1) or (64064.1)
Abbreviation: R_MI3PP Synonyms: _6 EC Number: 3.1.3.25 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi3p-D --> inost + pi Confidence Level: 4 NCD genes: LOCUS:_3613#TRANSCRIPT:1#ABBREVIATION:IMPA2# LOCUS:_3612#TRANSCRIPT:1#ABBREVIATION:IMPA1#ECNUMBERS:3.1.3.25# proteins: NAME:inositol(myo)-1(or 4)-monophosphatase 1#ABBREVIATION:Impa1.1# NAME:inositol(myo)-1(or 4)-monophosphatase 2#ABBREVIATION:Impa2.1# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:McAllister G, Whiting P, Hammond EA, Knowles MR, Atack JR, Bailey FJ, Maigetter R, Ragan CI.#TITLE:cDNA cloning of human and rat brain myo-inositol monophosphatase. Expression and characterization of the human recombinant enzyme#JOURNAL:Biochem J#YEAR:1992#PUBMED:1377913# AUTHORS:Yoshikawa T, Turner G, Esterling LE, Sanders AR, Detera-Wadleigh SD#TITLE:A novel human myo-inositol monophosphatase gene, IMP.18p, maps to a susceptibility region for bipolar disorder#JOURNAL:Mol Psychiatry#YEAR:1997#PUBMED:9322233# GENE ASSOCIATION: (3612.1) or (3613.1)
Abbreviation: R_NICRNS Synonyms: _0 SUBSYSTEM: NAD Metabolism Equation: [c] : atp + nicrns --> adp + h + nicrnt Confidence Level: 2 no gene or enzyme name known (in kegg) but reaction has to take place. IT genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_GGH_DASH_7DHFl Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [l] : 7dhf + h2o --> 6dhf + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-l# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (7DHF), lysosomal
Abbreviation: R_ARTFR12 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + hdcoa[c] --> R1coa_hs[c] + fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CHATn Synonyms: _3 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [n] : accoa + chol <==> ach + coa Confidence Level: 4 NJ genes: LOCUS:_1103#TRANSCRIPT:1#ABBREVIATION:CHAT#ECNUMBERS:2.3.1.6# LOCUS:_1103#TRANSCRIPT:4#ABBREVIATION:CHAT#ECNUMBERS:2.3.1.6# LOCUS:_1103#TRANSCRIPT:2#ABBREVIATION:CHAT#ECNUMBERS:2.3.1.6# LOCUS:_1103#TRANSCRIPT:3#ABBREVIATION:CHAT#ECNUMBERS:2.3.1.6# proteins: NAME:choline acetyltransferase#ABBREVIATION:Chat.1# NAME:choline acetyltransferase#ABBREVIATION:Chat.2# NAME:choline acetyltransferase#ABBREVIATION:Chat.3# NAME:choline acetyltransferase#ABBREVIATION:Chat.4# citations: AUTHORS:Dobransky T, Davis WL, Xiao GH, Rylett RJ.#TITLE:Expression, purification and characterization of recombinant human choline acetyltransferase: phosphorylation of the enzyme regulates catalytic activity.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10861222# GENE ASSOCIATION: (1103.4) or (1103.1) or (1103.3) or (1103.2)
Abbreviation: R_MAN1PT2 Synonyms: _6 EC Number: 2.7.7.22 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : gdp + h + man1p --> gdpmann + pi Confidence Level: 0 JLR genes: LOCUS:_29925#TRANSCRIPT:1#ABBREVIATION:GMPPB#ECNUMBERS:2.7.7.22# LOCUS:_29926#TRANSCRIPT:1#ABBREVIATION:GMPPA#ECNUMBERS:2.7.7.22# LOCUS:_29925#TRANSCRIPT:2#ABBREVIATION:GMPPB#ECNUMBERS:2.7.7.22# LOCUS:_29926#TRANSCRIPT:2#ABBREVIATION:GMPPA#ECNUMBERS:2.7.7.22# proteins: NAME:GDP-mannose pyrophosphorylase B#ABBREVIATION:Gmppb.1# NAME:GDP-mannose pyrophosphorylase B#ABBREVIATION:Gmppb.2# NAME:GDP-mannose pyrophosphorylase A#ABBREVIATION:Gmppa.1# NAME:GDP-mannose pyrophosphorylase A#ABBREVIATION:Gmppa.2# citations: none GENE ASSOCIATION: (29926.2) or (29926.1) or (29925.2) or (29925.1)
mannose-1-phosphate guanylyltransferase (GDP)
Abbreviation: R_EX_gd1b2_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gd1b2_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ENMAN6g Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : g1m6masnB1 + h2o --> glc1man + m5masnB1 Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Endomannosidase (m5masnB1-producing), Golgi apparatus
Abbreviation: R_PAIL45P_HStn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: pail45p_hs[c] <==> pail45p_hs[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
phosphatidylinositol 4,5-bisphosphate nuclear transport (diffusion)
Abbreviation: R_EX_arg_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : arg-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
L-serine/L-cysteine Na-dependent exchange (Cys-L in)
Abbreviation: R_CYSSERNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[e] + na1[e] + ser-L[c] --> cys-L[c] + na1[c] + ser-L[e] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
Abbreviation: R_EX_3aib_DASH_D_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 3aib-D <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DATPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: datp[c] <==> datp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SPHGNtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: sphgn[r] <==> sphgn[c] Confidence Level: 2 unknown mechanism - reflects interaction between different stages of endosomes and cytoplasm NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RETt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: retinol[e] --> retinol[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Retinol transport via faciliated diffusion
Abbreviation: R_HMGCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: hmgcoa[c] <==> hmgcoa[x] Confidence Level: 2 diffusion NJ genes: none proteins: none citations: AUTHORS:Biardi L, Krisans K#TITLE:Compartmentalization of cholesterol biosynthesis#JOURNAL:Journal of Biological Chemistry#YEAR:1996# AUTHORS:Kovacs WJ, Krisans S.#TITLE:Cholesterol biosynthesis and regulation: role of peroxisomes.#JOURNAL:Adv Exp Med Biol.#YEAR:2003#PUBMED:14713247# GENE ASSOCIATION:
Hydroxymethylglutaryl-CoA reversible peroxisomal transport
Abbreviation: R_GALASE7ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg17 --> gal + ksi_deg18 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_ACNAMtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: acnam[c] --> acnam[n] Confidence Level: 1 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_TETHEX3t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tethex3[e] <==> tethex3[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_DTDPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: dtdp[c] <==> dtdp[n] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GALGALGALTHCRMtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: galgalgalthcrm_hs[c] <==> galgalgalthcrm_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
galgalgalthcrm_hs intracellular transport
Abbreviation: R_NACHEXA5ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_d + (2) h2o --> acgal + cs_d_deg2 + h + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_GCHOLAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gchola[e] + hco3[c] <==> gchola[c] + hco3[e] Confidence Level: 4 NJ genes: LOCUS:_28234#TRANSCRIPT:1#ABBREVIATION:SLCO1B3# LOCUS:_6579#TRANSCRIPT:1#ABBREVIATION:SLCO1A2# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1A2#ABBREVIATION:Slco1a2.1# NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# NAME:solute carrier organic anion transporter family, member 1B3#ABBREVIATION:Slco1b3.1# citations: AUTHORS:Kullak-Ublick GA, Beuers U, Meier PJ, Domdey H, Paumgartner G.#TITLE:Assignment of the human organic anion transporting polypeptide (OATP) gene to chromosome 12p12 by fluorescence in situ hybridization.#JOURNAL:J Hepatol.#YEAR:1996#PUBMED:9007731# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (6579.1) or (28234.1) or (10599.1)
glycocholate transport via bicarbonate countertransport
Abbreviation: R_EX_10fthf7glu_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 10fthf7glu <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_10-formyltetrahydrofolate-[Glu](7) exchange
Abbreviation: R_EX_gmp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gmp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_4NPHte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _4nph[e] <==> 4nph[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TPI Synonyms: _0 EC Number: 5.3.1.1 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : dhap <==> g3p Confidence Level: 0 SMP genes: LOCUS:_7167#TRANSCRIPT:1#ABBREVIATION:TPI1#ECNUMBERS:5.3.1.1# LOCUS:_286016#TRANSCRIPT:1#ABBREVIATION:LOC286016# proteins: NAME:triosephosphate isomerase 1 #ABBREVIATION:Tpi1.1# NAME:similar to Triosephosphate isomerase#ABBREVIATION:Loc286016.1# citations: none GENE ASSOCIATION: (7167.1) or (286016.1)
Abbreviation: R_ACNGALACGLCGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acngalacglcgal14acglcgalgluside_hs[g] <==> acngalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SPH1Pte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: sph1p[c] <==> sph1p[e] Confidence Level: 2 unknown mechanism for transport, may be vesicular/caveolar/other ... intracellular transport and extracellular export mechanisms must exist - see for example: PMID: 15585321 NJ genes: none proteins: none citations: AUTHORS:Baumruker T, Bornancin F, Billich A.#TITLE:The role of sphingosine and ceramide kinases in inflammatory responses.#JOURNAL:Immunol Lett.#YEAR:2005#PUBMED:15585321# GENE ASSOCIATION:
Abbreviation: R_FUT92g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acngal14acglcgalgluside_hs + gdpfuc --> fucacngal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
Abbreviation: R_EX_galfucgalacglcgal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : galfucgalacglcgal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(Gal)4 (Glc)1 (GlcNAc)2 (LFuc)1 (Cer)1 exchange
Abbreviation: R_B_MANNASEly Synonyms: _5 EC Number: 3.2.1.25 SUBSYSTEM: N-Glycan Degradation Equation: [l] : h2o + mn --> acgam + man Confidence Level: 0 NCD genes: LOCUS:_4126#TRANSCRIPT:1#ABBREVIATION:MANBA#ECNUMBERS:3.2.1.25# proteins: NAME:mannosidase, beta A, lysosomal (ubiquitous)#ABBREVIATION:Manba.1-l# citations: AUTHORS:Alkhayat AH, Kraemer SA, Leipprandt JR, Macek M, Kleijer WJ, Friderici KH.#TITLE:Human beta-mannosidase cDNA characterization and first identification of a mutation associated with human beta-mannosidosis#JOURNAL:Hum Mol Genet#YEAR:1998#PUBMED:9384606# GENE ASSOCIATION: (4126.1)
Abbreviation: R_CLHCOtex Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[e] + (2) hco3[c] --> cl[c] + (2) hco3[e] Confidence Level: 2 SAB genes: LOCUS:_1836#TRANSCRIPT:1#ABBREVIATION:SLC26A2# LOCUS:_115019#TRANSCRIPT:1#ABBREVIATION:SLC26A9# LOCUS:_115019#TRANSCRIPT:2#ABBREVIATION:SLC26A9# LOCUS:_1811#TRANSCRIPT:1#ABBREVIATION:SLC26A3# LOCUS:_5172#TRANSCRIPT:1#ABBREVIATION:SLC26A4# LOCUS:_115111#TRANSCRIPT:1#ABBREVIATION:SLC26A7# LOCUS:_115111#TRANSCRIPT:2#ABBREVIATION:SLC26A7# LOCUS:_116369#TRANSCRIPT:1#ABBREVIATION:SLC26A8# LOCUS:_116369#TRANSCRIPT:2#ABBREVIATION:SLC26A8# proteins: NAME:solute carrier family 26 (sulfate transporter), member 2#ABBREVIATION:Slc26a2.1# NAME:solute carrier family 26, member 3#ABBREVIATION:Slc26a3.1# NAME:solute carrier family 26, member 4#ABBREVIATION:Slc26a4.1# NAME:solute carrier family 26, member 7#ABBREVIATION:Slc26a7.1# NAME:solute carrier family 26, member 7#ABBREVIATION:Slc26a7.2# NAME:solute carrier family 26, member 8#ABBREVIATION:Slc26a8.1# NAME:solute carrier family 26, member 8#ABBREVIATION:Slc26a8.2# NAME:solute carrier family 26, member 9#ABBREVIATION:Slc26a9.1# NAME:solute carrier family 26, member 9#ABBREVIATION:Slc26a9.2# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (116369.1) or (5172.1) or (116369.2) or (115019.2) or (1811.1) or (115019.1) or (1836.1) or (115111.1) or (115111.2)
chloride transport via bicarbonate countertransport
Abbreviation: R_FUCGALGBSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucgalgbside_hs[c] <==> fucgalgbside_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_IDOAASE3ly Synonyms: _1 EC Number: 3.2.1.76 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg22 --> hs_deg23 + idour Confidence Level: 4 NCD genes: LOCUS:_3425#TRANSCRIPT:1#ABBREVIATION:IDUA#ECNUMBERS:3.2.1.76# proteins: NAME:iduronidase, alpha-L-, lysosomal#ABBREVIATION:Idua.1-l# citations: AUTHORS:Scott HS, Anson DS, Orsborn AM, Nelson PV, Clements PR, Morris CP, Hopwood JJ#TITLE:Human alpha-L-iduronidase: cDNA isolation and expression#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1946389# AUTHORS:Clements PR, Brooks DA, McCourt PA, Hopwood JJ#TITLE:Immunopurification and characterization of human alpha-L-iduronidase with the use of monoclonal antibodies#JOURNAL:Biochem J#YEAR:1989#PUBMED:2470345# GENE ASSOCIATION: (3425.1)
Abbreviation: R_P450SCC1m Synonyms: _12 SUBSYSTEM: Cholesterol Metabolism Equation: [m] : chsterol + h + nadph + o2 <==> 20ahchsterol + h2o + nadp Confidence Level: 0 Acting on paired donors with incorporation of molecular oxygen With a reduced iron-sulfur protein as one donor, and incorporation of one atom of oxygen genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ABO9g Synonyms: _11 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucfucgalacglcgalacglcgal14acglcgalgluside_hs + (2) udpgal --> galgalfucfucgalacglcgalacglcgal14acglcgalgluside_hs + (2) h + (2) udp Confidence Level: 4 NJ genes: LOCUS:_28#TRANSCRIPT:1#ABBREVIATION:ABO# proteins: NAME:ABO blood group#ABBREVIATION:Abo.1# citations: AUTHORS:Lloyd KO.#TITLE:The chemistry and immunochemistry of blood group A, B, H, and Lewis antigens: past, present and future.#JOURNAL:Glycoconj J#YEAR:2000#PUBMED:11421346# AUTHORS:Yamamoto F.-I., Hakomori S.-I.;#TITLE:Sugar-nucleotide donor specificity of histo-blood group A and B transferases is based on amino acid substitutions#JOURNAL:J. Biol. Chem#YEAR:1990#PUBMED:2121736# AUTHORS:Patenaude S.I., Seto N.O.L., Borisova S.N., Szpacenko A., Marcus S.L., Palcic M.M., Evans S.V.;#TITLE:The structural basis for specificity in human ABO(H) blood group biosynthesis#JOURNAL:Nat. Struct. Biol#YEAR:2002#PUBMED:12198488# GENE ASSOCIATION: (28.1)
phosphatidylinositol-5-phosphate 3-kinase
Abbreviation: R_PI5P3K Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + pail5p_hs --> adp + h + pail35p_hs Confidence Level: 1 NCD genes: LOCUS:_5289#TRANSCRIPT:1#ABBREVIATION:PIK3C3# LOCUS:_5291#TRANSCRIPT:1#ABBREVIATION:PIK3CB#ECNUMBERS:2.7.1.137# LOCUS:_5290#TRANSCRIPT:1#ABBREVIATION:PIK3CA#ECNUMBERS:2.7.1.137# LOCUS:_5287#TRANSCRIPT:1#ABBREVIATION:PIK3C2B#ECNUMBERS:2.7.1.137# LOCUS:_5293#TRANSCRIPT:1#ABBREVIATION:PIK3CD#ECNUMBERS:2.7.1.137# LOCUS:_5286#TRANSCRIPT:1#ABBREVIATION:PIK3C2A#ECNUMBERS:2.7.1.137# LOCUS:_5294#TRANSCRIPT:1#ABBREVIATION:PIK3CG#ECNUMBERS:2.7.1.137# LOCUS:_23533#TRANSCRIPT:1#ABBREVIATION:P101-PI3K# LOCUS:_5288#TRANSCRIPT:1#ABBREVIATION:PIK3C2G#ECNUMBERS:2.7.1.137# LOCUS:_5295#TRANSCRIPT:1#ABBREVIATION:PIK3R1# LOCUS:_8503#TRANSCRIPT:1#ABBREVIATION:PIK3R3# LOCUS:_5296#TRANSCRIPT:1#ABBREVIATION:PIK3R2# LOCUS:_5295#TRANSCRIPT:2#ABBREVIATION:PIK3R1# LOCUS:_5295#TRANSCRIPT:3#ABBREVIATION:PIK3R1# LOCUS:_10870#TRANSCRIPT:1#ABBREVIATION:HCST# proteins: NAME:phosphoinositide-3-kinase (p110a, p85a)#ABBREVIATION:Pik3car1.1# NAME:phosphoinositide-3-kinase (p110a, p55a)#ABBREVIATION:Pik3car1.2# NAME:phosphoinositide-3-kinase (p110a, p50a)#ABBREVIATION:Pik3car.3# NAME:phosphoinositide-3-kinase (p110a, p85b)#ABBREVIATION:Pik3car2.1# NAME:phosphoinositide-3-kinase (p110a, p55c)#ABBREVIATION:Pik3car3.1# NAME:phosphoinositide-3-kinase (p110b, p85a)#ABBREVIATION:Pik3cbr1.1# NAME:phosphoinositide-3-kinase (p110b, p55a)#ABBREVIATION:Pik3cbr1.2# NAME:phosphoinositide-3-kinase (p110b, p50a)#ABBREVIATION:Pik3cbr1.3# NAME:phosphoinositide-3-kinase (p110b, p85b)#ABBREVIATION:Pik3cbr2.1# NAME:phosphoinositide-3-kinase (p110b, p55c)#ABBREVIATION:Pik3cbr3.1# NAME:phosphoinositide-3-kinase (p110c, p85a)#ABBREVIATION:Pik3cdr1.1# NAME:phosphoinositide-3-kinase (p110c, p55a)#ABBREVIATION:Pik3cdr1.2# NAME:phosphoinositide-3-kinase (p110c, p50a)#ABBREVIATION:Pik3cdr1.3# NAME:phosphoinositide-3-kinase (p110c, p85b)#ABBREVIATION:Pik3cdr2.1# NAME:phosphoinositide-3-kinase (p110c, p55c)#ABBREVIATION:Pik3cdr3.1# NAME:phosphoinositide-3-kinase (PIK3cg, p101)#ABBREVIATION:Pik3cgr5.1# NAME:phosphoinositide-3-kinase (PIK3CG, p87 adapter protein)#ABBREVIATION:Pik3cgap.1# NAME:phosphoinositide-3-kinase, class 2, alpha polypeptide#ABBREVIATION:Pik3c2a.1# NAME:phosphoinositide-3-kinase, class 2, beta polypeptide#ABBREVIATION:Pik3c2b.1# NAME:phosphoinositide-3-kinase, class 2, gamma polypeptide#ABBREVIATION:Pik3c2g.1# NAME:phosphoinositide-3-kinase, class 3#ABBREVIATION:Pik3c3.1# citations: AUTHORS:Tolias KF, Cantley LC#TITLE:Pathways for phosphoinositide synthesis#JOURNAL:Chem Phys Lipids#YEAR:1999#PUBMED:10358929# GENE ASSOCIATION: (5291.1 and 5295.3) or (5286.1) or (5296.1 and 5290.1) or (5293.1 and 5296.1) or (5288.1) or (5287.1) or (5293.1 and 8503.1) or (5293.1 and 5295.3) or (8503.1 and 5291.1) or (5295.2 and 5290.1) or (5289.1) or (5291.1 and 5295.1) or (5296.1 and 5291.1) or (23533.1 and 5294.1) or (5293.1 and 5295.1) or (5295.2 and 5293.1) or (5290.1 and 8503.1) or (5290.1 and 5295.3) or (10870.1 and 5294.1) or (5295.2 and 5291.1) or (5290.1 and 5295.1)
Abbreviation: R_ALAt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + na1[e] --> ala-L[c] + na1[c] Confidence Level: 4 tv Mendz pg. 201 Reversibility is unclear. genes: LOCUS:_84889#TRANSCRIPT:1#ABBREVIATION:SLC7A3# LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_6542#TRANSCRIPT:1#ABBREVIATION:SLC7A2# LOCUS:_6541#TRANSCRIPT:1#ABBREVIATION:SLC7A1# LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 1#ABBREVIATION:Slc7a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 2#ABBREVIATION:Slc7a2.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 3#ABBREVIATION:Slc7a3.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# AUTHORS:Albritton LM, Bowcock AM, Eddy RL, Morton CC, Tseng L, Farrer LA, Cavalli-Sforza LL, Shows TB, Cunningham JM#TITLE:The human cationic amino acid transporter (ATRC1): physical and genetic mapping to 13q12-q14#JOURNAL:Genomics#YEAR:1992#PUBMED:1348489# AUTHORS:Cariappa R, Heath-Monnig E, Furesz TC, Kamath SG, Smith CH#TITLE:Stable polarized expression of hCAT-1 in an epithelial cell line#JOURNAL:J Membr Biol#YEAR:2002#PUBMED:11891586# AUTHORS:Hoshide R, Ikeda Y, Karashima S, Matsuura T, Komaki S, Kishino T, Niikawa N, Endo F, Matsuda I#TITLE:Molecular cloning, tissue distribution, and chromosomal localization of human cationic amino acid transporter 2 (HCAT2)#JOURNAL:Genomics#YEAR:1996#PUBMED:8954799# AUTHORS:Furesz TC, Moe AJ, Smith CH#TITLE:Lysine uptake by human placental microvillous membrane: comparison of system y+ with basal membrane#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7534987# AUTHORS:Wolf S, Janzen A, Vekony N, Martine U, Strand D, Closs EI#TITLE:Expression of solute carrier 7A4 (SLC7A4) in the plasma membrane is not sufficient to mediate amino acid transport activity#JOURNAL:Biochem J#YEAR:2002#PUBMED:12049641# AUTHORS:Vekony N, Wolf S, Boissel JP, Gnauert K, Closs EI#TITLE:Human cationic amino acid transporter hCAT-3 is preferentially expressed in peripheral tissues#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11591158# AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (6542.1) or (81539.1) or (11254.1) or (54407.1) or (84889.1) or (55089.1) or (6541.1)
Abbreviation: R_EX_prostgf2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : prostgf2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLNALANaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[c] + gln-L[e] + na1[e] --> ala-L[e] + gln-L[c] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-alanine/L-glutamine Na-dependent exchange (Gln-L in)
Abbreviation: R_DNDPt58m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[m] + dgtp[c] --> adp[c] + dgtp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_glyc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : glyc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NADN Synonyms: _0 EC Number: 3.2.2.5 SUBSYSTEM: NAD Metabolism Equation: [c] : h2o + nad --> adprib + h + ncam Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Boyer CS, Moore GA, Moldeus P.#TITLE:Submitochondrial localization of the NAD+ glycohydrolase. Implications for the role of pyridine nucleotide hydrolysis in mitochondrial calcium fluxes.#JOURNAL:J Biol Chem. #YEAR:1993#PUBMED:8382685# GENE ASSOCIATION:
Abbreviation: R_25HVITD3tin_DASH_m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _25hvitd3[c] --> 25hvitd3[m] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_25-hydroxyvitamin D3 transport in mitochondria
Abbreviation: R_CLFORtex2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) cl[e] + for[c] --> (2) cl[c] + for[e] Confidence Level: 2 SAB genes: LOCUS:_65010#TRANSCRIPT:1#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:2#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:3#ABBREVIATION:SLC26A6# proteins: NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.1# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.2# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.3# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (65010.1) or (65010.3) or (65010.2)
chloride transport via formate countertransport (2:1)
Abbreviation: R_EX_succ_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : succ <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PUNP6 Synonyms: _0 EC Number: 2.4.2.1 SUBSYSTEM: Purine Catabolism Equation: [c] : din + pi <==> 2dr1p + hxan Confidence Level: 4 Specificity not completely determined. Can also catalyse ribosyltransferase reactions of the type catalysed by EC 2.4.2.5, nucleoside ribosyltransferase genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# citations: AUTHORS:Erion MD, Stoeckler JD, Guida WC, Walter RL, Ealick SE.#TITLE:Purine nucleoside phosphorylase. 2. Catalytic mechanism. #JOURNAL:Biochemistry#YEAR:1997#PUBMED:9305963# AUTHORS:Williams SR, Goddard JM, Martin DW Jr. #TITLE:Human purine nucleoside phosphorylase cDNA sequence and genomic clone characterization.#JOURNAL:Nucleic Acids Res.#YEAR:1984#PUBMED:6087295# AUTHORS:Canduri F, dos Santos DM, Silva RG, Mendes MA, Basso LA, Palma MS, de Azevedo WF, Santos DS.#TITLE:Structures of human purine nucleoside phosphorylase complexed with inosine and ddI.#JOURNAL:Biochem Biophys Res Commun. #YEAR:2004#PUBMED:14706628# GENE ASSOCIATION: (4860.1)
purine-nucleoside phosphorylase (Deoxyinosine)
Abbreviation: R_COUCOAFm Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : T4hcinnm + atp + coa --> amp + coucoa + ppi Confidence Level: 2 IT reaction involved in formation of 4-hydroxybenzoate from tyrosine. (EC 6.2.1.12) . genes: none proteins: none citations: AUTHORS:Meganathan R.#TITLE:Ubiquinone biosynthesis in microorganisms.#JOURNAL:FEMS Microbiol Lett. #YEAR:2001#PUBMED:11583838# AUTHORS:Loscher R, Heide L.#TITLE:Biosynthesis of p-Hydroxybenzoate from p-Coumarate and p-Coumaroyl-Coenzyme A in Cell-Free Extracts of Lithospermum erythrorhizon Cell Cultures. #JOURNAL:Plant Physiol.#YEAR:1994#PUBMED:12232327# AUTHORS:Booth, A. N., Masri, M. S., Robbins, D. J., Emerson, O. H., Jones, F. T. & DeEds, F.#TITLE:Urinary Phenolic Acid Metabolites of Tyrosine#JOURNAL:J. Biol. Chem#YEAR:1960# GENE ASSOCIATION:
Abbreviation: R_EX_gsn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gsn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACGALFUCGALACGALFUC12GAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: acgalfucgalacgalfuc12gal14acglcgalgluside_hs[e] <==> acgalfucgalacgalfuc12gal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_RTOT_3 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : R1coa_hs + R2coa_hs --> (2) Rtotal3coa Confidence Level: 0 User defined, tissue specific fluxes that can be customized w/ various ratios of R1 through R5 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PPBNGS Synonyms: _0 EC Number: 4.2.1.24 SUBSYSTEM: Heme Biosynthesis Equation: [c] : (2) 5aop --> h + (2) h2o + ppbng Confidence Level: 4 genes: LOCUS:_210#TRANSCRIPT:1#ABBREVIATION:ALAD#ECNUMBERS:4.2.1.24# LOCUS:_210#TRANSCRIPT:2#ABBREVIATION:ALAD#ECNUMBERS:4.2.1.24# proteins: NAME:aminolevulinate, delta-, dehydratase#ABBREVIATION:Alad.1# NAME:aminolevulinate, delta-, dehydratase#ABBREVIATION:Alad.2# citations: AUTHORS:Ishida N, Fujita H, Fukuda Y, Noguchi T, Doss M, Kappas A, Sassa S.#TITLE:Cloning and expression of the defective genes from a patient with delta-aminolevulinate dehydratase porphyria.#JOURNAL:J Clin Invest#YEAR:1992# GENE ASSOCIATION: (210.1) or (210.2)
Abbreviation: R_2HBO Synonyms: _0 EC Number: 1.1.1.27 SUBSYSTEM: Propanoate Metabolism Equation: [c] : 2hb + nad <==> 2obut + h + nadh Confidence Level: 4 NCD genes: LOCUS:_3945#TRANSCRIPT:1#ABBREVIATION:LDHB#ECNUMBERS:1.1.1.27# LOCUS:_3948#TRANSCRIPT:1#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_3939#TRANSCRIPT:1#ABBREVIATION:LDHA#ECNUMBERS:1.1.1.27# LOCUS:_160287#TRANSCRIPT:1#ABBREVIATION:LDHAL6A# LOCUS:_92483#TRANSCRIPT:1#ABBREVIATION:LDHAL6B# LOCUS:_3948#TRANSCRIPT:2#ABBREVIATION:LDHC#ECNUMBERS:1.1.1.27# LOCUS:_55293#TRANSCRIPT:1#ABBREVIATION:UEV3# proteins: NAME:lactate dehydrogenase A (liver, sk muscle)#ABBREVIATION:Ldha.1# NAME:lactate dehydrogenase B (heart, RBC)#ABBREVIATION:Ldhb.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.1# NAME:lactate dehydrogenase C (testis)#ABBREVIATION:Ldhc.2# NAME:lactate dehydrogenase A-like 6B (testis)#ABBREVIATION:Ldhal6b.1# NAME:lactate dehydrogenase A-like 6A#ABBREVIATION:Ldhal6a.1# NAME:ubiquitin-conjugating enzyme E2-like#ABBREVIATION:Uev3.1# NAME:lactate dehydrogenase (heart, RBC, brain, kidney, liver, sk muscle)#ABBREVIATION:LdhAB# citations: AUTHORS:Komoda T, Sakagishi Y, Mizushima H#TITLE:Determination of isoenzyme contents of lactic dehydrogenase activity and 2-hydroxybutyric dehydrogenase activity in lactic dehydrogenase preparations#JOURNAL:Clin Chim Acta#YEAR:1976#PUBMED:10108# AUTHORS:Naghizadeh F#TITLE:Oxidation of alpha-hydroxybutyrate by human serum#JOURNAL:Clin Chim Acta#YEAR:1977#PUBMED:21765# GENE ASSOCIATION: (92483.1) or (3948.2) or (55293.1) or (3945.1 and 3939.1) or (3939.1) or (160287.1) or (3945.1) or (3948.1)
Abbreviation: R_PPDOy Synonyms: _0 EC Number: 1.1.1.21 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : h + lald-D + nadph --> 12ppd-R + nadp Confidence Level: 4 NCD genes: LOCUS:_10327#TRANSCRIPT:1#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# LOCUS:_231#TRANSCRIPT:1#ABBREVIATION:AKR1B1#ECNUMBERS:1.1.1.21# LOCUS:_8574#TRANSCRIPT:1#ABBREVIATION:AKR7A2# LOCUS:_10327#TRANSCRIPT:2#ABBREVIATION:AKR1A1#ECNUMBERS:1.1.1.2# proteins: NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.1# NAME:aldo-keto reductase family 1, member a1#ABBREVIATION:Akr1a1.2# NAME:aldo-keto reductase family 1, member B1 (aldose reductase)#ABBREVIATION:Akr1b1.1# NAME:aldo-keto reductase family 7, member A2 (aflatoxin aldehyde reductase)#ABBREVIATION:Akr7a2.1# citations: AUTHORS:Vander Jagt DL, Robinson B, Taylor KK, Hunsaker LA.#TITLE:Reduction of trioses by NADPH-dependent aldo-keto reductases. Aldose reductase, methylglyoxal, and diabetic complications#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1537826# AUTHORS:Kelly VP, Sherratt PJ, Crouch DH, Hayes JD#TITLE:Novel homodimeric and heterodimeric rat gamma-hydroxybutyrate synthases that associate with the Golgi apparatus define a distinct subclass of aldo-keto reductase 7 family proteins.#JOURNAL:Biochem J#YEAR:2002#PUBMED:12071861# AUTHORS:Praml C, Savelyeva L, Perri P, Schwab M.#TITLE:Cloning of the human aflatoxin B1-aldehyde reductase gene at 1p35-1p36.1 in a region frequently altered in human tumor cells.#JOURNAL:Cancer Res#YEAR:1998#PUBMED:9823300# GENE ASSOCIATION: (8574.1) or (231.1) or (10327.2) or (10327.1)
Abbreviation: R_CPCTDTX Synonyms: _0 EC Number: 2.7.7.15 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : ctp + h + ntm2amep --> cmpntm2amep + ppi Confidence Level: 4 SAB genes: LOCUS:_5130#TRANSCRIPT:1#ABBREVIATION:PCYT1A# LOCUS:_9468#TRANSCRIPT:1#ABBREVIATION:PCYT1B#ECNUMBERS:2.7.7.15# proteins: NAME:phosphate cytidylyltransferase 1, choline, beta isoform#ABBREVIATION:Pcyt1b.1# NAME:phosphate cytidylyltransferase 1, choline, alpha isoform#ABBREVIATION:Pcyt1a.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Tan SA, Tan LG#TITLE:Distribution of ciliatine(2-aminoethylphosphonic acid) and phosphonalanine (2-amino-3-phosphonopropionic acid) in human tissues#JOURNAL:Clin Physiol Biochem#YEAR:1989# AUTHORS:Lykidis A, Murtis KG, Jackowski S#TITLE:Cloning and characterization of a second human CTP:Phosphocholine cytidylyltransferase#JOURNAL:Journal of Biological Chemistry#YEAR:1998# AUTHORS:Nakashima A, Hosaka K, Nikawa J#TITLE:Cloning of a human cDNA for CTP-Phosphoethanolamine cytidylyltransferase by complementation in vivo of a yeast mutant#JOURNAL:Journal of Biological Chemistry#YEAR:1997# GENE ASSOCIATION: (9468.1) or (5130.1)
Abbreviation: R_MG3er Synonyms: _1 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : g1m8masn + h2o --> glc-D + m8masn Confidence Level: 0 EC-3.2.1.- NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_din_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : din <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PCREATtmdiffir Synonyms: _0 SUBSYSTEM: Urea cycle/amino group metabolism Equation: pcreat[m] --> pcreat[c] Confidence Level: 2 SAB genes: none proteins: none citations: AUTHORS:Bessman SP, Carpenter CL#TITLE:The creatine-creatine phosphate energy shuttle#JOURNAL:Annu Rev Biochem#YEAR:1985#PUBMED:3896131# AUTHORS:Speer O, Neukomm LJ, Murphy RM, Zanolla E, Schlattner U, Henry H, Snow RJ, Wallimann T#TITLE:Creatine transporters: a reappraisal#JOURNAL:Mol Cell Biochem#YEAR:2004#PUBMED:14977199# TITLE:Biochemical pathways : an atlas of biochemistry and molecular biology #YEAR:1999# GENE ASSOCIATION:
Phosphocreatine transport to/from mitochondria via diffusion
Abbreviation: R_L_DASH_LACt4r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lac-L[e] + na1[e] <==> lac-L[c] + na1[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Poole RC, Halestrap AP#TITLE:Transport of lactate and other monocarboxylates across mammalian plasma membranes#JOURNAL:Am J Physiol#YEAR:1993#PUBMED:8476015# AUTHORS:Price NT, Jackson VN, Halestrap AP#TITLE:Cloning and sequencing of four new mammalian monocarboxylate transporter (MCT) homologues confirms the existence of a transporter family with an ancient past#JOURNAL:Biochem J#YEAR:1998#PUBMED:9425115# GENE ASSOCIATION:
L-lactate reversible transport via sodium symport
Abbreviation: R_EHGLAT2m Synonyms: _0 EC Number: 2.6.1.21 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : e4hglu + oaa --> 4h2oglt + asp-L Confidence Level: 2 NCD SAB oaa -> asp-L genes: LOCUS:_2806#TRANSCRIPT:1#ABBREVIATION:GOT2#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase, mitochondrial#ABBREVIATION:Got2.1-m# citations: AUTHORS:Takayama T, Fujita K, Suzuki K, Sakaguchi M, Fujie M, Nagai E, Watanabe S, Ichiyama A, Ogawa Y#TITLE:Control of oxalate formation from L-hydroxyproline in liver mitochondria#JOURNAL:J Am Soc Nephrol#YEAR:2003#PUBMED:12660328# AUTHORS:Knight J, Holmes RP#TITLE:Mitochondrial hydroxyproline metabolism: implications for primary hyperoxaluria#JOURNAL:Am J Nephrol#YEAR:2005#PUBMED:15849464# GENE ASSOCIATION: (2806.1)
L-erythro-4-Hydroxyglutamate:2-oxoglutarate aminotransferase 2, mitochondrial
Abbreviation: R_G14T20g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core4_pre6 + udpgal --> h + ksii_core4_pre7 + udp Confidence Level: 4 Keratan sulfate II biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
Abbreviation: R_SEASMETtn Synonyms: _0 SUBSYSTEM: Selenoamino acid metabolism Equation: seasmet[c] <==> seasmet[n] Confidence Level: 1 SAB based on yeast AMET transport genes: none proteins: none citations: none GENE ASSOCIATION:
Se-adenosyl-seleno-L-methionine nuclear transport
Abbreviation: R_KCC2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[e] + nh4[e] <==> cl[c] + nh4[c] Confidence Level: 4 NCD genes: LOCUS:_10723#TRANSCRIPT:1#ABBREVIATION:SLC12A7# LOCUS:_6560#TRANSCRIPT:1#ABBREVIATION:SLC12A4# LOCUS:_9990#TRANSCRIPT:1#ABBREVIATION:SLC12A6# proteins: NAME:solute carrier family 12 (potassium/chloride transporters), member 4#ABBREVIATION:Slc12a4.1-e# NAME:solute carrier family 12 (potassium/chloride transporters), member 6#ABBREVIATION:Slc12a6.1-e# NAME:solute carrier family 12 (potassium/chloride transporters), member 7#ABBREVIATION:Slc12a7.1-e# citations: AUTHORS:Gillen CM, Brill S, Payne JA, Forbush B 3rd#TITLE:Molecular cloning and functional expression of the K-Cl cotransporter from rabbit, rat, and human. A new member of the cation-chloride cotransporter family#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8663127# AUTHORS:Song L, Mercado A, Vazquez N, Xie Q, Desai R, George AL Jr, Gamba G, Mount DB#TITLE:Molecular, functional, and genomic characterization of human KCC2, the neuronal K-Cl cotransporter#JOURNAL:Brain Res Mol Brain Res#YEAR:2002#PUBMED:121106695# AUTHORS:Mount DB, Mercado A, Song L, Xu J, George AL Jr, Delpire E, Gamba G#TITLE:Cloning and characterization of KCC3 and KCC4, new members of the cation-chloride cotransporter gene family#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10347194# AUTHORS:Hiki K, D'Andrea RJ, Furze J, Crawford J, Woollatt E, Sutherland GR, Vadas MA, Gamble JR#TITLE:Cloning, characterization, and chromosomal location of a novel human K+-Cl- cotransporter#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10187864# AUTHORS:Race JE, Makhlouf FN, Logue PJ, Wilson FH, Dunham PB, Holtzman EJ#TITLE:Molecular cloning and functional characterization of KCC3, a new K-Cl cotransporter#JOURNAL:Am J Physiol#YEAR:1999#PUBMED:10600773# AUTHORS:Bergeron MJ, Gagnon E, Wallendorff B, Lapointe JY, Isenring P.#TITLE:Ammonium transport and pH regulation by K(+)-Cl(-) cotransporters#JOURNAL:Am J Physiol Renal Physiol#YEAR:2003#PUBMED:12657561# GENE ASSOCIATION: (6560.1) or (10723.1) or (9990.1)
Abbreviation: R_FAS180COA Synonyms: _0 EC Number: 2.3.1.86 SUBSYSTEM: Fatty acid elongation Equation: [c] : (3) h + malcoa + (2) nadph + pmtcoa --> co2 + coa + h2o + (2) nadp + stcoa Confidence Level: 4 NCD genes: LOCUS:_2194#TRANSCRIPT:1#ABBREVIATION:FASN#ECNUMBERS:2.3.1.85# proteins: NAME:fatty acid synthase#ABBREVIATION:Fasn.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Chakravarty B, Gu Z, Chirala SS, Wakil SJ, Quiocho FA.#TITLE:Human fatty acid synthase: structure and substrate selectivity of the thioesterase domain.#YEAR:2004#PUBMED:15507492# AUTHORS:Brink J, Ludtke SJ, Yang CY, Gu ZW, Wakil SJ, Chiu W.#TITLE:Quaternary structure of human fatty acid synthase by electron cryomicroscopy.#JOURNAL:Proc Natl Acad Sci#YEAR:2002#PUBMED:11756679# AUTHORS:Wakil SJ.#TITLE:Fatty acid synthase, a proficient multifunctional enzyme.#JOURNAL:Biochemistry#YEAR:1989#PUBMED:2669958# GENE ASSOCIATION: (2194.1)
Abbreviation: R_P5CRm Synonyms: _0 EC Number: 1.5.1.2 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : 1pyr5c + (2) h + nadph --> nadp + pro-L Confidence Level: 2 genes: LOCUS:_5831#TRANSCRIPT:1#ABBREVIATION:PYCR1#ECNUMBERS:1.5.1.2# LOCUS:_5831#TRANSCRIPT:2#ABBREVIATION:PYCR1#ECNUMBERS:1.5.1.2# proteins: NAME:pyrroline-5-carboxylate reductase 1#ABBREVIATION:Pycr1.1-m# NAME:pyrroline-5-carboxylate reductase 1#ABBREVIATION:Pycr1.2-m# citations: none GENE ASSOCIATION: (5831.1) or (5831.2)
Abbreviation: R_PTDCACRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: ptdcacrn[c] --> ptdcacrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (788.1)
Abbreviation: R_ALAt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + h[e] <==> ala-L[c] + h[c] Confidence Level: 2 NCD genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
L-alanine reversible transport via proton symport
Abbreviation: R_HSD3B13 Synonyms: _7 SUBSYSTEM: Steroid Metabolism Equation: [c] : 17ahprgnlone + nad --> 17ahprgstrn + h + nadh Confidence Level: 4 Microsomal rxn This is a conglomerated reaction - in actuality there is an intermediate: progen-5-ene-3,20,dione (C21H30O2) for pregnenolone 5-androstene-3,17-dione for dhea HSDB13 added by inference from Payne + Hales Endocrine Reviews (2004) 25(6):947-970 17ahprgnlone -> 17ahprgstrn: the catalysis of this reaction (3rd in HSD3 family that was added) was inferred from above paper NJ genes: LOCUS:_3283#TRANSCRIPT:1#ABBREVIATION:HSD3B1#ECNUMBERS:1.1.1.145,,5.3.# proteins: NAME:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 1#ABBREVIATION:Hsd3b1.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (3283.1)
Abbreviation: R_GLYtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: gly[c] <==> gly[x] Confidence Level: 2 TDV genes: none proteins: none citations: AUTHORS:Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP.#TITLE:Glycolate and glyoxylate metabolism in HepG2 cells.#YEAR:2004#PUBMED:15240345# GENE ASSOCIATION:
Abbreviation: R_RTOT_2 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : R2coa_hs + R4coa_hs --> (2) Rtotal2coa Confidence Level: 0 User defined, tissue specific fluxes that can be customized w/ various ratios of R1 through R5 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
aldehyde dehydrogenase (acetylaldehyde, NAD), mitochondrial
Abbreviation: R_ALDD2xm Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [m] : acald + h2o + nad --> ac + (2) h + nadh Confidence Level: 0 NCD genes: LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# citations: none GENE ASSOCIATION: (219.1) or (217.1)
Abbreviation: R_CITtam Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: cit[c] + mal-L[m] <==> cit[m] + mal-L[c] Confidence Level: 4 antiport with malate IF genes: LOCUS:_6576#TRANSCRIPT:1#ABBREVIATION:SLC25A1# proteins: NAME:solute carrier family 25 (mitochondrial carrier; citrate transporter), member 1 #ABBREVIATION:Slc25a1.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (6576.1)
Abbreviation: R_ASPNATm Synonyms: _2 EC Number: 2.3.1.17 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [m] : accoa + asp-L --> Nacasp + coa + h Confidence Level: 4 MM genes: none proteins: none citations: AUTHORS:Moreno A, Ross BD, Bluml S.#TITLE:Direct determination of the N-acetyl-L-aspartate synthesis rate in the human brain by (13)C MRS and [1-(13)C]glucose infusion.#YEAR:2001#PUBMED:11279290# GENE ASSOCIATION:
aspartate N-acetyltransferase, mitochondrial
Abbreviation: R_GSNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gsn[e] <==> gsn[c] Confidence Level: 4 IT genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (3177.1) or (2030.1)
guanosine facilated transport in cytosol
palmitoyl-CoA desaturase (n-C16:0CoA -> n-C16:1CoA)
Abbreviation: R_DESAT16_2 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + nadh + o2 + pmtcoa --> (2) h2o + hdcoa + nad Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_6319#TRANSCRIPT:1#ABBREVIATION:SCD# proteins: NAME:stearoyl-CoA desaturase#ABBREVIATION:Scd.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Zhang L, Ge L, Parimoo S, Stenn K, Prouty SM#TITLE:Human stearoyl-CoA desaturase: alternative transcripts generated from a single gene by usage of tandem polyadenylation sites#JOURNAL:Biochem J#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# AUTHORS:Wang Y, Kurdi-Haidar B, Oram JF.#TITLE:LXR-mediated activation of macrophage stearoyl-CoA desaturase generates unsaturated fatty acids that destabilize ABCA1.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:14967823# GENE ASSOCIATION: (6319.1)
Abbreviation: R_BMTer_U Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_U + memgacpail_hs --> (0.1) dolp_U + h + m2emgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_9488#TRANSCRIPT:1#ABBREVIATION:PIGB# proteins: NAME:phosphatidylinositol glycan, class B, endoplasmic reticulum#ABBREVIATION:Pigb.1-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Takahashi M, Inoue N, Ohishi K, Maeda Y, Nakamura N, Endo Y, Fujita T, Takeda J, Kinoshita T#TITLE:PIG-B, a membrane protein of the endoplasmic reticulum with a large lumenal domain, is involved in transferring the third mannose of the GPI anchor#JOURNAL:EMBO J#YEAR:1996#PUBMED:8861954# GENE ASSOCIATION: (9488.1)
Abbreviation: R_SIAASE3ly Synonyms: _4 EC Number: 3.2.1.18 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core2 --> acnam + ksii_core2_deg1 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_CERT2rt Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: gluside_hs[c] <==> gluside_hs[r] Confidence Level: 2 non-vesicular intracellular transport of glucocerebroside genes: LOCUS:_10087#TRANSCRIPT:1#ABBREVIATION:COL4A3BP# proteins: NAME:collagen, type IV, alpha 3 (Goodpasture antigen) binding protein#ABBREVIATION:Col4a3bp.1# citations: AUTHORS:Kumagai K, Yasuda S, Okemoto K, Nishijima M, Kobayashi S, Hanada K.#TITLE:CERT mediates intermembrane transfer of various molecular species of ceramides.#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15596449# AUTHORS:Hanada K, Kumagai K, Yasuda S, Miura Y, Kawano M, Fukasawa M, Nishijima M.#TITLE:Molecular machinery for non-vesicular trafficking of ceramide.#JOURNAL:Nature#YEAR:2003#PUBMED:14685229# AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# AUTHORS:van Meer G, Wolthoorn J, Degroote S.#TITLE:The fate and function of glycosphingolipid glucosylceramide.#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:2003#PUBMED:12803919# GENE ASSOCIATION: (10087.1)
Abbreviation: R_EX_2mcit_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 2mcit <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACHEe Synonyms: _1 EC Number: 3.1.1.7 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [e] : ach + h2o --> ac + chol + h Confidence Level: 4 NJ genes: LOCUS:_43#TRANSCRIPT:1#ABBREVIATION:ACHE#ECNUMBERS:3.1.1.7# LOCUS:_43#TRANSCRIPT:2#ABBREVIATION:ACHE#ECNUMBERS:3.1.1.7# proteins: NAME:acetylcholinesterase#ABBREVIATION:Ache.1# NAME:acetylcholinesterase#ABBREVIATION:Ache.2# citations: AUTHORS: Shafferman A, Kronman C, Flashner Y, Leitner M, Grosfeld H, Ordentlich A, Gozes Y, Cohen S, Ariel N, Barak D, et al.#TITLE:Mutagenesis of human acetylcholinesterase. Identification of residues involved in catalytic activity and in polypeptide folding.#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1517212# AUTHORS:Soreq H, Seidman S.#TITLE:Acetylcholinesterase--new roles for an old actor.#JOURNAL:Nat Rev Neurosci#YEAR:2001#PUBMED:11283752# GENE ASSOCIATION: (43.1) or (43.2)
Abbreviation: R_ST6GALNAC61 Synonyms: _6 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gd1a_hs --> cmp + gt1alpha_hs + h Confidence Level: 4 NJ genes: LOCUS:_30815#TRANSCRIPT:1#ABBREVIATION:SIAT7F# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 6 #ABBREVIATION:St6galnac6.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Harduin-Lepers A, Vallejo-Ruiz V, Krzewinski-Recchi MA, Smyn-Petit B, Julien S, Delannoy P#TITLE:The human sialyltransferase family#JOURNAL:Biochimie#YEAR:2001# GENE ASSOCIATION: (30815.1)
Abbreviation: R_GCHOLAt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gchola[e] + (2) na1[e] --> gchola[c] + (2) na1[c] Confidence Level: 4 electrogenic uptake NJ genes: LOCUS:_6555#TRANSCRIPT:1#ABBREVIATION:SLC10A2# LOCUS:_6554#TRANSCRIPT:1#ABBREVIATION:SLC10A1# proteins: NAME:solute carrier family 10 (sodium/bile acid cotransporter family), member 1#ABBREVIATION:Slc10a1.1# NAME:solute carrier family 10 (sodium/bile acid cotransporter family), member 2#ABBREVIATION:Slc10a2.1# citations: AUTHORS:Hagenbuch B, Dawson P.#TITLE:The sodium bile salt cotransport family SLC10.#JOURNAL:Pflugers Arch.#YEAR:2004#PUBMED:12851823# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:Molecular cloning, chromosomal localization, and functional characterization of a human liver Na+/bile acid cotransporter.#JOURNAL:J Clin Invest.#YEAR:1994#PUBMED:8132774# AUTHORS:Oelkers P, Kirby LC, Heubi JE, Dawson PA.#TITLE:Primary bile acid malabsorption caused by mutations in the ileal sodium-dependent bile acid transporter gene (SLC10A2).#JOURNAL:J Clin Invest.#YEAR:1997#PUBMED:9109432# AUTHORS:Wong MH, Oelkers P, Dawson PA.#TITLE:Identification of a mutation in the ileal sodium-dependent bile acid transporter gene that abolishes transport activity.#JOURNAL:J Biol Chem.#YEAR:1995#PUBMED:7592981# GENE ASSOCIATION: (6554.1) or (6555.1)
glycocholate transport via sodium cotransport
Abbreviation: R_FADH2tx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: fadh2[c] <==> fadh2[x] Confidence Level: 1 most likely NOT physiological - detailed information about the peroxisomal transporters has not yet been achieved - this transport is most likely dependent on a concomitant energy consuming reaction (human ABC peroxisomal transporters: PEX family - check in future for new research articles describing biochemistry and stoichiometry) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DLNLCGCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: dlnlcgcrn[c] --> dlnlcgcrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_S6T11g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre25 + paps --> h + ksi_pre26 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_DPGase Synonyms: _1 EC Number: 3.1.3.13 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : 23dpg + h2o --> 3pg + pi Confidence Level: 0 added EC num; also EC 5.4.2.4 -- NCD genes: LOCUS:_669#TRANSCRIPT:1#ABBREVIATION:BPGM#ECNUMBERS:5.4.2.4# LOCUS:_5224#TRANSCRIPT:1#ABBREVIATION:PGAM2#ECNUMBERS:5.4.2.1# LOCUS:_5223#TRANSCRIPT:1#ABBREVIATION:PGAM1#ECNUMBERS:5.4.2.1# LOCUS:_5224#TRANSCRIPT:2#ABBREVIATION:PGAM2#ECNUMBERS:5.4.2.1# LOCUS:_669#TRANSCRIPT:2#ABBREVIATION:BPGM#ECNUMBERS:5.4.2.4# proteins: NAME:brain phosphoglycerate mutase 1#ABBREVIATION:Pgam1.1# NAME:muscle phosphoglycerate mutase 2 (muscle)#ABBREVIATION:Pgam2.1# NAME:muscle phosphoglycerate mutase 2 (muscle)#ABBREVIATION:Pgam2.2# NAME:_2,3-bisphosphoglycerate mutase#ABBREVIATION:Bpgm.1# NAME:_2,3-bisphosphoglycerate mutase#ABBREVIATION:Bpgm.2# citations: none GENE ASSOCIATION: (669.2) or (5223.1) or (5224.2) or (5224.1) or (669.1)
Abbreviation: R_MAN1_7Ber Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : g1m8masn + h2o --> g1m7masnB + man Confidence Level: 0 NCD genes: LOCUS:_11253#TRANSCRIPT:1#ABBREVIATION:MAN1B1#ECNUMBERS:3.2.1.113# proteins: NAME:mannosidase, alpha, class 1B, member 1, endoplasmic reticulum (ubiquitous)#ABBREVIATION:Man1b1.1-er# citations: AUTHORS:Roth J, Zuber C, Guhl B, Fan JY, Ziak M.#TITLE:The importance of trimming reactions on asparagine-linked oligosaccharides for protein quality control.#JOURNAL:Histochem Cell Biol#YEAR:2002#PUBMED:11935292# AUTHORS:Gonzalez DS, Karaveg K, Vandersall-Nairn AS, Lal A, Moremen KW.#TITLE:Identification, expression, and characterization of a cDNA encoding human endoplasmic reticulum mannosidase I, the enzyme that catalyzes the first mannose trimming step in mammalian Asn-linked oligosaccharide biosynthesis.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10409699# GENE ASSOCIATION: (11253.1)
mannosidase I, endoplasmic reticulum (g1m7masnB-producing)
keratan sulfate I, degradation product 16_C160H258N11O137S7
Abbreviation: R_O2tn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: o2[c] <==> o2[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
S-Adenosyl-L-methionine:catechol O-methyltransferase
Abbreviation: R_SALMCOM Synonyms: _0 EC Number: 2.1.1.6 SUBSYSTEM: Tyrosine metabolism Equation: [c] : amet + nrpphr --> ahcys + h + normete-L Confidence Level: 1 SAB genes: LOCUS:_1312#TRANSCRIPT:1#ABBREVIATION:COMT#ECNUMBERS:2.1.1.6# LOCUS:_1312#TRANSCRIPT:2#ABBREVIATION:COMT#ECNUMBERS:2.1.1.6# LOCUS:_118881#TRANSCRIPT:1#ABBREVIATION:COMTD1# proteins: NAME:catechol-O-methyltransferase#ABBREVIATION:Comt.1# NAME:catechol-O-methyltransferase#ABBREVIATION:Comt.2# NAME:catechol-O-methyltransferase domain containing 1#ABBREVIATION:Comtd1.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (1312.1) or (118881.1) or (1312.2)
Abbreviation: R_B3GNT313g Synonyms: _9 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acgalfucgalacglcgal14acglcgalgluside_hs + udpgal --> galacgalfucgalacglcgal14acglcgalgluside_hs + h + udp Confidence Level: 2 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3#ABBREVIATION:B3gnt3.1# citations: AUTHORS:Yokoyama-Kobayashi M, Yamaguchi T, Sekine S, Kato S.#TITLE:Selection of cDNAs encoding putative type II membrane proteins on the cell surface from a human full-length cDNA bank.#JOURNAL:Gene#YEAR:1999#PUBMED:10072769# GENE ASSOCIATION: (10331.1)
Abbreviation: R_EX_pchol_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pchol_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Phosphatidylcholine (homo sapiens) exchange
Abbreviation: R_GK1 Synonyms: _0 EC Number: 2.7.4.8 SUBSYSTEM: Nucleotides Equation: [c] : atp + gmp <==> adp + gdp Confidence Level: 4 genes: LOCUS:_2987#TRANSCRIPT:1#ABBREVIATION:GUK1#ECNUMBERS:2.7.4.8# proteins: NAME:guanylate kinase#ABBREVIATION:Guk1.1# citations: AUTHORS:Brady WA, Kokoris MS, Fitzgibbon M, Black ME. #TITLE:Cloning, characterization, and modeling of mouse and human guanylate kinases.#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8663313# GENE ASSOCIATION: (2987.1)
Abbreviation: R_ADNK1 Synonyms: _0 EC Number: 2.7.1.20 SUBSYSTEM: Nucleotides Equation: [c] : adn + atp --> adp + amp + h Confidence Level: 4 _2-Aminoadenosine can also act as acceptor genes: LOCUS:_132#TRANSCRIPT:1#ABBREVIATION:ADK#ECNUMBERS:2.7.1.20# LOCUS:_132#TRANSCRIPT:2#ABBREVIATION:ADK#ECNUMBERS:2.7.1.20# proteins: NAME:adenosine kinase #ABBREVIATION:Adk.1# NAME:adenosine kinase#ABBREVIATION:Adk.2# citations: AUTHORS:McNally T, Helfrich RJ, Cowart M, Dorwin SA, Meuth JL, Idler KB, Klute KA, Simmer RL, Kowaluk EA, Halbert DN. #TITLE:Cloning and expression of the adenosine kinase gene from rat and human tissues.#JOURNAL:Biochem Biophys Res Commun#YEAR:1997#PUBMED:9070863# GENE ASSOCIATION: (132.1) or (132.2)
Abbreviation: R_HBZOPT10m Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 4hbz + decdp --> 3dphb + ppi Confidence Level: 4 Used to make Ubiquinone-10 EC 2.5.1.- IT genes: LOCUS:_27235#TRANSCRIPT:1#ABBREVIATION:COQ2# proteins: NAME:para-hydroxybenzoate - polyprenyltransferase, mitochondrial #ABBREVIATION:Coq2.1-m# citations: AUTHORS:Forsgren M, Attersand A, Lake S, Grunler J, Swiezewska E, Dallner G, Climent I.#TITLE:Isolation and functional expression of human COQ2, a gene encoding a polyprenyl transferase involved in the synthesis of CoQ. #JOURNAL:Biochem J.#YEAR:2004#PUBMED:15153069# AUTHORS:Kang DC, Takeshige K, Minakami S.#TITLE:An intermediate of ubiquinone biosynthesis exists in the microsomal fraction of HepG2 cells. #JOURNAL:J Biochem (Tokyo).#YEAR:1990#PUBMED:1965190# AUTHORS:Nambudiri AM, Ranganathan S, Rudney H#TITLE:The role of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity in the regulation of ubiquinone synthesis in human fibroblasts. #JOURNAL:J Biol Chem.#YEAR:1980#PUBMED:7380842# GENE ASSOCIATION: (27235.1)
Abbreviation: R_MALtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: mal-L[c] + pi[m] <==> mal-L[m] + pi[c] Confidence Level: 4 IF genes: LOCUS:_1468#TRANSCRIPT:1#ABBREVIATION:SLC25A10# proteins: NAME:Solute carrier family 25 (dicarboxylate transporter) member 10, mitochondrial #ABBREVIATION:Slc25a10.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (1468.1)
Abbreviation: R_2AMACSULT Synonyms: _1 SUBSYSTEM: Cysteine Metabolism Equation: [c] : 2amac + nadph + paps --> Lcyst + nadp + pap Confidence Level: 2 -this is a lumped rxn w/ an inferred reduction rxn that occurs in conjunction w/ sulfotransferase step; this reduction step has to occur in order for the transformation (2amac-->Lcyst) to be valid -used NADPH as electron donor since it is a common cofactor involved in biosynthetic steps utilizing oxygen (see Neema for further explanations) MM genes: none proteins: none citations: AUTHORS:Cooper AJ#TITLE:Biochemistry of sulfur-containing amino acids.#YEAR:1983#PUBMED:6351723# AUTHORS:Weinstein CL, Griffith OW.#TITLE:Cysteinesulfonate and beta-sulfopyruvate metabolism. Partitioning between decarboxylation, transamination, and reduction pathways.#YEAR:1988#PUBMED:3346220# GENE ASSOCIATION:
Abbreviation: R_PI45P5Pn Synonyms: _0 EC Number: 3.1.3.36 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail45p_hs --> pail4p_hs + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
phosphatidylinositol-4,5-bisphosphate 5-phosphatase, nuclear
Abbreviation: R_GLNtN1 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gln-L[e] + h[c] + (2) na1[e] <==> gln-L[c] + h[e] + (2) na1[c] Confidence Level: 4 SAB based om PMID 10823827 genes: LOCUS:_92745#TRANSCRIPT:1#ABBREVIATION:SLC38A5# LOCUS:_10991#TRANSCRIPT:1#ABBREVIATION:SLC38A3# proteins: NAME:solute carrier family 38, member 3#ABBREVIATION:Slc38a3.1# NAME:solute carrier family 38, member 5#ABBREVIATION:Slc38a5.1# citations: AUTHORS:Fei YJ, Sugawara M, Nakanishi T, Huang W, Wang H, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, genomic organization, and functional and electrogenic characteristics of human system N 1, a Na+- and H+-coupled glutamine transporter#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10823827# AUTHORS:Mackenzie B, Erickson JD#TITLE:Sodium-coupled neutral amino acid (System N/A) transporters of the SLC38 gene family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12845534# GENE ASSOCIATION: (92745.1) or (10991.1)
Abbreviation: R_C160CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + pmtcoa --> coa + pmtcrn Confidence Level: 4 genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (126129.1) or (1375.3) or (1375.1) or (1375.2) or (1374.1) or (1375.4)
Abbreviation: R_MGCHrm Synonyms: _0 EC Number: 4.2.1.18 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 3mgcoa + h2o <==> hmgcoa Confidence Level: 4 MM genes: LOCUS:_549#TRANSCRIPT:1#ABBREVIATION:AUH# proteins: NAME:AU RNA binding protein/enoyl-Coenzyme A hydratase (mito)#ABBREVIATION:Auh.1-m# citations: AUTHORS:IJlst L, Loupatty FJ, Ruiter JP, Duran M, Lehnert W, Wanders RJ. #TITLE:_3-Methylglutaconic aciduria type I is caused by mutations in AUH.#YEAR:2003#PUBMED:12434311# AUTHORS:Narisawa K, Gibson KM, Sweetman L, Nyhan WL, Duran M, Wadman SK. #TITLE:Deficiency of 3-methylglutaconyl-coenzyme A hydratase in two siblings with 3-methylglutaconic aciduria.#YEAR:1986#PUBMED:3082934# GENE ASSOCIATION: (549.1)
methylglutaconyl-CoA hydratase (reversible), mitochondrial
Abbreviation: R_PROD2m Synonyms: _0 EC Number: 1.5.99.8 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : fad + pro-L --> 1pyr5c + fadh2 + h Confidence Level: 2 JLR SAB m genes: LOCUS:_5625#TRANSCRIPT:1#ABBREVIATION:PRODH# proteins: NAME:proline dehydrogenase (oxidase) 1#ABBREVIATION:Prodh.1-m# citations: AUTHORS:Gogos JA, Santha M, Takacs Z, Beck KD, Luine V, Lucas LR, Nadler JV, Karayiorgou M.#TITLE:The gene encoding proline dehydrogenase modulates sensorimotor gating in mice. #JOURNAL:Nat Genet#YEAR:1999#PUBMED:10192398# GENE ASSOCIATION: (5625.1)
Abbreviation: R_PTDCACRNCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + ptdcacoa --> coa + ptdcacrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (1375.1) or (1375.4) or (1375.3) or (1374.1) or (126129.1) or (1375.2)
Abbreviation: R_GPAM_hs Synonyms: _1 EC Number: 2.3.1.15 SUBSYSTEM: Triacylglycerol Synthesis Equation: [c] : Rtotalcoa + glyc3p --> alpa_hs + coa Confidence Level: 4 NJ genes: LOCUS:_57678#TRANSCRIPT:1#ABBREVIATION:KIAA1560# proteins: NAME:glycerol 3-phosphate acyltransferase, mitochondrial#ABBREVIATION:Gpam.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Coleman RA, Lee DP#TITLE:Enzymes of triacylglycerol synthesis and their regulation#JOURNAL:Prog in Lipid Research#YEAR:2004# GENE ASSOCIATION: (57678.1)
Abbreviation: R_FACOAL204 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : arachd + atp + coa <==> amp + arachdcoa + ppi Confidence Level: 4 rev version so additional rxn doesn't need to be added to "unload" AA NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_EX_adprib_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : adprib <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
heparan sulfate, precursor 7_C73H109N4O58X
Abbreviation: R_PMI12346PH Synonyms: _0 EC Number: 3.6.1.52 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + ppmi12346p --> h + minohp + pi Confidence Level: 4 NCD genes: LOCUS:_9807#TRANSCRIPT:1#ABBREVIATION:IHPK1# LOCUS:_117283#TRANSCRIPT:1#ABBREVIATION:IHPK3# LOCUS:_9807#TRANSCRIPT:2#ABBREVIATION:IHPK1# proteins: NAME:inositol hexaphosphate kinase 1#ABBREVIATION:Ihpk1.1# NAME:inositol hexaphosphate kinase 1#ABBREVIATION:Ihpk1.2# NAME:inositol hexaphosphate kinase 3#ABBREVIATION:Ihpk3.1# citations: AUTHORS:Saiardi A, Nagata E, Luo HR, Snowman AM, Snyder SH#TITLE:Identification and characterization of a novel inositol hexakisphosphate kinase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11502751# GENE ASSOCIATION: (117283.1) or (9807.2) or (9807.1)
_5-diphosphoinositol-1,2,3,4,6-pentakisphosphate diphosphohydrolase
Abbreviation: R_UPPN Synonyms: _0 EC Number: 3.5.1.6 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : cala + (2) h + h2o --> ala-B + co2 + nh4 Confidence Level: 4 SAB balanced genes: LOCUS:_51733#TRANSCRIPT:1#ABBREVIATION:UPB1# proteins: NAME:ureidopropionase, beta#ABBREVIATION:Upb1.1# citations: AUTHORS:Vreken P, van Kuilenburg AB, Hamajima N, Meinsma R, van Lenthe H, Gohlich-Ratmann G, Assmann BE, Wevers RA, van Gennip AH#TITLE:cDNA cloning, genomic structure and chromosomal localization of the human BUP-1 gene encoding beta-ureidopropionase#JOURNAL:Biochim Biophys Acta#YEAR:1999#PUBMED:10542323# AUTHORS:Kuhara T#TITLE:Diagnosis and monitoring of inborn errors of metabolism using urease-pretreatment of urine, isotope dilution, and gas chromatography-mass spectrometry#JOURNAL:J Chromatogr B Analyt Technol Biomed Life Sci#YEAR:2002#PUBMED:12450676# GENE ASSOCIATION: (51733.1)
Abbreviation: R_FUC14GALACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fuc14galacglcgalgluside_hs[e] <==> fuc14galacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NTP3e Synonyms: _0 EC Number: 3.6.1.15 SUBSYSTEM: Nucleotides Equation: [e] : gtp + h2o --> gdp + h + pi Confidence Level: 4 genes: LOCUS:_124583#TRANSCRIPT:1#ABBREVIATION:CANT1# proteins: NAME:calcium activated nucleotidase 1 #ABBREVIATION:Cant1.1-e# citations: AUTHORS:Smith TM, Hicks-Berger CA, Kim S, Kirley TL.#TITLE:Cloning, expression, and characterization of a soluble calcium-activated nucleotidase, a human enzyme belonging to a new family of extracellular nucleotidases.#JOURNAL:Arch Biochem Biophys. #YEAR:2002#PUBMED:12234496# AUTHORS:Murphy DM, Ivanenkov VV, Kirley TL. #TITLE:Bacterial expression and characterization of a novel, soluble, calcium-binding, and calcium-activated human nucleotidase. #JOURNAL:Biochemistry#YEAR:2003#PUBMED:12600208# GENE ASSOCIATION: (124583.1)
Abbreviation: R_SPRMS Synonyms: _2 EC Number: 2.5.1.22 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : ametam + spmd --> 5mta + h + sprm Confidence Level: 4 NCD genes: LOCUS:_6611#TRANSCRIPT:1#ABBREVIATION:SMS#ECNUMBERS:2.5.1.22# proteins: NAME:spermine synthase#ABBREVIATION:Sms.1# citations: AUTHORS:Korhonen VP, Halmekyto M, Kauppinen L, Myohanen S, Wahlfors J, Keinanen T, Hyvonen T, Alhonen L, Eloranta T, Janne J#TITLE:Molecular cloning of a cDNA encoding human spermine synthase#JOURNAL:DNA Cell Biol#YEAR:1995#PUBMED:7546290# GENE ASSOCIATION: (6611.1)
Abbreviation: R_GALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: galgluside_hs[c] <==> galgluside_hs[g] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MM7Cag Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m7masnA --> m6masnC + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
Abbreviation: R_GLNLASEer Synonyms: _4 EC Number: 3.1.1.17 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [r] : guln + h <==> gullac + h2o Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Banhegyi G, Braun L, Csala M, Puskas F, Mandl J#TITLE:Ascorbate metabolism and its regulation in animals#JOURNAL:Free Radic Biol Med#YEAR:1997#PUBMED:9296457# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_EX_xylt_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : xylt <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PI345P5Pn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail345p_hs --> pail34p_hs + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
phosphatidylinositol-3,4,5-trisphosphate 5-phosphatase, nuclear
Abbreviation: R_EX_gal_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gal <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NAIt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: i[e] + (2) na1[e] <==> i[c] + (2) na1[c] Confidence Level: 4 NCD genes: LOCUS:_6528#TRANSCRIPT:1#ABBREVIATION:SLC5A5# proteins: NAME:solute carrier family 5 (sodium iodide symporter), member 5, extacellular#ABBREVIATION:Slc5a5.1-e# citations: AUTHORS:Leung DW, Loo DD, Hirayama BA, Zeuthen T, Wright EM#TITLE:Urea transport by cotransporters#JOURNAL:J Physiol#YEAR:2000#PUBMED:11034615# AUTHORS:Wright EM, Turk E#TITLE:The sodium/glucose cotransport family SLC5.#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12748858# AUTHORS:Wapnir IL, van de Rijn M, Nowels K, Amenta PS, Walton K, Montgomery K, Greco RS, Dohan O, Carrasco N#TITLE:Immunohistochemical profile of the sodium/iodide symporter in thyroid, breast, and other carcinomas using high density tissue microarrays and conventional sections#JOURNAL:J Clin Endocrinol Metab#YEAR:2003#PUBMED:12679487# AUTHORS:Dai G, Levy O, Carrasco N#TITLE:Cloning and characterization of the thyroid iodide transporter#JOURNAL:Nature#YEAR:1996#PUBMED:8559252# AUTHORS:Smanik PA, Liu Q, Furminger TL, Ryu K, Xing S, Mazzaferri EL, Jhiang SM#TITLE:Cloning of the human sodium lodide symporter#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8806637# AUTHORS:De La Vieja A, Dohan O, Levy O, Carrasco N#TITLE:Molecular analysis of the sodium/iodide symporter: impact on thyroid and extrathyroid pathophysiology#JOURNAL:Physiol Rev#YEAR:2000#PUBMED:10893432# AUTHORS:Eskandari S, Loo DD, Dai G, Levy O, Wright EM, Carrasco N.#TITLE:Thyroid Na+/I- symporter. Mechanism, stoichiometry, and specificity#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9341168# AUTHORS:Wright EM, Loo DD, Hirayama BA, Turk E#TITLE:Surprising versatility of Na+-glucose cotransporters: SLC5#JOURNAL:Physiology (Bethesda)#YEAR:2004#PUBMED:15546855# GENE ASSOCIATION: (6528.1)
Abbreviation: R_EX_whtststerone_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : whtststerone <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
L-Lactate dehydrogenase, cytosolic/mitochondrial
Abbreviation: R_L_DASH_LACDcm Synonyms: _0 EC Number: 1.1.2.3 SUBSYSTEM: Pyruvate Metabolism Equation: (2) ficytC[m] + lac-L[c] --> (2) focytC[m] + (2) h[c] + pyr[c] Confidence Level: 2 NCD/TDV genes: LOCUS:_124637#TRANSCRIPT:1#ABBREVIATION:FLJ32499# proteins: NAME:hypothetical protein FLJ32499, mitochondrial#ABBREVIATION:Flj32499.1-m# citations: none GENE ASSOCIATION: (124637.1)
Lactosylceramide alpha-2,3-sialyltransferase, Golgi apparatus
Abbreviation: R_SIAT9g Synonyms: _4 EC Number: 2.4.99.9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + galgluside_hs --> cmp + gm3_hs + h Confidence Level: 4 NJ genes: LOCUS:_8869#TRANSCRIPT:1#ABBREVIATION:SIAT9#ECNUMBERS:2.4.99.9# proteins: NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase 5#ABBREVIATION:St3gal5.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Ishii A, Ohta M, Watanabe Y, Matsuda K, Ishiyama K, Sakoe K, Nakamura M, Inokuchi J, Sanai Y, Saito M.#TITLE:Expression cloning and functional characterization of human cDNA for ganglioside GM3 synthase.#JOURNAL:J Biol Chem.#YEAR:1998#PUBMED:9822625# GENE ASSOCIATION: (8869.1)
Abbreviation: R_G14T17g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre35 + udpgal --> h + ksi_pre36 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (8704.2) or (8704.1) or (2683.1) or (9334.1)
Abbreviation: R_CYSGLTH Synonyms: _1 EC Number: 1.8.4.4 SUBSYSTEM: Cysteine Metabolism Equation: [c] : Lcystin + (2) gthrd <==> (2) cys-L + gthox Confidence Level: 4 reaction according to KEGG MM genes: none proteins: none citations: AUTHORS:Stipanuk MH#TITLE:Sulfur amino acid metabolism: pathways for production and removal of homocysteine and cysteine#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15189131# AUTHORS:Oshima RG, Rhead WJ, Thoene JG, Schneider JA.#TITLE:Cystine metabolism in human fibroblasts. Comparison of normal, cystinotic, and gamma-glutamylcysteine synethetase-deficient cells.#YEAR:1976#PUBMED:932033# GENE ASSOCIATION:
retinyl ester hydrolase (11-cis), extracellular
Abbreviation: R_RETH2e Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [e] : 11-cis-retfa + h2o --> Rtotal2 + h + retinol-cis-11 Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
L-Tryptophan,tetrahydrobiopterin:oxygen oxidoreductase (5-hydroxylating)
Abbreviation: R_TRPHYDRO2 Synonyms: _1 EC Number: 1.14.16.4 SUBSYSTEM: Tryptophan metabolism Equation: [c] : o2 + thbpt + trp-L --> 5htrp + thbpt4acam Confidence Level: 4 SAB unlumped version of TRPHYDRO1 genes: LOCUS:_7166#TRANSCRIPT:1#ABBREVIATION:TPH1#ECNUMBERS:1.14.16.4# LOCUS:_121278#TRANSCRIPT:1#ABBREVIATION:TPH2# proteins: NAME:tryptophan hydroxylase 1 (tryptophan 5-monooxygenase)#ABBREVIATION:Tph1.1# NAME:tryptophan hydroxylase 2#ABBREVIATION:Tph2.1# citations: AUTHORS:Wang L, Erlandsen H, Haavik J, Knappskog PM, Stevens RC#TITLE:Three-dimensional structure of human tryptophan hydroxylase and its implications for the biosynthesis of the neurotransmitters serotonin and melatonin#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12379098# AUTHORS:Thony B, Auerbach G, Blau N.#TITLE:Tetrahydrobiopterin biosynthesis, regeneration and functions.#JOURNAL:Biochem J#YEAR:2000#PUBMED:10727395# GENE ASSOCIATION: (7166.1) or (121278.1)
Abbreviation: R_CHOLATEt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cholate[e] + (2) na1[e] --> cholate[c] + (2) na1[c] Confidence Level: 4 electrogenic uptake NJ genes: LOCUS:_6555#TRANSCRIPT:1#ABBREVIATION:SLC10A2# LOCUS:_6554#TRANSCRIPT:1#ABBREVIATION:SLC10A1# proteins: NAME:solute carrier family 10 (sodium/bile acid cotransporter family), member 1#ABBREVIATION:Slc10a1.1# NAME:solute carrier family 10 (sodium/bile acid cotransporter family), member 2#ABBREVIATION:Slc10a2.1# citations: AUTHORS:Hagenbuch B, Dawson P.#TITLE:The sodium bile salt cotransport family SLC10.#JOURNAL:Pflugers Arch.#YEAR:2004#PUBMED:12851823# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:Molecular cloning, chromosomal localization, and functional characterization of a human liver Na+/bile acid cotransporter.#JOURNAL:J Clin Invest.#YEAR:1994#PUBMED:8132774# AUTHORS:Oelkers P, Kirby LC, Heubi JE, Dawson PA.#TITLE:Primary bile acid malabsorption caused by mutations in the ileal sodium-dependent bile acid transporter gene (SLC10A2).#JOURNAL:J Clin Invest.#YEAR:1997#PUBMED:9109432# AUTHORS:Wong MH, Oelkers P, Dawson PA.#TITLE:Identification of a mutation in the ileal sodium-dependent bile acid transporter gene that abolishes transport activity.#JOURNAL:J Biol Chem.#YEAR:1995#PUBMED:7592981# GENE ASSOCIATION: (6554.1) or (6555.1)
cholate transport via sodium cotransport
Abbreviation: R_ACONTm Synonyms: _0 EC Number: 4.2.1.3 SUBSYSTEM: Citric Acid Cycle Equation: [m] : cit <==> icit Confidence Level: 0 IF genes: LOCUS:_50#TRANSCRIPT:1#ABBREVIATION:ACO2#ECNUMBERS:4.2.1.3# proteins: NAME:aconitase 2, mitochondrial#ABBREVIATION:Aco2.1-m# citations: none GENE ASSOCIATION: (50.1)
Abbreviation: R_EX_glyc_DASH_S_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : glyc-S <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CSPG_Etly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: cspg_e[e] --> cspg_e[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate E transport, extracellular to lysosome
Abbreviation: R_P4504F123r Synonyms: _0 SUBSYSTEM: CYP Metabolism Equation: [r] : ebastine + h + nadph + o2 --> ebastineoh + h2o + nadp Confidence Level: 4 omega hydroxylation of ebastine NJ genes: LOCUS:_66002#TRANSCRIPT:1#ABBREVIATION:CYP4F12# proteins: NAME:cytochrome P450, family 4, subfamily F, polypeptide 12#ABBREVIATION:Cyp4f12.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# GENE ASSOCIATION: (66002.1)
Abbreviation: R_SERHL Synonyms: _0 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : ser-L --> 2amac + h2o Confidence Level: 4 MM genes: LOCUS:_10993#TRANSCRIPT:1#ABBREVIATION:SDS# proteins: NAME:serine dehydratase#ABBREVIATION:Sds.1# citations: AUTHORS:Ogawa H, Gomi T, Konishi K, Date T, Nakashima H, Nose K, Matsuda Y, Peraino C, Pitot HC, Fujioka M. #TITLE:Human liver serine dehydratase. cDNA cloning and sequence homology with hydroxyamino acid dehydratases from other sources. #YEAR:1989#PUBMED:2674117# GENE ASSOCIATION: (10993.1)
Abbreviation: R_25HVITD2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _25hvitd2[c] --> 25hvitd2[e] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_25-hydroxyvitamin D2 transport from cytoplasm
Abbreviation: R_DRIBt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: drib[e] <==> drib[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Brown NS, Bicknell R.#TITLE:Thymidine phosphorylase, 2-deoxy-D-ribose and angiogenesis#JOURNAL:Biochem J#YEAR:1998#PUBMED:9693094# GENE ASSOCIATION:
Abbreviation: R_NRVNCCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: nrvnccoa[c] <==> nrvnccoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_25HVITD2tin Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _25hvitd2[e] --> 25hvitd2[c] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_25-hydroxyvitamin D2 transport in cytoplasm
Abbreviation: R_PAPStg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: paps[c] <==> paps[g] Confidence Level: 4 NCD genes: LOCUS:_347734#TRANSCRIPT:1#ABBREVIATION:SLC35B2# proteins: NAME:solute carrier family 35, member B2, Golgi apparatus#ABBREVIATION:Slc35b2.1-g# citations: AUTHORS:Kamiyama S, Suda T, Ueda R, Suzuki M, Okubo R, Kikuchi N, Chiba Y, Goto S, Toyoda H, Saigo K, Watanabe M, Narimatsu H, Jigami Y, Nishihara S#TITLE:Molecular cloning and identification of 3'-phosphoadenosine 5'-phosphosulfate transporter#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12716889# GENE ASSOCIATION: (347734.1)
_3'-Phosphoadenylyl sufate Golgi transport
Abbreviation: R_XOLTRI24tc Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: xoltri24[r] <==> xoltri24[c] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_ac_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ac <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UNK2 Synonyms: _0 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : 2kmb + gln-L + h --> glu-L + met-L Confidence Level: 2 JEM has this reaction as TNSUNK genes: none proteins: none citations: AUTHORS:Myers RW, Abeles RH#TITLE:Conversion of 5-S-ethyl-5-thio-D-ribose to ethionine in Klebsiella pneumoniae. Basis for the selective toxicity of 5-S-ethyl-5-thio-D-ribose#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2543672# GENE ASSOCIATION:
Abbreviation: R_NTD1 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : dump + h2o --> duri + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_30833#TRANSCRIPT:1#ABBREVIATION:NT5C#ECNUMBERS:3.1.3.5# proteins: NAME:_5', 3'-nucleotidase, cytosolic #ABBREVIATION:Nt5C.1# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION: (30833.1)
Abbreviation: R_XSERtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: xser[r] --> xser[g] Confidence Level: 1 immunocytochemistry, subcellular fractionation, and electron microscopy have demonstrated that xylosylation begins in the ER and continues in the early Golgi [Kearns, J Biol Chem 1993] PMID: 8496172 NCD genes: none proteins: none citations: AUTHORS:Kearns AE, Vertel BM, Schwartz NB#TITLE:Topography of glycosylation and UDP-xylose production#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8496172# GENE ASSOCIATION:
Xyl-L-Ser (protein) transport from endoplasmic reticulum to Golgi apparatus
Abbreviation: R_DM_sTn_antigen_LPAREN_g_RPAREN_ Synonyms: _0 Equation: [g] : sTn_antigen --> Confidence Level: NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CYTK11n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : dcmp + dgtp <==> dcdp + dgdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_3MOPt2im Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _3mop[c] + h[c] --> 3mop[m] + h[m] Confidence Level: 4 MM genes: none proteins: none citations: AUTHORS:Hutson SM, Hall TR.#TITLE:Identification of the mitochondrial branched chain aminotransferase as a branched chain alpha-keto acid transport protein.#YEAR:1993#PUBMED:8428987# GENE ASSOCIATION:
_3-Methyl-2-oxopentanoate mitochondrial transport via proton symport
Abbreviation: R_PPPItn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: pppi[c] <==> pppi[n] Confidence Level: 2 it genes: none proteins: none citations: none GENE ASSOCIATION:
Inorganic triphosphate transport through nuclear pore
Abbreviation: R_STRDNCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: strdnc[e] <==> strdnc[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_GLBRAN Synonyms: _0 EC Number: 2.4.1.18 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : glygn1 --> glygn2 Confidence Level: 4 NCD genes: LOCUS:_2632#TRANSCRIPT:1#ABBREVIATION:GBE1#ECNUMBERS:2.4.1.18# proteins: NAME: glucan (1,4-alpha-), branching enzyme 1 (glycogen branching enzyme, Andersen disease, glycogen storage disease type IV) #ABBREVIATION:Gbe1.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Thon VJ, Khalil M, Cannon JF#TITLE:Isolation of human glycogen branching enzyme cDNAs by screening complementation in yeast.#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8463281# GENE ASSOCIATION: (2632.1)
_1,4-alpha-glucan branching enzyme (glygn1 -> glygn2)
Abbreviation: R_G1M7MASNCterg Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: g1m7masnC[r] --> g1m7masnC[g] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# GENE ASSOCIATION:
Abbreviation: R_DMANTIPYRINEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dmantipyrine[e] <==> dmantipyrine[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GGH_DASH_10FTHF7GLUe Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [e] : 10fthf7glu + h2o --> 10fthf6glu + glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-e# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (10FTHF7GLU), extracellular
Abbreviation: R_PI4P4Pn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail4p_hs --> pail_hs + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
phosphatidylinositol-4-phosphate 4-phosphatase, nuclear
Abbreviation: R_CSPG_Dtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: cspg_d[e] --> cspg_d[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate D transport, extracellular to lysosome
Abbreviation: R_NADPNe Synonyms: _0 EC Number: 3.2.2.6 SUBSYSTEM: NAD Metabolism Equation: [e] : h2o + nadp --> adprbp + h + ncam Confidence Level: 4 See EC 3.2.2.5 for NAD nucleosidase genes: LOCUS:_952#TRANSCRIPT:1#ABBREVIATION:CD38# proteins: NAME:cyclic ADP-ribose hydrolase#ABBREVIATION:Cadph.1# citations: AUTHORS:Kim UH, Han MK, Park BH, Kim HR, An NH.#TITLE:Function of NAD glycohydrolase in ADP-ribose uptake from NAD by human erythrocytes.#JOURNAL:Biochim Biophys Acta.#YEAR:1993#PUBMED:8394137# AUTHORS:Kontani K, Nishina H, Ohoka Y, Takahashi K, Katada T.#TITLE:NAD glycohydrolase specifically induced by retinoic acid in human leukemic HL-60 cells. Identification of the NAD glycohydrolase as leukocyte cell surface antigen CD38.#JOURNAL:J Biol Chem#YEAR:1993# GENE ASSOCIATION: (952.1)
Abbreviation: R_ALOX52 Synonyms: _2 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : 5HPET --> h2o + leuktrA4 Confidence Level: 4 cofactors + usage still not clear unknown mechanism - biochemistry supported by PMID: 2829172, 11160764 genes: LOCUS:_240#TRANSCRIPT:1#ABBREVIATION:ALOX5#ECNUMBERS:1.13.11.34# proteins: NAME:arachidonate 5-lipoxygenase#ABBREVIATION:Alox5.1# citations: TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Leff AR.#TITLE:Regulation of leukotrienes in the management of asthma: biology and clinical therapy.#JOURNAL:Annu Rev Med#YEAR:2001#PUBMED:11160764# GENE ASSOCIATION: (240.1)
Abbreviation: R_MM5cg Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m5masnB2 --> m4masn + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (10905.1) or (4121.1) or (57134.1)
Abbreviation: R_H6_APOS_ETer Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : em3gacpail_hs + pe_hs --> dag_hs + em2emgacpail_hs Confidence Level: 2 NCD genes: LOCUS:_23556#TRANSCRIPT:1#ABBREVIATION:PIGN# LOCUS:_23556#TRANSCRIPT:2#ABBREVIATION:PIGN# proteins: NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.1-r# NAME:phosphatidylinositol glycan, class N, endoplasmic reticulum#ABBREVIATION:Pign.2-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Hong Y, Maeda Y, Watanabe R, Ohishi K, Mishkind M, Riezman H, Kinoshita T#TITLE:Pig-n, a mammalian homologue of yeast Mcd4p, is involved in transferring phosphoethanolamine to the first mannose of the glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10574991# GENE ASSOCIATION: (23556.1) or (23556.2)
H6' phosphoethanolaminyl transferase, endoplasmic reticulum
Abbreviation: R_RTOT3 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : R3coa_hs --> Rtotalcoa Confidence Level: 0 User defined, tissue specific fluxes that can be customized w/ various ratios of R1 through R5 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_B3GNT11g Synonyms: _10 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : gal14acglcgalgluside_hs + uacgam --> acglcgal14acglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# citations: AUTHORS:Isshiki S, Kudo T, Nishihara S, Ikehara Y, Togayachi A, Furuya A, Shitara K, Kubota T, Watanabe M, Kitajima M, Narimatsu H.#TITLE:Lewis type 1 antigen synthase (beta3Gal-T5) is transcriptionally regulated by homeoproteins.#YEAR:2003#PUBMED:12855703# GENE ASSOCIATION: (10678.1)
Abbreviation: R_G14T8g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre8 + udpgal --> h + ksi_pre9 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
Abbreviation: R_3HKYNAKGAT Synonyms: _0 EC Number: 2.6.1.7 SUBSYSTEM: Tryptophan metabolism Equation: [c] : akg + hLkynr --> 42A3HP24DB + glu-L Confidence Level: 2 SAB genes: LOCUS:_883#TRANSCRIPT:1#ABBREVIATION:CCBL1#ECNUMBERS:2.6.1.64# LOCUS:_51166#TRANSCRIPT:1#ABBREVIATION:AADAT# LOCUS:_51166#TRANSCRIPT:2#ABBREVIATION:AADAT# proteins: NAME:cysteine conjugate-beta lyase; cytoplasmic (glutamine transaminase K, kyneurenine aminotransferase) #ABBREVIATION:Ccbl1.1# NAME:aminoadipate aminotransferase#ABBREVIATION:Aadat.1# NAME:aminoadipate aminotransferase#ABBREVIATION:Aadat.2# citations: AUTHORS:BONNER DM, JAKOBY WB#TITLE:Kynurenine transaminase from neurospora#JOURNAL:J Biol Chem#YEAR:1956#PUBMED:13357462# GENE ASSOCIATION: (51166.1) or (883.1) or (51166.2)
_3-Hydroxy-L-kynurenine:2-oxoglutarate aminotransferase
Abbreviation: R_GALC Synonyms: _4 EC Number: 3.2.1.46 SUBSYSTEM: Sphingolipid Metabolism Equation: [l] : galside_hs + h2o --> crm_hs + gal Confidence Level: 4 NJ genes: LOCUS:_2581#TRANSCRIPT:1#ABBREVIATION:GALC#ECNUMBERS:3.2.1.46# proteins: NAME:galactosylceramidase (Krabbe disease) #ABBREVIATION:Galc.1# citations: AUTHORS:Luzi P, Rafi MA, Wenger DA.#TITLE:Structure and organization of the human galactocerebrosidase (GALC) gene.#JOURNAL:Genomics#YEAR:1995#PUBMED:7601472# GENE ASSOCIATION: (2581.1)
N-acteylglutamate synthase, mitochondrial
Abbreviation: R_ACGSm Synonyms: _0 EC Number: 2.3.1.1 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : accoa + glu-L --> acglu + coa + h Confidence Level: 4 NCD genes: LOCUS:_162417#TRANSCRIPT:1#ABBREVIATION:NAGS# proteins: NAME:N-acetylglutamate synthase#ABBREVIATION:Nags.1-m# citations: AUTHORS:Caldovic L, Morizono H, Gracia Panglao M, Gallegos R, Yu X, Shi D, Malamy MH, Allewell NM, Tuchman M#TITLE:Cloning and expression of the human N-acetylglutamate synthase gene#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12459178# GENE ASSOCIATION: (162417.1)
Abbreviation: R_SQLSr Synonyms: _0 EC Number: 2.5.1.21 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : (2) frdp + h + nadph --> nadp + (2) ppi + sql Confidence Level: 4 This is a lumped reaction: Rxn 1: 2 frdp -> 2 ppi + presqualene diphosphate Rxn 2: presqualene diphosphate + NADPH -> squalene + NADP NCD ER version - NJ genes: LOCUS:_2222#TRANSCRIPT:1#ABBREVIATION:FDFT1#ECNUMBERS:2.5.1.21# proteins: NAME:farnesyl-diphosphate farnesyltransferase 1#ABBREVIATION:Fdft1.1# citations: AUTHORS:Biardi L, Krisans K#TITLE:Compartmentalization of cholesterol biosynthesis#JOURNAL:Journal of Biological Chemistry#YEAR:1996# AUTHORS:Cohen LH, Griffioen M, vanRoermund CW, Wanders RJ#TITLE:Subcellular localization of squalene synthase in human hepatoa cell line Hep G2#JOURNAL:Biochim Biophys Acta#YEAR:1992# AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (2222.1)
Abbreviation: R_OIVD2m Synonyms: _0 EC Number: 1.2.1.25 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 3mob + coa + nad --> co2 + ibcoa + nadh Confidence Level: 4 genes: LOCUS:_593#TRANSCRIPT:1#ABBREVIATION:BCKDHA# LOCUS:_1629#TRANSCRIPT:1#ABBREVIATION:DBT#ECNUMBERS:2.3.1.-# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_594#TRANSCRIPT:1#ABBREVIATION:BCKDHB# LOCUS:_594#TRANSCRIPT:2#ABBREVIATION:BCKDHB# proteins: NAME:dihydrolipoamide dehydrogenase, mitochondrial#ABBREVIATION:Dld.1-m# NAME:branched chain keto acid dehydrogenase E1, mitochondrial#ABBREVIATION:Bckdhab1-m# NAME:dihydrolipoamide branched chain transacylase (E2 component of branched chain keto acid dehydrogenase complex#ABBREVIATION:Dbt.1-m# NAME:branched chain keto acid dehydrogenase E1#ABBREVIATION:Bckdhab2-m# citations: AUTHORS:Lau KS, Herring WJ, Chuang JL, McKean M, Danner DJ, Cox RP, Chuang DT.#TITLE:Structure of the gene encoding dihydrolipoyl transacylase (E2) component of human branched chain alpha-keto acid dehydrogenase complex and characterization of an E2 pseudogene. #YEAR:1992#PUBMED:1429740# AUTHORS:Fisher CW, Chuang JL, Griffin TA, Lau KS, Cox RP, Chuang DT. #TITLE:Molecular phenotypes in cultured maple syrup urine disease cells. Complete E1 alpha cDNA sequence and mRNA and subunit contents of the human branched chain alpha-keto acid dehydrogenase complex. #YEAR:1989#PUBMED:2914958# GENE ASSOCIATION: (1629.1) and (594.1 and 593.1) and (1738.1) or (1629.1) and (1738.1) and (594.2 and 593.1)
_2-oxoisovalerate dehydrogenase (acylating; 3-methyl-2-oxobutanoate), mitochondrial
Abbreviation: R_ADRNCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: adrncoa[c] <==> adrncoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: AUTHORS:Sprecher H#TITLE:An update on the pathways of polyunsaturated fatty acid metabolism#JOURNAL:Curr Opn in Clinical Nutrition and Metabolic Care#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_DHEAStr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dheas[r] <==> dheas[c] Confidence Level: 1 specific mechanism not known NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_spc_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : spc_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
sphingosylphosphorylcholine (homo sapiens) exchange
Abbreviation: R_ABO4g Synonyms: _10 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fuc12gal14acglcgalgluside_hs + udpacgal --> acgalfuc12gal14acglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_28#TRANSCRIPT:1#ABBREVIATION:ABO# proteins: NAME:ABO blood group#ABBREVIATION:Abo.1# citations: AUTHORS:Lloyd KO.#TITLE:The chemistry and immunochemistry of blood group A, B, H, and Lewis antigens: past, present and future.#JOURNAL:Glycoconj J#YEAR:2000#PUBMED:11421346# AUTHORS:Yamamoto F.-I., Hakomori S.-I.;#TITLE:Sugar-nucleotide donor specificity of histo-blood group A and B transferases is based on amino acid substitutions#JOURNAL:J. Biol. Chem#YEAR:1990#PUBMED:2121736# AUTHORS:Patenaude S.I., Seto N.O.L., Borisova S.N., Szpacenko A., Marcus S.L., Palcic M.M., Evans S.V.;#TITLE:The structural basis for specificity in human ABO(H) blood group biosynthesis#JOURNAL:Nat. Struct. Biol#YEAR:2002#PUBMED:12198488# GENE ASSOCIATION: (28.1)
Abbreviation: R_CHOLtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: chol[r] <==> chol[c] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PPMI1346Ptn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: ppmi1346p[c] <==> ppmi1346p[n] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
diphosphatidylinositol tetrakisphosphate nuclear transport (diffusion)
Abbreviation: R_PHEMEtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: pheme[m] <==> pheme[c] Confidence Level: 2 TDV changed name to"tm" to indicate mitochondrial transport NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S4TASE1ly Synonyms: _5 EC Number: 3.1.6.12 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_a + h2o <==> cs_a_deg1 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_411#TRANSCRIPT:1#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# LOCUS:_411#TRANSCRIPT:2#ABBREVIATION:ARSB#ECNUMBERS:3.1.6.1# proteins: NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.1-l# NAME:arylsulfatase B, lysosomal#ABBREVIATION:Arsb.2-l# citations: AUTHORS:Schuchman EH, Jackson CE, Desnick RJ#TITLE:Human arylsulfatase B: MOPAC cloning, nucleotide sequence of a full-length cDNA, and regions of amino acid identity with arylsulfatases A and C#JOURNAL:Genomics#YEAR:1990#PUBMED:1968043# AUTHORS:Peters C, Schmidt B, Rommerskirch W, Rupp K, Zuhlsdorf M, Vingron M, Meyer HE, Pohlmann R, von Figura K#TITLE:Phylogenetic conservation of arylsulfatases. cDNA cloning and expression of human arylsulfatase B.#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2303452# GENE ASSOCIATION: (411.1) or (411.2)
Abbreviation: R_P4501B1r Synonyms: _1 EC Number: 1.14.14.1 SUBSYSTEM: Steroid Metabolism Equation: [r] : estradiol + h + nadph + o2 --> h2o + hestratriol + nadp Confidence Level: 4 _4 hydroxylation of 17 beta estradiol NJ genes: LOCUS:_1545#TRANSCRIPT:1#ABBREVIATION:CYP1B1#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 1, subfamily B, polypeptide 1#ABBREVIATION:Cyp1b1.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Tang YM, Wo YY, Stewart J, Hawkins AL, Griffin CA, Sutter TR, Greenlee WF.#TITLE:Isolation and characterization of the human cytochrome P450 CYP1B1 gene.#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8910454# GENE ASSOCIATION: (1545.1)
Abbreviation: R_CRNtuNa Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: crn[e] + na1[e] --> crn[c] + na1[c] Confidence Level: 4 SAB genes: LOCUS:_6584#TRANSCRIPT:1#ABBREVIATION:SLC22A5# proteins: NAME:solute carrier family 22 (organic cation transporter), member 5#ABBREVIATION:Slc22a5.1# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# GENE ASSOCIATION: (6584.1)
L-carnitine inward transport by Na+ symport
Abbreviation: R_SPMS Synonyms: _0 EC Number: 2.5.1.16 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : ametam + ptrc --> 5mta + h + spmd Confidence Level: 4 genes: LOCUS:_6723#TRANSCRIPT:1#ABBREVIATION:SRM#ECNUMBERS:2.5.1.16# proteins: NAME:spermidine synthase#ABBREVIATION:Srm.1# citations: AUTHORS:Myohanen S, Kauppinen L, Wahlfors J, Alhonen L, Janne J#TITLE:Human spermidine synthase gene: structure and chromosomal localization#JOURNAL:DNA Cell Biol#YEAR:1991#PUBMED:2069720# AUTHORS:Kauppinen L, Myohanen S, Halmekyto M, Alhonen L, Janne J#TITLE:Transgenic mice over-expressing the human spermidine synthase gene#JOURNAL:Biochem J#YEAR:1993#PUBMED:8343131# GENE ASSOCIATION: (6723.1)
Abbreviation: R_EX_antipyrene_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : antipyrene <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CYSGLNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[e] + gln-L[c] + na1[e] --> cys-L[c] + gln-L[e] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-cysteine/L-glutamine Na-dependent exchange (Cys-L in)
Abbreviation: R_RETH1e Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [e] : 9-cis-retfa + h2o --> Rtotal2 + h + retinol-9-cis Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
retinyl ester hydrolase (9-cis), extracellular
Abbreviation: R_EX_malttr_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : malttr <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NMNtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: nmn[c] <==> nmn[n] Confidence Level: 2 it genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PPCOACm Synonyms: _0 EC Number: 6.4.1.3 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : atp + hco3 + ppcoa --> adp + h + mmcoa-S + pi Confidence Level: 4 TV genes: LOCUS:_5095#TRANSCRIPT:1#ABBREVIATION:PCCA#ECNUMBERS:6.4.1.3# LOCUS:_5096#TRANSCRIPT:1#ABBREVIATION:PCCB#ECNUMBERS:6.4.1.3# proteins: NAME:propionyl Coenzyme A carboxylase, mitochondrial#ABBREVIATION:Pcc-m# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Lamhonwah AM, Barankiewicz TJ, Willard HF, Mahuran DJ, Quan F, Gravel RA#TITLE:Isolation of cDNA clones coding for the alpha and beta chains of human propionyl-CoA carboxylase: chromosomal assignments and DNA polymorphisms associated with PCCA and PCCB genes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1986#PUBMED:3460076# AUTHORS:Lamhonwah AM, Leclerc D, Loyer M, Clarizio R, Gravel RA#TITLE:Correction of the metabolic defect in propionic acidemia fibroblasts by microinjection of a full-length cDNA or RNA transcript encoding the propionyl-CoA carboxylase beta subunit#JOURNAL:Genomics#YEAR:1994#PUBMED:8188292# GENE ASSOCIATION: (5096.1 and 5095.1)
Propionyl-CoA carboxylase, mitochondrial
Abbreviation: R_S6TASE8ly Synonyms: _7 EC Number: 3.1.6.4 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e_deg1 + h2o <==> cs_e_deg2 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_DGSNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dgsn[e] <==> dgsn[c] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION:
Abbreviation: R_ENGASE2 Synonyms: _15 EC Number: 3.2.1.96 SUBSYSTEM: N-Glycan Degradation Equation: [c] : h2o + n2m2nm --> acgam + n2m2nmn Confidence Level: 0 NCD genes: LOCUS:_64772#TRANSCRIPT:1#ABBREVIATION:FLJ21865#ECNUMBERS:3.2.1.96# proteins: NAME:endo-beta-N-acetylglucosaminidase (thymus, spleen, pancreas, kidney, heart, sk muscle, placenta, brain)#ABBREVIATION:Flj21865.1# citations: AUTHORS:Suzuki T, Yano K, Sugimoto S, Kitajima K, Lennarz WJ, Inoue S, Inoue Y, Emori Y#TITLE:Endo-beta-N-acetylglucosaminidase, an enzyme involved in processing of free oligosaccharides in the cytosol#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2002#PUBMED:12114544# GENE ASSOCIATION: (64772.1)
Abbreviation: R_RPE Synonyms: _0 EC Number: 5.1.3.1 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : ru5p-D <==> xu5p-D Confidence Level: 0 SMP genes: LOCUS:_6120#TRANSCRIPT:1#ABBREVIATION:RPE#ECNUMBERS:5.1.3.1# LOCUS:_6120#TRANSCRIPT:2#ABBREVIATION:RPE#ECNUMBERS:5.1.3.1# proteins: NAME:ribulose-5-phosphate-3-epimerase#ABBREVIATION:Rpe.1# NAME:ribulose-5-phosphate-3-epimerase#ABBREVIATION:Rpe.2# citations: none GENE ASSOCIATION: (6120.1) or (6120.2)
Abbreviation: R_AAPSAS Synonyms: _0 SUBSYSTEM: Lysine Metabolism Equation: [c] : h + h2o + thp2c --> L2aadp6sa Confidence Level: 2 non-enzymatic reaction MM genes: none proteins: none citations: AUTHORS:IJlst L, de Kromme I, Oostheim W, Wanders RJ.#TITLE:Molecular cloning and expression of human L-pipecolate oxidase.#YEAR:2000#PUBMED:10772957# GENE ASSOCIATION:
Abbreviation: R_NACHEX10ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg7 --> (2) acgam + ksi_deg8 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3074.1) or (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_EX_thf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : thf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_10fthf5glu_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 10fthf5glu <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_10-formyltetrahydrofolate-[Glu](5) exchange
Abbreviation: R_ABUTt4_LPAREN_2_RPAREN_r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _4abut[e] + (2) na1[e] <==> 4abut[c] + (2) na1[c] Confidence Level: 4 SAB genes: LOCUS:_6529#TRANSCRIPT:1#ABBREVIATION:SLC6A1# LOCUS:_6540#TRANSCRIPT:1#ABBREVIATION:SLC6A13# LOCUS:_6538#TRANSCRIPT:1#ABBREVIATION:SLC6A11# LOCUS:_6539#TRANSCRIPT:1#ABBREVIATION:SLC6A12# proteins: NAME:solute carrier family 6 (neurotransmitter transporter, GABA), member 1#ABBREVIATION:Slc6a1.1# NAME:solute carrier family 6 (neurotransmitter transporter, GABA), member 11#ABBREVIATION:Slc6a11.1# NAME:solute carrier family 6 (neurotransmitter transporter, betaine/GABA), member 12#ABBREVIATION:Slc6a12.1# NAME:solute carrier family 6 (neurotransmitter transporter, GABA), member 13#ABBREVIATION:Slc6a13.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# GENE ASSOCIATION: (6529.1) or (6538.1) or (6540.1) or (6539.1)
_4-aminobutyrate reversible transport in via sodium symport (1:2)
Abbreviation: R_DHFtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dhf[c] <==> dhf[m] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
dihydrofolate reversible mitochondrial transport
Abbreviation: R_ESTRONEGLCtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: estroneglc[c] <==> estroneglc[r] Confidence Level: 2 unknown mechanism, however glucuronidated metabolites need to get out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GHMT3 Synonyms: _5 EC Number: 2.1.2.1 SUBSYSTEM: Lysine Metabolism Equation: [c] : 3htmelys + h --> 4tmeabut + gly Confidence Level: 2 MM-accoding to Hulse et. al (1978) genes: LOCUS:_6470#TRANSCRIPT:1#ABBREVIATION:SHMT1# LOCUS:_6470#TRANSCRIPT:2#ABBREVIATION:SHMT1# proteins: NAME:serine hydroxymethyltransferase 1 (soluble) #ABBREVIATION:Shmt1.1# NAME:serine hydroxymethyltransferase 1 (soluble)#ABBREVIATION:Shmt1.2# citations: AUTHORS:Hulse JD, Ellis SR, Henderson LM.#TITLE:Carnitine biosynthesis. beta-Hydroxylation of trimethyllysine by an alpha-ketoglutarate-dependent mitochondrial dioxygenase. #YEAR:1978#PUBMED:627563# GENE ASSOCIATION: (6470.1) or (6470.2)
Abbreviation: R_RTOTAL3CRNCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : Rtotal3crn + coa --> Rtotal3coa + crn Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# AUTHORS:Taroni F, Verderio E, Fiorucci S, Cavadini P, Finocchiaro G, Uziel G, Lamantea E, Gellera C, DiDonato S.#TITLE:Molecular characterization of inherited carnitine palmitoyltransferase II deficiency.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1992#PUBMED:1528846# AUTHORS:Fraser F, Padovese R, Zammit VA.#TITLE:Distinct kinetics of carnitine palmitoyltransferase i in contact sites and outer membranes of rat liver mitochondria.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11274214# GENE ASSOCIATION: (1376.1)
ribonucleoside-diphosphate reductase (CDP)
Abbreviation: R_RNDR3 Synonyms: _0 EC Number: 1.17.4.1 SUBSYSTEM: Nucleotides Equation: [c] : cdp + trdrd --> dcdp + h2o + trdox Confidence Level: 4 genes: LOCUS:_6241#TRANSCRIPT:1#ABBREVIATION:RRM2#ECNUMBERS:1.17.4.1# LOCUS:_6240#TRANSCRIPT:1#ABBREVIATION:RRM1#ECNUMBERS:1.7.14.1# proteins: NAME:ribonucleotide reductase #ABBREVIATION:Rrm12.1# citations: AUTHORS:Shao J, Zhou B, Zhu L, Qiu W, Yuan YC, Xi B, Yen Y.#TITLE: In vitro characterization of enzymatic properties and inhibition of the p53R2 subunit of human ribonucleotide reductase. #JOURNAL:Cancer Res. #YEAR:2004#PUBMED:14729598# GENE ASSOCIATION: (6240.1 and 6241.1)
Abbreviation: R_DOLPMT3_Ler Synonyms: _2 EC Number: 2.4.1.130 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolmanp_L + (0.1) m6mpdol_L --> (0.1) dolp_L + h + (0.1) m7mpdol_L Confidence Level: 0 NCD genes: LOCUS:_79087#TRANSCRIPT:1#ABBREVIATION:ALG12# proteins: NAME:alpha-1,6-mannosyltransferase, endoplasmic reticulum#ABBREVIATION:Alg12.1-er# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION: (79087.1)
Abbreviation: R_GGH_DASH_10FTHF5GLUl Synonyms: _0 SUBSYSTEM: Folate Metabolism Equation: [l] : 10fthf5glu + (4) h2o --> 10fthf + (4) glu-L Confidence Level: 4 REMOVES EITHER ONE OR TWO GLUTAMYL RESIDUES WITH A REACTION (WILL ONLY REPRESENT 1) - last step from 5-glutamyl to monoglutamyl is in one step IT genes: LOCUS:_8836#TRANSCRIPT:1#ABBREVIATION:GGH#ECNUMBERS:3.4.19.9# proteins: NAME:gamma-glutamyl hydrolase (conjugase, folylpolygammaglutamyl hydrolase) #ABBREVIATION:Ggh.1-l# citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# AUTHORS:Rhee MS, Lindau-Shepard B, Chave KJ, Galivan J, Ryan TJ.#TITLE:Characterization of human cellular gamma-glutamyl hydrolase.#JOURNAL:Mol Pharmacol. #YEAR:1998#PUBMED:9614206# AUTHORS:Chandler CJ, Wang TT, Halsted CH.#TITLE:Pteroylpolyglutamate hydrolase from human jejunal brush borders. Purification and characterization. #JOURNAL:J Biol Chem. #YEAR:1986#PUBMED:2867095# GENE ASSOCIATION: (8836.1)
Gamma-glutamyl hydrolase (10FTHF5GLU), lysosomal
Abbreviation: R_DHCR71r Synonyms: _3 EC Number: 1.3.1.21 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : ddsmsterol + h + nadph --> dsmsterol + nadp Confidence Level: 4 lanosterol -> cholesterol pathway see paper refs Moebius et al PNAS 95:1899-1902 - check/re-check lit for more specific details (NADPH known to be cofactor - also NADH?, possibly rev?) NJ genes: LOCUS:_1717#TRANSCRIPT:1#ABBREVIATION:DHCR7#ECNUMBERS:1.3.1.21# proteins: NAME:_7-dehydrocholesterol reductase#ABBREVIATION:Dhcr7.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# AUTHORS:Moebius FF, Fitzky BU, Lee, JN, Paik YK, Glossmann H#TITLE:Molecular cloning and expression of the human delta-7-sterol reductase#JOURNAL:PNAS#YEAR:1998# GENE ASSOCIATION: (1717.1)
Abbreviation: R_CDS Synonyms: _7 EC Number: 2.7.7.41 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : ctp + h + pa_hs --> cdpdag_hs + ppi Confidence Level: 4 ER membrane - but on cytosolic surface NJ genes: LOCUS:_1040#TRANSCRIPT:1#ABBREVIATION:CDS1#ECNUMBERS:2.7.7.41# proteins: NAME:CDP diacylglycerol synthase#ABBREVIATION:Cds1.1# citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Kent C#TITLE:Eukaryotic phospholipid biosynthesis#JOURNAL:Annu Rev Biochem#YEAR:1995# AUTHORS:Halford S, Dulai KS, Daw SC, Fitzgibbon J, Hunt DM#TITLE:Isolation and chromosomal localization of two human cdp-diacylglycerol synthase genes#JOURNAL:Genomics#YEAR:1998# AUTHORS:Weeks R, Dowhan W, Shen H, Balantac N, Meengs B, Nudelman E, Leung DW#TITLE:Isolation and expression of an isoform of human CDP-diacylglycerol synthease cDNA#JOURNAL:DNA Cell Biol#YEAR:1997# GENE ASSOCIATION: (1040.1)
Abbreviation: R_AHCYStn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: ahcys[c] <==> ahcys[n] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
S-adenosyl-L-homocysteine nuclear transport
Abbreviation: R_G1M8MASNterg Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: g1m8masn[r] --> g1m8masn[g] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
g1m8masn transport from endoplasmic reticulum to Golgi apparatus
Abbreviation: R_ARACHCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: arachcrn[c] --> arachcrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_TMNDNCCOAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: tmndnccoa[c] <==> tmndnccoa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
chondroitin sulfate E (GalNAc4,6diS-GlcA), precursor 5a_C45H64N2O51S5X
Abbreviation: R_S6T23g Synonyms: _3 EC Number: 2.8.2.17 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_e_pre5a + paps --> cspg_e + h + pap Confidence Level: 4 NCD genes: LOCUS:_56548#TRANSCRIPT:1#ABBREVIATION:CHST7# proteins: NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 7, Golgi apparatus#ABBREVIATION:Chst7.1-g# citations: AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Tsutsumi K, Shimakawa H, Kitagawa H, Sugahara K#TITLE:Functional expression and genomic structure of human chondroitin 6-sulfotransferase#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9883891# AUTHORS:Kitagawa H, Fujita M, Ito N, Sugahara K#TITLE:Molecular cloning and expression of a novel chondroitin 6-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781596# AUTHORS:Bhakta S, Bartes A, Bowman KG, Kao WM, Polsky I, Lee JK, Cook BN, Bruehl RE, Rosen SD, Bertozzi CR, Hemmerich S#TITLE:Sulfation of N-acetylglucosamine by chondroitin 6-sulfotransferase 2 (GST-5)#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10956661# GENE ASSOCIATION: (56548.1)
Abbreviation: R_GLUNm Synonyms: _0 EC Number: 3.5.1.2 SUBSYSTEM: Glutamate metabolism Equation: [m] : gln-L + h2o --> glu-L + nh4 Confidence Level: 4 genes: LOCUS:_27165#TRANSCRIPT:1#ABBREVIATION:GA#ECNUMBERS:3.5.1.2# LOCUS:_2744#TRANSCRIPT:1#ABBREVIATION:GLS#ECNUMBERS:3.5.1.2# LOCUS:_27165#TRANSCRIPT:2#ABBREVIATION:GA# proteins: NAME:glutaminase, mitochondrial#ABBREVIATION:Gls.1-m# NAME:liver mitochondrial glutaminase#ABBREVIATION:Ga.1-m# NAME:liver mitochondrial glutaminase#ABBREVIATION:Ga.2-m# citations: AUTHORS:Holcomb T, Taylor L, Trohkimoinen J, Curthoys NP.#TITLE:Isolation, characterization and expression of a human brain mitochondrial glutaminase cDNA. #YEAR:2000#PUBMED:10719215# AUTHORS:Zacharias DP, Lima MM, Souza AL Jr, de Abranches Oliveira Santos ID, Enokiara M, Michalany N, Curi R.#TITLE:Human cutaneous melanoma expresses a significant phosphate-dependent glutaminase activity: a comparison with the surrounding skin of the same patient. #YEAR:2003#PUBMED:12579526# AUTHORS:Aledo JC, Gomez-Fabre PM, Olalla L, Marquez J. #TITLE:Identification of two human glutaminase loci and tissue-specific expression of the two related genes. #YEAR:2005#PUBMED:PMID:# GENE ASSOCIATION: (2744.1) or (27165.2) or (27165.1)
Abbreviation: R_DHFR Synonyms: _0 EC Number: 1.5.1.3 SUBSYSTEM: Folate Metabolism Equation: [c] : dhf + h + nadph <==> nadp + thf Confidence Level: 2 The enzyme from animals and some micro-organisms also slowly reduces folate to 5,6,7,8-tetrahydrofolate genes: LOCUS:_1719#TRANSCRIPT:1#ABBREVIATION:DHFR#ECNUMBERS:1.5.1.3# proteins: NAME:dihydrofolate reductase #ABBREVIATION:Dhfr.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Funanage VL, Myoda TT, Moses PA, Cowell HR.#TITLE:Assignment of the human dihydrofolate reductase gene to the q11----q22 region of chromosome 5.#JOURNAL:Mol Cell Biol. #YEAR:1984#PUBMED:6504041# GENE ASSOCIATION: (1719.1)
Abbreviation: R_PYDXPP Synonyms: _0 SUBSYSTEM: Vitamin B6 Metabolism Equation: [c] : h2o + pydx5p --> pi + pydx Confidence Level: 2 genes: LOCUS:_57026#TRANSCRIPT:1#ABBREVIATION:PDXP#ECNUMBERS:3.1.3.-# proteins: NAME:Pyridoxal Phosphatase #ABBREVIATION:PdxP.1# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# AUTHORS:Jang YM, Kim DW, Kang TC, Won MH, Baek NI, Moon BJ, Choi SY, Kwon OS.#TITLE:Human pyridoxal phosphatase. Molecular cloning, functional expression, and tissue distribution.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:14522954# GENE ASSOCIATION: (57026.1)
Abbreviation: R_PHETA1m Synonyms: _5 EC Number: 2.6.1.58 SUBSYSTEM: Phenylalanine metabolism Equation: [m] : akg + phe-L <==> glu-L + phpyr Confidence Level: 2 SAB m genes: LOCUS:_6898#TRANSCRIPT:1#ABBREVIATION:TAT#ECNUMBERS:2.6.1.5# LOCUS:_2806#TRANSCRIPT:1#ABBREVIATION:GOT2#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase, mitochondrial#ABBREVIATION:Got2.1-m# NAME:tyrosine aminotransferase#ABBREVIATION:Tat.1-m# citations: AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# GENE ASSOCIATION: (2806.1) or (6898.1)
Abbreviation: R_HCO3_CLt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[c] + hco3[e] <==> cl[e] + hco3[c] Confidence Level: 4 MM genes: LOCUS:_6522#TRANSCRIPT:1#ABBREVIATION:SLC4A2# LOCUS:_83697#TRANSCRIPT:1#ABBREVIATION:SLC4A9# LOCUS:_6508#TRANSCRIPT:1#ABBREVIATION:SLC4A3# LOCUS:_6521#TRANSCRIPT:1#ABBREVIATION:SLC4A1# LOCUS:_6508#TRANSCRIPT:2#ABBREVIATION:SLC4A3# proteins: NAME:solute carrier family 4, anion exchanger, member 1 (erythrocyte membrane protein band 3, Diego blood group), extracellular #ABBREVIATION:Slc4a1.1-e# NAME:solute carrier family 4, anion exchanger, member 2 (erythrocyte membrane protein band 3-like 1), extracellullar#ABBREVIATION:Slc4a2.1-e# NAME:solute carrier family 4, anion exchanger, member 3, extracellular #ABBREVIATION:Slc4a3.1-e# NAME:solute carrier family 4, anion exchanger, member 3, extracellular #ABBREVIATION:Slc4a3.2-e# NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 9, extracellular #ABBREVIATION:Slc4a9.1-e# citations: AUTHORS:Romero MF, Fulton CM, Boron WF.#TITLE:The SLC4 family of HCO 3 - transporters.#YEAR:2004#PUBMED:14722772# AUTHORS:Alper SL, Darman RB, Chernova MN, Dahl NK. #TITLE:The AE gene family of Cl/HCO3- exchangers. #YEAR:2003#PUBMED:12027221# AUTHORS:Parker MD, Ourmozdi EP, Tanner MJ.#TITLE:Human BTR1, a new bicarbonate transporter superfamily member and human AE4 from kidney.#YEAR:2001#PUBMED:11302728# GENE ASSOCIATION: (6522.1) or (83697.1) or (6521.1) or (6508.2) or (6508.1)
bicarbonate transport (Cl-/HCO3- exchange)
Abbreviation: R_G14T9g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre11 + udpgal --> h + ksi_pre12 + udp Confidence Level: 4 Keratan sulfate I biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (8703.1) or (2683.1) or (8704.2) or (8704.1) or (9334.1)
Abbreviation: R_CLS_hs Synonyms: _0 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : cdpdag_hs + pglyc_hs --> clpn_hs + cmp + h Confidence Level: 0 NJ genes: LOCUS:_54675#TRANSCRIPT:1#ABBREVIATION:C20orf155# proteins: NAME:putative cardiolipin synthase#ABBREVIATION:C20orf155.1# citations: AUTHORS:Schlame M, Rua D, Greenberg ML.#TITLE:The biosynthesis and functional role of cardiolipin.#JOURNAL:Prog Lipid Res.#YEAR:2000#PUBMED:10799718# GENE ASSOCIATION: (54675.1)
Abbreviation: R_PROSTGE2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hco3[c] + prostge2[e] <==> hco3[e] + prostge2[c] Confidence Level: 4 NJ genes: LOCUS:_6579#TRANSCRIPT:1#ABBREVIATION:SLCO1A2# LOCUS:_28231#TRANSCRIPT:1#ABBREVIATION:SLCO4A1# LOCUS:_28232#TRANSCRIPT:1#ABBREVIATION:SLCO3A1# LOCUS:_6578#TRANSCRIPT:1#ABBREVIATION:SLCO2A1# proteins: NAME:solute carrier organic anion transporter family, member 2A1#ABBREVIATION:Slco2a1.1# NAME:solute carrier organic anion transporter family, member 1A2#ABBREVIATION:Slco1a2.1# NAME:solute carrier organic anion transporter family, member 3A1#ABBREVIATION:Slco3a1.1# NAME:solute carrier organic anion transporter family, member 4A1#ABBREVIATION:Slco4a1.1# citations: AUTHORS:Lu R, Kanai N, Bao Y, Schuster VL.#TITLE:Cloning, in vitro expression, and tissue distribution of a human prostaglandin transporter cDNA(hPGT).#JOURNAL:J Clin Invest.#YEAR:1996#PUBMED:8787677# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (28231.1) or (6578.1) or (28232.1) or (6579.1)
Abbreviation: R_VD3 Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [c] : pd3 <==> vitd3 Confidence Level: 4 This reaction takes place in skin and needs body temperature based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.194 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_EX_dmantipyrine_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dmantipyrine <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_udp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : udp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLNTHRNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gln-L[e] + na1[e] + thr-L[c] --> gln-L[c] + na1[c] + thr-L[e] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-threonine/L-glutamine Na-dependent exchange (Gln-L in)
Abbreviation: R_FATP1t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hdca[c] + na1[c] <==> hdca[e] + na1[e] Confidence Level: 4 electroneutral transport NJ genes: LOCUS:_11000#TRANSCRIPT:1#ABBREVIATION:SLC27A3# LOCUS:_10999#TRANSCRIPT:1#ABBREVIATION:SLC27A4# LOCUS:_376497#TRANSCRIPT:1#ABBREVIATION:SLC27A1# LOCUS:_376497#TRANSCRIPT:2#ABBREVIATION:SLC27A1# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 4#ABBREVIATION:Slc27a4.1# NAME:solute carrier family 27 (fatty acid transporter), member 3#ABBREVIATION:Slc27a3.1# NAME:solute carrier family 27 (fatty acid transporter), member 1#ABBREVIATION:Slc27a1hs.1# NAME:solute carrier family 27 (fatty acid transporter), member 1#ABBREVIATION:Slc27a1hs.2# citations: AUTHORS:Fitscher BA, Riedel HD, Young KC, Stremmel W.#TITLE:Tissue distribution and cDNA cloning of a human fatty acid transport protein (hsFATP4).#JOURNAL:Biochim Biophys Acta.#YEAR:1998#PUBMED:9878842# AUTHORS:Stahl A.#TITLE:A current review of fatty acid transport proteins (SLC27).#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12856180# AUTHORS:Pohl J, Ring A, Hermann T, Stremmel W.#TITLE:Role of FATP in parenchymal cell fatty acid uptake.#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15522816# GENE ASSOCIATION: (376497.2) or (10999.1) or (376497.1) or (11000.1)
Abbreviation: R_GCALDD Synonyms: _0 EC Number: 1.2.1.21 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : gcald + h2o + nad --> glyclt + (2) h + nadh Confidence Level: 2 JLR genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Brent J#TITLE:Current management of ethylene glycol poisoning#JOURNAL:Drugs#YEAR:2001#PUBMED:11434452# GENE ASSOCIATION: (218.1) or (223.1) or (8854.2) or (224.1) or (222.1) or (8854.1) or (221.1) or (216.1) or (8854.3) or (501.1) or (220.1)
Abbreviation: R_EX_tetpent3_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tetpent3 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TDPm Synonyms: _0 EC Number: 3.6.1.15 SUBSYSTEM: Thiamine Metabolism Equation: [m] : h2o + thmpp --> h + pi + thmmp Confidence Level: 2 _01-25-05 IT genes: none proteins: none citations: AUTHORS:Barile M, Valenti D, Brizio C, Quagliariello E, Passarella S. #TITLE:Rat liver mitochondria can hydrolyse thiamine pyrophosphate to thiamine monophosphate which can cross the mitochondrial membrane in a carrier-mediated process.#JOURNAL:FEBS Lett.#YEAR:1998#PUBMED:9755848# GENE ASSOCIATION:
Abbreviation: R_ST8SIA56g Synonyms: _4 EC Number: 2.4.99.8 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acngal14acglcgalgluside_hs + cmpacna --> acnacngal14acglcgalgluside_hs + cmp + h Confidence Level: 4 NJ genes: LOCUS:_29906#TRANSCRIPT:1#ABBREVIATION:SIAT8E# proteins: NAME:ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 5 #ABBREVIATION:St8sia5.1# citations: AUTHORS:Kim YJ, Kim KS, Do S, Kim CH, Kim SK, Lee YC.#TITLE:Molecular cloning and expression of human alpha2,8-sialyltransferase (hST8Sia V).#JOURNAL:Biochem Biophys Res Commun.#YEAR:1997#PUBMED:9199191# GENE ASSOCIATION: (29906.1)
Abbreviation: R_S2T4g Synonyms: _0 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre11 + (2) paps --> (2) h + hs_pre12 + (2) pap Confidence Level: 4 EC 2.8.2- NCD genes: LOCUS:_9653#TRANSCRIPT:1#ABBREVIATION:HS2ST1# proteins: NAME:heparan sulfate 2-O-sulfotransferase 1, Golgi apparatus#ABBREVIATION:Hs2st1.1-g# citations: AUTHORS:Kobayashi M, Sugumaran G, Liu J, Shworak NW, Silbert JE, Rosenberg RD#TITLE:Molecular cloning and characterization of a human uronyl 2-sulfotransferase that sulfates iduronyl and glucuronyl residues in dermatan/chondroitin sulfate#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10187838# AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Rong J, Habuchi H, Kimata K, Lindahl U, Kusche-Gullberg M#TITLE:Expression of heparan sulphate L-iduronyl 2-O-sulphotransferase in human kidney 293 cells results in increased D-glucuronyl 2-O-sulphation#JOURNAL:Biochem J#YEAR:2000#PUBMED:10677367# AUTHORS:Seki N, Ohira M, Nagase T, Ishikawa K, Miyajima N, Nakajima D, Nomura N, Ohara O#TITLE:Characterization of cDNA clones in size-fractionated cDNA libraries from human brain.#JOURNAL:DNA Res#YEAR:1997#PUBMED:9455484# GENE ASSOCIATION: (9653.1)
Abbreviation: R_FKYNH Synonyms: _1 EC Number: 3.5.1.9 SUBSYSTEM: Tryptophan metabolism Equation: [c] : Lfmkynr + h2o --> Lkynr + for + h Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_HPDCACRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: hpdcacrn[c] --> hpdcacrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (788.1)
Abbreviation: R_CYTD Synonyms: _0 EC Number: 3.5.4.5 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : cytd + h + h2o --> nh4 + uri Confidence Level: 2 genes: LOCUS:_978#TRANSCRIPT:1#ABBREVIATION:CDA#ECNUMBERS:3.5.4.5# LOCUS:_57379#TRANSCRIPT:1#ABBREVIATION:AICDA# proteins: NAME:cytidine deaminase #ABBREVIATION:Cda.1# NAME:activation-induced cytidine deaminase #ABBREVIATION:Aicda.1# citations: AUTHORS:Kuhn K, Bertling WM, Emmrich F.#TITLE:Cloning of a functional cDNA for human cytidine deaminase (CDD) and its use as a marker of monocyte/macrophage differentiation.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1993#PUBMED:8422236# AUTHORS:Saccone S, Besati C, Andreozzi L, Della Valle G, Garattini E, Terao M.#TITLE:Assignment of the human cytidine deaminase (CDA) gene to chromosome 1 band p35-p36.2.#JOURNAL:Genomics#YEAR:1994#PUBMED:8001985# AUTHORS:Laliberte J, Momparler RL.#TITLE:Human cytidine deaminase: purification of enzyme, cloning, and expression of its complementary DNA.#JOURNAL:Cancer Res#YEAR:1994#PUBMED:7923172# AUTHORS:Ge Y, Jensen TL, Stout ML, Flatley RM, Grohar PJ, Ravindranath Y, Matherly LH, Taub JW. #TITLE:The role of cytidine deaminase and GATA1 mutations in the increased cytosine arabinoside sensitivity of Down syndrome myeloblasts and leukemia cell lines.#JOURNAL:Cancer Res. #YEAR:2004#PUBMED:14744791# AUTHORS:Brar SS, Watson M, Diaz M.#TITLE:Activation-induced cytosine deaminase (AID) is actively exported out of the nucleus but retained by the induction of DNA breaks.#JOURNAL:J Biol Chem.#YEAR:2004#PUBMED:15087440# GENE ASSOCIATION: (978.1) or (57379.1)
Abbreviation: R_PAFH Synonyms: _3 EC Number: 3.1.1.47 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : h2o + paf_hs --> ac + ak2lgchol_hs + h Confidence Level: 4 NJ genes: LOCUS:_5049#TRANSCRIPT:1#ABBREVIATION:PAFAH1B2#ECNUMBERS:3.1.1.47# LOCUS:_5048#TRANSCRIPT:1#ABBREVIATION:PAFAH1B1#ECNUMBERS:3.1.1.47# LOCUS:_5051#TRANSCRIPT:1#ABBREVIATION:PAFAH2#ECNUMBERS:3.1.1.47# LOCUS:_5050#TRANSCRIPT:1#ABBREVIATION:PAFAH1B3# proteins: NAME:platelet-activating factor aceylthydrolase, isoform 1b#ABBREVIATION:PAFAH1B.1# NAME:platelet-activating factor acetylhydrolase, isoform 1b, beta-gamma#ABBREVIATION:Pafah1b12.1# NAME:platelet-activating factor acetylhydrolase 2#ABBREVIATION:Pafah2.1# citations: AUTHORS:Adachi H, Tsujimoto M, Hattori M, Arai H, Inoue K.#TITLE:cDNA cloning of human cytosolic platelet-activating factor acetylhydrolase gamma-subunit and its mRNA expression in human tissues.#JOURNAL:Biochem Biophys Res Commun#YEAR:1995#PUBMED:7669037# AUTHORS:Hattori M, Adachi H, Tsujimoto M, Arai H, Inoue K.#TITLE:Abstract Miller-Dieker lissencephaly gene encodes a subunit of brain platelet-activating factor acetylhydrolase [corrected]#JOURNAL:Nature#YEAR:1994#PUBMED:8028668# AUTHORS:Hattori K, Adachi H, Matsuzawa A, Yamamoto K, Tsujimoto M, Aoki J, Hattori M, Arai H, Inoue K.#TITLE:cDNA cloning and expression of intracellular platelet-activating factor (PAF) acetylhydrolase II. Its homology with plasma PAF acetylhydrolase.#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8955149# GENE ASSOCIATION: (5051.1) or (5049.1 and 5050.1) or (5049.1 and 5050.1 and 5048.1)
Abbreviation: R_DOPAMT Synonyms: _0 EC Number: 2.1.1.6 SUBSYSTEM: Tyrosine metabolism Equation: [c] : amet + dopa --> 3moxtyr + ahcys + h Confidence Level: 4 SAB genes: LOCUS:_1312#TRANSCRIPT:1#ABBREVIATION:COMT#ECNUMBERS:2.1.1.6# LOCUS:_1312#TRANSCRIPT:2#ABBREVIATION:COMT#ECNUMBERS:2.1.1.6# proteins: NAME:catechol-O-methyltransferase#ABBREVIATION:Comt.1# NAME:catechol-O-methyltransferase#ABBREVIATION:Comt.2# citations: AUTHORS:Bertocci B, Miggiano V, Da Prada M, Dembic Z, Lahm HW, Malherbe P#TITLE:Human catechol-O-methyltransferase: cloning and expression of the membrane-associated form#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1847521# GENE ASSOCIATION: (1312.1) or (1312.2)
Abbreviation: R_GCC2cm Synonyms: _0 EC Number: 1.8.1.4 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : dhlam + nad <==> h + lpam + nadh Confidence Level: 4 NCD genes: LOCUS:_2731#TRANSCRIPT:1#ABBREVIATION:GLDC#ECNUMBERS:1.4.4.2# LOCUS:_275#TRANSCRIPT:1#ABBREVIATION:AMT#ECNUMBERS:2.1.2.10# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_2653#TRANSCRIPT:1#ABBREVIATION:GCSH# proteins: NAME:glycine dehydrogenase (decarboxylating; glycine decarboxylase, glycine cleavage system protein P), mitochondrial #ABBREVIATION:Gldc.1-m# NAME:aminomethyltransferase (glycine cleavage system protein T), mitochondrial #ABBREVIATION:Amt.1-m# NAME:glycine cleavage system protein H (aminomethyl carrier) #ABBREVIATION:Gcsh.1-m# NAME:dihydrolipoamide dehydrogenase, mitochondrial#ABBREVIATION:Dld.1-m# citations: AUTHORS:Lee HH, Kim do J, Ahn HJ, Ha JY, Suh SW.#TITLE:Crystal structure of T-protein of the glycine cleavage system. Cofactor binding, insights into H-protein recognition, and molecular basis for understanding nonketotic hyperglycinemia.#YEAR:2004#PUBMED:15355973# GENE ASSOCIATION: (275.1) and (1738.1) and (2731.1) and (2653.1)
glycine-cleavage complex (lipoamide), mitochondrial
Abbreviation: R_DOLP_Uter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: (0.1) dolp_U[r] --> (0.1) dolp_U[c] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION:
Abbreviation: R_GLYCtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: glyc[c] <==> glyc[m] Confidence Level: 0 TDV genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_H2CO3D2m Synonyms: _0 SUBSYSTEM: Miscellaneous Equation: [m] : h + hco3 <==> h2co3 Confidence Level: 2 IT (pka (H2CO3) = 6.4) genes: none proteins: none citations: none GENE ASSOCIATION:
carboxylic acid dissociation, mitochondrial
Abbreviation: R_EX_bhb_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : bhb <==> Confidence Level: IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_4NPHSFte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _4nphsf[e] <==> 4nphsf[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NDPK5 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + dgdp <==> adp + dgtp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.2) or (10201.1) or (4831.1 and 4830.2) or (4832.1) or (4831.1 and 4830.1) or (29922.1)
nucleoside-diphosphate kinase (ATP:dGDP)
Abbreviation: R_S6T7g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre13 + paps --> h + ksi_pre14 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_DURIPP Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [c] : duri + pi <==> 2dr1p + ura Confidence Level: 4 JLR genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# LOCUS:_151531#TRANSCRIPT:1#ABBREVIATION:UPP2# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# NAME:uridine phosphorylase 2 #ABBREVIATION:Upp2.1# citations: AUTHORS:Erion MD, Stoeckler JD, Guida WC, Walter RL, Ealick SE.#TITLE:Purine nucleoside phosphorylase. 2. Catalytic mechanism. #JOURNAL:Biochemistry#YEAR:1997#PUBMED:9305963# AUTHORS:Williams SR, Goddard JM, Martin DW Jr. #TITLE:Human purine nucleoside phosphorylase cDNA sequence and genomic clone characterization.#JOURNAL:Nucleic Acids Res.#YEAR:1984#PUBMED:6087295# AUTHORS:Russell RL, Cao D, Zhang D, Handschumacher RE, Pizzorno G.#TITLE:Uridine phosphorylase association with vimentin. Intracellular distribution and localization.#JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11278417# AUTHORS:Johansson M.#TITLE:Identification of a novel human uridine phosphorylase. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12849978# GENE ASSOCIATION: (4860.1) or (151531.1)
Abbreviation: R_O2Stn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: o2s[c] <==> o2s[n] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Archibald F.#TITLE:Oxygen toxicity and the health and survival of eukaryote cells: a new piece is added to the puzzle. #JOURNAL:Proc Natl Acad Sci U S A#YEAR:2003#PUBMED:12939409# GENE ASSOCIATION:
superoxide anion transport via diffusion (nucleus)
Abbreviation: R_DNDPt3m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: datp[c] + dcdp[m] --> datp[m] + dcdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_LACZe Synonyms: _0 EC Number: 3.2.1.23 SUBSYSTEM: Galactose metabolism Equation: [e] : h2o + lcts --> gal + glc-D Confidence Level: 0 Some enzymes in this group hydrolyse a-L-arabinosides; some animal enzymes also hydrolyse b-D-fucosides and b-D-glucosides; cf. EC 3.2.1.108 lactase genes: LOCUS:_3938#TRANSCRIPT:1#ABBREVIATION:LCT#ECNUMBERS:3.2.1.23# proteins: NAME:lactase, extracellular (intestine)#ABBREVIATION:Lct.1-e# citations: none GENE ASSOCIATION: (3938.1)
Abbreviation: R_EX_acgalfucgalacgalfucgalacglcgal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : acgalfucgalacgalfucgalacglcgal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_bilglcur_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : bilglcur <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GALASE12ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg32 --> gal + ksi_deg33 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_GAL3ST12 Synonyms: _4 EC Number: 2.8.2.11 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : digalside_hs + paps --> digalsgalside_hs + h + pap Confidence Level: 4 NJ genes: LOCUS:_9514#TRANSCRIPT:1#ABBREVIATION:CST#ECNUMBERS:2.8.2.11# proteins: NAME:galactose-3-O-sulfotransferase 1#ABBREVIATION:Gal3st1.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Honke K, Tsuda M, Hirahara Y, Ishii A, Makita A, Wada Y.#TITLE:Molecular cloning and expression of cDNA encoding human 3'-phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase.#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9030544# GENE ASSOCIATION: (9514.1)
Abbreviation: R_EX_avite2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : avite2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TMNDNCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tmndnc[e] <==> tmndnc[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_MI4PP Synonyms: _6 EC Number: 3.1.3.25 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + mi4p-D --> inost + pi Confidence Level: 4 NCD genes: LOCUS:_3613#TRANSCRIPT:1#ABBREVIATION:IMPA2# LOCUS:_3612#TRANSCRIPT:1#ABBREVIATION:IMPA1#ECNUMBERS:3.1.3.25# proteins: NAME:inositol(myo)-1(or 4)-monophosphatase 1#ABBREVIATION:Impa1.1# NAME:inositol(myo)-1(or 4)-monophosphatase 2#ABBREVIATION:Impa2.1# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:McAllister G, Whiting P, Hammond EA, Knowles MR, Atack JR, Bailey FJ, Maigetter R, Ragan CI.#TITLE:cDNA cloning of human and rat brain myo-inositol monophosphatase. Expression and characterization of the human recombinant enzyme#JOURNAL:Biochem J#YEAR:1992#PUBMED:1377913# AUTHORS:Yoshikawa T, Turner G, Esterling LE, Sanders AR, Detera-Wadleigh SD#TITLE:A novel human myo-inositol monophosphatase gene, IMP.18p, maps to a susceptibility region for bipolar disorder#JOURNAL:Mol Psychiatry#YEAR:1997#PUBMED:9322233# GENE ASSOCIATION: (3612.1) or (3613.1)
Abbreviation: R_FTHFDH Synonyms: _0 EC Number: 1.5.1.6 SUBSYSTEM: Folate Metabolism Equation: [c] : 10fthf + h2o + nadp --> co2 + h + nadph + thf Confidence Level: 4 IT genes: LOCUS:_10840#TRANSCRIPT:1#ABBREVIATION:FTHFD# proteins: NAME:formyltetrahydrofolate dehydrogenase #ABBREVIATION:Fthfdh.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Johlin FC, Swain E, Smith C, Tephly TR. #TITLE:Studies on the mechanism of methanol poisoning: purification and comparison of rat and human liver 10-formyltetrahydrofolate dehydrogenase.#JOURNAL:Mol Pharmacol. #YEAR:1989#PUBMED:2733692# GENE ASSOCIATION: (10840.1)
Abbreviation: R_SUCRe Synonyms: _0 EC Number: 3.2.1.26 SUBSYSTEM: Galactose metabolism Equation: [e] : h2o + sucr --> fru + glc-D Confidence Level: 4 NCD genes: LOCUS:_8972#TRANSCRIPT:1#ABBREVIATION:MGAM# LOCUS:_6476#TRANSCRIPT:1#ABBREVIATION:SI#ECNUMBERS:3.2.1.48,3.2.1.10# proteins: NAME:maltase-glucoamylase (alpha-glucosidase), extracellular (intestine)#ABBREVIATION:Mgam.1-e# NAME:sucrase-isomaltase (alpha-glucosidase), extracellular (sm intestine)#ABBREVIATION:Si.1-e# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Wu GD, Wang W, Traber PG#TITLE:Isolation and characterization of the human sucrase-isomaltase gene and demonstration of intestine-specific transcriptional elements#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1560017# AUTHORS:Naim HY, Sterchi EE, Lentze MJ#TITLE:Structure, biosynthesis, and glycosylation of human small intestinal maltase-glucoamylase#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3143729# GENE ASSOCIATION: (6476.1) or (8972.1)
sucrose hydrolyzing enxyme, extracellular
Abbreviation: R_ARTFR11 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : pmtcoa --> R1coa_hs Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_i_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : i <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_gt1a_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gt1a_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DOLP_Lter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: (0.1) dolp_L[r] --> (0.1) dolp_L[c] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION:
Abbreviation: R_XOLDIOLONEtm Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: xoldiolone[m] <==> xoldiolone[r] Confidence Level: 2 cholesterol precursor, transport by "diffusion" -> actually beleived to be flip-flop mechanism (PMID: 12840657). NJ genes: none proteins: none citations: AUTHORS:Hamilton JA.#TITLE:Fast flip-flop of cholesterol and fatty acids in membranes: implications for membrane transport proteins.#JOURNAL:Curr Opin Lipidol. #YEAR:2003#PUBMED:12840657# GENE ASSOCIATION:
Abbreviation: R_EX_pglyc_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pglyc_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
phosphatidylglycerol (homo sapiens) exchange
Abbreviation: R_EX_sl_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : sl-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ST3GAL62g Synonyms: _4 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : cmpacna + galacglcgal14acglcgalgluside_hs --> acngalacglcgal14acglcgalgluside_hs + cmp + h Confidence Level: 4 NJ genes: LOCUS:_10402#TRANSCRIPT:1#ABBREVIATION:SIAT10# proteins: NAME:sialyltransferase 10 (alpha-2,3-sialyltransferase VI)#ABBREVIATION:Siat10.1# citations: AUTHORS:Okajima T, Fukumoto S, Miyazaki H, Ishida H, Kiso M, Furukawa K, Urano T, Furukawa K.#TITLE:Molecular cloning of a novel alpha2,3-sialyltransferase (ST3Gal VI) that sialylates type II lactosamine structures on glycoproteins and glycolipids.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10206952# GENE ASSOCIATION: (10402.1)
Abbreviation: R_TETTET6COAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: tettet6coa[c] <==> tettet6coa[x] Confidence Level: 2 interaction between peroxisomes and ER (some enzymes on the outer membrane) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
({[(mannosyl),(phosphoethanolaminyl)]-dimannosyl},{phosphoethanolaminyl})-mannosyl-glucosaminyl-acylphosphatidylinositol-Protein (M4B)_C59H110N3O40P3FULLRCO2FULLR2CO2X
Abbreviation: R_AM6SAD Synonyms: _5 EC Number: 1.2.1.32 SUBSYSTEM: Tryptophan metabolism Equation: [c] : am6sa + h2o + nad --> amuco + (2) h + nadh Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION:
aminomuconate-semialdehyde dehydrogenase
Abbreviation: R_LCADim Synonyms: _0 EC Number: 1.2.1.21 SUBSYSTEM: Pyruvate Metabolism Equation: [m] : h2o + lald-L + nad --> (2) h + lac-L + nadh Confidence Level: 2 NCD genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_7915#TRANSCRIPT:1#ABBREVIATION:ALDH5A1#ECNUMBERS:1.2.1.24# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# LOCUS:_4329#TRANSCRIPT:1#ABBREVIATION:ALDH6A1#ECNUMBERS:1.2.1.27# LOCUS:_5832#TRANSCRIPT:1#ABBREVIATION:ALDH18A1# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_7915#TRANSCRIPT:2#ABBREVIATION:ALDH5A1# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# NAME:aldehyde dehydrogenase 5 family, member A1 (succinate-semialdehyde dehydrogenase), mitochondrial#ABBREVIATION:Aldh5a1.1-m# NAME:aldehyde dehydrogenase 5 family, member A1 (succinate-semialdehyde dehydrogenase), mitochondrial#ABBREVIATION:Aldh5a1.2-m# NAME:aldehyde dehydrogenase 18 family, member A1#ABBREVIATION:Aldh18a1.1-m# NAME:Aldehyde dehydrogenase 6 family, member A1, mitochondrial#ABBREVIATION:Aldh6a1.1-m# citations: AUTHORS:Ewaschuk JB, Naylor JM, Zello GA#TITLE:D-lactate in human and ruminant metabolism#JOURNAL:J Nutr#YEAR:2005#PUBMED:15987839# AUTHORS:Christopher MM, Eckfeldt JH, Eaton JW.#TITLE:Propylene glycol ingestion causes D-lactic acidosis#JOURNAL:Lab Invest.#YEAR:1990#PUBMED:2296157# AUTHORS:Ray S, Ray M.#TITLE:Oxidation of lactaldehyde by cytosolic aldehyde dehydrogenase and inhibition of cytosolic and mitochondrial aldehyde dehydrogenase by metabolites.#JOURNAL:Biochim Biophys Acta.#YEAR:1984#PUBMED:6487654# GENE ASSOCIATION: (4329.1) or (7915.1) or (8659.1) or (5832.1) or (217.1) or (7915.2) or (219.1) or (8659.2)
Abbreviation: R_SBTD_D2 Synonyms: _0 EC Number: 1.1.1.14 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : nad + sbt-D --> fru + h + nadh Confidence Level: 0 NCD genes: LOCUS:_6652#TRANSCRIPT:1#ABBREVIATION:SORD#ECNUMBERS:1.1.1.14# proteins: NAME:sorbitol dehydrogenase#ABBREVIATION:Sord.1# citations: AUTHORS:El-Kabbani O, Darmanin C, Chung RP#TITLE:Sorbitol dehydrogenase: structure, function and ligand design#JOURNAL:Curr Med Chem#YEAR:2004#PUBMED:14965227# GENE ASSOCIATION: (6652.1)
D-sorbitol dehydrogenase (D-fructose producing)
Abbreviation: R_HSD17B1 Synonyms: _1 EC Number: 1.1.1.62 SUBSYSTEM: Steroid Metabolism Equation: [c] : estrone + h + nadph <==> estradiol + nadp Confidence Level: 4 microsomal NADP as cofactor HSD type 1 recheck/confirm comparmentalization - type 1: cytosol, types 2,3: microsomes paper ref notes reversibility: Payne and Hales Endocrine Reviews (2004): 25(6): 947-970 NJ genes: LOCUS:_3292#TRANSCRIPT:1#ABBREVIATION:HSD17B1# LOCUS:_7923#TRANSCRIPT:1#ABBREVIATION:HSD17B8#ECNUMBERS:1.1.1.62# proteins: NAME:hydroxysteroid (17-beta) dehydrogenase 1#ABBREVIATION:Hsd17b1.1# NAME:hydroxysteroid (17-beta) dehydrogenase 8#ABBREVIATION:Hsd17b8.1# citations: AUTHORS:Adamski J, Leenders F Carstensen JF, Kaumann M, Markus MM, Husen B, Tesdorpf JG, Seedor U, deLaunoit Y, Jakob F#TITLE:Steroids, fatty acyl-CoA, and sterols are substrates of 80-kDa multifunctional protein#JOURNAL:Steroids#YEAR:1997# AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# AUTHORS:Andersson S, Moghrabi N#TITLE:Physiology and molecular genetics of 17beta-hydroxysteroid dehydrogenases#JOURNAL:Steroids#YEAR:1997# GENE ASSOCIATION: (3292.1) or (7923.1)
Abbreviation: R_C3STDH1Pr Synonyms: _1 EC Number: 5.3.3.1 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : 4mzym_int1 + nadp --> 4mzym_int2 + co2 + h + nadph Confidence Level: 4 NCD ER version NJ genes: LOCUS:_50814#TRANSCRIPT:1#ABBREVIATION:NSDHL# proteins: NAME:NAD(P) dependent steroid dehydrogenase#ABBREVIATION:Nsdhl.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# AUTHORS:Caldas H, Herman GE#TITLE:NSDHL, an eznyme invovled in cholesterol biosynthesis, traffics through the Golgi and accumulates on ER membranes on the surface of lipid droplets#JOURNAL:Human Molecular Genetics#YEAR:2003# AUTHORS:Konig A, Happle R, Bornholdt D, Engel H, Grzeschik KH#TITLE:Mutations in the NSDHL gene, encoding a 3-beta-hydroxysteroid dehydrogenase, cause CHILD syndrome#JOURNAL:Am J Med Genet#YEAR:2000# GENE ASSOCIATION: (50814.1)
C-3 sterol dehydrogenase (4-methylzymosterol)
Abbreviation: R_MALTSULtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: mal-L[c] + tsul[m] <==> mal-L[m] + tsul[c] Confidence Level: 4 MM genes: LOCUS:_1468#TRANSCRIPT:1#ABBREVIATION:SLC25A10# proteins: NAME:Solute carrier family 25 (dicarboxylate transporter) member 10, mitochondrial #ABBREVIATION:Slc25a10.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# AUTHORS:Crompton M, Palmieri F, Capano M, Quagliariello E.#TITLE:The transport of sulphate and sulphite in rat liver mitochondria. #YEAR:1974#PUBMED:4441366# GENE ASSOCIATION: (1468.1)
Malate:thiosulfate antiport, mitochondrial
Abbreviation: R_HMGCOASi Synonyms: _0 EC Number: 4.1.3.5 SUBSYSTEM: Cholesterol Metabolism Equation: [c] : aacoa + accoa + h2o --> coa + h + hmgcoa Confidence Level: 4 NCD New assignment: EC-2.3.3.10 EC-4.1.3.5 (created 1961, deleted 2002). Enzyme Commission Made irreversible to match KEGG data for S. aureus genes: LOCUS:_3157#TRANSCRIPT:1#ABBREVIATION:HMGCS1#ECNUMBERS:4.1.3.5# LOCUS:_3157#TRANSCRIPT:2#ABBREVIATION:HMGCS1#ECNUMBERS:4.1.3.5# proteins: NAME:_3-hydroxy-3-methylglutaryl-Coenzyme A synthase 1 (soluble)#ABBREVIATION:Hmgcs1.1# NAME:_3-hydroxy-3-methylglutaryl-Coenzyme A synthase 1 (soluble)#ABBREVIATION:Hmgcs1.2# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# GENE ASSOCIATION: (3157.2) or (3157.1)
_2-oxoisovalerate dehydrogenase (acylating; 3-methyl-2-oxopentanoate), mitochondrial
Abbreviation: R_OIVD3m Synonyms: _0 EC Number: 1.2.1.25 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 3mop + coa + nad --> 2mbcoa + co2 + nadh Confidence Level: 4 genes: LOCUS:_593#TRANSCRIPT:1#ABBREVIATION:BCKDHA# LOCUS:_1629#TRANSCRIPT:1#ABBREVIATION:DBT#ECNUMBERS:2.3.1.-# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_594#TRANSCRIPT:1#ABBREVIATION:BCKDHB# LOCUS:_594#TRANSCRIPT:2#ABBREVIATION:BCKDHB# proteins: NAME:dihydrolipoamide dehydrogenase, mitochondrial#ABBREVIATION:Dld.1-m# NAME:branched chain keto acid dehydrogenase E1, mitochondrial#ABBREVIATION:Bckdhab1-m# NAME:dihydrolipoamide branched chain transacylase (E2 component of branched chain keto acid dehydrogenase complex#ABBREVIATION:Dbt.1-m# NAME:branched chain keto acid dehydrogenase E1#ABBREVIATION:Bckdhab2-m# citations: AUTHORS:Lau KS, Herring WJ, Chuang JL, McKean M, Danner DJ, Cox RP, Chuang DT.#TITLE:Structure of the gene encoding dihydrolipoyl transacylase (E2) component of human branched chain alpha-keto acid dehydrogenase complex and characterization of an E2 pseudogene. #YEAR:1992#PUBMED:1429740# AUTHORS:Fisher CW, Chuang JL, Griffin TA, Lau KS, Cox RP, Chuang DT. #TITLE:Molecular phenotypes in cultured maple syrup urine disease cells. Complete E1 alpha cDNA sequence and mRNA and subunit contents of the human branched chain alpha-keto acid dehydrogenase complex. #YEAR:1989#PUBMED:2914958# GENE ASSOCIATION: (1629.1) and (594.1 and 593.1) and (1738.1) or (1629.1) and (1738.1) and (594.2 and 593.1)
Abbreviation: R_BILDGLCURtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: bildglcur[c] <==> bildglcur[r] Confidence Level: 2 unknown mechanism, however glucuronidated metabolites need to get out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PNTEH Synonyms: _0 SUBSYSTEM: CoA Catabolism Equation: [c] : h2o + ptth --> cysam + pnto-R Confidence Level: 4 EC 3.5.1- KEGG RXN R02973 RM genes: LOCUS:_8876#TRANSCRIPT:1#ABBREVIATION:VNN1# LOCUS:_8875#TRANSCRIPT:1#ABBREVIATION:VNN2#ECNUMBERS:3.5.1.-# LOCUS:_55350#TRANSCRIPT:1#ABBREVIATION:VNN3# LOCUS:_55350#TRANSCRIPT:2#ABBREVIATION:VNN3# LOCUS:_8875#TRANSCRIPT:2#ABBREVIATION:VNN2# LOCUS:_55350#TRANSCRIPT:3#ABBREVIATION:VNN3# proteins: NAME:vanin 2#ABBREVIATION:Van2.1# NAME:vanin 2#ABBREVIATION:Van2.2# NAME:vanin 1#ABBREVIATION:Van1.1# NAME:vanin 3#ABBREVIATION:Van3.1# NAME:vanin 3#ABBREVIATION:Van3.2# NAME:vanin 3#ABBREVIATION:Van3.3# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Maras B, Barra D, Dupre S, Pitari G.#TITLE:Is pantetheinase the actual identity of mouse and human vanin-1 proteins?#JOURNAL:FEBS Lett. #YEAR:1999#PUBMED:10567687# AUTHORS:Martin F, Malergue F, Pitari G, Philippe JM, Philips S, Chabret C, Granjeaud S, Mattei MG, Mungall AJ, Naquet P, Galland F.#TITLE:Vanin genes are clustered (human 6q22-24 and mouse 10A2B1) and encode isoforms of pantetheinase ectoenzymes.#JOURNAL:Immunogenetics#YEAR:2001#PUBMED:11491533# GENE ASSOCIATION: (8876.1) and (8875.2) and (55350.3) or (8876.1) and (8875.2) and (55350.2) or (8876.1) and (8875.1) and (55350.1) or (8876.1) and (55350.2) and (8875.1) or (8875.2) and (8876.1) and (55350.1) or (8876.1) and (55350.3) and (8875.1)
Abbreviation: R_Uritn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: uri[c] <==> uri[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ALR Synonyms: _1 EC Number: 1.1.1.2 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : h2o + mthgxl + nadp --> (2) h + nadph + pyr Confidence Level: 4 MM genes: LOCUS:_231#TRANSCRIPT:1#ABBREVIATION:AKR1B1#ECNUMBERS:1.1.1.21# proteins: NAME:aldo-keto reductase family 1, member B1 (aldose reductase)#ABBREVIATION:Akr1b1.1# citations: AUTHORS:Vander Jagt DL, Robinson B, Taylor KK, Hunsaker LA.#TITLE:Reduction of trioses by NADPH-dependent aldo-keto reductases. Aldose reductase, methylglyoxal, and diabetic complications#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1537826# GENE ASSOCIATION: (231.1)
Acetylcholine secretion via secretory vesicle (ATP driven)
Abbreviation: R_ACHVESSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) ach[c] + (2) atp[c] + (2) h2o[c] --> (3) ach[e] + (2) adp[c] + (2) h[c] + (2) pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of ach from the cell. It leaves out the vesicle because that is not a compartment. ATP:H ratio is assumed as 1:3 for vacuolar ATPase and substrate:h ratio is 1:2. This gives a 3:2 ratio for substrate:ATP genes: LOCUS:_6572#TRANSCRIPT:1#ABBREVIATION:SLC18A3# proteins: NAME:solute carrier family 18 (vesicular acetylcholine), member 3#ABBREVIATION:Slc18a3.1# citations: AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# AUTHORS:Eiden LE, Schafer MK, Weihe E, Schutz B#TITLE:The vesicular amine transporter family (SLC18): amine/proton antiporters required for vesicular accumulation and regulated exocytotic secretion of monoamines and acetylcholine#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12827358# GENE ASSOCIATION: (6572.1)
Abbreviation: R_IMPC Synonyms: _0 EC Number: 3.5.4.10 SUBSYSTEM: IMP Biosynthesis Equation: [c] : h2o + imp <==> fprica Confidence Level: 4 genes: LOCUS:_471#TRANSCRIPT:1#ABBREVIATION:ATIC# proteins: NAME:_5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase #ABBREVIATION:AtiC.1# citations: AUTHORS:Cheong CG, Wolan DW, Greasley SE, Horton PA, Beardsley GP, Wilson IA.#TITLE:Crystal structures of human bifunctional enzyme aminoimidazole-4-carboxamide ribonucleotide transformylase/IMP cyclohydrolase in complex with potent sulfonyl-containing antifolates.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14966129# AUTHORS:Bulock KG, Beardsley GP, Anderson KS.#TITLE:The kinetic mechanism of the human bifunctional enzyme ATIC (5-amino-4-imidazolecarboxamide ribonucleotide transformylase/inosine 5'-monophosphate cyclohydrolase). A surprising lack of substrate channeling.#JOURNAL:J Biol Chem.#YEAR:2002#PUBMED:11948179# AUTHORS:Sugita T, Aya H, Ueno M, Ishizuka T, Kawashima K.#TITLE:Characterization of molecularly cloned human 5-aminoimidazole-4-carboxamide ribonucleotide transformylase.#JOURNAL:J Biochem (Tokyo). #YEAR:1997#PUBMED:9378707# GENE ASSOCIATION: (471.1)
Abbreviation: R_FACOAL2252 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + clpnd + coa <==> amp + clpndcoa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_LALDO Synonyms: _0 SUBSYSTEM: Pyruvate Metabolism Equation: [c] : gthrd + lald-D + nad <==> h + lgt-S + nadh Confidence Level: 0 EC 1.2.1.1 NCD genes: LOCUS:_128#TRANSCRIPT:1#ABBREVIATION:ADH5#ECNUMBERS:1.1.1.1# proteins: NAME:alcohol dehydrogenase V#ABBREVIATION:Adh5# citations: none GENE ASSOCIATION: (128.1)
D-Lactaldehyde:NAD+ oxidoreductase (glutathione-formylating)
Abbreviation: R_CYSASNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[c] + cys-L[e] + na1[e] --> asn-L[e] + cys-L[c] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-cysteine/L-asparagine Na-dependent exchange (Cys-L in)
Abbreviation: R_GTHRDtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: gthrd[c] <==> gthrd[r] Confidence Level: 1 unknown mechanism - NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TMNDNCCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + tmndnccoa --> coa + tmndnccrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_ARTFR204 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (2) fadh2[m] + (2) h[m] + (2) nadph[m] + strdnccoa[c] --> (1.125) R2coa_hs[c] + (2) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PYRt2p Synonyms: _0 SUBSYSTEM: D-alanine metabolism Equation: h[c] + pyr[c] <==> h[x] + pyr[x] Confidence Level: 4 NCD genes: LOCUS:_9194#TRANSCRIPT:1#ABBREVIATION:SLC16A7# LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 7#ABBREVIATION:Slc16a7.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1#ABBREVIATION:Slc16a1.1# citations: AUTHORS:McClelland GB, Khanna S, Gonzalez GF, Butz CE, Brooks GA#TITLE:Peroxisomal membrane monocarboxylate transporters: evidence for a redox shuttle system?#JOURNAL:Biochem Biophys Res Commun#YEAR:2003#PUBMED:12705896# AUTHORS:Lin RY, Vera JC, Chaganti RS, Golde DW#TITLE:Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9786900# AUTHORS:Halestrap AP, Meredith D#TITLE:The SLC16 gene family-from monocarboxylate transporters (MCTs) to aromatic amino acid transporters and beyond#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739169# GENE ASSOCIATION: (6566.1) or (9194.1)
pyruvate peroxisomal transport via proton symport
Abbreviation: R_ACOATA Synonyms: _0 EC Number: 2.3.1.38 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : ACP + accoa <==> acACP + coa Confidence Level: 4 tv rxn is now charge balanced; removed h - NCD genes: LOCUS:_2194#TRANSCRIPT:1#ABBREVIATION:FASN#ECNUMBERS:2.3.1.85# proteins: NAME:fatty acid synthase#ABBREVIATION:Fasn.1# citations: AUTHORS:Chakravarty B, Gu Z, Chirala SS, Wakil SJ, Quiocho FA.#TITLE:Human fatty acid synthase: structure and substrate selectivity of the thioesterase domain.#YEAR:2004#PUBMED:15507492# AUTHORS:Brink J, Ludtke SJ, Yang CY, Gu ZW, Wakil SJ, Chiu W.#TITLE:Quaternary structure of human fatty acid synthase by electron cryomicroscopy.#JOURNAL:Proc Natl Acad Sci#YEAR:2002#PUBMED:11756679# GENE ASSOCIATION: (2194.1)
Abbreviation: R_ADRNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: adrn[e] <==> adrn[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_GPIMTer_L Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_L + gacpail_hs --> (0.1) dolp_L + h + mgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_93183#TRANSCRIPT:1#ABBREVIATION:PIGM# LOCUS:_54965#TRANSCRIPT:1#ABBREVIATION:PIGX# proteins: NAME:phosphatidylinositol glycan, class M, endoplasmic reticulum#ABBREVIATION:Pigm.1-r# NAME:phosphatidylinositol glycan, class X, endoplasmic reticulum#ABBREVIATION:Pigx.1-r# citations: AUTHORS:Maeda Y, Watanabe R, Harris CL, Hong Y, Ohishi K, Kinoshita K, Kinoshita T#TITLE:PIG-M transfers the first mannose to glycosylphosphatidylinositol on the lumenal side of the ER#JOURNAL:EMBO J#YEAR:2001#PUBMED:11226175# AUTHORS:Ashida H, Hong Y, Murakami Y, Shishioh N, Sugimoto N, Kim YU, Maeda Y, Kinoshita T#TITLE:Mammalian PIG-X and yeast Pbn1p are the essential components of glycosylphosphatidylinositol-mannosyltransferase I#JOURNAL:Mol Biol Cell#YEAR:2005#PUBMED:15635094# GENE ASSOCIATION: (93183.1) and (54965.1)
Abbreviation: R_ARTCOAL1 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : Rtotal + coa <==> Rtotalcoa Confidence Level: 0 HR fatty acid metabolism - R groups = acyl groups, Rtotal, Rtotal2, Rtotal3 correspond to three possible acyl groups added to glycerol Carbon stoichiometry normalized wrt C16 R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_arach_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : arach <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_ppa_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ppa <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GCHOLAtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: gchola[x] <==> gchola[c] Confidence Level: 2 unknown mechanism, however bile acids need to be secreted out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PYRt2r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[e] + pyr[e] <==> h[c] + pyr[c] Confidence Level: 4 JLR genes: LOCUS:_9194#TRANSCRIPT:1#ABBREVIATION:SLC16A7# LOCUS:_9123#TRANSCRIPT:1#ABBREVIATION:SLC16A3# LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 7#ABBREVIATION:Slc16a7.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1#ABBREVIATION:Slc16a1.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 3#ABBREVIATION:Slc16a3.1-e# citations: AUTHORS:Lin RY, Vera JC, Chaganti RS, Golde DW#TITLE:Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9786900# AUTHORS:Halestrap AP, Price NT#TITLE:The proton-linked monocarboxylate transporter (MCT) family: structure, function and regulation#JOURNAL:Biochem J#YEAR:1999#PUBMED:10510291# AUTHORS:Garcia CK, Li X, Luna J, Francke U#TITLE:cDNA cloning of the human monocarboxylate transporter 1 and chromosomal localization of the SLC16A1 locus to 1p13.2-p12#JOURNAL:Genomics#YEAR:1994#PUBMED:7835905# AUTHORS:Poole RC, Halestrap AP#TITLE:Transport of lactate and other monocarboxylates across mammalian plasma membranes#JOURNAL:Am J Physiol#YEAR:1993#PUBMED:8476015# AUTHORS:Ritzhaupt A, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:The characterization of butyrate transport across pig and human colonic luminal membrane#JOURNAL:J Physiol#YEAR:1998#PUBMED:9508842# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification of a monocarboxylate transporter isoform type 1 (MCT1) on the luminal membrane of human and pig colon#JOURNAL:Biochem Soc Trans#YEAR:1998#PUBMED:9649795# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification and characterization of a monocarboxylate transporter (MCT1) in pig and human colon: its potential to transport L-lactate as well as butyrate#JOURNAL:J Physiol#YEAR:1998#PUBMED:9824713# AUTHORS:Halestrap AP, Meredith D#TITLE:The SLC16 gene family-from monocarboxylate transporters (MCTs) to aromatic amino acid transporters and beyond#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739169# AUTHORS:Manning Fox JE, Meredith D, Halestrap AP#TITLE:Characterisation of human monocarboxylate transporter 4 substantiates its role in lactic acid efflux from skeletal muscle#JOURNAL:J Physiol#YEAR:2000#PUBMED:11101640# AUTHORS:Price NT, Jackson VN, Halestrap AP#TITLE:Cloning and sequencing of four new mammalian monocarboxylate transporter (MCT) homologues confirms the existence of a transporter family with an ancient past#JOURNAL:Biochem J#YEAR:1998#PUBMED:9425115# GENE ASSOCIATION: (6566.1) or (9123.1) or (9194.1)
pyruvate reversible transport via proton symport
Abbreviation: R_NACHEXA6ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_d_deg4 + (2) h2o --> acgal + cs_d_deg6 + h + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_AMACRp Synonyms: _0 EC Number: 5.1.99.4 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : cholcoar --> cholcoas Confidence Level: 4 see Russell bile acid biosynthesis review uniprot NJ genes: LOCUS:_23600#TRANSCRIPT:1#ABBREVIATION:AMACR# LOCUS:_23600#TRANSCRIPT:2#ABBREVIATION:AMACR# proteins: NAME:alpha-methylacyl-CoA racemase#ABBREVIATION:Amacr.1# NAME:alpha-methylacyl-CoA racemase#ABBREVIATION:Amacr.2# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:VanVeldhoven PP, Croes K, Casteels M, Mannaerts GP#TITLE:_2-methylacyl racemase: a couple assay based on the use of pristanoyl-CoA oxidase/peroxidase and reinvestigation of its subcellular distribution in rat and human liver#JOURNAL:Biochimica et Biophysica Acta#YEAR:1997# GENE ASSOCIATION: (23600.1) or (23600.2)
Abbreviation: R_NADtru Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: nad[r] --> nad[c] Confidence Level: 1 diffusion - but unidirectional output from ER (to avoid loops w/ NAD/NADP) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_MCLACCYSR Synonyms: _0 SUBSYSTEM: Cysteine Metabolism Equation: [c] : cys-L + mercplac + nad --> h + mercplaccys + nadh Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Hannestad U, Martensson J, Sjodahl R, Sorbo B.#TITLE:_3-mercaptolactate cysteine disulfiduria: biochemical studies on affected and unaffected members of a family. #YEAR:1981#PUBMED:6945862# GENE ASSOCIATION:
Abbreviation: R_GASNASE3ly Synonyms: _8 EC Number: 3.5.1.26 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg1 --> Asn-X-Ser/Thr + ksi_deg2 Confidence Level: 4 NCD genes: LOCUS:_175#TRANSCRIPT:1#ABBREVIATION:AGA#ECNUMBERS:3.5.1.26# proteins: NAME:aspartylglucosaminidase, lysosomal#ABBREVIATION:Aga.1-l# citations: AUTHORS:Fisher KJ, Tollersrud OK, Aronson NN Jr#TITLE:Cloning and sequence analysis of a cDNA for human glycosylasparaginase. A single gene encodes the subunits of this lysosomal amidase#JOURNAL:FEBS Lett.#YEAR:1990#PUBMED:2401370# AUTHORS:Ikonen E, Baumann M, Gron K, Syvanen AC, Enomaa N, Halila R, Aula P, Peltonen L#TITLE:Aspartylglucosaminuria: cDNA encoding human aspartylglucosaminidase and the missense mutation causing the disease.#JOURNAL:EMBO J#YEAR:1991#PUBMED:1703489# AUTHORS:Tollersrud OK, Heiskanen T, Peltonen L#TITLE:Human leucocyte glycosylasparaginase is an alpha/beta-heterodimer of 19 kDa alpha-subunit and 17 and 18 kDa beta-subunit.#JOURNAL:Biochem J#YEAR:1994#PUBMED:8002961# GENE ASSOCIATION: (175.1)
Abbreviation: R_H5MTer_L Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_L + emgacpail_hs --> (0.1) dolp_L + h + memgacpail_hs Confidence Level: 4 NCD genes: LOCUS:_55650#TRANSCRIPT:1#ABBREVIATION:PIGV# proteins: NAME:phosphatidylinositol glycan, class V, endoplasmic reticulum#ABBREVIATION:Pigv.1-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Kang JY, Hong Y, Ashida H, Shishioh N, Murakami Y, Morita YS, Maeda Y, Kinoshita T#TITLE:IG-V involved in transferring the second mannose in glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15623507# GENE ASSOCIATION: (55650.1)
Abbreviation: R_UDPGLDCg Synonyms: _1 EC Number: 4.1.1.35 SUBSYSTEM: Nucleotide Sugar Metabolism Equation: [g] : h + udpglcur --> co2 + udpxyl Confidence Level: 4 NCD genes: LOCUS:_80146#TRANSCRIPT:1#ABBREVIATION:UXS1#ECNUMBERS:EC,4.1.1.35# proteins: NAME:UDP-glucuronate decarboxylase , Golgi apparatus#ABBREVIATION:Uxs1.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Silbert JE, DeLuca S#TITLE:The synthesis of uridine diphosphate xylose by particulate preparations from mouse mast-cell tumors#JOURNAL:Biochim Biophys Acta#YEAR:1967#PUBMED:4293108# AUTHORS:Kearns AE, Vertel BM, Schwartz NB#TITLE:Topography of glycosylation and UDP-xylose production#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8496172# AUTHORS:Moriarity JL, Hurt KJ, Resnick AC, Storm PB, Laroy W, Schnaar RL, Snyder SH#TITLE:UDP-glucuronate decarboxylase, a key enzyme in proteoglycan synthesis: cloning, characterization, and localization#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11877387# GENE ASSOCIATION: (80146.1)
UDP-D-glucuronate decarboxylase, Golgi apparatus
Abbreviation: R_NACUP Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: nac[e] --> nac[c] Confidence Level: 2 JLR genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_MAN2_7Cer Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : g1m8masn + h2o --> g1m7masnC + man Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Spiro RG#TITLE:Glucose residues as key determinants in the biosynthesis and quality control of glycoproteins with N-linked oligosaccharides.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11007802# AUTHORS:Roth J, Zuber C, Guhl B, Fan JY, Ziak M.#TITLE:The importance of trimming reactions on asparagine-linked oligosaccharides for protein quality control.#JOURNAL:Histochem Cell Biol#YEAR:2002#PUBMED:11935292# GENE ASSOCIATION:
mannosidase II, endoplasmic reticulum (g1m7masnC-producing)
Abbreviation: R_HISTASE Synonyms: _0 EC Number: 1.4.3.6 SUBSYSTEM: Histidine Metabolism Equation: [c] : h2o + hista + o2 --> h2o2 + im4act + nh4 Confidence Level: 4 SAB genes: LOCUS:_314#TRANSCRIPT:1#ABBREVIATION:AOC2#ECNUMBERS:1.4.3.6# LOCUS:_26#TRANSCRIPT:1#ABBREVIATION:ABP1#ECNUMBERS:1.4.3.6# LOCUS:_314#TRANSCRIPT:2#ABBREVIATION:AOC2# proteins: NAME:amiloride binding protein 1 (amine oxidase (copper-containing))#ABBREVIATION:Abp1.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.2# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# AUTHORS:Zimatkin SM, Anichtchik OV#TITLE:Alcohol-histamine interactions#JOURNAL:Alcohol Alcohol#YEAR:1999#PUBMED:10344773# GENE ASSOCIATION: (26.1) or (314.1) or (314.2)
Abbreviation: R_VALTA Synonyms: _0 EC Number: 2.6.1.42 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [c] : akg + val-L <==> 3mob + glu-L Confidence Level: 4 genes: LOCUS:_586#TRANSCRIPT:1#ABBREVIATION:BCAT1#ECNUMBERS:2.6.1.26# proteins: NAME:branched chain aminotransferase 1, cytosolic #ABBREVIATION:Bcat1.1# citations: AUTHORS:Bledsoe RK, Dawson PA, Hutson SM.#TITLE:Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm). #YEAR:1997#PUBMED:9165094# AUTHORS:Naylor SL, Shows TB.#TITLE:Branched-chain aminotransferase deficiency in Chinese hamster cells complemented by two independent genes on human chromosomes 12 and 19. #YEAR:1980#PUBMED:6933702# GENE ASSOCIATION: (586.1)
Abbreviation: R_VALtec Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: val-L[e] <==> val-L[c] Confidence Level: 4 SAB genes: LOCUS:_124935#TRANSCRIPT:1#ABBREVIATION:SLC43A2# LOCUS:_8501#TRANSCRIPT:1#ABBREVIATION:SLC43A1# proteins: NAME:solute carrier family 43, member 2#ABBREVIATION:Slc43a2.1# NAME:solute carrier family 43, member 1#ABBREVIATION:Slc43a1.1# citations: AUTHORS:Bodoy S, Martin L, Zorzano A, Palacin M, Estevez R, Bertran J#TITLE:Identification of LAT4, a novel amino acid transporter with system L activity#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15659399# AUTHORS:Babu E, Kanai Y, Chairoungdua A, Kim do K, Iribe Y, Tangtrongsup S, Jutabha P, Li Y, Ahmed N, Sakamoto S, Anzai N, Nagamori S, Endou H#TITLE:Identification of a novel system L amino acid transporter structurally distinct from heterodimeric amino acid transporters#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12930836# AUTHORS:Hediger MA, Romero MF, Peng JB, Rolfs A, Takanaga H, Bruford EA#TITLE:The ABCs of solute carriers: physiological, pathological and therapeutic implications of human membrane transport proteinsIntroduction#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14624363# GENE ASSOCIATION: (8501.1) or (124935.1)
L-valine transport via diffusion (extracellular to cytosol)
((N-acetyl-D-glucosaminyl)4-(alpha-D-mannosyl)2-beta-D-mannosyl-diacetylchitobiosyl)-L-asparagine (protein)_C66H109N6O45X
Abbreviation: R_SPHS1Ptr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: sphs1p[c] <==> sphs1p[r] Confidence Level: 2 unknown mechanism, however sphs1p is known to have intracellular and extracellular activity and signal transduction functions (PMID: 10751414 or PMID: 15585321). NJ genes: none proteins: none citations: AUTHORS:Baumruker T, Bornancin F, Billich A.#TITLE:The role of sphingosine and ceramide kinases in inflammatory responses.#JOURNAL:Immunol Lett.#YEAR:2005#PUBMED:15585321# AUTHORS:Quest AF, Leyton L, Parraga M.#TITLE:Caveolins, caveolae, and lipid rafts in cellular transport, signaling, and disease.#JOURNAL:Biochem Cell Biol.#YEAR:2004#PUBMED:15052333# GENE ASSOCIATION:
Abbreviation: R_FUCACNGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucacngal14acglcgalgluside_hs[g] <==> fucacngal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLYCLTtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: glyclt[c] --> glyclt[x] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP.#TITLE:Glycolate and glyoxylate metabolism in HepG2 cells.#YEAR:2004#PUBMED:15240345# GENE ASSOCIATION:
Abbreviation: R_NACHEXA1ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_a + (2) h2o --> acgal + cs_a_deg2 + h + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_DPROOp Synonyms: _0 SUBSYSTEM: Arginine and Proline Metabolism Equation: [x] : o2 + pro-D --> 1p2cbxl + h + h2o2 Confidence Level: 4 MM genes: LOCUS:_1610#TRANSCRIPT:1#ABBREVIATION:DAO#ECNUMBERS:1.4.3.3# proteins: NAME:D-amino-acid oxidase#ABBREVIATION:Dao.1-x# citations: none GENE ASSOCIATION: (1610.1)
Abbreviation: R_UGT1A1r Synonyms: _6 EC Number: 2.4.1.17 SUBSYSTEM: Steroid Metabolism Equation: [r] : estrone + udpglcur --> estroneglc + udp Confidence Level: 4 NJ genes: LOCUS:_54575#TRANSCRIPT:1#ABBREVIATION:UGT1A10# LOCUS:_54576#TRANSCRIPT:1#ABBREVIATION:UGT1A8# proteins: NAME:UDP glycosyltransferase 1 family, polypeptide A10#ABBREVIATION:Ugt1a10.1# NAME:UDP glycosyltransferase 1 family, polypeptide A8#ABBREVIATION:Ugt1a8.1# citations: AUTHORS:Basu NK, Kubota S, Meselhy MR, Ciotti M, Chowdhury B, Hartori M, Owens IS.#TITLE:Free Full Text Gastrointestinally distributed UDP-glucuronosyltransferase 1A10, which metabolizes estrogens and nonsteroidal anti-inflammatory drugs, depends upon phosphorylation.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15117964# AUTHORS:Mackenzie PI, Owens IS, Burchell B, Bock KW, Bairoch A, Belanger A, Fournel-Gigleux S, Green M, Hum DW, Iyanagi T, Lancet D, Louisot P, Magdalou J, Chowdhury JR, Ritter JK, Schachter H, Tephly TR, Tipton KF, Nebert DW.#TITLE:The UDP glycosyltransferase gene superfamily: recommended nomenclature update based on evolutionary divergence.#JOURNAL:Pharmacogenetics#YEAR:1997#PUBMED:9295054# GENE ASSOCIATION: (54575.1) or (54576.1)
UDP-N-acetyl-D-glucosamine 2-epimerase (Hydrolysis)
Abbreviation: R_UAG2EMAi Synonyms: _0 EC Number: 5.1.3.14 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : h2o + uacgam --> acmana + h + udp Confidence Level: 4 genes: LOCUS:_10020#TRANSCRIPT:1#ABBREVIATION:GNE#ECNUMBERS:5.1.3.14,2.7.1.60# proteins: NAME:glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase#ABBREVIATION:Gne.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Seppala R, Lehto VP, Gahl WA#TITLE:Mutations in the human UDP-N-acetylglucosamine 2-epimerase gene define the disease sialuria and the allosteric site of the enzyme#JOURNAL:Am J Hum Genet#YEAR:1999#PUBMED:10330343# AUTHORS:Lucka L, Krause M, Danker K, Reutter W, Horstkorte R#TITLE:Primary structure and expression analysis of human UDP-N-acetyl-glucosamine-2-epimerase/N-acetylmannosamine kinase, the bifunctional enzyme in neuraminic acid biosynthesis#JOURNAL:FEBS Lett#YEAR:1999#PUBMED:10431835# GENE ASSOCIATION: (10020.1)
Abbreviation: R_SPTix Synonyms: _0 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [x] : pyr + ser-L --> ala-L + hpyr Confidence Level: 4 genes: LOCUS:_189#TRANSCRIPT:1#ABBREVIATION:AGXT#ECNUMBERS:2.6.1.44# proteins: NAME:alanine-glyoxylate aminotransferase (oxalosis I; hyperoxaluria I; glycolicaciduria; serine-pyruvate aminotransferase), peroxisomal #ABBREVIATION:Agxt.1-x# citations: AUTHORS:Holmes RP, Assimos DG#TITLE:Glyoxylate synthesis, and its modulation and influence on oxalate synthesis#JOURNAL:J Urol#YEAR:1998#PUBMED:9783918# AUTHORS:Lumb MJ, Danpure CJ. #TITLE:Functional synergism between the most common polymorphism in human alanine:glyoxylate aminotransferase and four of the most common disease-causing mutations.#YEAR:2000#PUBMED:10960483# GENE ASSOCIATION: (189.1)
serine-pyruvate aminotransferase (irreversible), peroxisomal
Abbreviation: R_CLHCO3tex2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) cl[e] + hco3[c] --> (2) cl[c] + hco3[e] Confidence Level: 2 SAB genes: LOCUS:_65010#TRANSCRIPT:1#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:2#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:3#ABBREVIATION:SLC26A6# proteins: NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.1# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.2# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.3# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (65010.1) or (65010.3) or (65010.2)
chloride transport via bicarbonate countertransport (2:1)
Abbreviation: R_PMI12346PHn Synonyms: _0 EC Number: 3.6.1.52 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + ppmi12346p --> h + minohp + pi Confidence Level: 4 NCD genes: LOCUS:_9807#TRANSCRIPT:1#ABBREVIATION:IHPK1# LOCUS:_117283#TRANSCRIPT:1#ABBREVIATION:IHPK3# LOCUS:_51447#TRANSCRIPT:1#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:3#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:2#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:5#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:4#ABBREVIATION:IHPK2# LOCUS:_51447#TRANSCRIPT:6#ABBREVIATION:IHPK2# LOCUS:_9807#TRANSCRIPT:2#ABBREVIATION:IHPK1# proteins: NAME:inositol hexaphosphate kinase 1, nuclear#ABBREVIATION:Ihpk1.1-n# NAME:inositol hexaphosphate kinase 1#ABBREVIATION:Ihpk1.2-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.1-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.2-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.3-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.4-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.5-n# NAME:inositol hexaphosphate kinase 2, nuclear#ABBREVIATION:Ihpk2.6-n# NAME:inositol hexaphosphate kinase 3 , nuclear#ABBREVIATION:Ihpk3.1-n# citations: AUTHORS:Saiardi A, Nagata E, Luo HR, Snowman AM, Snyder SH#TITLE:Identification and characterization of a novel inositol hexakisphosphate kinase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11502751# GENE ASSOCIATION: (51447.3) or (9807.2) or (51447.4) or (51447.6) or (9807.1) or (51447.5) or (117283.1) or (51447.1) or (51447.2)
_5-diphosphoinositol-1,2,3,4,6-pentakisphosphate diphosphohydrolase, nuclear
Abbreviation: R_PSFLIPm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[c] + h2o[c] + ps_hs[c] --> adp[c] + h[c] + pi[c] + ps_hs[m] Confidence Level: 4 See PMID: 10856717 - flippase activity no detailed data on which compartment ATP hydrolysis occurs, so for the time being assumed to be cytosolic NJ genes: LOCUS:_10396#TRANSCRIPT:1#ABBREVIATION:ATP8A1#ECNUMBERS:3.6.3.1# LOCUS:_57194#TRANSCRIPT:1#ABBREVIATION:ATP10A#ECNUMBERS:3.6.3.1# proteins: NAME:ATPase, aminophospholipid transporter (APLT), Class I, type 8A, member 1#ABBREVIATION:Atp8a1.1# NAME:ATPase, Class V, type 10A#ABBREVIATION:Atp10a.1# citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# AUTHORS:Herzing LB, Kim SJ, Cook EH Jr, Ledbetter DH.#TITLE:The human aminophospholipid-transporting ATPase gene ATP10C maps adjacent to UBE3A and exhibits similar imprinted expression.#JOURNAL:Am J Hum Genet#YEAR:2001#PUBMED:11353404# AUTHORS:Mouro I, Halleck MS, Schlegel RA, Mattei MG, Williamson P, Zachowski A, Devaux P, Cartron JP, Colin Y.#TITLE:Cloning, expression, and chromosomal mapping of a human ATPase II gene, member of the third subfamily of P-type ATPases and orthologous to the presumed bovine and murine aminophospholipid translocase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10198212# GENE ASSOCIATION: (10396.1) or (57194.1)
Abbreviation: R_S6T12g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre28 + paps --> h + ksi_pre29 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.1) or (9435.1) or (10164.1) or (4166.1) or (23563.2)
Abbreviation: R_NTD7l Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleic acid degradation Equation: [l] : amp + h2o --> adn + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:R.L. Pisoni#TITLE:Lysosomal Nucleic Acid and Phosphate Metabolism and Related Metabolic Reactions, Chapter 9#YEAR:1996# GENE ASSOCIATION: (53.1)
Abbreviation: R_EX_h_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : h <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SERtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: ser-L[c] <==> ser-L[x] Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Xue HH, Sakaguchi T, Fujie M, Ogawa H, Ichiyama A.#TITLE:Flux of the L-serine metabolism in rabbit, human, and dog livers. Substantial contributions of both mitochondrial and peroxisomal serine:pyruvate/alanine:glyoxylate aminotransferase.#YEAR:1999#PUBMED:10347152# GENE ASSOCIATION:
Abbreviation: R_P45019A2r Synonyms: _4 EC Number: 1.14.14.1 SUBSYSTEM: Steroid Metabolism Equation: [r] : nadph + (2) o2 + tststerone --> estradiol + for + (2) h2o + nadp Confidence Level: 4 uniprot - states "membrane bound" - non-specific acts on androstenedione and testosterone formate production inferred from cyp P450 review paper Korzekwa J Steroid Biochemistry (1993) 44:367-73-RETURN FOLLOWING DEEPER LIT SEARCH H2O added for balance purposes - in analogy to other P450 reactions NJ genes: LOCUS:_1588#TRANSCRIPT:1#ABBREVIATION:CYP19A1# LOCUS:_1588#TRANSCRIPT:2#ABBREVIATION:CYP19A1# proteins: NAME:cytochrome P450, family 19, subfamily A, polypeptide 1#ABBREVIATION:Cyp19a1.1# NAME:cytochrome P450, family 19, subfamily A, polypeptide 1#ABBREVIATION:Cyp19a1.2# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# AUTHORS:Korzekwa KR, Trager WF, Mancewicz J, Osawa Y#TITLE:Studies on the mechanism of aromatase and other cytochrome P450 mediated deformylation reactions#JOURNAL:J Steroid Biochem Mol Biol#YEAR:1993# GENE ASSOCIATION: (1588.1) or (1588.2)
Abbreviation: R_CAMPt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + camp[c] + h2o[c] --> adp[c] + camp[e] + h[c] + pi[c] Confidence Level: 4 IT genes: LOCUS:_10257#TRANSCRIPT:1#ABBREVIATION:ABCC4# LOCUS:_10057#TRANSCRIPT:1#ABBREVIATION:ABCC5# LOCUS:_10057#TRANSCRIPT:2#ABBREVIATION:ABCC5# proteins: NAME:ATP-binding cassette, sub-family C (CFTR/MRP), member 4 #ABBREVIATION:Abcc4.1-e# NAME:ATP-binding cassette, sub-family C (CFTR/MRP), member 5 #ABBREVIATION:Abcc5.1-e# NAME:ATP-binding cassette, sub-family C (CFTR/MRP), member 5 #ABBREVIATION:Abcc5.2-e# citations: AUTHORS:Chen ZS, Lee K, Kruh GD.#TITLE:Transport of cyclic nucleotides and estradiol 17-beta-D-glucuronide by multidrug resistance protein 4. Resistance to 6-mercaptopurine and 6-thioguanine.#JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11447229# AUTHORS:Reid G, Wielinga P, Zelcer N, van der Heijden I, Kuil A, de Haas M, Wijnholds J, Borst P. #TITLE:The human multidrug resistance protein MRP4 functions as a prostaglandin efflux transporter and is inhibited by nonsteroidal antiinflammatory drugs.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2003#PUBMED:12835412# AUTHORS:Dazert P, Meissner K, Vogelgesang S, Heydrich B, Eckel L, Bohm M, Warzok R, Kerb R, Brinkmann U, Schaeffeler E, Schwab M, Cascorbi I, Jedlitschky G, Kroemer HK. #TITLE:Expression and localization of the multidrug resistance protein 5 (MRP5/ABCC5), a cellular export pump for cyclic nucleotides, in human heart.#JOURNAL:Am J Pathol. #YEAR:2003#PUBMED:14507663# AUTHORS:Meyer Zu Schwabedissen HE, Grube M, Heydrich B, Linnemann K, Fusch C, Kroemer HK, Jedlitschky G. #TITLE:Expression, localization, and function of MRP5 (ABCC5), a transporter for cyclic nucleotides, in human placenta and cultured human trophoblasts: effects of gestational age and cellular differentiation. #JOURNAL:Am J Pathol.#YEAR:2005#PUBMED:15631998# AUTHORS:Wielinga PR, van der Heijden I, Reid G, Beijnen JH, Wijnholds J, Borst P. #TITLE:Characterization of the MRP4- and MRP5-mediated transport of cyclic nucleotides from intact cells. #JOURNAL: J Biol Chem. #YEAR:2003#PUBMED:12637526# GENE ASSOCIATION: (10257.1) or (10057.2) or (10057.1)
Abbreviation: R_ADPtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: adp[c] <==> adp[x] Confidence Level: 1 most likely NOT physiological - detailed information about the peroxisomal transporters has not yet been achieved - this transport is most likely dependent on a concomitant energy consuming reaction (human ABC peroxisomal transporters: PEX family - check in future for new research articles describing biochemistry and stoichiometry) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ADNCYC Synonyms: _3 EC Number: 4.6.1.1 SUBSYSTEM: Nucleotides Equation: [c] : atp --> camp + ppi Confidence Level: 4 JLR genes: LOCUS:_112#TRANSCRIPT:1#ABBREVIATION:ADCY6#ECNUMBERS:4.6.1.1# LOCUS:_113#TRANSCRIPT:1#ABBREVIATION:ADCY7#ECNUMBERS:4.6.1.1# LOCUS:_111#TRANSCRIPT:1#ABBREVIATION:ADCY5#ECNUMBERS:4.6.1.1# LOCUS:_55811#TRANSCRIPT:1#ABBREVIATION:SAC#ECNUMBERS:4.6.1.1# LOCUS:_114#TRANSCRIPT:1#ABBREVIATION:ADCY8#ECNUMBERS:4.6.1.1# LOCUS:_115#TRANSCRIPT:1#ABBREVIATION:ADCY9#ECNUMBERS:4.6.1.1# LOCUS:_108#TRANSCRIPT:1#ABBREVIATION:ADCY2#ECNUMBERS:4.6.1.1# LOCUS:_109#TRANSCRIPT:1#ABBREVIATION:ADCY3#ECNUMBERS:4.6.1.1# LOCUS:_107#TRANSCRIPT:1#ABBREVIATION:ADCY1#ECNUMBERS:4.6.1.1# LOCUS:_196883#TRANSCRIPT:1#ABBREVIATION:ADCY4#ECNUMBERS:4.6.1.1# LOCUS:_112#TRANSCRIPT:2#ABBREVIATION:ADCY6#ECNUMBERS:4.6.1.1# proteins: NAME:adenylate cyclase 1 (brain) #ABBREVIATION:Adcy1.1# NAME:adenylate cyclase 2 (brain) #ABBREVIATION:Adcy2.1# NAME:adenylate cyclase 3 #ABBREVIATION:Adcy3.1 # NAME:adenylate cyclase 4 #ABBREVIATION:Adcy4.1# NAME:adenylate cyclase 5 #ABBREVIATION:Adcy5.1# NAME:adenylate cyclase 6 #ABBREVIATION:Adcy6.1# NAME:adenylate cyclase 6#ABBREVIATION:Adcy6.2# NAME:adenylate cyclase 7 #ABBREVIATION:Adcy7.1# NAME:adenylate cyclase 8 (brain) #ABBREVIATION:Adcy8.1# NAME:adenylate cyclase 9 #ABBREVIATION:Adcy9.1# NAME:testicular soluble adenylyl cyclase #ABBREVIATION:Sac.1# citations: AUTHORS:Ding Q, Gros R, Chorazyczewski J, Ferguson SS, Feldman RD. #TITLE:Isoform-specific regulation of adenylyl cyclase function by disruption of membrane trafficking.#JOURNAL:Mol Pharmacol.#YEAR:2005#PUBMED:15547246# AUTHORS:Ludwig MG, Seuwen K. #TITLE:Characterization of the human adenylyl cyclase gene family: cDNA, gene structure, and tissue distribution of the nine isoforms. #JOURNAL:J Recept Signal Transduct Res#YEAR:2002#PUBMED:12503609# AUTHORS:Gaudin C, Homcy CJ, Ishikawa Y.#TITLE:Mammalian adenylyl cyclase family members are randomly located on different chromosomes.#JOURNAL:Hum Genet.#YEAR:1994#PUBMED:7959689# AUTHORS:Stengel D, Parma J, Gannage MH, Roeckel N, Mattei MG, Barouki R, Hanoune J.#TITLE:Different chromosomal localization of two adenylyl cyclase genes expressed in human brain.#JOURNAL:Hum Genet. #YEAR:1992#PUBMED:1427768# AUTHORS:Katsel PL, Tagliente TM, Schwarz TE, Craddock-Royal BD, Patel ND, Maayani S.#TITLE:Molecular and biochemical evidence for the presence of type III adenylyl cyclase in human platelets. #JOURNAL:Platelets#YEAR:2003#PUBMED:12623444# AUTHORS:Patrizio M, Colucci M, Levi G.#TITLE:Human immunodeficiency virus type 1 Tat protein decreases cyclic AMP synthesis in rat microglia cultures.#JOURNAL:J Neurochem.#YEAR:2001#PUBMED:11299302# AUTHORS:Raimundo S, Giray J, Volff JN, Schwab M, Altenbuchner J, Ratge D, Wisser H.#TITLE:Cloning and sequence of partial cDNAs encoding the human type V and VI adenylyl cyclases and subsequent RNA-quantification in various tissues.#JOURNAL:Clin Chim Acta. #YEAR:1999#PUBMED:10481931# AUTHORS:Hellevuo K, Berry R, Sikela JM, Tabakoff B.#TITLE:Localization of the gene for a novel human adenylyl cyclase (ADCY7) to chromosome 16.#JOURNAL:Hum Genet. #YEAR:1995#PUBMED:7860067# AUTHORS:Defer N, Marinx O, Stengel D, Danisova A, Iourgenko V, Matsuoka I, Caput D, Hanoune J.#TITLE:Molecular cloning of the human type VIII adenylyl cyclase.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8076676# AUTHORS:Cumbay MG, Watts VJ.#TITLE:Novel regulatory properties of human type 9 adenylate cyclase.#JOURNAL:J Pharmacol Exp Ther.#YEAR:2004#PUBMED:14996950# AUTHORS:Toyota T, Hattori E, Meerabux J, Yamada K, Saito K, Shibuya H, Nankai M, Yoshikawa T.#TITLE:Molecular analysis, mutation screening, and association study of adenylate cyclase type 9 gene (ADCY9) in mood disorders.#JOURNAL:Am J Med Genet. #YEAR:2002#PUBMED:11840511# AUTHORS:Jaiswal BS, Conti M.#TITLE:Identification and functional analysis of splice variants of the germ cell soluble adenylyl cyclase. #JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11423534# AUTHORS:Litvin TN, Kamenetsky M, Zarifyan A, Buck J, Levin LR.#TITLE:Kinetic properties of "soluble" adenylyl cyclase. Synergism between calcium and bicarbonate.#JOURNAL:J Biol Chem.#YEAR:2003#PUBMED:12609998# GENE ASSOCIATION: (112.1) or (55811.1) or (109.1) or (112.2) or (114.1) or (108.1) or (113.1) or (107.1) or (196883.1) or (115.1) or (111.1)
Abbreviation: R_FAOXC2251836m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (2) coa + dcsptn1coa + (2) h2o + (2) nad --> (2) accoa + (2) h + lnlncgcoa + (2) nadh Confidence Level: 4 _2 rounds of beta-ox, plus a loss of 2 degrees of desatuartion, so no fad is oxidized peroxisomal (very long chain FA) c22:5,n-6 -> c18:3, n-6 - final '6' in abbreviation indicates omega6 FA 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_NRVNCCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + nrvnccrn --> crn + nrvnccoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_SPH1Ptr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: sph1p[c] <==> sph1p[r] Confidence Level: 2 unknown mechanism for transport, may be vesicular/caveolar/other ... intracellular transport and extracellular export mechanisms must exist - see for example: PMID: 15585321 NJ genes: none proteins: none citations: AUTHORS:Baumruker T, Bornancin F, Billich A.#TITLE:The role of sphingosine and ceramide kinases in inflammatory responses.#JOURNAL:Immunol Lett.#YEAR:2005#PUBMED:15585321# AUTHORS:Quest AF, Leyton L, Parraga M.#TITLE:Caveolins, caveolae, and lipid rafts in cellular transport, signaling, and disease.#JOURNAL:Biochem Cell Biol.#YEAR:2004#PUBMED:15052333# GENE ASSOCIATION:
Abbreviation: R_ALOX12R Synonyms: _2 EC Number: 1.13.11.31 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : arachd + o2 --> 12RHPET Confidence Level: 4 NJ genes: LOCUS:_242#TRANSCRIPT:1#ABBREVIATION:ALOX12B# proteins: NAME:arachidonate 12-lipoxygenase, 12R#ABBREVIATION:Alox12b.1# citations: TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Boeglin WE, Kim RB, Brash AR.#TITLE:A 12R-lipoxygenase in human skin: mechanistic evidence, molecular cloning, and expression.#JOURNAL:Proc Natl Acad Sci#YEAR:1998#PUBMED:9618483# GENE ASSOCIATION: (242.1)
Abbreviation: R_EX_taxol_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : taxol <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
linoleic acid ACP (all cis)_C29H51N2O8PRS
Abbreviation: R_PI345P3P Synonyms: _0 EC Number: 3.1.3.67 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail345p_hs --> pail45p_hs + pi Confidence Level: 4 NCD genes: LOCUS:_5728#TRANSCRIPT:1#ABBREVIATION:PTEN# proteins: NAME:phosphatase and tensin homolog (mutated in multiple advanced cancers 1) #ABBREVIATION:Pten.1# citations: AUTHORS:Ono H, Katagiri H, Funaki M, Anai M, Inukai K, Fukushima Y, Sakoda H, Ogihara T, Onishi Y, Fujishiro M, Kikuchi M, Oka Y, Asano T#TITLE:Regulation of phosphoinositide metabolism, Akt phosphorylation, and glucose transport by PTEN (phosphatase and tensin homolog deleted on chromosome 10) in 3T3-L1 adipocytes#JOURNAL:Mol Endocrinol#YEAR:2001#PUBMED:11463863# GENE ASSOCIATION: (5728.1)
phosphatidylinositol-3,4,5-trisphosphate 3-phosphatase
Abbreviation: R_WHTTDCAte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: whttdca[e] <==> whttdca[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ALATA_L Synonyms: _0 EC Number: 2.6.1.2 SUBSYSTEM: Glutamate metabolism Equation: [c] : akg + ala-L <==> glu-L + pyr Confidence Level: 4 genes: LOCUS:_2875#TRANSCRIPT:1#ABBREVIATION:GPT1#ECNUMBERS:2.6.1.2# LOCUS:_84706#TRANSCRIPT:1#ABBREVIATION:GPT2#ECNUMBERS:2.6.1.2# proteins: NAME:glutamic-pyruvate transaminase (alanine aminotransferase) 1#ABBREVIATION:Gpt1.1# NAME:glutamic-pyruvate transaminase (alanine aminotransferase) 2#ABBREVIATION:Gpt2.1# citations: AUTHORS:Yang RZ, Blaileanu G, Hansen BC, Shuldiner AR, Gong DW.#TITLE:cDNA cloning, genomic structure, chromosomal mapping, and functional expression of a novel human alanine aminotransferase.#JOURNAL:Genomics#YEAR:2002#PUBMED:11863375# GENE ASSOCIATION: (2875.1) or (84706.1)
Abbreviation: R_PIK5n Synonyms: _3 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + pail_hs --> adp + h + pail5p_hs Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
Abbreviation: R_FUT95g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucfucgalacglcgal14acglcgalgluside_hs + gdpfuc --> fucfucfucgalacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
Abbreviation: R_CRVNCtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: crvnc[e] <==> crvnc[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_EX_lipoate_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lipoate <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FPGS2 Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [c] : 5thf + atp + glu-L --> 6thf + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_TCYNTtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: tcynt[m] --> tcynt[c] Confidence Level: 2 thiocyanate is found excreted in urine (PMID: 6351723) MM genes: none proteins: none citations: AUTHORS:Cooper AJ#TITLE:Biochemistry of sulfur-containing amino acids.#YEAR:1983#PUBMED:6351723# GENE ASSOCIATION:
Thiocyanate transport via diffusion (mitochondrial)
Abbreviation: R_S3T3g Synonyms: _5 EC Number: 2.8.2.23 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre15 + paps --> h + hspg + pap Confidence Level: 4 NCD genes: LOCUS:_9955#TRANSCRIPT:1#ABBREVIATION:HS3ST3A1#ECNUMBERS:2.8.2.23# LOCUS:_9953#TRANSCRIPT:1#ABBREVIATION:HS3ST3B1#ECNUMBERS:2.8.2.23# LOCUS:_9952#TRANSCRIPT:1#ABBREVIATION:HS3ST3B2#ECNUMBERS:2.8.2.23# LOCUS:_64711#TRANSCRIPT:1#ABBREVIATION:HS3ST6# LOCUS:_9954#TRANSCRIPT:1#ABBREVIATION:HS3ST3A2#ECNUMBERS:2.8.2.23# LOCUS:_9951#TRANSCRIPT:1#ABBREVIATION:HS3ST4#ECNUMBERS:2.8.2.23# proteins: NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 3A1, Golgi apparatus#ABBREVIATION:Hs3st3a1.1-g# NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 3A2, Golgi apparatus#ABBREVIATION:Hs3st3a2.1-g# NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 3B1, Golgi apparatus#ABBREVIATION:Hs3st3b1.1-g# NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 3B2#ABBREVIATION:Hs3st3b2.1-g# NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 4, Golgi apparatus#ABBREVIATION:Hs3st4.1-g# NAME:heparan sulfate (glucosamine) 3-O-sulfotransferase 6, Golgi apparatus#ABBREVIATION:Hs3st6.1-g# citations: AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Shworak NW, Liu J, Fritze LM, Schwartz JJ, Zhang L, Logeart D, Rosenberg RD#TITLE:Molecular cloning and expression of mouse and human cDNAs encoding heparan sulfate D-glucosaminyl 3-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9346953# AUTHORS:Liu J, Shworak NW, Sinay P, Schwartz JJ, Zhang L, Fritze LM, Rosenberg RD#TITLE:Expression of heparan sulfate D-glucosaminyl 3-O-sulfotransferase isoforms reveals novel substrate specificities#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:9988768# AUTHORS:Liu J, Shriver Z, Blaiklock P, Yoshida K, Sasisekharan R, Rosenberg RD#TITLE:Heparan sulfate D-glucosaminyl 3-O-sulfotransferase-3A sulfates N-unsubstituted glucosamine residues#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10608887# AUTHORS:Xu D, Tiwari V, Xia G, Clement C, Shukla D, Liu J#TITLE:Characterization of heparan sulphate 3-O-sulphotransferase isoform 6 and its role in assisting the entry of herpes simplex virus type 1#JOURNAL:Biochem J#YEAR:2005#PUBMED:15303968# GENE ASSOCIATION: (9951.1) or (9952.1) or (9955.1) or (9953.1) or (9954.1) or (64711.1)
Abbreviation: R_FUT91g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : gal14acglcgalgluside_hs + gdpfuc --> fucgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
Abbreviation: R_ADSL2 Synonyms: _0 EC Number: 4.3.2.2 SUBSYSTEM: IMP Biosynthesis Equation: [c] : 25aics --> aicar + fum Confidence Level: 4 genes: LOCUS:_158#TRANSCRIPT:1#ABBREVIATION:ADSL#ECNUMBERS:4.3.2.2# LOCUS:_158#TRANSCRIPT:2#ABBREVIATION:ADSL# proteins: NAME:adenylosuccinate lyase#ABBREVIATION:Adsl.1# NAME:adenylosuccinate lyase#ABBREVIATION:Adsl.2# citations: AUTHORS:Barton JW, Hart IM, Patterson D.#TITLE:Mapping of a locus correcting lack of phosphoribosylaminoimidazole carboxylase activity in Chinese hamster ovary cell Ade-D mutants to human chromosome 4. #JOURNAL:Genomics#YEAR:1991#PUBMED:2004782# AUTHORS:Stone RL, Aimi J, Barshop BA, Jaeken J, Van den Berghe G, Zalkin H, Dixon JE.#TITLE:A mutation in adenylosuccinate lyase associated with mental retardation and autistic features.#JOURNAL:Nat Genet. #YEAR:1992#PUBMED:1302001# GENE ASSOCIATION: (158.1) or (158.2)
Abbreviation: R_H2O2tn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: h2o2[c] <==> h2o2[n] Confidence Level: 2 it genes: none proteins: none citations: AUTHORS:Ohno Y, Gallin JI. #TITLE:Diffusion of extracellular hydrogen peroxide into intracellular compartments of human neutrophils. Studies utilizing the inactivation of myeloperoxidase by hydrogen peroxide and azide.#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2989289# GENE ASSOCIATION:
Abbreviation: R_HXPRT Synonyms: _0 EC Number: 2.4.2.8 SUBSYSTEM: Salvage Pathway Equation: [c] : hxan + prpp --> imp + ppi Confidence Level: 4 Guanine (see GUAPRT) and 6-mercaptopurine can replace hypoxanthine genes: LOCUS:_3251#TRANSCRIPT:1#ABBREVIATION:HPRT1#ECNUMBERS:2.4.2.8# proteins: NAME:hypoxanthine phosphoribosyltransferase 1 (Lesch-Nyhan syndrome) #ABBREVIATION:HprT1.1# citations: AUTHORS:Wilson JM, Kobayashi R, Fox IH, Kelley WN.#TITLE: Human hypoxanthine-guanine phosphoribosyltransferase. #JOURNAL:J Biol Chem.#YEAR:1983#PUBMED:6853490# AUTHORS:Kim SH, Moores JC, David D, Respess JG, Jolly DJ, Friedmann T.#TITLE:The organization of the human HPRT gene.#JOURNAL:Nucleic Acids Res#YEAR:1986#PUBMED:3008106# GENE ASSOCIATION: (3251.1)
hypoxanthine phosphoribosyltransferase (Hypoxanthine)
Abbreviation: R_ALCD2if Synonyms: _0 EC Number: 1.1.1.1 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : etoh + nad --> acald + h + nadh Confidence Level: 0 NCD genes: LOCUS:_130#TRANSCRIPT:1#ABBREVIATION:ADH6#ECNUMBERS:1.1.1.1# LOCUS:_126#TRANSCRIPT:1#ABBREVIATION:ADH1C#ECNUMBERS:1.1.1.1# LOCUS:_125#TRANSCRIPT:1#ABBREVIATION:ADH1B#ECNUMBERS:1.1.1.1# LOCUS:_128#TRANSCRIPT:1#ABBREVIATION:ADH5#ECNUMBERS:1.1.1.1# LOCUS:_127#TRANSCRIPT:1#ABBREVIATION:ADH4#ECNUMBERS:1.1.1.1# LOCUS:_124#TRANSCRIPT:1#ABBREVIATION:ADH1A#ECNUMBERS:1.1.1.1# LOCUS:_131#TRANSCRIPT:1#ABBREVIATION:ADH7#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:1#ABBREVIATION:ZADH2# LOCUS:_137872#TRANSCRIPT:1#ABBREVIATION:ADHFE1#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:2#ABBREVIATION:ZADH2# proteins: NAME:alcohol dehydrogenase IV #ABBREVIATION:Adh4# NAME:alcohol dehydrogenase V#ABBREVIATION:Adh5# NAME:alcohol dehydrogenase 1A (class I), alpha polypeptide#ABBREVIATION:Adh1a.1# NAME:alcohol dehydrogenase IB (class I), beta polypeptide#ABBREVIATION:Adh1b.1# NAME:alcohol dehydrogenase 1C (class I), gamma polypeptide#ABBREVIATION:Adh1c.1# NAME:alcohol dehydrogenase 6 (class V)#ABBREVIATION:Adh6.1# NAME:alcohol dehydrogenase 7 (class IV), mu or sigma polypeptide#ABBREVIATION:Adh7.1# NAME:alcohol dehydrogenase, iron containing, 1 (liver)#ABBREVIATION:Adhfe1.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ab.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ac# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1bc# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.1# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.2# citations: AUTHORS:Deng Y, Wang Z, Gu S, Ji C, Ying K, Xie Y, Mao Y#TITLE:Cloning and characterization of a novel human alcohol dehydrogenase gene (ADHFe1)#JOURNAL:DNA Seq#YEAR:2002#PUBMED:12592711# AUTHORS:Ramchandani VA, Bosron WF, Li TK#TITLE:Research advances in ethanol metabolism#JOURNAL:Pathol Biol (Paris)#YEAR:2001#PUBMED:11762128# GENE ASSOCIATION: (126.1 and 124.1) or (131.1) or (126.1) or (125.1 and 126.1) or (128.1) or (137872.1) or (125.1) or (284273.2) or (127.1) or (124.1) or (125.1 and 124.1) or (284273.1) or (130.1) or (126.1)
alcohol dehydrogenase, forward rxn (ethanol -> acetaldehyde)
Abbreviation: R_NAt3_1g Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: h[g] + na1[c] <==> h[c] + na1[g] Confidence Level: 4 JLR genes: LOCUS:_23315#TRANSCRIPT:1#ABBREVIATION:SLC9A8# LOCUS:_84679#TRANSCRIPT:1#ABBREVIATION:SLC9A7# proteins: NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 7#ABBREVIATION:Slc9a7.1-g# NAME:solute carrier family 9 (sodium/hydrogen exchanger), isoform 8#ABBREVIATION:Slc9a8.1-g# citations: AUTHORS:Numata M, Orlowski J#TITLE:Molecular cloning and characterization of a novel (Na+,K+)/H+ exchanger localized to the trans-Golgi network#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11279194# AUTHORS:Nakamura N, Tanaka S, Teko Y, Mitsui K, Kanazawa H.#TITLE:Four Na+/H+ exchanger isoforms are distributed to Golgi and post-Golgi compartments and are involved in organelle pH regulation.#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15522866# GENE ASSOCIATION: (84679.1) or (23315.1)
Abbreviation: R_TMNDNCCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + tmndnccrn --> crn + tmndnccoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_10FTHFtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _10fthf[c] <==> 10fthf[m] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_10-Formyltetrahydrofolate mitochondrial transport via diffusion
Abbreviation: R_NDPK5n Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [n] : atp + dgdp <==> adp + dgtp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.1# NAME:Nucleosinde diphosphate kinase AB, nuclear#ABBREVIATION:NME12_n.2# citations: none GENE ASSOCIATION: (4831.1 and 4830.1) or (4830.2 and 4831.1)
nucleoside-diphosphate kinase (ATP:dGDP), nuclear
Abbreviation: R_GCC2am Synonyms: _0 EC Number: 1.4.4.2 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : gly + h + lpam <==> alpam + co2 Confidence Level: 4 NCD genes: LOCUS:_2731#TRANSCRIPT:1#ABBREVIATION:GLDC#ECNUMBERS:1.4.4.2# LOCUS:_275#TRANSCRIPT:1#ABBREVIATION:AMT#ECNUMBERS:2.1.2.10# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_2653#TRANSCRIPT:1#ABBREVIATION:GCSH# proteins: NAME:glycine dehydrogenase (decarboxylating; glycine decarboxylase, glycine cleavage system protein P), mitochondrial #ABBREVIATION:Gldc.1-m# NAME:aminomethyltransferase (glycine cleavage system protein T), mitochondrial #ABBREVIATION:Amt.1-m# NAME:glycine cleavage system protein H (aminomethyl carrier) #ABBREVIATION:Gcsh.1-m# NAME:dihydrolipoamide dehydrogenase, mitochondrial#ABBREVIATION:Dld.1-m# citations: AUTHORS:Lee HH, Kim do J, Ahn HJ, Ha JY, Suh SW.#TITLE:Crystal structure of T-protein of the glycine cleavage system. Cofactor binding, insights into H-protein recognition, and molecular basis for understanding nonketotic hyperglycinemia.#YEAR:2004#PUBMED:15355973# GENE ASSOCIATION: (275.1) and (1738.1) and (2731.1) and (2653.1)
Abbreviation: R_LINKDEG4ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e_deg7 + (5) h2o --> (2) gal + glcur + (2) h + (2) so4 + xyl-D Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
Abbreviation: R_HMGCOAtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: hmgcoa[c] <==> hmgcoa[m] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Biardi L, Krisans K#TITLE:Compartmentalization of cholesterol biosynthesis#JOURNAL:Journal of Biological Chemistry#YEAR:1996# AUTHORS:Kovacs WJ, Krisans S.#TITLE:Cholesterol biosynthesis and regulation: role of peroxisomes.#JOURNAL:Adv Exp Med Biol.#YEAR:2003#PUBMED:14713247# GENE ASSOCIATION:
Hydroxymethylglutaryl-CoA reversible mitochondrial transport
Abbreviation: R_EX_1mncam_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 1mncam <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GTHP Synonyms: _0 EC Number: 1.11.1.9 SUBSYSTEM: Glutathione Metabolism Equation: [c] : (2) gthrd + h2o2 <==> gthox + (2) h2o Confidence Level: 4 NCD genes: LOCUS:_2876#TRANSCRIPT:1#ABBREVIATION:GPX1#ECNUMBERS:1.11.1.9# LOCUS:_2879#TRANSCRIPT:1#ABBREVIATION:GPX4# LOCUS:_2877#TRANSCRIPT:1#ABBREVIATION:GPX2#ECNUMBERS:1.11.1.9# LOCUS:_5052#TRANSCRIPT:1#ABBREVIATION:PRDX1# LOCUS:_7001#TRANSCRIPT:1#ABBREVIATION:PRDX2# LOCUS:_7001#TRANSCRIPT:2#ABBREVIATION:PRDX2# LOCUS:_7001#TRANSCRIPT:3#ABBREVIATION:PRDX2# LOCUS:_5052#TRANSCRIPT:3#ABBREVIATION:PRDX1# LOCUS:_5052#TRANSCRIPT:2#ABBREVIATION:PRDX1# LOCUS:_2876#TRANSCRIPT:2#ABBREVIATION:GPX1#ECNUMBERS:1.11.1.9# proteins: NAME:glutathione peroxidase 1#ABBREVIATION:Gpx1.1# NAME:glutathione peroxidase 1#ABBREVIATION:Gpx1.2# NAME:glutathione peroxidase 2 (gastrointestinal)#ABBREVIATION:Gpx2.1# NAME:glutathione peroxidase 4 (phospholipid hydroperoxidase)#ABBREVIATION:Gpx4.1# NAME:Peroxiredoxin1#ABBREVIATION:Prdx1.1# NAME:Peroxiredoxin1#ABBREVIATION:Prdx1.2# NAME:Peroxiredoxin1#ABBREVIATION:Prdx1.3# NAME:Peroxiredoxin2#ABBREVIATION:Prdx2.1# NAME:Peroxiredoxin2#ABBREVIATION:Prdx2.2# NAME:Peroxiredoxin2#ABBREVIATION:Prdx2.3# citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# GENE ASSOCIATION: (7001.3) or (5052.3) or (2877.1) or (2876.2) or (5052.2) or (2876.1) or (5052.1) or (7001.1) or (2879.1) or (7001.2)
L-glutamine/L-asparagine Na-dependent exchange (Asn-L in)
Abbreviation: R_ASNGLNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: asn-L[e] + gln-L[c] + na1[e] --> asn-L[c] + gln-L[e] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
Abbreviation: R_CHSTEROLt2 Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: chsterol[r] <==> chsterol[m] Confidence Level: 2 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: AUTHORS:Jefcoate C.#TITLE:High-flux mitochondrial cholesterol trafficking, a specialized function of the adrenal cortex.#JOURNAL:J Clin Invest#YEAR:2002#PUBMED:12370263# AUTHORS:Strauss JF 3rd, Kishida T, Christenson LK, Fujimoto T, Hiroi H.#TITLE:START domain proteins and the intracellular trafficking of cholesterol in steroidogenic cells.#JOURNAL:Mol Cell Endocrinol#YEAR:2003#PUBMED:12770731# GENE ASSOCIATION:
Abbreviation: R_GMPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: gmp[c] <==> gmp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_G14T5g Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre9 + udpgal --> h + ksii_core2_pre10 + udp Confidence Level: 4 Keratan sulfate II biosynthesis NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Mengle-Gaw L, McCoy-Haman MF, Tiemeier DC#TITLE:Genomic structure and expression of human beta-1,4-galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1903938# AUTHORS:Masri KA, Appert HE, Fukuda MN#TITLE:Identification of the full-length coding sequence for human galactosyltransferase (beta-N-acetylglucosaminide: beta 1,4-galactosyltransferase)#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3144273# AUTHORS:Appert HE, Rutherford TJ, Tarr GE, Wiest JS, Thomford NR, McCorquodale DJ#TITLE:Isolation of a cDNA coding for human galactosyltransferase#JOURNAL:Biochem Biophys Res Commun#YEAR:1986#PUBMED:3094506# AUTHORS:Almeida R, Amado M, David L, Levery SB, Holmes EH, Merkx G, van Kessel AG, Rygaard E, Hassan H, Bennett E, Clausen H#TITLE:A family of human beta4-galactosyltransferases. Cloning and expression of two novel UDP-galactose:beta-n-acetylglucosamine beta1, 4-galactosyltransferases, beta4Gal-T2 and beta4Gal-T3#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9405390# AUTHORS:Sato T, Furukawa K, Bakker H, Van den Eijnden DH, Van Die I.#TITLE:Molecular cloning of a human cDNA encoding beta-1,4-galactosyltransferase with 37% identity to mammalian UDP-Gal:GlcNAc beta-1,4-galactosyltransferase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9435216# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (9334.1) or (2683.1) or (8704.2) or (8704.1) or (8703.1)
Abbreviation: R_CHSTEROLSULT Synonyms: _0 EC Number: 2.8.2.2 SUBSYSTEM: Steroid Metabolism Equation: [c] : chsterol + paps --> chsterols + h + pap Confidence Level: 4 NJ genes: LOCUS:_6820#TRANSCRIPT:1#ABBREVIATION:SULT2B1# proteins: NAME:sulfotransferase family, cytosolic, 2B, member 1#ABBREVIATION:Sult2b1.1# citations: AUTHORS:Her C, Wood TC, Eichler EE, Mohrenweiser HW, Ramagli LS, Siciliano MJ, Weinshilboum RM.#TITLE:Human hydroxysteroid sulfotransferase SULT2B1: two enzymes encoded by a single chromosome 19 gene.#JOURNAL:Genomics#YEAR:1998#PUBMED:9799594# AUTHORS:Fuda H, Lee YC, Shimizu C, Javitt NB, Strott CA.#TITLE:Mutational analysis of human hydroxysteroid sulfotransferase SULT2B1 isoforms reveals that exon 1B of the SULT2B1 gene produces cholesterol sulfotransferase, whereas exon 1A yields pregnenolone sulfotransferase.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12145317# GENE ASSOCIATION: (6820.1)
Abbreviation: R_EX_urea_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : urea <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PGCD Synonyms: _0 EC Number: 1.1.1.95 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : 3pg + nad --> 3php + h + nadh Confidence Level: 4 genes: LOCUS:_26227#TRANSCRIPT:1#ABBREVIATION:PHGDH#ECNUMBERS:1.1.1.95# proteins: NAME:phosphoglycerate dehydrogenase #ABBREVIATION:Phgdh.1# citations: AUTHORS:Cho HM, Jun DY, Bae MA, Ahn JD, Kim YH.#TITLE:Nucleotide sequence and differential expression of the human 3-phosphoglycerate dehydrogenase gene.#YEAR:2000#PUBMED:10713460# GENE ASSOCIATION: (26227.1)
Abbreviation: R_AGPSx Synonyms: _11 EC Number: 2.5.1.26 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [x] : adhap_hs + alkylR1oh --> Rtotal + akdhap_hs + h Confidence Level: 4 NJ genes: LOCUS:_8540#TRANSCRIPT:1#ABBREVIATION:AGPS#ECNUMBERS:2.5.1.26# proteins: NAME:alkylglycerone phosphate synthase#ABBREVIATION:Agps.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:deVet ECJM, vandenBosch H#TITLE:Alkyl-dihydroxyacetonephosphate synthase#JOURNAL:Cell Biochemsitry and Biophysics#YEAR:2000# AUTHORS:deVet ECJM, Ijlst L, Oostheim W, Wanders RJA, vandenBosch H#TITLE:Alkyl-dihydroxyacetonephosphate synthase#JOURNAL:Journal of Biological Chemistry#YEAR:1998# AUTHORS:Hajra AK#TITLE:Glycerolipid biosynthesis in peroxisomes#JOURNAL:Prog Lipid Res#YEAR:1995# GENE ASSOCIATION: (8540.1)
Abbreviation: R_UMPK4n Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : gtp + ump <==> gdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_FADtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: fad[c] <==> fad[x] Confidence Level: 1 most likely NOT physiological - detailed information about the peroxisomal transporters has not yet been achieved - this transport is most likely dependent on a concomitant energy consuming reaction (human ABC peroxisomal transporters: PEX family - check in future for new research articles describing biochemistry and stoichiometry) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLUVESSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + glu-L[c] + h2o[c] --> adp[c] + glu-L[e] + h[c] + pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of glu from the cell. It leaves out the vesicle because a compartment is not available for that. ATP demand is not known stoichiometrically. genes: LOCUS:_246213#TRANSCRIPT:1#ABBREVIATION:SLC17A8# LOCUS:_57084#TRANSCRIPT:1#ABBREVIATION:SLC17A6# LOCUS:_57030#TRANSCRIPT:1#ABBREVIATION:SLC17A7# proteins: NAME:solute carrier family 17 (sodium-dependent inorganic phosphate cotransporter), member 6#ABBREVIATION:Slc17a6.1# NAME:solute carrier family 17 (sodium-dependent inorganic phosphate cotransporter), member 7#ABBREVIATION:Slc17a7.1# NAME:solute carrier family 17 (sodium-dependent inorganic phosphate cotransporter), member 8#ABBREVIATION:Slc17a8.1# citations: AUTHORS:Reimer RJ, Edwards RH#TITLE:Organic anion transport is the primary function of the SLC17/type I phosphate transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12811560# AUTHORS:Bai L, Xu H, Collins JF, Ghishan FK#TITLE:Molecular and functional analysis of a novel neuronal vesicular glutamate transporter#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11432869# AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# GENE ASSOCIATION: (57084.1) or (246213.1) or (57030.1)
L-glutamate secretion via secretory vesicle (ATP driven)
Abbreviation: R_S6T6g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre10 + paps --> h + ksi_pre11 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_PPPGOm Synonyms: _0 EC Number: 1.3.3.4 SUBSYSTEM: Heme Biosynthesis Equation: [m] : (3) o2 + (2) pppg9 --> (6) h2o + (2) ppp9 Confidence Level: 2 NCD genes: LOCUS:_5498#TRANSCRIPT:1#ABBREVIATION:PPOX#ECNUMBERS:1.3.3.4# proteins: NAME:protoporphyrinogen oxidase #ABBREVIATION:Ppox.1-m# citations: AUTHORS:Dailey TA, Dailey HA.#TITLE:Human protoporphyrinogen oxidase: expression, purification, and characterization of the cloned enzyme.#JOURNAL:Protein Sci#YEAR:1996#PUBMED:8771201# GENE ASSOCIATION: (5498.1)
protoporphyrinogen oxidase, mitochondrial
Abbreviation: R_NDPK4 Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [c] : atp + dtdp <==> adp + dttp Confidence Level: 4 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_29922#TRANSCRIPT:1#ABBREVIATION:NME7# LOCUS:_4831#TRANSCRIPT:1#ABBREVIATION:NME2# LOCUS:_4830#TRANSCRIPT:1#ABBREVIATION:NME1# LOCUS:_4832#TRANSCRIPT:1#ABBREVIATION:NME3# LOCUS:_10201#TRANSCRIPT:1#ABBREVIATION:NME6# LOCUS:_29922#TRANSCRIPT:2#ABBREVIATION:NME7# LOCUS:_4830#TRANSCRIPT:2#ABBREVIATION:NME1# proteins: NAME:Nucleoside diphosphate kinase AB #ABBREVIATION:NME12.1# NAME:Nucleoside diphosphate kinase AB#ABBREVIATION:NME12.2# NAME:Nucleoside diphosphate kinase 3#ABBREVIATION:NME3.1# NAME:Nucleoside diphosphate kinase 6#ABBREVIATION:NME6.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.1# NAME:Nucleoside phosphate kinase 7#ABBREVIATION:NME7.2# citations: none GENE ASSOCIATION: (29922.2) or (10201.1) or (4831.1 and 4830.1) or (4832.1) or (4831.1 and 4830.2) or (29922.1)
nucleoside-diphosphate kinase (ATP:dTDP)
heparan sulfate, degradation product 13_C55H83N3O61S5
carbamoyl phosphate transport, endoplasmic reticulum
Abbreviation: R_CBPter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: cbp[c] + pi[c] + ppi[c] <==> cbp[r] + pi[r] + ppi[r] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Lucius RW, Waddell ID, Burchell A, Nordlie RC#TITLE:The hepatic glucose-6-phosphatase system in Ehrlich-ascites-tumour-bearing mice#JOURNAL:Biochem J.#YEAR:1993#PUBMED:8384451# AUTHORS:Foster JD, Nordlie RC#TITLE:The biochemistry and molecular biology of the glucose-6-phosphatase system.#JOURNAL:Exp Biol Med (Maywood)#YEAR:2002#PUBMED:12192101# GENE ASSOCIATION:
Abbreviation: R_INSTt4_2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: inost[e] + (2) na1[e] <==> inost[c] + (2) na1[c] Confidence Level: 4 NCD genes: LOCUS:_115584#TRANSCRIPT:1#ABBREVIATION:SLC5A11# proteins: NAME:solute carrier family 5 (sodium/glucose cotransporter), member 11#ABBREVIATION:Slc5a11.1-e# citations: AUTHORS:Coady MJ, Wallendorff B, Gagnon DG, Lapointe JY#TITLE:Identification of a novel Na+/myo-inositol cotransporter#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12133831# AUTHORS:Roll P, Massacrier A, Pereira S, Robaglia-Schlupp A, Cau P, Szepetowski P#TITLE:New human sodium/glucose cotransporter gene (KST1): identification, characterization, and mutation analysis in ICCA (infantile convulsions and choreoathetosis) and BFIC (benign familial infantile convulsions) families#JOURNAL:Gene#YEAR:2002#PUBMED:12039040# GENE ASSOCIATION: (115584.1)
inositol-1,4,5-trisphosphate 3-kinase, nucleus
Abbreviation: R_MI145PKn Synonyms: _1 EC Number: 2.7.1.127 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + mi145p --> adp + h + mi1345p Confidence Level: 4 NCD genes: LOCUS:_253430#TRANSCRIPT:1#ABBREVIATION:IPMK# proteins: NAME:inositol polyphosphate multikinase, nucleus#ABBREVIATION:Ipmk.1-n# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:Chang SC, Miller AL, Feng Y, Wente SR, Majerus PW. #TITLE:The human homolog of the rat inositol phosphate multikinase is an inositol 1,3,4,6-tetrakisphosphate 5-kinase#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12223481# AUTHORS:Nalaskowski MM, Deschermeier C, Fanick W, Mayr GW#TITLE:The human homologue of yeast ArgRIII protein is an inositol phosphate multikinase with predominantly nuclear localization#JOURNAL:Biochem J#YEAR:2002#PUBMED:12027805# GENE ASSOCIATION: (253430.1)
Abbreviation: R_ARTFR42 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + odecoa[c] --> (1.125) R4coa_hs[c] + fad[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PPItx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: ppi[c] <==> ppi[x] Confidence Level: 1 diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FUCASE2ly Synonyms: _1 EC Number: 3.2.1.51 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi --> fuc-L + ksi_deg1 Confidence Level: 4 NCD genes: LOCUS:_2517#TRANSCRIPT:1#ABBREVIATION:FUCA1#ECNUMBERS:3.2.1.51# proteins: NAME:alpha-fucosidase 1, lysosomal (tissue)#ABBREVIATION:Fuca1.1-l# citations: AUTHORS:Johnson SW, Alhadeff JA.#TITLE:Mammalian alpha-L-fucosidases#JOURNAL:Comp Biochem Physiol B#YEAR:1991#PUBMED:1769200# AUTHORS:Occhiodoro T, Beckmann KR, Morris CP, Hopwood JJ#TITLE:Human alpha-L-fucosidase: complete coding sequence from cDNA clones#JOURNAL:Biochem Biophys Res Commun#YEAR:1989#PUBMED:2803312# AUTHORS:Fukushima H, de Wet JR, O'Brien JS#TITLE:Molecular cloning of a cDNA for human alpha-L-fucosidase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1985#PUBMED:2983333# AUTHORS:Khunsook S, Alhadeff JA, Bean BS#TITLE:Purification and characterization of human seminal plasma alpha-L-fucosidase.#JOURNAL:Mol Hum Reprod#YEAR:2002#PUBMED:11870229# GENE ASSOCIATION: (2517.1)
Abbreviation: R_ARTFR212 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: dcsptn1coa[c] + (3) fadh2[m] + (2) h[m] + (2) nadph[m] --> (1.375) R2coa_hs[c] + (3) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TDP Synonyms: _4 EC Number: 3.6.1.15 SUBSYSTEM: Thiamine Metabolism Equation: [c] : h2o + thmpp --> h + pi + thmmp Confidence Level: 4 _01-25-05 IT AMF genes: none proteins: none citations: AUTHORS:Bettendorff L, Mastrogiacomo F, Kish SJ, Grisar T.#TITLE:Thiamine, thiamine phosphates, and their metabolizing enzymes in human brain. #JOURNAL:J Neurochem. #YEAR:1996#PUBMED:8522961# AUTHORS:Laforenza U, Mazzarello P, Patrini C, Poloni M, Casadei GP, Rindi G.#TITLE:Different distribution of thiaminpyrophosphatase activity in neuronal and glial cell enriched fractions from human and rat brain: an isoelectric focusing investigation. #JOURNAL:Basic Appl Histochem.#YEAR:1990#PUBMED:2171493# GENE ASSOCIATION:
Abbreviation: R_CYTK13 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : datp + dcmp <==> dadp + dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_3SALACBOXL Synonyms: _0 EC Number: 4.1.1.15 SUBSYSTEM: Taurine and hypotaurine metabolism Equation: [c] : 3sala + h --> co2 + hyptaur Confidence Level: 4 SAB genes: LOCUS:_2572#TRANSCRIPT:1#ABBREVIATION:GAD2#ECNUMBERS:4.1.1.15# LOCUS:_2571#TRANSCRIPT:1#ABBREVIATION:GAD1#ECNUMBERS:4.1.1.15# LOCUS:_51380#TRANSCRIPT:1#ABBREVIATION:CSAD# LOCUS:_2571#TRANSCRIPT:2#ABBREVIATION:GAD1#ECNUMBERS:4.1.1.15# proteins: NAME:glutamate decarboxylase 1 (brain, 67kDa)#ABBREVIATION:Gad1.1# NAME:glutamate decarboxylase 1 (brain, 67kDa)#ABBREVIATION:Gad1.2# NAME:glutamate decarboxylase 2 (pancreatic islets and brain, 65kDa)#ABBREVIATION:Gad2.1# NAME:cysteine sulfinic acid decarboxylase#ABBREVIATION:Csad.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (51380.1) or (2571.1) or (2572.1) or (2571.2)
inositol-1,4,5-triphosphate 6-kinase, nucleus
Abbreviation: R_MI145P6Kn Synonyms: _1 EC Number: 2.7.1.151 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + mi145p --> adp + h + mi1456p Confidence Level: 4 NCD genes: LOCUS:_253430#TRANSCRIPT:1#ABBREVIATION:IPMK# proteins: NAME:inositol polyphosphate multikinase, nucleus#ABBREVIATION:Ipmk.1-n# citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# AUTHORS:Chang SC, Miller AL, Feng Y, Wente SR, Majerus PW. #TITLE:The human homolog of the rat inositol phosphate multikinase is an inositol 1,3,4,6-tetrakisphosphate 5-kinase#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12223481# AUTHORS:Nalaskowski MM, Deschermeier C, Fanick W, Mayr GW#TITLE:The human homologue of yeast ArgRIII protein is an inositol phosphate multikinase with predominantly nuclear localization#JOURNAL:Biochem J#YEAR:2002#PUBMED:12027805# GENE ASSOCIATION: (253430.1)
Abbreviation: R_S4T1g Synonyms: _1 EC Number: 2.8.2.5 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_pre + (2) paps --> cs_a,b,e_pre1 + (2) h + (2) pap Confidence Level: 4 NCD genes: LOCUS:_50515#TRANSCRIPT:1#ABBREVIATION:CHST11# LOCUS:_55501#TRANSCRIPT:1#ABBREVIATION:CHST12# LOCUS:_166012#TRANSCRIPT:1#ABBREVIATION:CHST13# LOCUS:_113189#TRANSCRIPT:1#ABBREVIATION:D4ST1# proteins: NAME:carbohydrate (chondroitin 4) sulfotransferase 11, Golgi#ABBREVIATION:Chst11.1-g# NAME:carbohydrate (chondroitin 4) sulfotransferase 12, Golgi apparatus#ABBREVIATION:Chst12.1-g# NAME:carbohydrate (chondroitin 4) sulfotransferase 13, Golgi apparatus#ABBREVIATION:Chst13.1-g# NAME:dermatan 4 sulfotransferase 1, Golgi apparatus#ABBREVIATION:D4st.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Yamauchi S, Mita S, Matsubara T, Fukuta M, Habuchi H, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of chondroitin 4-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10722746# AUTHORS:Okuda T, Mita S, Yamauchi S, Matsubara T, Yagi F, Yamamori D, Fukuta M, Kuroiwa A, Matsuda Y, Habuchi O#TITLE:Molecular cloning, expression, and chromosomal mapping of human chondroitin 4-sulfotransferase, whose expression pattern in human tissues is different from that of chondroitin 6-sulfotransferase#JOURNAL:J Biochem (Tokyo)#YEAR:2000#PUBMED:11056388# AUTHORS:Hiraoka N, Nakagawa H, Ong E, Akama TO, Fukuda MN, Fukuda M#TITLE:Molecular cloning and expression of two distinct human chondroitin 4-O-sulfotransferases that belong to the HNK-1 sulfotransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781601# AUTHORS:Kang HG, Evers MR, Xia G, Baenziger JU, Schachner M#TITLE:Molecular cloning and characterization of chondroitin-4-O-sulfotransferase-3. A novel member of the HNK-1 family of sulfotransferases#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12080076# AUTHORS:Evers MR, Xia G, Kang HG, Schachner M, Baenziger JU#TITLE:Molecular cloning and characterization of a dermatan-specific N-acetylgalactosamine 4-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:1147079# AUTHORS:Mikami T, Mizumoto S, Kago N, Kitagawa H, Sugahara K.#TITLE:Specificities of three distinct human chondroitin/dermatan N-acetylgalactosamine 4-O-sulfotransferases demonstrated using partially desulfated dermatan sulfate as an acceptor: implication of differential roles in dermatan sulfate biosynthesis.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12847091# GENE ASSOCIATION: (55501.1) or (166012.1) or (50515.1) or (113189.1)
Abbreviation: R_EX_phyt_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : phyt <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_P45011B12m Synonyms: _3 EC Number: 1.14.15.4 SUBSYSTEM: Steroid Metabolism Equation: [m] : 11docrtsl + h + nadph + o2 --> crtsl + h2o + nadp Confidence Level: 4 Payne and Hales (2004) Mol Bio of Steroidogenic Enzymes 25(6):947-970 H2O added for balancing NJ genes: LOCUS:_1584#TRANSCRIPT:1#ABBREVIATION:CYP11B1#ECNUMBERS:1.14.15.4# proteins: NAME:cytochrome P450, family 11, subfamily B, polypeptide 1#ABBREVIATION:Cyp11b1.1# citations: AUTHORS:Payne AH, Hales DB#TITLE:Overview of steroidogenic enzymes in the pathway from cholesterol to active steroid hormones#JOURNAL:Endocrine Reviews#YEAR:2004# GENE ASSOCIATION: (1584.1)
Abbreviation: R_PCHOLPg_hs Synonyms: _2 EC Number: 3.1.4.4 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [g] : h2o + pchol_hs --> chol + h + pa_hs Confidence Level: 4 NJ genes: LOCUS:_5337#TRANSCRIPT:1#ABBREVIATION:PLD1#ECNUMBERS:3.1.4.4# proteins: NAME:phospholipase D1, phosphatidylcholine-specific#ABBREVIATION:Pld1.1# citations: AUTHORS:Cases S, Stone SJ, Zhou P, Yen E, Tow B, Lardizabal KD, Voelker T, Farese RV Jr.#TITLE:Cloning of DGAT2, a second mammalian diacylglycerol acyltransferase, and related family members.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11481335# AUTHORS:Hammond SM, Altshuller YM, Sung TC, Rudge SA, Rose K, Engebrecht J, Morris AJ, Frohman MA.#TITLE:Human ADP-ribosylation factor-activated phosphatidylcholine-specific phospholipase D defines a new and highly conserved gene family.#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:8530346# GENE ASSOCIATION: (5337.1)
Abbreviation: R_NTD6l Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleic acid degradation Equation: [l] : damp + h2o --> dad-2 + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:R.L. Pisoni#TITLE:Lysosomal Nucleic Acid and Phosphate Metabolism and Related Metabolic Reactions, Chapter 9#YEAR:1996# GENE ASSOCIATION: (53.1)
Abbreviation: R_ETHAK Synonyms: _0 EC Number: 2.7.1.82 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : atp + etha --> adp + ethamp + h Confidence Level: 4 NCD genes: LOCUS:_1119#TRANSCRIPT:1#ABBREVIATION:CHK#ECNUMBERS:2.7.1.32# LOCUS:_55500#TRANSCRIPT:1#ABBREVIATION:EKI1#ECNUMBERS:2.7.1.82# LOCUS:_55224#TRANSCRIPT:1#ABBREVIATION:ETNK2# LOCUS:_1119#TRANSCRIPT:2#ABBREVIATION:CHK# proteins: NAME:choline kinase#ABBREVIATION:Chk.1# NAME:choline kinase#ABBREVIATION:Chk.2# NAME:ethanolamine kinase#ABBREVIATION:Etnk1# NAME:ethanolamine kinase#ABBREVIATION:Etnk2# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Kent C#TITLE:Eukaryotic phospholipid biosynthesis#JOURNAL:Annu Rev Biochem#YEAR:1995# GENE ASSOCIATION: (1119.2) or (55500.1) or (1119.1) or (55224.1)
Abbreviation: R_SGPL11r Synonyms: _3 EC Number: 4.1.2.27 SUBSYSTEM: Sphingolipid Metabolism Equation: [r] : sph1p --> ethamp + hxdcal Confidence Level: 4 NJ genes: LOCUS:_8879#TRANSCRIPT:1#ABBREVIATION:SGPL1# proteins: NAME:sphingosine-1-phosphate lyase 1 #ABBREVIATION:Sgpl.1# citations: AUTHORS:Van Veldhoven PP, Gijsbers S, Mannaerts GP, Vermeesch JR, Brys V.#TITLE:Human sphingosine-1-phosphate lyase: cDNA cloning, functional expression studies and mapping to chromosome 10q22(1).#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:11018465# AUTHORS:Reiss U, Oskouian B, Zhou J, Gupta V, Sooriyakumaran P, Kelly S, Wang E, Merrill AH Jr, Saba JD.#TITLE:Sphingosine-phosphate lyase enhances stress-induced ceramide generation and apoptosis.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14570870# GENE ASSOCIATION: (8879.1)
Abbreviation: R_FAOXC18280m Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (5) coa + (3) fad + (5) h2o + lneldccoa + (5) nad --> (5) accoa + (3) fadh2 + (5) h + (5) nadh + occoa Confidence Level: 4 c18:2, transe -> c8:0 - 5 rounds of beta ox, lose 2 degrees of unsat - only 3 fad oxidized 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: none GENE ASSOCIATION: (34.1) or (35.1)
Abbreviation: R_DMGtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dmgly[c] <==> dmgly[m] Confidence Level: 2 MM genes: none proteins: none citations: none GENE ASSOCIATION:
dimethylglycine transport via diffusion (cytosol to mitochondria)
Abbreviation: R_GLCNACASE5ly Synonyms: _4 EC Number: 3.2.1.50 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg24 --> acgam + hs_deg25 Confidence Level: 4 NCD genes: LOCUS:_4669#TRANSCRIPT:1#ABBREVIATION:NAGLU#ECNUMBERS:3.2.1.50# proteins: NAME:N-acetylglucosaminidase, alpha- (Sanfilippo disease IIIB), lysosome#ABBREVIATION:Naglu.1-l# citations: AUTHORS:Weber B, Blanch L, Clements PR, Scott HS, Hopwood JJ#TITLE:Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B)#JOURNAL:Hum Mol Genet#YEAR:1996#PUBMED:8776591# AUTHORS:Sasaki T, Sukegawa K, Masue M, Fukuda S, Tomatsu S, Orii T#TITLE:Purification and partial characterization of alpha-N-acetylglucosaminidase from human liver#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1783617# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION: (4669.1)
Abbreviation: R_NMNS Synonyms: _0 EC Number: 2.4.2.12 SUBSYSTEM: NAD Metabolism Equation: [c] : h + ncam + prpp --> nmn + ppi Confidence Level: 4 genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_ARGt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: arg-L[e] + na1[e] --> arg-L[c] + na1[c] Confidence Level: 2 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# proteins: NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# AUTHORS:Van Winkle LJ#TITLE:Amino acid transport regulation and early embryo development#JOURNAL:Biol Reprod#YEAR:2001#PUBMED:11133652# GENE ASSOCIATION: (11254.1)
L-arginine transport in via sodium symport
Abbreviation: R_HDCAter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: hdca[r] --> hdca[c] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_crmp_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : crmp_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_chtn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : chtn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLPASE1 Synonyms: _0 EC Number: 2.4.1.1 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : glygn2 + (3) pi --> dxtrn + (3) g1p Confidence Level: 4 NCD genes: LOCUS:_5836#TRANSCRIPT:1#ABBREVIATION:PYGL#ECNUMBERS:2.4.1.1# LOCUS:_5837#TRANSCRIPT:1#ABBREVIATION:PYGM#ECNUMBERS:2.4.1.1# LOCUS:_5834#TRANSCRIPT:1#ABBREVIATION:PYGB#ECNUMBERS:2.4.1.1# proteins: NAME:glycogen phosphorylase (brain)#ABBREVIATION:Pygb.1# NAME:glycogen phosphorylase (Hers disease, glycogen storage disease type VI) (liver)#ABBREVIATION:Pygl.1# NAME:glycogen phosphorylase (McArdle syndrome, glycogen storage disease type V) (muscle)#ABBREVIATION:Pygm.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Newgard CB, Littman DR, van Genderen C, Smith M, Fletterick RJ#TITLE:Human brain glycogen phosphorylase. Cloning, sequence analysis, chromosomal mapping, tissue expression, and comparison with the human liver and muscle isozymes#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3346228# GENE ASSOCIATION: (5834.1) or (5837.1) or (5836.1)
glycogen phosphorylase (glygn2 -> dxtrn)
Abbreviation: R_ESTRADIOLGLCtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: estradiolglc[c] <==> estradiolglc[r] Confidence Level: 2 unknown mechanism, however glucuronidated metabolites need to get out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NADPHtxu Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: nadph[c] --> nadph[x] Confidence Level: 1 diffusion - unidirectional to avoid NADP/NAD loops w/ free hydrogens NJ genes: none proteins: none citations: AUTHORS:Geisbrecht BV, Gould SJ#TITLE:The human PICD gene encodes a cytoplasmic and peroxisomal NADP(+)-dependent isocitrate dehydrogenase#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10521434# GENE ASSOCIATION:
Abbreviation: R_ARTFR55 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: (3) fadh2[m] + (2) h[m] + (2) nadph[m] + tetpent3coa[c] --> (1.5) R5coa_hs[c] + (3) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ALDSTRNte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: aldstrn[c] <==> aldstrn[e] Confidence Level: 2 specific mechanism not known NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ABUTD Synonyms: _1 EC Number: 1.2.1.19 SUBSYSTEM: beta-Alanine metabolism Equation: [c] : 4abutn + h2o + nad --> 4abut + (2) h + nadh Confidence Level: 4 JLR genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# proteins: NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# citations: AUTHORS:Lin SW, Chen JC, Hsu LC, Hsieh CL, Yoshida A.#TITLE:Human gamma-aminobutyraldehyde dehydrogenase (ALDH9): cDNA sequence, genomic organization, polymorphism, chromosomal localization, and tissue expression.#YEAR:1996#PUBMED:8786138# GENE ASSOCIATION: (223.1)
Abbreviation: R_NACHEXA19ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg36 --> acgam + h + ksi_deg38 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_NRPPHRVESSEC Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) atp[c] + (2) h2o[c] + (3) nrpphr[c] --> (2) adp[c] + (2) h[c] + (3) nrpphr[e] + (2) pi[c] Confidence Level: 4 SAB This reaction represents the ATP driven secretion of nrpphr from the cell. It leaves out the vesicle because that is not a compartment. ATP:H ratio is assumed as 1:3 for vacuolar ATPase and substrate:h ratio is 1:2. This gives a 3:2 ratio for substrate:ATP genes: LOCUS:_6571#TRANSCRIPT:1#ABBREVIATION:SLC18A2# LOCUS:_6570#TRANSCRIPT:1#ABBREVIATION:SLC18A1# proteins: NAME:solute carrier family 18 (vesicular monoamine), member 1#ABBREVIATION:Slc18a1.1# NAME:solute carrier family 18 (vesicular monoamine), member 2#ABBREVIATION:Slc18a2.1# citations: AUTHORS:Wagner CA, Finberg KE, Breton S, Marshansky V, Brown D, Geibel JP#TITLE:Renal vacuolar H+-ATPase#JOURNAL:Physiol Rev#YEAR:2004#PUBMED:15383652# AUTHORS:Eiden LE, Schafer MK, Weihe E, Schutz B#TITLE:The vesicular amine transporter family (SLC18): amine/proton antiporters required for vesicular accumulation and regulated exocytotic secretion of monoamines and acetylcholine#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12827358# GENE ASSOCIATION: (6570.1) or (6571.1)
Noradrenaline secretion via secretory vesicle (ATP driven)
Abbreviation: R_BAAT1x Synonyms: _1 EC Number: 2.3.1.65 SUBSYSTEM: Bile Acid Biosynthesis Equation: [x] : cholcoa + gly --> coa + gchola Confidence Level: 4 glycine or taurine as substrates, produces glycocholic or taurocholic acids respectively NJ genes: LOCUS:_570#TRANSCRIPT:1#ABBREVIATION:BAAT#ECNUMBERS:2.3.1.65# proteins: NAME:bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase)#ABBREVIATION:Baat.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Falany CN, Johnson MR, Barnes S, Diasio RB#TITLE:Glycine and taurine conugation of bile acids by a single enzyme#JOURNAL:Journal of Biological Chemistry#YEAR:1994# GENE ASSOCIATION: (570.1)
Abbreviation: R_CSNATm Synonyms: _0 EC Number: 2.3.1.7 SUBSYSTEM: Fatty Acid Metabolism Equation: [m] : acrn + coa <==> accoa + crn Confidence Level: 4 TDV genes: LOCUS:_1384#TRANSCRIPT:1#ABBREVIATION:CRAT#ECNUMBERS:2.3.1.7# proteins: NAME:carnitine acetyltransferase, mitochondrial#ABBREVIATION:Crat.1-m# citations: AUTHORS:Lysiak W, Toth PP, Suelter CH, Bieber LL.#TITLE:Quantitation of the efflux of acylcarnitines from rat heart, brain, and liver mitochondria.#JOURNAL:J Biol Chem#YEAR:1986#PUBMED:3759988# GENE ASSOCIATION: (1384.1)
Abbreviation: R_PIK4 Synonyms: _3 EC Number: 2.7.1.67 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : atp + pail_hs --> adp + h + pail4p_hs Confidence Level: 4 NJ genes: LOCUS:_5297#TRANSCRIPT:1#ABBREVIATION:PIK4CA#ECNUMBERS:2.7.1.67,2.7.1.68# LOCUS:_5298#TRANSCRIPT:1#ABBREVIATION:PIK4CB# LOCUS:_55361#TRANSCRIPT:1#ABBREVIATION:PI4KII# proteins: NAME:phosphatidylinositol 4-kinase, catalytic, alpha polypeptide#ABBREVIATION:Pik4ca.1# NAME:phosphatidylinositol 4-kinase, catalytic, beta polypeptide#ABBREVIATION:Pik4cb.1# NAME:phosphtaidylinositol 4-kinase, type II#ABBREVIATION:Pi4kii.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Wong K, Cantley LC#TITLE:Cloning and characterization of a human phosphatidylinositol 4-kinase#JOURNAL:Journal of Biological Chemsitry#YEAR:1994# AUTHORS:Minogue S, Anderson JS, Waugh MG, dosSantos M, Corless S, Cramer R, Hsuan JJ#TITLE:Cloning of a human type II phosphatidylinositol 4-kinase reveals a novel lipid kinase family#JOURNAL:Journal of Biological Chemistry#YEAR:2001# AUTHORS:Nakagawa T, Goto K, Kondo H#TITLE:Cloning, expression, and localization of 230-kDa phosphatidylinositol 4-kinase#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8662589# AUTHORS:Burriss Garrett RJ, Redman CM#TITLE:Localization of enzymes involved in polyphosphoinositids metabolism on the cytoplasmic surface of the human erythrocyte membrane#JOURNAL:Biochim Biophys Acta#YEAR:1975#PUBMED:164238# AUTHORS:Wei YJ, Sun HQ, Yamamoto M, Wlodarski P, Kunii K, Martinez M, Barylko B, Albanesi JP, Yin HL. #TITLE:ype II phosphatidylinositol 4-kinase beta is a cytosolic and peripheral membrane protein that is recruited to the plasma membrane and activated by Rac-GTP#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12324459# GENE ASSOCIATION: (5298.1) or (55361.1) or (5297.1)
Abbreviation: R_COAtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: coa[c] <==> coa[x] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FPGS2m Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [m] : 5thf + atp + glu-L --> 6thf + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_XOLTRI25te Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: xoltri25[c] <==> xoltri25[e] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_A_MANASEly Synonyms: _6 EC Number: 3.2.1.24 SUBSYSTEM: N-Glycan Degradation Equation: [l] : (2) h2o + m2mn --> (2) man + mn Confidence Level: 0 NCD genes: LOCUS:_4125#TRANSCRIPT:1#ABBREVIATION:MAN2B1#ECNUMBERS:3.2.1.24# proteins: NAME:mannosidase, alpha, class 2B, member 1, lysosomal (ubiquitous)#ABBREVIATION:Man2b1.1-l# citations: AUTHORS:Liao YF, Lal A, Moremen KW.#TITLE:Cloning, expression, purification, and characterization of the human broad specificity lysosomal acid alpha-mannosidase#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8910458# GENE ASSOCIATION: (4125.1)
Abbreviation: R_TYRt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + tyr-L[e] --> na1[c] + tyr-L[c] Confidence Level: 2 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# proteins: NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# AUTHORS:Van Winkle LJ#TITLE:Amino acid transport regulation and early embryo development#JOURNAL:Biol Reprod#YEAR:2001#PUBMED:11133652# GENE ASSOCIATION: (11254.1)
L-tyrosine transport in via sodium symport
Abbreviation: R_NDPK3m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + cdp <==> adp + ctp Confidence Level: 2 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:CDP), mitochondrial
Abbreviation: R_ADK3m Synonyms: _0 SUBSYSTEM: Nucleotides Equation: [m] : amp + gtp <==> adp + gdp Confidence Level: 4 NCD genes: LOCUS:_50808#TRANSCRIPT:1#ABBREVIATION:AK3L1# proteins: NAME:adenylate kinase 3 like 1 #ABBREVIATION:Ak3L1.1-m# citations: AUTHORS:Noma T, Fujisawa K, Yamashiro Y, Shinohara M, Nakazawa A, Gondo T, Ishihara T, Yoshinobu K.#TITLE:Structure and expression of human mitochondrial adenylate kinase targeted to the mitochondrial matrix. #JOURNAL:Biochem J.#YEAR:2001#PUBMED:11485571# GENE ASSOCIATION: (50808.1)
Abbreviation: R_CYTDt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cytd[e] <==> cytd[c] Confidence Level: 4 IT genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (3177.1) or (2030.1)
Abbreviation: R_PRISTANALtx Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: pristanal[x] <==> pristanal[c] Confidence Level: 1 Mechanism not known, see PMID 11591435 for justification. NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6T24g Synonyms: _3 EC Number: 2.8.2.17 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_e_pre4 + paps --> cs_e_pre5b + h + pap Confidence Level: 4 NCD genes: LOCUS:_56548#TRANSCRIPT:1#ABBREVIATION:CHST7# LOCUS:_9469#TRANSCRIPT:1#ABBREVIATION:CHST3# proteins: NAME:carbohydrate (chondroitin 6) sulfotransferase 3, Golgi#ABBREVIATION:Chst3.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 7, Golgi apparatus#ABBREVIATION:Chst7.1-g# citations: AUTHORS:Habuchi O, Hirahara Y, Uchimura K, Fukuta M#TITLE:Enzymatic sulfation of galactose residue of keratan sulfate by chondroitin 6-sulfotransferase#JOURNAL:Glycobiology#YEAR:1996#PUBMED:8991509# AUTHORS:Fukuta M, Kobayashi Y, Uchimura K, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of human chondroitin 6-sulfotransferase#JOURNAL:Biochim Biophys Acta#YEAR:1998#PUBMED:9714738# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Tsutsumi K, Shimakawa H, Kitagawa H, Sugahara K#TITLE:Functional expression and genomic structure of human chondroitin 6-sulfotransferase#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9883891# AUTHORS:Kitagawa H, Fujita M, Ito N, Sugahara K#TITLE:Molecular cloning and expression of a novel chondroitin 6-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781596# AUTHORS:Bhakta S, Bartes A, Bowman KG, Kao WM, Polsky I, Lee JK, Cook BN, Bruehl RE, Rosen SD, Bertozzi CR, Hemmerich S#TITLE:Sulfation of N-acetylglucosamine by chondroitin 6-sulfotransferase 2 (GST-5)#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10956661# GENE ASSOCIATION: (9469.1) or (56548.1)
Abbreviation: R_FAOXC183806x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : (5) coa + (5) h2o + lnlncgcoa + (5) nad + (2) o2 --> (5) accoa + (5) h + (2) h2o2 + (5) nadh + occoa Confidence Level: 4 _5 rounds of beta ox, 3 degrees loss of saturation -> only 2 fad oxidized to fadh2 - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) peroxisomal (very long chain FA) c18:3,n-6 -> c8:0, 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (30.1 and 51.2 and 1962.1) or (51.1 and 3295.1 and 30.1) or (51.1 and 30.1 and 1962.1)
Abbreviation: R_PETHCT Synonyms: _0 EC Number: 2.7.7.14 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : ctp + ethamp + h --> cdpea + ppi Confidence Level: 4 NCD genes: LOCUS:_5833#TRANSCRIPT:1#ABBREVIATION:PCYT2# proteins: NAME:phosphate cytidylyltransferase 2, ethanolamine#ABBREVIATION:Pcyt2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Kent C#TITLE:Eukaryotic phospholipid biosynthesis#JOURNAL:Annu Rev Biochem#YEAR:1995# AUTHORS:Nakashima A, Hosaka K, Nikawa J#TITLE:Cloning of a human cDNA for CTP-Phosphoethanolamine cytidylyltransferase by complementation in vivo of a yeast mutant#JOURNAL:Journal of Biological Chemistry#YEAR:1997# GENE ASSOCIATION: (5833.1)
Abbreviation: R_RETI3 Synonyms: _0 EC Number: 5.2.1.7 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : retinol <==> retinol-cis-13 Confidence Level: 4 IT genes: none proteins: none citations: AUTHORS:Blaner WS.#TITLE:Cellular metabolism and actions of 13-cis-retinoic acid. #JOURNAL:J Am Acad Dermatol. #YEAR:2001#PUBMED:11606944# GENE ASSOCIATION:
Abbreviation: R_LPS3e Synonyms: _1 EC Number: 3.1.1.23 SUBSYSTEM: Fatty Acid Metabolism Equation: [e] : h2o + mag_hs --> Rtotal2 + glyc + h Confidence Level: 2 NJ genes: LOCUS:_3990#TRANSCRIPT:1#ABBREVIATION:LIPC#ECNUMBERS:3.1.1.3# proteins: NAME:lipase, hepatic#ABBREVIATION:Lipc.1# citations: AUTHORS:Ben-Zeev O, Doolittle MH.#TITLE:Maturation of hepatic lipase. Formation of functional enzyme in the endoplasmic reticulum is the rate-limiting step in its secretion.#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14630921# GENE ASSOCIATION: (3990.1)
Abbreviation: R_GLYAMDTRc Synonyms: _3 EC Number: 2.1.4.1 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : arg-L + gly <==> gudac + orn Confidence Level: 4 SAB c genes: LOCUS:_2628#TRANSCRIPT:1#ABBREVIATION:GATM#ECNUMBERS:2.1.4.1# proteins: NAME:glycine amidinotransferase (L-arginine:glycine amidinotransferase)#ABBREVIATION:Gatm.1# citations: AUTHORS:Isbrandt D, von Figura K#TITLE:Cloning and sequence analysis of human guanidinoacetate N-methyltransferase cDNA#JOURNAL:Biochim Biophys Acta#YEAR:1995#PUBMED:8547310# GENE ASSOCIATION: (2628.1)
Abbreviation: R_GLCAT4g Synonyms: _1 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_d_pre2 + udpglcur --> cs_d_pre3 + h + udp Confidence Level: 4 EC 2.4.1.226 NCD genes: LOCUS:_22856#TRANSCRIPT:1#ABBREVIATION:CHSY1# LOCUS:_54480#TRANSCRIPT:1#ABBREVIATION:CSGlcA-T# LOCUS:_79586#TRANSCRIPT:1#ABBREVIATION:CHPF# LOCUS:_337876#TRANSCRIPT:1#ABBREVIATION:CSS3# proteins: NAME:chondroitin sulfate glucuronyltransferase, Golgi apparatus#ABBREVIATION:Csglca-t.1-g# NAME:carbohydrate (chondroitin) synthase 1, Golgi apparatus#ABBREVIATION:Chsy1.1-g# NAME:chondroitin polymerizing factor, Golgi apparatus#ABBREVIATION:Chpf.1-g# NAME:chondroitin sulfate synthase 3, Golgi apparatus#ABBREVIATION:Css3.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Gotoh M, Yada T, Sato T, Akashima T, Iwasaki H, Mochizuki H, Inaba N, Togayachi A, Kudo T, Watanabe H, Kimata K, Narimatsu H#TITLE:Molecular cloning and characterization of a novel chondroitin sulfate glucuronyltransferase that transfers glucuronic acid to N-acetylgalactosamine#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12145278# AUTHORS:Kitagawa H, Uyama T, Sugahara K#TITLE:Molecular cloning and expression of a human chondroitin synthase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11514575# AUTHORS:Kitagawa H, Izumikawa T, Uyama T, Sugahara K#TITLE:Molecular cloning of a chondroitin polymerizing factor that cooperates with chondroitin synthase for chondroitin polymerization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12716890# AUTHORS:Yada T, Sato T, Kaseyama H, Gotoh M, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-3. Molecular cloning and characterization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12907687# AUTHORS:Yada T, Gotoh M, Sato T, Shionyu M, Go M, Kaseyama H, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12761225# GENE ASSOCIATION: (54480.1) or (337876.1) or (79586.1) and (22856.1)
Abbreviation: R_EX_hom_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hom-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_octa_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : octa <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLCAT9g Synonyms: _2 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre7 + udpglcur --> h + hs_pre8 + udp Confidence Level: 4 EC 2.4.1.225 NCD genes: LOCUS:_2131#TRANSCRIPT:1#ABBREVIATION:EXT1# LOCUS:_2132#TRANSCRIPT:1#ABBREVIATION:EXT2#ECNUMBERS:2.4.1.-# LOCUS:_2132#TRANSCRIPT:2#ABBREVIATION:EXT2# proteins: NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.1-g# NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.2-g# citations: AUTHORS:McCormick C, Duncan G, Goutsos KT, Tufaro F#TITLE:The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10639137# AUTHORS:Kobayashi S, Morimoto K, Shimizu T, Takahashi M, Kurosawa H, Shirasawa T#TITLE:Association of EXT1 and EXT2, hereditary multiple exostoses gene products, in Golgi apparatus#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679296# AUTHORS:Ahn J, Ludecke HJ, Lindow S, Horton WA, Lee B, Wagner MJ, Horsthemke B, Wells DE#TITLE:Cloning of the putative tumour suppressor gene for hereditary multiple exostoses (EXT1)#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7550340# AUTHORS:McCormick C, Leduc Y, Martindale D, Mattison K, Esford LE, Dyer AP, Tufaro F#TITLE:The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate#JOURNAL:Nat Genet#YEAR:1998#PUBMED:9620772# AUTHORS:Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K#TITLE:The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9756849# AUTHORS:Stickens D, Clines G, Burbee D, Ramos P, Thomas S, Hogue D, Hecht JT, Lovett M, Evans GA#TITLE:The EXT2 multiple exostoses gene defines a family of putative tumour suppressor genes#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8782816# GENE ASSOCIATION: (2131.1 and 2132.1) or (2131.1 and 2132.2)
heparan sulfate, precursor 8_C79H116N4O64X
Abbreviation: R_TYRCBOX Synonyms: _0 EC Number: 4.1.1.25 SUBSYSTEM: Tyrosine metabolism Equation: [c] : h + tyr-L --> co2 + tym Confidence Level: 4 SAB KEGG also says EC 4.1.1.28 genes: LOCUS:_1644#TRANSCRIPT:1#ABBREVIATION:DDC#ECNUMBERS:4.1.1.28# proteins: NAME:aromatic-L-amino-acid decarboxylase#ABBREVIATION:Ddc.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (1644.1)
Abbreviation: R_TSULt4_3 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (3) na1[e] + tsul[e] <==> (3) na1[c] + tsul[c] Confidence Level: 4 NCD genes: LOCUS:_6561#TRANSCRIPT:1#ABBREVIATION:SLC13A1# proteins: NAME:solute carrier family 13 (sodium/sulfate symporters), member 1#ABBREVIATION:Slc13a1.1-e# citations: AUTHORS:Lee A, Beck L, Markovich D#TITLE:The human renal sodium sulfate cotransporter (SLC13A1; hNaSi-1) cDNA and gene: organization, chromosomal localization, and functional characterization#JOURNAL:Genomics#YEAR:2000#PUBMED:11161786# AUTHORS:Pajor AM#TITLE:Molecular properties of sodium/dicarboxylate cotransporters#JOURNAL:J Membr Biol#YEAR:2000#PUBMED:10811962# GENE ASSOCIATION: (6561.1)
thiosulfate transport via sodium symport
Abbreviation: R_EX_lgnc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lgnc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DIGALSGALSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: digalsgalside_hs[c] <==> digalsgalside_hs[e] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) - extracellular transport is also made available since metabolites can get to outer plasma membrane NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_GLYCTO1p Synonyms: _0 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [x] : glyclt + o2 --> glx + h2o2 Confidence Level: 4 tv NCD genes: LOCUS:_54363#TRANSCRIPT:1#ABBREVIATION:HAO1#ECNUMBERS:1.1.3.15# LOCUS:_51179#TRANSCRIPT:1#ABBREVIATION:HAO2# LOCUS:_51179#TRANSCRIPT:2#ABBREVIATION:HAO2# proteins: NAME:hydroxyacid oxidase 2 (long chain), peroxisomal (liver, kidney)#ABBREVIATION:Hao2.1-x# NAME:hydroxyacid oxidase 2 (long chain), peroxisomal (liver, kidney)#ABBREVIATION:Hao2.2-x# NAME:hydroxyacid oxidase (glycolate oxidase) 1, peroxisome#ABBREVIATION:Hao1.1-x# citations: AUTHORS:Jones JM, Morrell JC, Gould SJ#TITLE:Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10777549# AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# AUTHORS:Ushijima Y#TITLE:Identity of aliphatic L- -hydroxyacid oxidase and glycolate oxidase from rat livers#JOURNAL:Arch Biochem Biophys#YEAR:1973#PUBMED:4705431# GENE ASSOCIATION: (51179.1) or (54363.1) or (51179.2)
Abbreviation: R_NDPK5m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + dgdp <==> adp + dgtp Confidence Level: 2 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:dGDP), mitochondrial
Abbreviation: R_DOLDPP_Uer Synonyms: _0 EC Number: 3.6.1.43 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) doldp_U + h2o --> (0.1) dolp_U + h + pi Confidence Level: 0 NCD genes: LOCUS:_57171#TRANSCRIPT:1#ABBREVIATION:DOLPP1# proteins: NAME:Dolichol pyrophosphate phosphatase, endoplasmic reticulum#ABBREVIATION:Dolpp1.1-er# citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION: (57171.1)
Dolichyl-diphosphate phosphohydrolase, human (uterus)
Abbreviation: R_S23T2g Synonyms: _0 EC Number: 2.4.99.4 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : cmpacna + core2 --> cmp + h + ksii_core2_pre1 Confidence Level: 4 NCD genes: LOCUS:_6483#TRANSCRIPT:1#ABBREVIATION:SIAT4B#ECNUMBERS:2.4.99.4# LOCUS:_6482#TRANSCRIPT:1#ABBREVIATION:SIAT4A#ECNUMBERS:2.4.99.4# LOCUS:_6484#TRANSCRIPT:1#ABBREVIATION:SIAT4C#ECNUMBERS:2.4.99.4# LOCUS:_6482#TRANSCRIPT:2#ABBREVIATION:SIAT4A#ECNUMBERS:2.4.99.4# proteins: NAME:sialyltransferase 4A (beta-galactoside alpha-2,3-sialyltransferase), Golgi apparatus (placenta, liver, sk muscle)#ABBREVIATION:Siat4a.1-g# NAME:_3-sialyltransferase), Golgi apparatus (placenta, liver, sk muscle)#ABBREVIATION:Siat4a.2-g# NAME:sialyltransferase 4B, Golgi apparatus#ABBREVIATION:Siat4b.1-g# NAME:ST3 beta-galactoside alpha-2,3-sialyltransferase, Golgi#ABBREVIATION:Siat4c.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Kitagawa H, Paulson JC#TITLE:Differential expression of five sialyltransferase genes in human tissues#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8027041# AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Shang J, Qiu R, Wang J, Liu J, Zhou R, Ding H, Yang S, Zhang S, Jin C#TITLE:Molecular cloning and expression of Galbeta1,3GalNAc alpha2, 3-sialyltransferase from human fetal liver#JOURNAL:Eur J Biochem#YEAR:1999#PUBMED:10504389# AUTHORS:Kim YJ, Kim KS, Kim SH, Kim CH, Ko JH, Choe IS, Tsuji S, Lee YC#TITLE:Molecular cloning and expression of human Gal beta 1,3GalNAc alpha 2,3-sialytransferase (hST3Gal II)#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8920913# AUTHORS:Kitagawa H, Paulson JC#TITLE:Cloning of a novel alpha 2,3-sialyltransferase that sialylates glycoprotein and glycolipid carbohydrate groups#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8288606# AUTHORS:Sasaki K, Watanabe E, Kawashima K, Sekine S, Dohi T, Oshima M, Hanai N, Nishi T, Hasegawa M#TITLE:Expression cloning of a novel Gal beta (1-3/1-4) GlcNAc alpha 2,3-sialyltransferase using lectin resistance selection#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:7901202# GENE ASSOCIATION: (6484.1) or (6482.1) or (6483.1) or (6482.2)
beta-galactoside alpha-2,3-sialyltransferase (core 2)
Abbreviation: R_FUCGALFUCGALACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucgalfucgalacglcgalgluside_hs[g] <==> fucgalfucgalacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_2AMACHYD Synonyms: _0 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [c] : 2amac + h2o --> nh4 + pyr Confidence Level: 4 SAB see PMID 500639: this reaction is spontaneous genes: none proteins: none citations: AUTHORS:Gavaret JM, Cahnmann HJ, Nunez J#TITLE:The fate of the "lost side chain" during thyroid hormonogenesis#JOURNAL:J Biol Chem#YEAR:1979#PUBMED:500639# GENE ASSOCIATION:
Abbreviation: R_EX_peplys_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : peplys <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CCA_D3tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: cca_d3[m] --> cca_d3[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Calcitroic acid transport from mitochondria
Abbreviation: R_GALSIDEtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: galside_hs[c] <==> galside_hs[l] Confidence Level: 1 unknown mechanism for transport - not known if it is energy dependent or not, recycling and IC transport is important to get it to other areas of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CSNATp Synonyms: _0 EC Number: 2.3.1.7 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : accoa + crn <==> acrn + coa Confidence Level: 2 NCD genes: LOCUS:_1384#TRANSCRIPT:2#ABBREVIATION:CRAT#ECNUMBERS:2.3.1.7# proteins: NAME:carnitine acetyltransferase, perixosomal#ABBREVIATION:Crat.2-x# citations: AUTHORS:Corti O, Finocchiaro G, Rossi E, Zuffardi O, DiDonato S.#TITLE:Molecular cloning of cDNAs encoding human carnitine acetyltransferase and mapping of the corresponding gene to chromosome 9q34.1.#JOURNAL:Genomics#YEAR:1994#PUBMED:7829107# GENE ASSOCIATION: (1384.2)
carnitine O-acetyltransferase, reverse direction, peroxisomal
Abbreviation: R_VITD3tm3 Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: vitd3[c] --> vitd3[m] Confidence Level: 2 I am not sure how vitamin D3 is transported from cytosol in mitochondria based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_ACNMLr Synonyms: _0 EC Number: 4.1.3.3 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acnam <==> acmana + pyr Confidence Level: 2 JLR genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
_3 alpha-hydroxysteroid dehydrogenase (type 3)
Abbreviation: R_HSD3A1r Synonyms: _0 SUBSYSTEM: Steroid Metabolism Equation: [r] : andrstandn + h + nadh <==> andrstrn + nad Confidence Level: 4 Microsomal rxn see PMID: 14643063 substrate specificity still not clear (nad vs nadp) NJ genes: LOCUS:_1109#TRANSCRIPT:1#ABBREVIATION:AKR1C4#ECNUMBERS:1.1.1.225# proteins: NAME:aldo-keto reductase family 1, member C4#ABBREVIATION:Akr1c4.1# citations: AUTHORS:Belanger A, Pelletier G, Labrie F, Barbier O, Chouinard S.#TITLE:Inactivation of androgens by UDP-glucuronosyltransferase enzymes in humans.#JOURNAL:Trends Endocrinol Metab.#YEAR:2003#PUBMED:14643063# AUTHORS:Khanna M, Qin KN, Wang RW, Cheng KC.#TITLE:Substrate specificity, gene structure, and tissue-specific distribution of multiple human 3 alpha-hydroxysteroid dehydrogenases.#JOURNAL:J Biol Chem. #YEAR:1995#PUBMED:7650035# GENE ASSOCIATION: (1109.1)
Abbreviation: R_THYPX Synonyms: _0 SUBSYSTEM: Tyrosine metabolism Equation: [c] : (2) h + h2o2 + (2) i --> (2) h2o + iodine Confidence Level: 4 NCD genes: LOCUS:_7173#TRANSCRIPT:1#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:2#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:5#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:4#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:3#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# proteins: NAME:thyroid peroxidase#ABBREVIATION:Tpo.1# NAME:thyroid peroxidase#ABBREVIATION:Tpo.2# NAME:thyroid peroxidase#ABBREVIATION:Tpo.3# NAME:thyroid peroxidase#ABBREVIATION:Tpo.4# NAME:thyroid peroxidase#ABBREVIATION:Tpo.5# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Kimura S, Kotani T, McBride OW, Umeki K, Hirai K, Nakayama T, Ohtaki S#TITLE:Human thyroid peroxidase: complete cDNA and protein sequence, chromosome mapping, and identification of two alternately spliced mRNAs#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1987#PUBMED:3475693# AUTHORS:Magnusson RP, Chazenbalk GD, Gestautas J, Seto P, Filetti S, DeGroot LJ, Rapoport B. #TITLE:Molecular cloning of the complementary deoxyribonucleic acid for human thyroid peroxidase.#JOURNAL:Mol Endocrinol#YEAR:1987#PUBMED:3153466# GENE ASSOCIATION: (7173.4) or (7173.1) or (7173.3) or (7173.2) or (7173.5)
Abbreviation: R_NACHEX8ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e_deg2 + h2o --> acgal + cs_e_deg3 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3074.1) or (3073.1)
Abbreviation: R_PRDXl Synonyms: _0 EC Number: 1.11.1.7 SUBSYSTEM: Miscellaneous Equation: [l] : h2o2 + meoh --> fald + (2) h2o Confidence Level: 0 RS/TV (6/3/05) Peroxidases can act on a variety of substrates which are oxidized, not just methanol. genes: LOCUS:_4353#TRANSCRIPT:1#ABBREVIATION:MPO#ECNUMBERS:1.11.1.7# proteins: NAME:Myeloperoxidase, lysosome#ABBREVIATION:Mpo.1-l# citations: AUTHORS:Burner U, Furtmuller PG, Kettle AJ, Koppenol WH, Obinger C.#TITLE:Mechanism of reaction of myeloperoxidase with nitrite#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10777476# AUTHORS:Arnhold J.#TITLE:Properties, functions, and secretion of human myeloperoxidase#JOURNAL:Biochemistry (Mosc)#YEAR:2004#PUBMED:14972011# GENE ASSOCIATION: (4353.1)
Abbreviation: R_DOPAtu Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dopa[e] <==> dopa[c] Confidence Level: 4 SAB electrogenic, Na indepdendent & rev NJ genes: LOCUS:_6582#TRANSCRIPT:1#ABBREVIATION:SLC22A2# LOCUS:_6582#TRANSCRIPT:2#ABBREVIATION:SLC22A2# LOCUS:_6581#TRANSCRIPT:1#ABBREVIATION:SLC22A3# proteins: NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.1# NAME:solute carrier family 22 (organic cation transporter), member 2#ABBREVIATION:Slc22a2.2# NAME:solute carrier family 22 (extraneuronal monoamine transporter), member 3#ABBREVIATION:Slc22a3.1# citations: AUTHORS:Koepsell H, Endou H#TITLE:The SLC22 drug transporter family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12883891# GENE ASSOCIATION: (6582.1) or (6581.1) or (6582.2)
Abbreviation: R_GACPAILter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: gacpail_hs[c] --> gacpail_hs[r] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Murakami Y, Siripanyapinyo U, Hong Y, Kang JY, Ishihara S, Nakakuma H, Maeda Y, Kinoshita T#TITLE:PIG-W is critical for inositol acylation but not for flipping of glycosylphosphatidylinositol-anchor#JOURNAL:Mol Biol Cell#YEAR:2003#PUBMED:14517336# GENE ASSOCIATION:
glucosaminyl-acylphosphatidylinositol ER flippase
Abbreviation: R_C3STDH1r Synonyms: _1 EC Number: 5.3.3.1 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : 4mzym_int1 + nad --> 4mzym_int2 + co2 + h + nadh Confidence Level: 4 NCD ER version NJ genes: LOCUS:_50814#TRANSCRIPT:1#ABBREVIATION:NSDHL# proteins: NAME:NAD(P) dependent steroid dehydrogenase#ABBREVIATION:Nsdhl.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (50814.1)
Abbreviation: R_PHYQt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + phyQ[e] --> adp[c] + h[c] + phyQ[c] + pi[c] Confidence Level: 4 genes: none proteins: none citations: AUTHORS:David A. Bender#TITLE:Vitamin K#YEAR:2003# GENE ASSOCIATION:
Abbreviation: R_THMDt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + thymd[e] --> na1[c] + thymd[c] Confidence Level: 4 SAB genes: LOCUS:_9154#TRANSCRIPT:1#ABBREVIATION:SLC28A1# LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# LOCUS:_9154#TRANSCRIPT:2#ABBREVIATION:SLC28A1# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 1, extracellular #ABBREVIATION:Slc28a1.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 1 #ABBREVIATION:Slc28a1.2-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (9154.1) or (9154.2) or (64078.1)
thymidine transport in via sodium symport
Abbreviation: R_GQ1Btg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: gq1b_hs[c] <==> gq1b_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_MM6B2g Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m6masnB2 --> m5masnB2 + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (10905.1) or (4121.1) or (57134.1)
inositol-3,4,5,6-tetrakisphosphate 1-kinase
Abbreviation: R_MI3456PK Synonyms: _7 EC Number: 2.7.1.134 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + mi3456p --> adp + h + mi13456p Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# GENE ASSOCIATION:
_3-Hydroxykynurenamine:oxygen oxidoreductase(deaminating)(flavin-containing)
Abbreviation: R_3HXKYNOXDA Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Tryptophan metabolism Equation: [c] : 3hxkynam + o2 --> 48dhoxquin + h2o2 + nh4 Confidence Level: 1 SAB genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# proteins: NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: AUTHORS:Smith DJ, Salmi M, Bono P, Hellman J, Leu T, Jalkanen S.#TITLE:Cloning of vascular adhesion protein 1 reveals a novel multifunctional adhesion molecule#JOURNAL: J Exp Med#YEAR:1998#PUBMED:9653080# AUTHORS:Lyles GA#TITLE:Mammalian plasma and tissue-bound semicarbazide-sensitive amine oxidases: biochemical, pharmacological and toxicological aspects#JOURNAL:Int J Biochem Cell Biol#YEAR:1996#PUBMED:8920635# AUTHORS:Buffoni F, Ignesti G#TITLE:Biochemical aspects and functional role of the copper-containing amine oxidases#JOURNAL:Inflammopharmacology#YEAR:2003#PUBMED:15035803# GENE ASSOCIATION: (4128.1) or (4129.1)
Abbreviation: R_FAOXC180 Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : coa + fad + h2o + nad + stcoa --> accoa + fadh2 + h + nadh + pmtcoa Confidence Level: 4 TV Other enzymes: 1.3.3.6, 4.2.1.74, 4.2.1.17, 1.1.1.35, 1.1.1.211 genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Kelly DP, Kim JJ, Billadello JJ, Hainline BE, Chu TW, Strauss AW#TITLE:Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3035565# AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# GENE ASSOCIATION: (35.1) or (35.1) or (34.1) or (34.1)
Abbreviation: R_EICOSTETCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + eicostetcoa --> coa + eicostetcrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_GLPASE2 Synonyms: _0 EC Number: 2.4.1.1 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [c] : glygn3 + (7) h2o --> Tyr-ggn + (7) glc-D Confidence Level: 4 NCD genes: LOCUS:_5836#TRANSCRIPT:1#ABBREVIATION:PYGL#ECNUMBERS:2.4.1.1# LOCUS:_5837#TRANSCRIPT:1#ABBREVIATION:PYGM#ECNUMBERS:2.4.1.1# LOCUS:_5834#TRANSCRIPT:1#ABBREVIATION:PYGB#ECNUMBERS:2.4.1.1# proteins: NAME:glycogen phosphorylase (brain)#ABBREVIATION:Pygb.1# NAME:glycogen phosphorylase (Hers disease, glycogen storage disease type VI) (liver)#ABBREVIATION:Pygl.1# NAME:glycogen phosphorylase (McArdle syndrome, glycogen storage disease type V) (muscle)#ABBREVIATION:Pygm.1# citations: AUTHORS:Newgard CB, Littman DR, van Genderen C, Smith M, Fletterick RJ#TITLE:Human brain glycogen phosphorylase. Cloning, sequence analysis, chromosomal mapping, tissue expression, and comparison with the human liver and muscle isozymes#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3346228# GENE ASSOCIATION: (5836.1) or (5837.1) or (5834.1)
Abbreviation: R_BPNT Synonyms: _0 EC Number: 3.1.3.7 SUBSYSTEM: Nucleotides Equation: [c] : h2o + pap --> amp + pi Confidence Level: 4 genes: LOCUS:_10380#TRANSCRIPT:1#ABBREVIATION:BPNT1#ECNUMBERS:3.1.3.7# proteins: NAME:_3'(2'), 5'-bisphosphate nucleotidase 1 #ABBREVIATION:Bpnt1# citations: AUTHORS:Spiegelberg BD, Xiong JP, Smith JJ, Gu RF, York JD.#TITLE:Cloning and characterization of a mammalian lithium-sensitive bisphosphate 3'-nucleotidase inhibited by inositol 1,4-bisphosphate#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10224133# GENE ASSOCIATION: (10380.1)
Abbreviation: R_IMACTD Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Histidine Metabolism Equation: [c] : h2o + im4act + nad --> (2) h + im4ac + nadh Confidence Level: 2 SAB 2 H's on RHS are there to balance--not certain genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (216.1) or (8854.1) or (224.1) or (8854.2) or (223.1) or (501.1) or (8854.3)
Abbreviation: R_ORNt3m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h[c] + orn[m] <==> h[m] + orn[c] Confidence Level: 4 NCD genes: LOCUS:_83884#TRANSCRIPT:1#ABBREVIATION:SLC25A2# LOCUS:_10166#TRANSCRIPT:1#ABBREVIATION:SLC25A15# proteins: NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 2#ABBREVIATION:Slc25a2.1-m# NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15#ABBREVIATION:Slc25a15.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (83884.1) or (10166.1)
ornithine mitochondrial transport via proton antiport
Abbreviation: R_LCYSTAT Synonyms: _0 EC Number: 2.6.1.1 SUBSYSTEM: Cysteine Metabolism Equation: [c] : Lcyst + akg <==> 3spyr + glu-L Confidence Level: 2 described in PMID: 3346220 and KEGG MM genes: LOCUS:_2805#TRANSCRIPT:1#ABBREVIATION:GOT1#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase #ABBREVIATION:Got1.1# citations: AUTHORS:Weinstein CL, Griffith OW.#TITLE:Cysteinesulfonate and beta-sulfopyruvate metabolism. Partitioning between decarboxylation, transamination, and reduction pathways.#YEAR:1988#PUBMED:3346220# GENE ASSOCIATION: (2805.1)
L-Cysteate:2-oxoglutarate aminotransferase
Abbreviation: R_MALTly Synonyms: _0 EC Number: 3.2.1.20 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [l] : h2o + malt --> (2) glc-D Confidence Level: 4 IF genes: LOCUS:_2548#TRANSCRIPT:1#ABBREVIATION:GAA#ECNUMBERS:3.2.1.20# proteins: NAME:alpha glucosidase; acid (Pompe disease, glycogen storage disease type II), lysosome#ABBREVIATION:Gaa.1-l# citations: AUTHORS:Hoefsloot LH, Hoogeveen-Westerveld M, Kroos MA, van Beeumen J, Reuser AJ, Oostra BA#TITLE:Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex#JOURNAL:EMBO J#YEAR:1988#PUBMED:3049072# GENE ASSOCIATION: (2548.1)
Abbreviation: R_AQCOBALt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: aqcobal[e] --> aqcobal[c] Confidence Level: 2 IT Cobalamin is normally bound to a transcobalami (I-III). Uptake is normally via endocytoces (via lysosome). It is thought that the accobal is release from lysosome in unbounded form but references are contradictory. genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Carmel R, Green R, Rosenblatt DS, Watkins D.#TITLE:Update on cobalamin, folate, and homocysteine.#JOURNAL:Hematology (Am Soc Hematol Educ Program).#YEAR:2003#PUBMED:14633777# GENE ASSOCIATION:
Abbreviation: R_ASPDxt Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: asp-D[x] <==> asp-D[c] Confidence Level: 2 MM genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACSRTNMT Synonyms: _0 EC Number: 2.1.1.4 SUBSYSTEM: Tryptophan metabolism Equation: [c] : Nacsertn + amet --> ahcys + h + melatn Confidence Level: 4 SAB genes: LOCUS:_438#TRANSCRIPT:1#ABBREVIATION:ASMT#ECNUMBERS:2.1.1.4# proteins: NAME:acetylserotonin O-methyltransferase #ABBREVIATION:Asmt.1# citations: AUTHORS:Donohue SJ, Roseboom PH, Illnerova H, Weller JL, Klein DC#TITLE:Human hydroxyindole-O-methyltransferase: presence of LINE-1 fragment in a cDNA clone and pineal mRNA#JOURNAL:DNA Cell Biol#YEAR:1993#PUBMED:8397829# GENE ASSOCIATION: (438.1)
S-Adenosyl-L-homocysteine:N-acetylserotonin O-methyltransferase
Abbreviation: R_EX_7dhf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 7dhf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GALGT1 Synonyms: _3 EC Number: 2.4.1.92 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : galgluside_hs + udpacgal --> ga2_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_2583#TRANSCRIPT:1#ABBREVIATION:GALGT#ECNUMBERS:2.4.1.92# proteins: NAME:UDP-N-acetyl-alpha-D-galactosamine:(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase (GalNAc-T)#ABBREVIATION:Galgt.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Furukawa K, Soejima H, Niikawa N, Shiku H.#TITLE:Genomic organization and chromosomal assignment of the human beta1, 4-N-acetylgalactosaminyltransferase gene. Identification of multiple transcription units.#JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8702839# AUTHORS:Nagata Y, Yamashiro S, Yodoi J, Lloyd KO, Shiku H, Furukawa K.#TITLE:Expression cloning of beta 1,4 N-acetylgalactosaminyltransferase cDNAs that determine the expression of GM2 and GD2 gangliosides.#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1601877# GENE ASSOCIATION: (2583.1)
Abbreviation: R_FACOAL150 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + ptdca <==> amp + ppi + ptdcacoa Confidence Level: 4 AR genes: LOCUS:_2182#TRANSCRIPT:1#ABBREVIATION:ACSL4# LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# LOCUS:_2181#TRANSCRIPT:1#ABBREVIATION:ACSL3# LOCUS:_2181#TRANSCRIPT:2#ABBREVIATION:ACSL3# LOCUS:_22305#TRANSCRIPT:1#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# LOCUS:_22305#TRANSCRIPT:2#ABBREVIATION:ACSL6#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.1# NAME:acyl-CoA synthetase long-chain family member 4#ABBREVIATION:Acsl4.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.2# NAME:acyl-CoA synthetase long-chain family member 6#ABBREVIATION:Acsl6.1# NAME:acyl-CoA synthetase long-chain family member 6 #ABBREVIATION:Acsl6.2# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (22305.2) or (2182.1) or (2180.1) or (2181.2) or (2181.1) or (22305.1)
Iodide:hydrogen-peroxide oxidoreductase 2
Abbreviation: R_IDHPOXOX2b Synonyms: _0 EC Number: 1.11.1.8 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 3ityr-L + iodine --> 35diotyr + h + i Confidence Level: 4 SAB H + I instead of HI genes: LOCUS:_7173#TRANSCRIPT:1#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:2#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:5#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:4#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# LOCUS:_7173#TRANSCRIPT:3#ABBREVIATION:TPO#ECNUMBERS:1.11.1.7# proteins: NAME:thyroid peroxidase#ABBREVIATION:Tpo.1# NAME:thyroid peroxidase#ABBREVIATION:Tpo.2# NAME:thyroid peroxidase#ABBREVIATION:Tpo.3# NAME:thyroid peroxidase#ABBREVIATION:Tpo.4# NAME:thyroid peroxidase#ABBREVIATION:Tpo.5# citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# AUTHORS:Nakamura M, Yamazaki I, Kotani T, Ohtaki S#TITLE:Thyroid peroxidase selects the mechanism of either 1- or 2-electron oxidation of phenols, depending on their substituents#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2997169# GENE ASSOCIATION: (7173.4) or (7173.1) or (7173.3) or (7173.2) or (7173.5)
Abbreviation: R_DHORD9 Synonyms: _0 SUBSYSTEM: Pyrimidine Biosynthesis Equation: dhor-S[c] + q10[m] --> orot[c] + q10h2[m] Confidence Level: 4 _06-15-05 - EC 1.3.3.1 IT based on Bader et al, 1998, Prot. Expr. Purif,., 13,414-422 (I guess it is ubiquinone but i am not sure ) (needs FMN as cofactor) v(enzyme is located in inner-membrane of mitochondria, reaction take place in cytosol (Fig. 8 Rawls et al PMID: 10727948 ) genes: LOCUS:_1723#TRANSCRIPT:1#ABBREVIATION:DHODH#ECNUMBERS:1.3.3.1# proteins: NAME:Dihydroorotate degydrogenase#ABBREVIATION:DhodH.1# citations: AUTHORS:Bader B, Knecht W, Fries M, Loffler M.#TITLE: Expression, purification, and characterization of histidine-tagged rat and human flavoenzyme dihydroorotate dehydrogenase.#JOURNAL:Protein Expr Purif.#YEAR:1998#PUBMED:9693067# AUTHORS:Loffler M, Jockel J, Schuster G, Becker C.#TITLE:Dihydroorotat-ubiquinone oxidoreductase links mitochondria in the biosynthesis of pyrimidine nucleotides.#JOURNAL:Mol Cell Biochem.#YEAR:1997# AUTHORS:Rawls J, Knecht W, Diekert K, Lill R, Loffler M.#TITLE:Requirements for the mitochondrial import and localization of dihydroorotate dehydrogenase.#JOURNAL:Eur J Biochem. #YEAR:2005#PUBMED:10727948# GENE ASSOCIATION: (1723.1)
dihydoorotic acid dehydrogenase (quinone10)
Abbreviation: R_EX_sarcs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : sarcs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_dgchol_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dgchol <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PI4P3Kn Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : atp + pail4p_hs --> adp + h + pail34p_hs Confidence Level: 2 NCD Notice that contrary to some standards in texts, KEGG, etc, the Rtotal group accounts for the whole fatty acyl CoA (so the CHO only accounts for the core backbone), manner in which Rtotal was produced accounts for O and H balances. Formation of the Rtotal group (rxn flux)accounted for mass balance for CoA NJ changed EC from 2.7.1.149 (pail5p) to 2.7.1.68 (pail4p) - NCD genes: none proteins: none citations: AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION:
phosphatidylinositol 4-phosphate 3-kinase, nuclear
prostaglandine E2 transport (ATP-dependent)
Abbreviation: R_PROSTGE2t3 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + prostge2[c] --> adp[c] + h[c] + pi[c] + prostge2[e] Confidence Level: 4 IT genes: LOCUS:_10257#TRANSCRIPT:1#ABBREVIATION:ABCC4# proteins: NAME:ATP-binding cassette, sub-family C (CFTR/MRP), member 4 #ABBREVIATION:Abcc4.1-e# citations: AUTHORS:Chen ZS, Lee K, Kruh GD.#TITLE:Transport of cyclic nucleotides and estradiol 17-beta-D-glucuronide by multidrug resistance protein 4. Resistance to 6-mercaptopurine and 6-thioguanine.#JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11447229# AUTHORS:Reid G, Wielinga P, Zelcer N, van der Heijden I, Kuil A, de Haas M, Wijnholds J, Borst P. #TITLE:The human multidrug resistance protein MRP4 functions as a prostaglandin efflux transporter and is inhibited by nonsteroidal antiinflammatory drugs.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2003#PUBMED:12835412# AUTHORS:Wielinga PR, van der Heijden I, Reid G, Beijnen JH, Wijnholds J, Borst P. #TITLE:Characterization of the MRP4- and MRP5-mediated transport of cyclic nucleotides from intact cells. #JOURNAL: J Biol Chem. #YEAR:2003#PUBMED:12637526# GENE ASSOCIATION: (10257.1)
Abbreviation: R_S6TASE9ly Synonyms: _7 EC Number: 3.1.6.4 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e_deg5 + h2o <==> cs_e_deg6 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_UMPK5n Synonyms: _9 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : datp + ump <==> dadp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_C14STRr Synonyms: _1 EC Number: 1.3.1.70 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : 44mctr + h + nadph --> 44mzym + nadp Confidence Level: 4 NCD ER version NJ genes: LOCUS:_7108#TRANSCRIPT:1#ABBREVIATION:TM7SF2# proteins: NAME:transmembrane 7 superfamily member 2#ABBREVIATION:Tm7sf2.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (7108.1)
Abbreviation: R_EX_triodthy_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : triodthy <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_5homeprazole_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 5homeprazole <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ARTFR207 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : arachcoa --> (1.25) R2coa_hs Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TMDPP Synonyms: _0 EC Number: 2.4.2.4 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : pi + thymd <==> 2dr1p + thym Confidence Level: 4 genes: LOCUS:_1890#TRANSCRIPT:1#ABBREVIATION:ECGF1# proteins: NAME:thymidine phosphorylase (endothelial cell growth factor 1 (platelet-derived)) #ABBREVIATION:Ecgf1.1# citations: AUTHORS:Brown NS, Bicknell R.#TITLE:Thymidine phosphorylase, 2-deoxy-D-ribose and angiogenesis#JOURNAL:Biochem J#YEAR:1998#PUBMED:9693094# AUTHORS:Kubilus J, Lee LD, Baden HP#TITLE:Purification of thymidine phosphorylase from human amniochorion#JOURNAL:Biochim Biophys Acta#YEAR:1978#PUBMED:718961# AUTHORS:Gan TE, Hallam L, Pilkington GR, Van der Weyden MB#TITLE:A rapid and simple radiometric assay for thymidine phosphorylase of human peripheral blood cells#JOURNAL:Clin Chim Acta#YEAR:1981#PUBMED:7028324# GENE ASSOCIATION: (1890.1)
Abbreviation: R_GALASE10ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg26 --> gal + ksi_deg27 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_ACACT8p Synonyms: _0 EC Number: 2.3.1.16 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : 3ohodcoa + coa --> accoa + pmtcoa Confidence Level: 4 NCD genes: LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# proteins: NAME:acetyl-Coenzyme A acyltransferase 1#ABBREVIATION:Acaa1.1# citations: AUTHORS:Bout A, Franse MM, Collins J, Blonden L, Tager JM, Benne R.#TITLE:Characterization of the gene encoding human peroxisomal 3-oxoacyl-CoA thiolase (ACAA). No large DNA rearrangement in a thiolase-deficient patient.#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1679347# GENE ASSOCIATION: (30.1)
acetyl-CoA acyltransferase (hexadecanoyl-CoA), peroxisomal
Abbreviation: R_HS4ly Synonyms: _4 EC Number: 3.10.1.1 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg18 --> h + hs_deg19 + so4 Confidence Level: 4 NCD genes: LOCUS:_6448#TRANSCRIPT:1#ABBREVIATION:SGSH# proteins: NAME:N-sulfoglucosamine sulfohydrolase (sulfamidase), lysosomal#ABBREVIATION:Sgsh.1-l# citations: AUTHORS:Scott HS, Blanch L, Guo XH, Freeman C, Orsborn A, Baker E, Sutherland GR, Morris CP, Hopwood JJ#TITLE:Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7493035# AUTHORS:Bielicki J, Hopwood JJ, Melville EL, Anson DS#TITLE:Recombinant human sulphamidase: expression, amplification, purification and characterization#JOURNAL:Biochem J#YEAR:1998#PUBMED:9405287# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION: (6448.1)
Abbreviation: R_DNDPt31m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + dcdp[c] --> atp[c] + dcdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_S6TASE14ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg15 <==> h + ksi_deg16 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_ACCOAtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: accoa[c] <==> accoa[r] Confidence Level: 0 transport between cytosol and Golgi/ER presumed presumed facilitated transport, but unknown co-substrates, see PMID: 12739170, Hirabayashi et al NJ changed name from ACCOArt to ACCOAtr for consistency - NCD genes: LOCUS:_9197#TRANSCRIPT:1#ABBREVIATION:SLC33A1# proteins: NAME:acetyl-CoA transporter/O-acetylator#ABBREVIATION:Slc33a1.1# citations: none GENE ASSOCIATION: (9197.1)
Abbreviation: R_MM7B1g Synonyms: _8 EC Number: 3.2.1.113 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : h2o + m7masnB --> m6masnB1 + man Confidence Level: 0 NCD genes: LOCUS:_4121#TRANSCRIPT:1#ABBREVIATION:MAN1A1# LOCUS:_10905#TRANSCRIPT:1#ABBREVIATION:MAN1A2# LOCUS:_57134#TRANSCRIPT:1#ABBREVIATION:MAN1C1# proteins: NAME:mannosidase, alpha, class 1A, member 1, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a1.1-g# NAME:mannosidase, alpha, class 1A, member 2, Golgi apparatus (ubiquitous)#ABBREVIATION:Man1a2.1-g# NAME:mannosidase, alpha, class 1C, member 1, Golgi apparatus (all tested except lung, muscle, pancreas)#ABBREVIATION:Man1c1.1-g# citations: AUTHORS:Tremblay LO, Herscovics A.#TITLE:Characterization of a cDNA encoding a novel human Golgi alpha 1, 2-mannosidase (IC) involved in N-glycan biosynthesis#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10915796# AUTHORS:Tremblay LO, Campbell Dyke N, Herscovics A.#TITLE:Molecular cloning, chromosomal mapping and tissue-specific expression of a novel human alpha1,2-mannosidase gene involved in N-glycan maturation.#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9592125# GENE ASSOCIATION: (4121.1) or (57134.1) or (10905.1)
Abbreviation: R_NDPK6m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + dudp <==> adp + dutp Confidence Level: 2 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:dUDP), mitochondrial
Abbreviation: R_HACD1m Synonyms: _0 EC Number: 1.1.1.35 SUBSYSTEM: Tryptophan metabolism Equation: [m] : aacoa + h + nadh <==> 3hbcoa + nad Confidence Level: 1 Same as EC 1.1.1.157 SAB mito genes: LOCUS:_3030#TRANSCRIPT:1#ABBREVIATION:HADHA#ECNUMBERS:4.2.1.17,1.1.1.35# LOCUS:_3032#TRANSCRIPT:1#ABBREVIATION:HADHB# LOCUS:_3033#TRANSCRIPT:1#ABBREVIATION:HADHSC# LOCUS:_3028#TRANSCRIPT:1#ABBREVIATION:HADH2# proteins: NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit #ABBREVIATION:Hadha.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit #ABBREVIATION:Hadhb.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase, type II#ABBREVIATION:Hadh2.1-m# NAME:L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain #ABBREVIATION:Hadhsc.1-m# citations: none GENE ASSOCIATION: (3028.1) or (3033.1) or (3030.1) and (3032.1)
_3-hydroxyacyl-CoA dehydrogenase (acetoacetyl-CoA) (mitochondria)
Abbreviation: R_CHOLtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: chol[g] <==> chol[c] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NTD5 Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : dtmp + h2o --> pi + thymd Confidence Level: 2 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_30833#TRANSCRIPT:1#ABBREVIATION:NT5C#ECNUMBERS:3.1.3.5# proteins: NAME:_5', 3'-nucleotidase, cytosolic #ABBREVIATION:Nt5C.1# citations: AUTHORS:Oka J, Matsumoto A, Hosokawa Y, Inoue S. #TITLE:Molecular cloning of human cytosolic purine 5'-nucleotidase.#JOURNAL:Biochem Biophys Res Commun. #YEAR:1994# GENE ASSOCIATION: (30833.1)
Abbreviation: R_EX_3aib_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 3aib <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FUT911g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucfucgalacglc13galacglcgal14acglcgalgluside_hs + gdpfuc --> fucfucfucgalacglc13galacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
Abbreviation: R_T4HCINNOX Synonyms: _0 EC Number: 1.14.18.1 SUBSYSTEM: Stilbene, coumarine and lignin biosynthesis Equation: [c] : (2) T4hcinnm + o2 --> (2) 34dhcinm Confidence Level: 1 SAB genes: LOCUS:_7299#TRANSCRIPT:1#ABBREVIATION:TYR#ECNUMBERS:1.14.18.1# proteins: NAME:tyrosinase#ABBREVIATION:Tyr.1# citations: none GENE ASSOCIATION: (7299.1)
Abbreviation: R_FA120ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : ddcaACP + h2o <==> ACP + ddca + h Confidence Level: 4 NCD rxn is now charge balanced - NCD genes: LOCUS:_55301#TRANSCRIPT:1#ABBREVIATION:THEDC1# proteins: NAME:s-acyl fatty acid synthase thioesterase#ABBREVIATION:Thedc1.1# citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION: (55301.1)
Abbreviation: R_S6T20g Synonyms: _3 EC Number: 2.8.2.17 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_c_pre3 + paps --> cspg_c + h + pap Confidence Level: 4 NCD genes: LOCUS:_56548#TRANSCRIPT:1#ABBREVIATION:CHST7# LOCUS:_9469#TRANSCRIPT:1#ABBREVIATION:CHST3# proteins: NAME:carbohydrate (chondroitin 6) sulfotransferase 3, Golgi#ABBREVIATION:Chst3.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 7, Golgi apparatus#ABBREVIATION:Chst7.1-g# citations: AUTHORS:Habuchi O, Hirahara Y, Uchimura K, Fukuta M#TITLE:Enzymatic sulfation of galactose residue of keratan sulfate by chondroitin 6-sulfotransferase#JOURNAL:Glycobiology#YEAR:1996#PUBMED:8991509# AUTHORS:Fukuta M, Kobayashi Y, Uchimura K, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of human chondroitin 6-sulfotransferase#JOURNAL:Biochim Biophys Acta#YEAR:1998#PUBMED:9714738# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Tsutsumi K, Shimakawa H, Kitagawa H, Sugahara K#TITLE:Functional expression and genomic structure of human chondroitin 6-sulfotransferase#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9883891# AUTHORS:Kitagawa H, Fujita M, Ito N, Sugahara K#TITLE:Molecular cloning and expression of a novel chondroitin 6-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781596# AUTHORS:Bhakta S, Bartes A, Bowman KG, Kao WM, Polsky I, Lee JK, Cook BN, Bruehl RE, Rosen SD, Bertozzi CR, Hemmerich S#TITLE:Sulfation of N-acetylglucosamine by chondroitin 6-sulfotransferase 2 (GST-5)#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10956661# GENE ASSOCIATION: (9469.1) or (56548.1)
Abbreviation: R_XYLK Synonyms: _3 EC Number: 2.7.1.17 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [c] : atp + xylu-D --> adp + h + xu5p-D Confidence Level: 4 genes: LOCUS:_9942#TRANSCRIPT:1#ABBREVIATION:XYLB# proteins: NAME:xylulokinase homolog (H. influenzae)#ABBREVIATION:Xylb.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (9942.1)
_1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
Abbreviation: R_P5CDm Synonyms: _0 EC Number: 1.5.1.12 SUBSYSTEM: Glutamate metabolism Equation: [m] : 1pyr5c + (2) h2o + nad --> glu-L + h + nadh Confidence Level: 4 NCD genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: AUTHORS:Hu CA, Lin WW, Valle D.#TITLE:Cloning, characterization, and expression of cDNAs encoding human delta 1-pyrroline-5-carboxylate dehydrogenase.#YEAR:1996#PUBMED:8621661# AUTHORS:Geraghty MT, Vaughn D, Nicholson AJ, Lin WW, Jimenez-Sanchez G, Obie C, Flynn MP, Valle D, Hu CA.#TITLE:Mutations in the Delta1-pyrroline 5-carboxylate dehydrogenase gene cause type II hyperprolinemia.#YEAR:1998#PUBMED:9700195# GENE ASSOCIATION: (8659.1) or (8659.2)
Abbreviation: R_EX_coumarin_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : coumarin <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_his_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : his-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NADNe Synonyms: _0 EC Number: 3.2.2.5 SUBSYSTEM: NAD Metabolism Equation: [e] : h2o + nad --> adprib + h + ncam Confidence Level: 4 genes: LOCUS:_952#TRANSCRIPT:1#ABBREVIATION:CD38# proteins: NAME:cyclic ADP-ribose hydrolase#ABBREVIATION:Cadph.1# citations: AUTHORS:Kim UH, Han MK, Park BH, Kim HR, An NH.#TITLE:Function of NAD glycohydrolase in ADP-ribose uptake from NAD by human erythrocytes.#JOURNAL:Biochim Biophys Acta.#YEAR:1993#PUBMED:8394137# AUTHORS:Kontani K, Nishina H, Ohoka Y, Takahashi K, Katada T.#TITLE:NAD glycohydrolase specifically induced by retinoic acid in human leukemic HL-60 cells. Identification of the NAD glycohydrolase as leukocyte cell surface antigen CD38.#JOURNAL:J Biol Chem#YEAR:1993# GENE ASSOCIATION: (952.1)
Abbreviation: R_EX_pydam_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pydam <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LRAT2 Synonyms: _0 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : pchol_hs + retinol-9-cis --> 9-cis-retfa + lpchol_hs Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Lecithin retinol acyltransferase (9-cis)
Abbreviation: R_EX_bildglcur_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : bildglcur <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACACT1rm Synonyms: _0 EC Number: 2.3.1.9 SUBSYSTEM: Tryptophan metabolism Equation: [m] : (2) accoa <==> aacoa + coa Confidence Level: 0 TDV genes: LOCUS:_3030#TRANSCRIPT:1#ABBREVIATION:HADHA#ECNUMBERS:4.2.1.17,1.1.1.35# LOCUS:_3032#TRANSCRIPT:1#ABBREVIATION:HADHB# LOCUS:_38#TRANSCRIPT:1#ABBREVIATION:ACAT1#ECNUMBERS:2.3.1.9# proteins: NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit #ABBREVIATION:Hadha.1-m# NAME:hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit #ABBREVIATION:Hadhb.1-m# NAME:acetyl-Coenzyme A acetyltransferase 1 (acetoacetyl Coenzyme A thiolase)#ABBREVIATION:Acat1.1-m# citations: none GENE ASSOCIATION: (3030.1) and (3032.1) or (38.1)
Abbreviation: R_PYDXNtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: pydxn[e] <==> pydxn[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# AUTHORS:Mehansho H, Henderson LM.#TITLE:Transport and accumulation of pyridoxine and pyridoxal by erythrocytes.#JOURNAL:J Biol Chem. #YEAR:1980#PUBMED:J Biol Chem.# GENE ASSOCIATION:
Abbreviation: R_4PYRDX Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _4pyrdx[c] + atp[c] + h2o[c] --> 4pyrdx[e] + adp[c] + h[c] + pi[c] Confidence Level: 2 IT Reaction correct??? (active transport) genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ink SL, Henderson LM.#TITLE:Vitamin B6 metabolism.#JOURNAL:Annu Rev Nutr.#YEAR:1984#PUBMED:6380540# GENE ASSOCIATION:
Abbreviation: R_CTPS2 Synonyms: _0 EC Number: 6.3.4.2 SUBSYSTEM: Pyrimidine Biosynthesis Equation: [c] : atp + gln-L + h2o + utp --> adp + ctp + glu-L + (2) h + pi Confidence Level: 4 genes: LOCUS:_56474#TRANSCRIPT:1#ABBREVIATION:CTPS2# LOCUS:_56474#TRANSCRIPT:2#ABBREVIATION:CTPS2# proteins: NAME:CTP synthase II #ABBREVIATION:Ctps2.1# NAME:CTP synthase II #ABBREVIATION:Ctps2.2# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:van Kuilenburg AB, Meinsma R, Vreken P, Waterham HR, van Gennip AH. #TITLE:Identification of a cDNA encoding an isoform of human CTP synthetase.#JOURNAL:Biochim Biophys Acta.#YEAR:2000#PUBMED:10899599# GENE ASSOCIATION: (56474.1) or (56474.2)
Abbreviation: R_EX_tdchola_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tdchola <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6T14g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre34 + paps --> h + ksi_pre35 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_GALU Synonyms: _0 EC Number: 2.7.7.9 SUBSYSTEM: Galactose metabolism Equation: [c] : g1p + h + utp <==> ppi + udpg Confidence Level: 0 genes: LOCUS:_7359#TRANSCRIPT:1#ABBREVIATION:UGP1#ECNUMBERS:2.7.7.9# LOCUS:_7360#TRANSCRIPT:1#ABBREVIATION:UGP2#ECNUMBERS:2.7.7.9# LOCUS:_7360#TRANSCRIPT:2#ABBREVIATION:UGP2#ECNUMBERS:2.7.7.9# proteins: NAME:UDP-glucose pyrophosphorylase 2#ABBREVIATION:Ugp2.1# NAME:UDP-glucose pyrophosphorylase 2#ABBREVIATION:Ugp2.2# NAME:UDP-glucose pyrophosphorylase 1#ABBREVIATION:Ugp1.1# citations: none GENE ASSOCIATION: (7360.1) or (7359.1) or (7360.2)
UTP-glucose-1-phosphate uridylyltransferase
Abbreviation: R_CITtbm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: cit[c] + pep[m] <==> cit[m] + pep[c] Confidence Level: 4 antiport with PEP IF genes: LOCUS:_6576#TRANSCRIPT:1#ABBREVIATION:SLC25A1# proteins: NAME:solute carrier family 25 (mitochondrial carrier; citrate transporter), member 1 #ABBREVIATION:Slc25a1.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (6576.1)
Abbreviation: R_C161CRN2t Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: hdd2crn[c] --> hdd2crn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_TDPDRR Synonyms: _5 EC Number: 1.1.1.133 SUBSYSTEM: Nucleotide Sugar Metabolism Equation: [c] : dtdp4d6dm + h + nadph --> dtdprmn + nadp Confidence Level: 1 JLR genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Tonetti M, Sturla L, Bisso A, Zanardi D, Benatti U, De Flora A#TITLE:The metabolism of 6-deoxyhexoses in bacterial and animal cells#JOURNAL:Biochimie#YEAR:1998#PUBMED:9893952# GENE ASSOCIATION:
Abbreviation: R_PI45PLC Synonyms: _6 EC Number: 3.1.4.11 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail45p_hs --> dag_hs + h + mi145p Confidence Level: 4 NCD genes: LOCUS:_5335#TRANSCRIPT:1#ABBREVIATION:PLCG1#ECNUMBERS:3.1.4.11# LOCUS:_5331#TRANSCRIPT:1#ABBREVIATION:PLCB3# LOCUS:_23236#TRANSCRIPT:1#ABBREVIATION:PLCB1#ECNUMBERS:3.1.4.11# LOCUS:_5330#TRANSCRIPT:1#ABBREVIATION:PLCB2#ECNUMBERS:3.1.4.11# LOCUS:_5333#TRANSCRIPT:1#ABBREVIATION:PLCD1# LOCUS:_5332#TRANSCRIPT:1#ABBREVIATION:PLCB4# LOCUS:_5336#TRANSCRIPT:1#ABBREVIATION:PLCG2#ECNUMBERS:3.1.4.3# LOCUS:_89869#TRANSCRIPT:1#ABBREVIATION:PLCZ1# LOCUS:_257068#TRANSCRIPT:1#ABBREVIATION:PLCXD2# LOCUS:_5334#TRANSCRIPT:1#ABBREVIATION:PLCL1# LOCUS:_5332#TRANSCRIPT:2#ABBREVIATION:PLCB4# LOCUS:_84812#TRANSCRIPT:1#ABBREVIATION:PLCD4# LOCUS:_23007#TRANSCRIPT:1#ABBREVIATION:PLCL3# LOCUS:_51196#TRANSCRIPT:1#ABBREVIATION:PLCE1# LOCUS:_23236#TRANSCRIPT:2#ABBREVIATION:PLCB1# LOCUS:_113026#TRANSCRIPT:1#ABBREVIATION:PLCD3# LOCUS:_5335#TRANSCRIPT:2#ABBREVIATION:PLCG1# LOCUS:_9651#TRANSCRIPT:1#ABBREVIATION:PLCL4# proteins: NAME:phospholipase C, delta 4#ABBREVIATION:Plcd4.1# NAME:phospholipase C, delta 1#ABBREVIATION:Plcd1.1# NAME:phospholipase C-like 3#ABBREVIATION:Plcl3.1# NAME:phospholipase C, epsilon 1#ABBREVIATION:Plce1.1# NAME:phospholipase C, gamma 2 (phosphatidylinositol-specific)#ABBREVIATION:Plcg2.1# NAME:phospholipase C, beta 4#ABBREVIATION:Plcb4.1# NAME:phospholipase C, beta 4#ABBREVIATION:Plcb4.2# NAME:phospholipase C, beta 1 (phosphoinositide-specific)#ABBREVIATION:Plcb1.1# NAME:phospholipase C, beta 1 (phosphoinositide-specific)#ABBREVIATION:Plcb1.2# NAME:phospholipase C, zeta 1#ABBREVIATION:Plcz1.1# NAME:phospholipase C, delta 3#ABBREVIATION:Plcd3.1# NAME:phosphatidylinositol-specific phospholipase C, X domain containing 2 #ABBREVIATION:Plcxd2.1# NAME:phospholipase C, beta 3 (phosphatidylinositol-specific)#ABBREVIATION:Plcb3.1# NAME:phospholipase C, gamma 1#ABBREVIATION:Plcg1.1# NAME:phospholipase C, gamma 1#ABBREVIATION:Plcg1.2# NAME:phospholipase C-like 1#ABBREVIATION:Plcl1.1# NAME:phospholipase C, beta 2#ABBREVIATION:Plcb2.1# NAME:phospholipase C-like 4#ABBREVIATION:Plcl4.1# citations: AUTHORS:Cheng HF, Jiang MJ, Chen CL, Liu SM, Wong LP, Lomasney JW, King K#TITLE:Cloning and identification of amino acid residues of human phospholipase C delta 1 essential for catalysis#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7890667# AUTHORS:Ishikawa S, Takahashi T, Ogawa M, Nakamura Y#TITLE:Genomic structure of the human PLCD1 (phospholipase C delta 1) locus on 3p22-->p21.3#JOURNAL:Cytogenet Cell Genet#YEAR:1997#PUBMED:9345909# AUTHORS:Kim H, Suh PG, Ryu SH, Park SH#TITLE:Assignment of the human PLC delta4 gene (PLCD4) to human chromosome band 2q35 by fluorescence in situ hybridization#JOURNAL:Cytogenet Cell Genet.#YEAR:1999#PUBMED:10702683# AUTHORS:Leung DW, Tompkins C, Brewer J, Ball A, Coon M, Morris V, Waggoner D, Singer JW#TITLE:Phospholipase C delta-4 overexpression upregulates ErbB1/2 expression, Erk signaling pathway, and proliferation in MCF-7 cells#JOURNAL:Mol Cancer#YEAR:2004#PUBMED:15140260# AUTHORS:Song C, Hu CD, Masago M, Kariyai K, Yamawaki-Kataoka Y, Shibatohge M, Wu D, Satoh T, Kataoka T#TITLE:Regulation of a novel human phospholipase C, PLCepsilon, through membrane targeting by Ras#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11022048# AUTHORS:Lopez I, Mak EC, Ding J, Hamm HE, Lomasney JW#TITLE:A novel bifunctional phospholipase c that is regulated by Galpha 12 and stimulates the Ras/mitogen-activated protein kinase pathway#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11022047# AUTHORS:Hernandez D, Egan SE, Yulug IG, Fisher EM#TITLE:Mapping the gene that encodes phosphatidylinositol-specific phospholipase C-gamma 2 in the human and the mouse#JOURNAL:Genomics#YEAR:1994#PUBMED:7835906# AUTHORS:Alvarez RA, Ghalayini AJ, Xu P, Hardcastle A, Bhattacharya S, Rao PN, Pettenati MJ, Anderson RE, Baehr W.#TITLE:cDNA sequence and gene locus of the human retinal phosphoinositide-specific phospholipase-C beta 4 (PLCB4)#JOURNAL:Genomics#YEAR:1995#PUBMED:8530101# AUTHORS:Peruzzi D, Aluigi M, Manzoli L, Billi AM, Di Giorgio FP, Morleo M, Martelli AM, Cocco L#TITLE:Molecular characterization of the human PLC beta1 gene#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12213492# AUTHORS:Caricasole A, Sala C, Roncarati R, Formenti E, Terstappen GC#TITLE:Cloning and characterization of the human phosphoinositide-specific phospholipase C-beta 1 (PLC beta 1)#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:11118617# AUTHORS:Peruzzi D, Calabrese G, Faenza I, Manzoli L, Matteucci A, Gianfrancesco F, Billi AM, Stuppia L, Palka G, Cocco L#TITLE:Identification and chromosomal localisation by fluorescence in situ hybridisation of human gene of phosphoinositide-specific phospholipase C beta(1)#JOURNAL:Biochim Biophy Acta#YEAR:2000#PUBMED:10760467# AUTHORS:Saunders CM, Larman MG, Parrington J, Cox LJ, Royse J, Blayney LM, Swann K, Lai FA#TITLE:PLC zeta: a sperm-specific trigger of Ca(2+) oscillations in eggs and embryo development#JOURNAL:Development#YEAR:2002#PUBMED:12117804# AUTHORS:Cox LJ, Larman MG, Saunders CM, Hashimoto K, Swann K, Lai FA#TITLE:Sperm phospholipase Czeta from humans and cynomolgus monkeys triggers Ca2+ oscillations, activation and development of mouse oocytes#JOURNAL:Reproduction#YEAR:2002#PUBMED:12416999# AUTHORS:Kim H, Suh PG, Ryu SH, Park SH#TITLE:Assignment of the human PLC delta3 gene (PLCD3) to human chromosome band 17q21 by fluorescence in situ hybridization#JOURNAL:Cytogenet Cell Genet#YEAR:1999#PUBMED:10702670# AUTHORS:Carozzi AJ, Kriz RW, Webster C, Parker PJ#TITLE:Identification, purification and characterization of a novel phosphatidylinositol-specific phospholipase C, a third member of the beta subfamily#JOURNAL:Eur J Biochem#YEAR:1992#PUBMED:13333955# AUTHORS:Weber G, Friedman E, Grimmond S, Hayward NK, Phelan C, Skogseid B, Gobl A, Zedenius J, Sandelin K, Teh BT, et al.#TITLE:The phospholipase C beta 3 gene located in the MEN1 region shows loss of expression in endocrine tumours#JOURNAL:Hum Mol Genet#YEAR:1994#PUBMED:7849701# AUTHORS:Sinke RJ, Geurts van Kessel AG#TITLE:Localization of the human phosphatidylinositol-specific phospholipase c beta 3 gene (PLCB3) within chromosome band 11q13#JOURNAL:Genoics#YEAR:1995#PUBMED:7789993# AUTHORS:Lagercrantz J, Carson E, Phelan C, Grimmond S, Rosen A, Dare E, Nordenskjold M, Hayward NK, Larsson C, Weber G#TITLE:Genomic organization and complete cDNA sequence of the human phosphoinositide-specific phospholipase C beta 3 gene (PLCB3)#JOURNAL:Genomics#YEAR:1995#PUBMED:7607669# AUTHORS:Mazuruk K, Schoen TJ, Chader GJ, Rodriguez IR#TITLE:Structural organization and expression of the human phosphatidylinositol-specific phospholipase C beta-3 gene#JOURNAL:Biochem Biophys Res Commun#YEAR:1995#PUBMED:7612006# AUTHORS:Burgess WH, Dionne CA, Kaplow J, Mudd R, Friesel R, Zilberstein A, Schlessinger J, Jaye M#TITLE:Characterization and cDNA cloning of phospholipase C-gamma, a major substrate for heparin-binding growth factor 1 (acidic fibroblast growth factor)-activated tyrosine kinase#JOURNAL:Mol Cell Biol#YEAR:1990#PUBMED:2167438# AUTHORS:Kohno T, Otsuka T, Takano H, Yamamoto T, Hamaguchi M, Terada M, Yokota J#TITLE:Identification of a novel phospholipase C family gene at chromosome 2q33 that is homozygously deleted in human small cell lung carcinoma#JOURNAL:Hum Mol Genet#YEAR:1995#PUBMED:7633416# AUTHORS:Park D, Jhon DY, Kriz R, Knopf J, Rhee SG#TITLE:Cloning, sequencing, expression, and Gq-independent activation of phospholipase C-beta 2#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1644792# GENE ASSOCIATION: (5331.1) or (5332.1) or (9651.1) or (113026.1) or (89869.1) or (5333.1) or (5330.1) or (5335.2) or (5336.1) or (257068.1) or (23236.1) or (5335.1) or (5334.1) or (84812.1) or (5332.2) or (23007.1) or (23236.2) or (51196.1)
phosphatidylinositol 4,5-bisphosphate phospholipase C
Abbreviation: R_GALASE1ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: N-Glycan Degradation Equation: [l] : (2) h2o + l2n2m2mn --> (2) gal + n2m2mn Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_S6TASE5ly Synonyms: _7 EC Number: 3.1.6.4 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_c_deg3 + h2o <==> cs_c_deg4 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_DOLMANP_Uter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: (0.1) dolmanp_U[c] --> (0.1) dolmanp_U[r] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION:
dolichol-phosphate mannose flippase (uterus)
N-acetylputrescine: oxygen oxireductase (deaminating)
Abbreviation: R_APRTO2 Synonyms: _0 EC Number: 1.4.3.4 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : aprut + h2o + o2 --> h2o2 + n4abutn + nh4 Confidence Level: 1 NCD genes: LOCUS:_4129#TRANSCRIPT:1#ABBREVIATION:MAOB#ECNUMBERS:1.4.3.4# LOCUS:_4128#TRANSCRIPT:1#ABBREVIATION:MAOA#ECNUMBERS:1.4.3.4# proteins: NAME:monoamine oxidase A#ABBREVIATION:MaoA.1# NAME:monoamine oxidase B#ABBREVIATION:MaoB.1# citations: none GENE ASSOCIATION: (4128.1) or (4129.1)
Abbreviation: R_ACGALFUCGALACGALFUCGALACGLCGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acgalfucgalacgalfucgalacglcgal14acglcgalgluside_hs[g] <==> acgalfucgalacgalfucgalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ARTFR53 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: eicostetcoa[c] + (2) fadh2[m] + (2) h[m] + (2) nadph[m] --> (1.25) R5coa_hs[c] + (2) fad[m] + (2) nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SMPD4 Synonyms: _0 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : h2o + spc_hs --> cholp + h + sphings Confidence Level: 2 reaction details not determined - elucidated from PMID: 9972865, 12069827 NJ genes: none proteins: none citations: AUTHORS:Meyer zu Heringdorf D, Himmel HM, Jakobs KH.#TITLE:Sphingosylphosphorylcholine-biological functions and mechanisms of action.#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12069827# AUTHORS:Rodriguez-Lafrasse C, Vanier MT.#TITLE:Sphingosylphosphorylcholine in Niemann-Pick disease brain: accumulation in type A but not in type B.#JOURNAL:Neurochem Res.#YEAR:1999#PUBMED:9972865# GENE ASSOCIATION:
phosphatidylinositol 4-phosphate phospholipase C
Abbreviation: R_PI4PLC Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : h2o + pail4p_hs --> dag_hs + h + mi14p Confidence Level: 4 NCD genes: LOCUS:_5335#TRANSCRIPT:1#ABBREVIATION:PLCG1#ECNUMBERS:3.1.4.11# LOCUS:_5331#TRANSCRIPT:1#ABBREVIATION:PLCB3# LOCUS:_23236#TRANSCRIPT:1#ABBREVIATION:PLCB1#ECNUMBERS:3.1.4.11# LOCUS:_5330#TRANSCRIPT:1#ABBREVIATION:PLCB2#ECNUMBERS:3.1.4.11# LOCUS:_5333#TRANSCRIPT:1#ABBREVIATION:PLCD1# LOCUS:_5332#TRANSCRIPT:1#ABBREVIATION:PLCB4# LOCUS:_5336#TRANSCRIPT:1#ABBREVIATION:PLCG2#ECNUMBERS:3.1.4.3# LOCUS:_89869#TRANSCRIPT:1#ABBREVIATION:PLCZ1# LOCUS:_257068#TRANSCRIPT:1#ABBREVIATION:PLCXD2# LOCUS:_5334#TRANSCRIPT:1#ABBREVIATION:PLCL1# LOCUS:_5332#TRANSCRIPT:2#ABBREVIATION:PLCB4# LOCUS:_84812#TRANSCRIPT:1#ABBREVIATION:PLCD4# LOCUS:_23007#TRANSCRIPT:1#ABBREVIATION:PLCL3# LOCUS:_51196#TRANSCRIPT:1#ABBREVIATION:PLCE1# LOCUS:_23236#TRANSCRIPT:2#ABBREVIATION:PLCB1# LOCUS:_113026#TRANSCRIPT:1#ABBREVIATION:PLCD3# LOCUS:_5335#TRANSCRIPT:2#ABBREVIATION:PLCG1# LOCUS:_9651#TRANSCRIPT:1#ABBREVIATION:PLCL4# proteins: NAME:phospholipase C, delta 4#ABBREVIATION:Plcd4.1# NAME:phospholipase C, delta 1#ABBREVIATION:Plcd1.1# NAME:phospholipase C-like 3#ABBREVIATION:Plcl3.1# NAME:phospholipase C, epsilon 1#ABBREVIATION:Plce1.1# NAME:phospholipase C, gamma 2 (phosphatidylinositol-specific)#ABBREVIATION:Plcg2.1# NAME:phospholipase C, beta 4#ABBREVIATION:Plcb4.1# NAME:phospholipase C, beta 4#ABBREVIATION:Plcb4.2# NAME:phospholipase C, beta 1 (phosphoinositide-specific)#ABBREVIATION:Plcb1.1# NAME:phospholipase C, beta 1 (phosphoinositide-specific)#ABBREVIATION:Plcb1.2# NAME:phospholipase C, zeta 1#ABBREVIATION:Plcz1.1# NAME:phospholipase C, delta 3#ABBREVIATION:Plcd3.1# NAME:phosphatidylinositol-specific phospholipase C, X domain containing 2 #ABBREVIATION:Plcxd2.1# NAME:phospholipase C, beta 3 (phosphatidylinositol-specific)#ABBREVIATION:Plcb3.1# NAME:phospholipase C, gamma 1#ABBREVIATION:Plcg1.1# NAME:phospholipase C, gamma 1#ABBREVIATION:Plcg1.2# NAME:phospholipase C-like 1#ABBREVIATION:Plcl1.1# NAME:phospholipase C, beta 2#ABBREVIATION:Plcb2.1# NAME:phospholipase C-like 4#ABBREVIATION:Plcl4.1# citations: AUTHORS:Cheng HF, Jiang MJ, Chen CL, Liu SM, Wong LP, Lomasney JW, King K#TITLE:Cloning and identification of amino acid residues of human phospholipase C delta 1 essential for catalysis#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7890667# AUTHORS:Ishikawa S, Takahashi T, Ogawa M, Nakamura Y#TITLE:Genomic structure of the human PLCD1 (phospholipase C delta 1) locus on 3p22-->p21.3#JOURNAL:Cytogenet Cell Genet#YEAR:1997#PUBMED:9345909# AUTHORS:Kim H, Suh PG, Ryu SH, Park SH#TITLE:Assignment of the human PLC delta4 gene (PLCD4) to human chromosome band 2q35 by fluorescence in situ hybridization#JOURNAL:Cytogenet Cell Genet.#YEAR:1999#PUBMED:10702683# AUTHORS:Leung DW, Tompkins C, Brewer J, Ball A, Coon M, Morris V, Waggoner D, Singer JW#TITLE:Phospholipase C delta-4 overexpression upregulates ErbB1/2 expression, Erk signaling pathway, and proliferation in MCF-7 cells#JOURNAL:Mol Cancer#YEAR:2004#PUBMED:15140260# AUTHORS:Song C, Hu CD, Masago M, Kariyai K, Yamawaki-Kataoka Y, Shibatohge M, Wu D, Satoh T, Kataoka T#TITLE:Regulation of a novel human phospholipase C, PLCepsilon, through membrane targeting by Ras#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11022048# AUTHORS:Lopez I, Mak EC, Ding J, Hamm HE, Lomasney JW#TITLE:A novel bifunctional phospholipase c that is regulated by Galpha 12 and stimulates the Ras/mitogen-activated protein kinase pathway#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11022047# AUTHORS:Hernandez D, Egan SE, Yulug IG, Fisher EM#TITLE:Mapping the gene that encodes phosphatidylinositol-specific phospholipase C-gamma 2 in the human and the mouse#JOURNAL:Genomics#YEAR:1994#PUBMED:7835906# AUTHORS:Alvarez RA, Ghalayini AJ, Xu P, Hardcastle A, Bhattacharya S, Rao PN, Pettenati MJ, Anderson RE, Baehr W.#TITLE:cDNA sequence and gene locus of the human retinal phosphoinositide-specific phospholipase-C beta 4 (PLCB4)#JOURNAL:Genomics#YEAR:1995#PUBMED:8530101# AUTHORS:Peruzzi D, Aluigi M, Manzoli L, Billi AM, Di Giorgio FP, Morleo M, Martelli AM, Cocco L#TITLE:Molecular characterization of the human PLC beta1 gene#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12213492# AUTHORS:Caricasole A, Sala C, Roncarati R, Formenti E, Terstappen GC#TITLE:Cloning and characterization of the human phosphoinositide-specific phospholipase C-beta 1 (PLC beta 1)#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:11118617# AUTHORS:Peruzzi D, Calabrese G, Faenza I, Manzoli L, Matteucci A, Gianfrancesco F, Billi AM, Stuppia L, Palka G, Cocco L#TITLE:Identification and chromosomal localisation by fluorescence in situ hybridisation of human gene of phosphoinositide-specific phospholipase C beta(1)#JOURNAL:Biochim Biophy Acta#YEAR:2000#PUBMED:10760467# AUTHORS:Saunders CM, Larman MG, Parrington J, Cox LJ, Royse J, Blayney LM, Swann K, Lai FA#TITLE:PLC zeta: a sperm-specific trigger of Ca(2+) oscillations in eggs and embryo development#JOURNAL:Development#YEAR:2002#PUBMED:12117804# AUTHORS:Cox LJ, Larman MG, Saunders CM, Hashimoto K, Swann K, Lai FA#TITLE:Sperm phospholipase Czeta from humans and cynomolgus monkeys triggers Ca2+ oscillations, activation and development of mouse oocytes#JOURNAL:Reproduction#YEAR:2002#PUBMED:12416999# AUTHORS:Kim H, Suh PG, Ryu SH, Park SH#TITLE:Assignment of the human PLC delta3 gene (PLCD3) to human chromosome band 17q21 by fluorescence in situ hybridization#JOURNAL:Cytogenet Cell Genet#YEAR:1999#PUBMED:10702670# AUTHORS:Carozzi AJ, Kriz RW, Webster C, Parker PJ#TITLE:Identification, purification and characterization of a novel phosphatidylinositol-specific phospholipase C, a third member of the beta subfamily#JOURNAL:Eur J Biochem#YEAR:1992#PUBMED:13333955# AUTHORS:Weber G, Friedman E, Grimmond S, Hayward NK, Phelan C, Skogseid B, Gobl A, Zedenius J, Sandelin K, Teh BT, et al.#TITLE:The phospholipase C beta 3 gene located in the MEN1 region shows loss of expression in endocrine tumours#JOURNAL:Hum Mol Genet#YEAR:1994#PUBMED:7849701# AUTHORS:Sinke RJ, Geurts van Kessel AG#TITLE:Localization of the human phosphatidylinositol-specific phospholipase c beta 3 gene (PLCB3) within chromosome band 11q13#JOURNAL:Genoics#YEAR:1995#PUBMED:7789993# AUTHORS:Lagercrantz J, Carson E, Phelan C, Grimmond S, Rosen A, Dare E, Nordenskjold M, Hayward NK, Larsson C, Weber G#TITLE:Genomic organization and complete cDNA sequence of the human phosphoinositide-specific phospholipase C beta 3 gene (PLCB3)#JOURNAL:Genomics#YEAR:1995#PUBMED:7607669# AUTHORS:Mazuruk K, Schoen TJ, Chader GJ, Rodriguez IR#TITLE:Structural organization and expression of the human phosphatidylinositol-specific phospholipase C beta-3 gene#JOURNAL:Biochem Biophys Res Commun#YEAR:1995#PUBMED:7612006# AUTHORS:Burgess WH, Dionne CA, Kaplow J, Mudd R, Friesel R, Zilberstein A, Schlessinger J, Jaye M#TITLE:Characterization and cDNA cloning of phospholipase C-gamma, a major substrate for heparin-binding growth factor 1 (acidic fibroblast growth factor)-activated tyrosine kinase#JOURNAL:Mol Cell Biol#YEAR:1990#PUBMED:2167438# AUTHORS:Kohno T, Otsuka T, Takano H, Yamamoto T, Hamaguchi M, Terada M, Yokota J#TITLE:Identification of a novel phospholipase C family gene at chromosome 2q33 that is homozygously deleted in human small cell lung carcinoma#JOURNAL:Hum Mol Genet#YEAR:1995#PUBMED:7633416# AUTHORS:Park D, Jhon DY, Kriz R, Knopf J, Rhee SG#TITLE:Cloning, sequencing, expression, and Gq-independent activation of phospholipase C-beta 2#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1644792# GENE ASSOCIATION: (5331.1) or (5332.1) or (23007.1) or (113026.1) or (89869.1) or (84812.1) or (9651.1) or (5335.2) or (5336.1) or (257068.1) or (23236.1) or (5335.1) or (5334.1) or (5330.1) or (5332.2) or (5333.1) or (23236.2) or (51196.1)
Abbreviation: R_MI13456PK Synonyms: _0 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + mi13456p --> adp + h + minohp Confidence Level: 2 EC 2.7.1.- NCD genes: none proteins: none citations: AUTHORS:Irvine RF, Moor RM, Pollock WK, Smith PM, Wreggett KA#TITLE:Inositol phosphates: proliferation, metabolism and function#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:1988#PUBMED:2906139# GENE ASSOCIATION:
inositol-1,3,4,5,6-pentakisphosphate 2-kinase
Abbreviation: R_GGLUCT Synonyms: _3 EC Number: 2.3.2.4 SUBSYSTEM: Glutathione Metabolism Equation: gluala[e] --> 5oxpro[c] + ala-L[c] Confidence Level: 4 SAB genes: LOCUS:_2655#TRANSCRIPT:1#ABBREVIATION:GCTG#ECNUMBERS:2.3.2.4# proteins: NAME:gamma-glutamylcyclotransferase#ABBREVIATION:Gctg.1# citations: AUTHORS:York MJ, Crossley MJ, Hyslop SJ, Fisher ML, Kuchel PW#TITLE:gamma-Glutamylcyclotransferase: inhibition by D-beta-aminoglutaryl-L-alanine and analysis of the solvent kinetic isotope effect#JOURNAL:Eur J Biochem#YEAR:1989#PUBMED:2570694# GENE ASSOCIATION: (2655.1)
Abbreviation: R_GluForTx Synonyms: _4 EC Number: 2.1.2.5 SUBSYSTEM: Histidine Metabolism Equation: [c] : forglu + h + thf --> 5forthf + glu-L Confidence Level: 4 SAB genes: LOCUS:_10841#TRANSCRIPT:1#ABBREVIATION:FTCD#ECNUMBERS:2.1.2.5,4.3.1.4# LOCUS:_10841#TRANSCRIPT:2#ABBREVIATION:FTCD# proteins: NAME:formiminotransferase cyclodeaminase#ABBREVIATION:Ftcd.1# NAME:formiminotransferase cyclodeaminase#ABBREVIATION:Ftcd.2# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Hilton JF, Christensen KE, Watkins D, Raby BA, Renaud Y, de la Luna S, Estivill X, MacKenzie RE, Hudson TJ, Rosenblatt DS#TITLE:The molecular basis of glutamate formiminotransferase deficiency#JOURNAL:Hum Mutat#YEAR:2003#PUBMED:12815595# GENE ASSOCIATION: (10841.1) or (10841.2)
Abbreviation: R_DM_Ser_FSLASH_Thr_LPAREN_ly_RPAREN_ Synonyms: _0 Equation: [l] : Ser/Thr --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S2TASE5ly Synonyms: _1 EC Number: 3.1.6.18 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_d_deg6 + h2o --> cs_c_deg5 + h + so4 Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Shaklee PN, Glaser JH, Conrad HE#TITLE:A sulfatase specific for glucuronic acid 2-sulfate residues in glycosaminoglycans#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:4019466# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver glucuronate 2-sulphatase. Purification, characterization and catalytic properties#JOURNAL:Biochem J#YEAR:1989#PUBMED:2497731# GENE ASSOCIATION:
Abbreviation: R_EX_pe_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : pe_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
phosphatidylethanolamine (homo sapiens) exchange
Abbreviation: R_NACHEX27ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Hyaluronan Metabolism Equation: [l] : h2o + ha_deg1 --> acgam + ha_pre1 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Franke I, Resch A, Dassler T, Maier T, Bock A.#TITLE:YfiK from Escherichia coli promotes export of O-acetylserine and cysteine.#JOURNAL:J Bacteriol.#YEAR:2003#PUBMED:12562784# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3073.1) or (3074.1)
Abbreviation: R_XOLTRI24te Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: xoltri24[c] <==> xoltri24[e] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GRTTx Synonyms: _0 EC Number: 2.5.1.10 SUBSYSTEM: Cholesterol Metabolism Equation: [x] : grdp + ipdp --> frdp + ppi Confidence Level: 4 peroxisomal version - NJ genes: LOCUS:_2224#TRANSCRIPT:1#ABBREVIATION:FDPS#ECNUMBERS:2.5.1.1,,2.5.1.# proteins: NAME:farnesyl diphosphate synthase (farnesyl pyrophosphate synthetase, dimethylallyltranstransferase, geranyltranstransferase)#ABBREVIATION:Fdps.1# citations: AUTHORS:Biardi L, Krisans K#TITLE:Compartmentalization of cholesterol biosynthesis#JOURNAL:Journal of Biological Chemistry#YEAR:1996# AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (2224.1)
Abbreviation: R_HSD3B7 Synonyms: _2 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : nad + xol7a --> h + nadh + xol7aone Confidence Level: 4 genes: LOCUS:_80270#TRANSCRIPT:1#ABBREVIATION:HSD3B7# proteins: NAME:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7#ABBREVIATION:Hsd3b7.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (80270.1)
Abbreviation: R_C226CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : c226coa + crn --> c226crn + coa Confidence Level: 4 TDV genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (126129.1) or (1375.3) or (1374.1) or (1375.2) or (1375.1) or (1375.4)
Abbreviation: R_FUT12g Synonyms: _4 EC Number: 2.4.1.69 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacglcgalgluside_hs + gdpfuc --> fucgalacglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2523#TRANSCRIPT:1#ABBREVIATION:FUT1#ECNUMBERS:2.4.1.69# proteins: NAME:fucosyltransferase 1#ABBREVIATION:Fut1.1# citations: AUTHORS:Larsen RD, Ernst LK, Nair RP, Lowe JB.#TITLE:Molecular cloning, sequence, and expression of a human GDP-L-fucose:beta-D-galactoside 2-alpha-L-fucosyltransferase cDNA that can form the H blood group antigen.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1990#PUBMED:2118655# GENE ASSOCIATION: (2523.1)
Abbreviation: R_DNDPt61m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dgtp[c] + dtdp[m] --> dgtp[m] + dtdp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_ARTFR210 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: fadh2[m] + h[m] + lnlccoa[c] + nadph[m] --> (1.125) R2coa_hs[c] + fad[m] + nadp[m] Confidence Level: 0 Carbon stoichiometry normalized wrt C16:0 The concomitant oxidation of fadh2 is to account for the normalization to a saturated fatty acid (later in the mitochondria, the full beta oxidation cycle will occur, and the additional round of fad reduction will be balanced to account for the respective degree of desaturation). For example a 9,15 fatty acid would have 2fadh2(m) -> 2fad(m). The concomittant exchange for nadph is to account for cases in which the fatty acid has alternating odd and even double bonds (all human fatty acids?). For example a 9,12 fatty acid would have: fadh2(m) + nadph(m) + h(m) -> fad(m) + nadp(m). The nadph is from 2,4 dienoyl-CoA reductase (human gene: DECR1, gene ID 1666). PMID 7818482 (Koivuranta et al 1994) identified this as a mitochondrial gene, Voet and Voet claim this is peroxisomal (not directly supported with primary literature, so it is presumed to be mitochondrial). For all of the R-group reactions which lead to subsequent fatty acid oxidation, 3,2 enoyl-CoA isomerase activity (human gene: DCI, gene ID 1632) is "inherently" incorporated into the reactions. R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Glycerophosphodiester phosphodiesterase (Glycerophosphocholine)
Abbreviation: R_GPDDA1 Synonyms: _0 EC Number: 3.1.4.46 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : g3pc + h2o --> chol + glyc3p + h Confidence Level: 0 JLR genes: none proteins: none citations: AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_34DHOXPEGOX Synonyms: _0 EC Number: 1.1.1.1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 34dhmald + h + nadh <==> 34dhoxpeg + nad Confidence Level: 4 SAB genes: LOCUS:_130#TRANSCRIPT:1#ABBREVIATION:ADH6#ECNUMBERS:1.1.1.1# LOCUS:_126#TRANSCRIPT:1#ABBREVIATION:ADH1C#ECNUMBERS:1.1.1.1# LOCUS:_125#TRANSCRIPT:1#ABBREVIATION:ADH1B#ECNUMBERS:1.1.1.1# LOCUS:_128#TRANSCRIPT:1#ABBREVIATION:ADH5#ECNUMBERS:1.1.1.1# LOCUS:_127#TRANSCRIPT:1#ABBREVIATION:ADH4#ECNUMBERS:1.1.1.1# LOCUS:_124#TRANSCRIPT:1#ABBREVIATION:ADH1A#ECNUMBERS:1.1.1.1# LOCUS:_131#TRANSCRIPT:1#ABBREVIATION:ADH7#ECNUMBERS:1.1.1.1# LOCUS:_137872#TRANSCRIPT:1#ABBREVIATION:ADHFE1#ECNUMBERS:1.1.1.1# proteins: NAME:alcohol dehydrogenase IV #ABBREVIATION:Adh4# NAME:alcohol dehydrogenase V#ABBREVIATION:Adh5# NAME:alcohol dehydrogenase 6 (class V)#ABBREVIATION:Adh6.1# NAME:alcohol dehydrogenase 7 (class IV), mu or sigma polypeptide#ABBREVIATION:Adh7.1# NAME:alcohol dehydrogenase, iron containing, 1 (liver)#ABBREVIATION:Adhfe1.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ab.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ac# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1bc# citations: AUTHORS:Mardh G, Dingley AL, Auld DS, Vallee BL#TITLE:Human class II (pi) alcohol dehydrogenase has a redox-specific function in norepinephrine metabolism#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1986#PUBMED:3466164# GENE ASSOCIATION: (130.1) or (127.1) or (125.1 and 124.1) or (131.1) or (126.1 and 124.1) or (128.1) or (137872.1) or (125.1 and 126.1)
_3,4-Dihydroxyphenylethyleneglycol:NAD+ oxidoreductase
Abbreviation: R_DCIm Synonyms: _2 EC Number: 5.3.3.8 SUBSYSTEM: Fatty Acid Metabolism Equation: [m] : dd2coa <==> dd3coa Confidence Level: 2 NJ genes: LOCUS:_1632#TRANSCRIPT:1#ABBREVIATION:DCI#ECNUMBERS:5.3.3.8# proteins: NAME:dodecenoyl-Coenzyme A delta isomerase#ABBREVIATION:Dci.1# citations: AUTHORS:Partanen ST, Novikov DK, Popov AN, Mursula AM, Hiltunen JK, Wierenga RK.#TITLE:The 1.3 A crystal structure of human mitochondrial Delta3-Delta2-enoyl-CoA isomerase shows a novel mode of binding for the fatty acyl group.#JOURNAL:J Mol Biol#YEAR:2004#PUBMED:15351645# GENE ASSOCIATION: (1632.1)
Abbreviation: R_EX_4pyrdx_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 4pyrdx <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TRPO2 Synonyms: _0 EC Number: 1.13.11.11 SUBSYSTEM: Tryptophan metabolism Equation: [c] : o2 + trp-L --> Lfmkynr Confidence Level: 4 NCD genes: LOCUS:_6999#TRANSCRIPT:1#ABBREVIATION:TDO2#ECNUMBERS:1.13.11.11# proteins: NAME:tryptophan 2,3-dioxygenase#ABBREVIATION:Tdo2.1# citations: AUTHORS:Comings DE, Muhleman D, Dietz G, Sherman M, Forest GL#TITLE:Sequence of human tryptophan 2,3-dioxygenase (TDO2): presence of a glucocorticoid response-like element composed of a GTT repeat and an intronic CCCCT repeat#JOURNAL:Genomics#YEAR:1995#PUBMED:8666386# GENE ASSOCIATION: (6999.1)
L-Tryptophan:oxygen 2,3-oxidoreductase (decyclizing)
Abbreviation: R_CHOLPtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: cholp[c] <==> cholp[g] Confidence Level: 1 unknown mechanism for transport - no strong evidence to indicate further lysosomal or golgi metabolism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GALNACT3g Synonyms: _6 EC Number: 2.4.1.175 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_c_pre2 + uacgam --> cs_c_pre3 + h + udp Confidence Level: 4 NCD genes: LOCUS:_22856#TRANSCRIPT:1#ABBREVIATION:CHSY1# LOCUS:_55454#TRANSCRIPT:1#ABBREVIATION:GALNACT-2# LOCUS:_55790#TRANSCRIPT:1#ABBREVIATION:ChGn# LOCUS:_79586#TRANSCRIPT:1#ABBREVIATION:CHPF# LOCUS:_337876#TRANSCRIPT:1#ABBREVIATION:CSS3# proteins: NAME:chondroitin sulfate GalNAcT-2, Golgi apparatus#ABBREVIATION:Galnact-2.1-g# NAME:chondroitin beta1,4 N-acetylgalactosaminyltransferase, Golgi apparatus#ABBREVIATION:Chgn.1-g# NAME:carbohydrate (chondroitin) synthase 1, Golgi apparatus#ABBREVIATION:Chsy1.1-g# NAME:chondroitin polymerizing factor, Golgi apparatus#ABBREVIATION:Chpf.1-g# NAME:chondroitin sulfate synthase 3, Golgi apparatus#ABBREVIATION:Css3.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Sato T, Gotoh M, Kiyohara K, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Togayachi A, Kudo T, Asada M, Watanabe H, Imamura T, Kimata K, Narimatsu H#TITLE:Differential roles of two N-acetylgalactosaminyltransferases, CSGalNAcT-1, and a novel enzyme, CSGalNAcT-2. Initiation and elongation in synthesis of chondroitin sulfate#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12446672# AUTHORS:Uyama T, Kitagawa H, Tamura Ji J, Sugahara K#TITLE:Molecular cloning and expression of human chondroitin N-acetylgalactosaminyltransferase: the key enzyme for chain initiation and elongation of chondroitin/dermatan sulfate on the protein linkage region tetrasaccharide shared by heparin/heparan sulfate#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11788602# AUTHORS:Gotoh M, Sato T, Akashima T, Iwasaki H, Kameyama A, Mochizuki H, Yada T, Inaba N, Zhang Y, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Nishihara S, Watanabe H, Kimata K, Narimatsu H#TITLE:Enzymatic synthesis of chondroitin with a novel chondroitin sulfate N-acetylgalactosaminyltransferase that transfers N-acetylgalactosamine to glucuronic acid in initiation and elongation of chondroitin sulfate synthesis#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12163485# AUTHORS:Kitagawa H, Uyama T, Sugahara K#TITLE:Molecular cloning and expression of a human chondroitin synthase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11514575# AUTHORS:Kitagawa H, Izumikawa T, Uyama T, Sugahara K#TITLE:Molecular cloning of a chondroitin polymerizing factor that cooperates with chondroitin synthase for chondroitin polymerization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12716890# AUTHORS:Yada T, Sato T, Kaseyama H, Gotoh M, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-3. Molecular cloning and characterization#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12907687# AUTHORS:Yada T, Gotoh M, Sato T, Shionyu M, Go M, Kaseyama H, Iwasaki H, Kikuchi N, Kwon YD, Togayachi A, Kudo T, Watanabe H, Narimatsu H, Kimata K#TITLE:Chondroitin sulfate synthase-2. Molecular cloning and characterization of a novel human glycosyltransferase homologous to chondroitin sulfate glucuronyltransferase, which has dual enzymatic activities#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12761225# GENE ASSOCIATION: (55790.1) or (79586.1) and (22856.1) or (55454.1) or (337876.1)
Abbreviation: R_AG13T15g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre33 + uacgam --> h + ksi_pre34 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
Abbreviation: R_ARGtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: arg-L[c] + h[m] <==> arg-L[m] + h[c] Confidence Level: 4 TDV genes: LOCUS:_83884#TRANSCRIPT:1#ABBREVIATION:SLC25A2# LOCUS:_10166#TRANSCRIPT:1#ABBREVIATION:SLC25A15# proteins: NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 2#ABBREVIATION:Slc25a2.1-m# NAME:solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15#ABBREVIATION:Slc25a15.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (83884.1) or (10166.1)
arginine mitochondrial transport via ornithine carrier
Abbreviation: R_NACHEXA4ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_c_deg3 + (2) h2o --> acgal + cs_c_deg5 + h + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_GALt1r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gal[e] <==> gal[c] Confidence Level: 4 NCD genes: LOCUS:_6515#TRANSCRIPT:1#ABBREVIATION:SLC2A3# LOCUS:_6514#TRANSCRIPT:1#ABBREVIATION:SLC2A2# LOCUS:_6513#TRANSCRIPT:1#ABBREVIATION:SLC2A1# LOCUS:_81031#TRANSCRIPT:1#ABBREVIATION:SLC2A10# LOCUS:_29988#TRANSCRIPT:1#ABBREVIATION:SLC2A8# proteins: NAME:solute carrier family 2 (facilitated glucose transporter), member 1, plasma membrane#ABBREVIATION:Slc2a1.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 2, extracellular#ABBREVIATION:Slc2a2.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 3, extracellular#ABBREVIATION:Slc2a3.1-e# NAME:solute carrier family 2, (facilitated glucose transporter) member 8, extracellular#ABBREVIATION:Slc2a8.1-e# NAME:solute carrier family 2 (facilitated glucose transporter), member 10, extracellular#ABBREVIATION:Slc2a10.1-e# citations: AUTHORS:Hosokawa M, Thorens B#TITLE:Glucose release from GLUT2-null hepatocytes: characterization of a major and a minor pathway#JOURNAL:Am J Physiol Endocrinol Metab#YEAR:2002#PUBMED:11882499# AUTHORS:Guillam MT, Burcelin R, Thorens B#TITLE:Normal hepatic glucose production in the absence of GLUT2 reveals an alternative pathway for glucose release from hepatocytes#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9770484# AUTHORS:Uldry M, Thorens B#TITLE:The SLC2 family of facilitated hexose and polyol transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12750891# AUTHORS:Flier JS, Mueckler MM, Usher P, Lodish HF#TITLE:Elevated levels of glucose transport and transporter messenger RNA are induced by ras or src oncogenes#JOURNAL:Science#YEAR:1987#PUBMED:3103217# AUTHORS:Maher F, Vannucci SJ, Simpson IA#TITLE:Glucose transporter proteins in brain#JOURNAL:FASEB J#YEAR:1994#PUBMED:7926364# AUTHORS:Mueckler M, Caruso C, Baldwin SA, Panico M, Blench I, Morris HR, Allard WJ, Lienhard GE, Lodish HF#TITLE:Sequence and structure of a human glucose transporter#JOURNAL:Science#YEAR:1985#PUBMED:3839598# AUTHORS:Uldry M, Ibberson M, Hosokawa M, Thorens B#TITLE:GLUT2 is a high affinity glucosamine transporter#JOURNAL:FEBS Lett#YEAR:2002#PUBMED:12135767# AUTHORS:Fukumoto H, Seino S, Imura H, Seino Y, Eddy RL, Fukushima Y, Byers MG, Shows TB, Bell GI#TITLE:Sequence, tissue distribution, and chromosomal localization of mRNA encoding a human glucose transporter-like protein#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1988#PUBMED:3399500# AUTHORS:Johnson JH, Newgard CB, Milburn JL, Lodish HF, Thorens B#TITLE:The high Km glucose transporter of islets of Langerhans is functionally similar to the low affinity transporter of liver and has an identical primary sequence#JOURNAL:J Biol Chem#YEAR:1990#PUBMED:2182619# AUTHORS:Thorens B, Cheng ZQ, Brown D, Lodish HF#TITLE:Liver glucose transporter: a basolateral protein in hepatocytes and intestine and kidney cells#JOURNAL:Am J Physiol#YEAR:1990#PUBMED:1701966# AUTHORS:Haber RS, Weinstein SP, O'Boyle E, Morgello S#TITLE:Tissue distribution of the human GLUT3 glucose transporte#JOURNAL:Endocrinology#YEAR:1993#PUBMED:8504756# AUTHORS:Heijnen HF, Oorschot V, Sixma JJ, Slot JW, James DE#TITLE:Thrombin stimulates glucose transport in human platelets via the translocation of the glucose transporter GLUT-3 from alpha-granules to the cell surface#JOURNAL:J Cell Biol#YEAR:1997#PUBMED:9230074# AUTHORS:Kayano T, Fukumoto H, Eddy RL, Fan YS, Byers MG, Shows TB, Bell GI#TITLE:Evidence for a family of human glucose transporter-like proteins. Sequence and gene localization of a protein expressed in fetal skeletal muscle and other tissues#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3170580# AUTHORS:Stuart CA, Wen G, Jiang J#TITLE:GLUT3 protein and mRNA in autopsy muscle specimens#JOURNAL:Metabolism#YEAR:1999#PUBMED:10421229# AUTHORS:Doege H, Schurmann A, Bahrenberg G, Brauers A, Joost HG#TITLE:GLUT8, a novel member of the sugar transport facilitator family with glucose transport activity#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10821868# AUTHORS:Carayannopoulos MO, Chi MM, Cui Y, Pingsterhaus JM, McKnight RA, Mueckler M, Devaskar SU, Moley KH#TITLE:GLUT8 is a glucose transporter responsible for insulin-stimulated glucose uptake in the blastocyst#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10860996# AUTHORS:Dawson PA, Mychaleckyj JC, Fossey SC, Mihic SJ, Craddock AL, Bowden DW#TITLE:Sequence and functional analysis of GLUT10: a glucose transporter in the Type 2 diabetes-linked region of chromosome 20q12-13.1#JOURNAL:Mol Genet Metab#YEAR:2001#PUBMED:11592815# AUTHORS:McVie-Wylie AJ, Lamson DR, Chen YT#TITLE:Molecular cloning of a novel member of the GLUT family of transporters, SLC2a10 (GLUT10), localized on chromosome 20q13.1: a candidate gene for NIDDM susceptibility#JOURNAL:Genomics#YEAR:2001#PUBMED:11247674# GENE ASSOCIATION: (29988.1) or (6513.1) or (6515.1) or (6514.1) or (81031.1)
Abbreviation: R_FATP9t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[c] + nrvnc[c] <==> na1[e] + nrvnc[e] Confidence Level: 4 electroneutral transport NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# LOCUS:_10998#TRANSCRIPT:1#ABBREVIATION:SLC27A5# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 5#ABBREVIATION:Slc27a5.1# NAME:solute carrier family 27 (fatty acid transporter), member 2#ABBREVIATION:Slc27a2.1# citations: AUTHORS:Steinberg SJ, Wang SJ, McGuinness MC, Watkins PA.#TITLE:Human liver-specific very-long-chain acyl-coenzyme A synthetase: cDNA cloning and characterization of a second enzymatically active protein.#JOURNAL:Mol Genet Metab.#YEAR:1999#PUBMED:10479480# AUTHORS:Stahl A.#TITLE:A current review of fatty acid transport proteins (SLC27).#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12856180# AUTHORS:Pohl J, Ring A, Hermann T, Stremmel W.#TITLE:Role of FATP in parenchymal cell fatty acid uptake.#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15522816# GENE ASSOCIATION: (10998.1) or (11001.1)
Abbreviation: R_PIK5 Synonyms: _3 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [c] : atp + pail_hs --> adp + h + pail5p_hs Confidence Level: 4 NCD genes: LOCUS:_8394#TRANSCRIPT:1#ABBREVIATION:PIP5K1A#ECNUMBERS:2.7.1.68# LOCUS:_23396#TRANSCRIPT:1#ABBREVIATION:PIP5K1C# LOCUS:_8395#TRANSCRIPT:1#ABBREVIATION:PIP5K1B# proteins: NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, gamma #ABBREVIATION:Pip5k1c.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, beta#ABBREVIATION:Pip5k1b.1# NAME:phosphatidylinositol-4-phosphate 5-kinase, type I, alpha#ABBREVIATION:Pip5k1a.1# citations: AUTHORS:Tolias KF, Cantley LC#TITLE:Pathways for phosphoinositide synthesis#JOURNAL:Chem Phys Lipids#YEAR:1999#PUBMED:10358929# AUTHORS:Tolias KF, Rameh LE, Ishihara H, Shibasaki Y, Chen J, Prestwich GD, Cantley LC, Carpenter CL#TITLE:Type I phosphatidylinositol-4-phosphate 5-kinases synthesize the novel lipids phosphatidylinositol 3,5-bisphosphate and phosphatidylinositol 5-phosphate#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9660759# GENE ASSOCIATION: (8394.1) or (23396.1) or (8395.1)
Abbreviation: R_SIAASE4ly Synonyms: _4 EC Number: 3.2.1.18 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core4 --> acnam + ksii_core4_deg1 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_NNATn Synonyms: _0 EC Number: 2.7.7.18 SUBSYSTEM: NAD Metabolism Equation: [n] : atp + h + nicrnt --> dnad + ppi Confidence Level: 4 IT genes: LOCUS:_64802#TRANSCRIPT:1#ABBREVIATION:NMNAT1#ECNUMBERS:2.7.7.1# proteins: NAME:Nicotinamide nucleotide adenylyltransferase 1 #ABBREVIATION:Nmnat1.1# citations: AUTHORS:Zhang X, Kurnasov OV, Karthikeyan S, Grishin NV, Osterman AL, Zhang H. #TITLE:Structural characterization of a human cytosolic NMN/NaMN adenylyltransferase and implication in human NAD biosynthesis.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:12574164# AUTHORS: Emanuelli M, Carnevali F, Saccucci F, Pierella F, Amici A, Raffaelli N, Magni G. #TITLE:Molecular cloning, chromosomal localization, tissue mRNA levels, bacterial expression, and enzymatic properties of human NMN adenylyltransferase.#JOURNAL:J Biol Chem.#YEAR:2001#PUBMED:11027696# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# GENE ASSOCIATION: (64802.1)
Abbreviation: R_EX_cyan_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : cyan <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PDE1 Synonyms: _0 EC Number: 3.1.4.17 SUBSYSTEM: Nucleotides Equation: [c] : camp + h2o --> amp + h Confidence Level: 4 NCD genes: LOCUS:_5139#TRANSCRIPT:1#ABBREVIATION:PDE3A#ECNUMBERS:3.1.4.17# LOCUS:_5144#TRANSCRIPT:1#ABBREVIATION:PDE4D# LOCUS:_27115#TRANSCRIPT:1#ABBREVIATION:PDE7B# LOCUS:_5138#TRANSCRIPT:1#ABBREVIATION:PDE2A# LOCUS:_5143#TRANSCRIPT:1#ABBREVIATION:PDE4C# LOCUS:_5153#TRANSCRIPT:1#ABBREVIATION:PDE1B#ECNUMBERS:3.1.4.17# LOCUS:_5142#TRANSCRIPT:1#ABBREVIATION:PDE4B# LOCUS:_5137#TRANSCRIPT:1#ABBREVIATION:PDE1C# LOCUS:_5136#TRANSCRIPT:1#ABBREVIATION:PDE1A#ECNUMBERS:3.1.4.17# LOCUS:_10846#TRANSCRIPT:1#ABBREVIATION:PDE10A# LOCUS:_5140#TRANSCRIPT:1#ABBREVIATION:PDE3B# LOCUS:_5141#TRANSCRIPT:1#ABBREVIATION:PDE4A# LOCUS:_50940#TRANSCRIPT:1#ABBREVIATION:PDE11A# LOCUS:_8622#TRANSCRIPT:1#ABBREVIATION:PDE8B# LOCUS:_5151#TRANSCRIPT:1#ABBREVIATION:PDE8A# LOCUS:_5151#TRANSCRIPT:2#ABBREVIATION:PDE8A# LOCUS:_5140#TRANSCRIPT:2#ABBREVIATION:PDE3B# LOCUS:_5151#TRANSCRIPT:3#ABBREVIATION:PDE8A# LOCUS:_5151#TRANSCRIPT:5#ABBREVIATION:PDE8A# LOCUS:_5151#TRANSCRIPT:4#ABBREVIATION:PDE8A# LOCUS:_5136#TRANSCRIPT:2#ABBREVIATION:PDE1A# LOCUS:_5150#TRANSCRIPT:1#ABBREVIATION:PDE7A#ECNUMBERS:3.1.4.17# LOCUS:_5150#TRANSCRIPT:2#ABBREVIATION:PDE7A#ECNUMBERS:3.1.4.17# proteins: NAME:phosphodiesterase 4A, cAMP-specific (phosphodiesterase E2 dunce homolog, Drosophila) #ABBREVIATION:Pde4A.1# NAME:phosphodiesterase 4B, cAMP-specific (phosphodiesterase E4 dunce homolog, Drosophila) #ABBREVIATION:Pde4B.1# NAME:phosphodiesterase 4C, cAMP-specific (phosphodiesterase E1 dunce homolog, Drosophila) #ABBREVIATION:Pde4C.1# NAME:phosphodiesterase 4D, cAMP-specific (phosphodiesterase E3 dunce homolog, Drosophila) #ABBREVIATION:Pde4D.1# NAME:phosphodiesterase 3A, cGMP-inhibited #ABBREVIATION:Pde3A.1# NAME:phosphodiesterase 3B, cGMP-inhibited #ABBREVIATION:Pde3B.1# NAME:phosphodiesterase 3B, cGMP-inhibited#ABBREVIATION:Pde3B.2# NAME:phosphodiesterase 2A, cGMP-stimulated #ABBREVIATION:Pde2A.1# NAME:phosphodiesterase 1A, calmodulin-dependent #ABBREVIATION:Pde1A.1# NAME:phosphodiesterase 1A, calmodulin-dependent #ABBREVIATION:Pde1A.2# NAME:phosphodiesterase 1B, calmodulin-dependent #ABBREVIATION:Pde1B.1# NAME:phosphodiesterase 1C, calmodulin-dependent 70kDa #ABBREVIATION:Pde1C.1# NAME:phosphodiesterase 7B #ABBREVIATION:Pde7B.1# NAME:phosphodiesterase 7A isoform a #ABBREVIATION:Pde7A.1# NAME:phosphodiesterase 7A isoform a #ABBREVIATION:Pde7A.2# NAME:phosphodiesterase 10A #ABBREVIATION:Pde10A# NAME:phosphodiesterase 8B #ABBREVIATION:Pde8b.1# NAME:phosphodiesterase 8A #ABBREVIATION:Pde8a.1# NAME:phosphodiesterase 8A#ABBREVIATION:Pde8a.2# NAME:phosphodiesterase 8A#ABBREVIATION:Pde8a.3# NAME:phosphodiesterase 8A#ABBREVIATION:Pde8a.4# NAME:phosphodiesterase 8A#ABBREVIATION:Pde8a.5# NAME:phosphodiesterase 11A #ABBREVIATION:Pde11A.1# citations: AUTHORS:White JB, Thompson WJ, Pittler SJ. #TITLE:Characterization of 3',5' cyclic nucleotide phosphodiesterase activity in Y79 retinoblastoma cells: absence of functional PDE6.#JOURNAL:Mol Vis.#YEAR:2004#PUBMED:15480303# AUTHORS:Secchiero P, Zella D, Curreli S, Mirandola P, Capitani S, Gallo RC, Zauli G. #TITLE:Pivotal role of cyclic nucleoside phosphodiesterase 4 in Tat-mediated CD4+ T cell hyperactivation and HIV type 1 replication.#JOURNAL:Proc Natl Acad Sci U S A. #YEAR:2000#PUBMED:11114167# AUTHORS:Pryzwansky KB, Madden VJ#TITLE:Type 4A cAMP-specific phosphodiesterase is stored in granules of human neutrophils and eosinophils.#JOURNAL:Cell Tissue Res#YEAR:2003#PUBMED:12764607# AUTHORS:Lefievre L, de Lamirande E, Gagnon C.#TITLE:Presence of cyclic nucleotide phosphodiesterases PDE1A, existing as a stable complex with calmodulin, and PDE3A in human spermatozoa.#JOURNAL:Biol Reprod. #YEAR:2002#PUBMED:12135876# AUTHORS:Wechsler J, Choi YH, Krall J, Ahmad F, Manganiello VC, Movsesian MA.#TITLE:Isoforms of cyclic nucleotide phosphodiesterase PDE3A in cardiac myocytes.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12154085# AUTHORS:Shakur Y, Takeda K, Kenan Y, Yu ZX, Rena G, Brandt D, Houslay MD, Degerman E, Ferrans VJ, Manganiello VC. #TITLE:Membrane localization of cyclic nucleotide phosphodiesterase 3 (PDE3). Two N-terminal domains are required for the efficient targeting to, and association of, PDE3 with endoplasmic reticulum.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10952971# AUTHORS:Scapin G, Patel SB, Chung C, Varnerin JP, Edmondson SD, Mastracchio A, Parmee ER, Singh SB, Becker JW, Van der Ploeg LH, Tota MR.#TITLE:Crystal structure of human phosphodiesterase 3B: atomic basis for substrate and inhibitor specificity. #JOURNAL:Biochemistry. #YEAR:2004#PUBMED:15147193# AUTHORS:Miki T, Taira M, Hockman S, Shimada F, Lieman J, Napolitano M, Ward D, Taira M, Makino H, Manganiello VC.#TITLE:Characterization of the cDNA and gene encoding human PDE3B, the cGIP1 isoform of the human cyclic GMP-inhibited cyclic nucleotide phosphodiesterase family.#JOURNAL:Genomics#YEAR:1996#PUBMED:8884271# AUTHORS:Rosman GJ, Martins TJ, Sonnenburg WK, Beavo JA, Ferguson K, Loughney K. #TITLE:Isolation and characterization of human cDNAs encoding a cGMP-stimulated 3',5'-cyclic nucleotide phosphodiesterase.#JOURNAL:Gene#YEAR:1997#PUBMED:9210593# AUTHORS:Manns JM, Brenna KJ, Colman RW, Sheth SB. #TITLE:Differential regulation of human platelet responses by cGMP inhibited and stimulated cAMP phosphodiesterases. #JOURNAL:Thromb Haemost.#YEAR:2002#PUBMED:12038792# AUTHORS:Yu J, Wolda SL, Frazier AL, Florio VA, Martins TJ, Snyder PB, Harris EA, McCaw KN, Farrell CA, Steiner B, Bentley JK, Beavo JA, Ferguson K, Gelinas R.#TITLE:Identification and characterisation of a human calmodulin-stimulated phosphodiesterase PDE1B1.#JOURNAL:Cell Signal#YEAR:1997#PUBMED:9419816# AUTHORS:Goraya TA, Masada N, Ciruela A, Cooper DM.#TITLE:Sustained entry of Ca2+ is required to activate Ca2+-calmodulin-dependent phosphodiesterase 1A.#JOURNAL: J Biol Chem. #YEAR:2004#PUBMED:15272012# AUTHORS:Kotera J, Sasaki T, Kobayashi T, Fujishige K, Yamashita Y, Omori K.#TITLE:Subcellular localization of cyclic nucleotide phosphodiesterase type 10A variants, and alteration of the localization by cAMP-dependent protein kinase-dependent phosphorylation. #JOURNAL:J Biol Chem.#YEAR:2004#PUBMED:14604994# AUTHORS:Gardner C, Robas N, Cawkill D, Fidock M.#TITLE:Cloning and characterization of the human and mouse PDE7B, a novel cAMP-specific cyclic nucleotide phosphodiesterase.#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10872825# AUTHORS:Smith SJ, Brookes-Fazakerley S, Donnelly LE, Barnes PJ, Barnette MS, Giembycz MA.#TITLE:Ubiquitous expression of phosphodiesterase 7A in human proinflammatory and immune cells#JOURNAL:Am J Physiol Lung Cell Mol Physiol.#YEAR:2003#PUBMED:12388353# AUTHORS:Gamanuma M, Yuasa K, Sasaki T, Sakurai N, Kotera J, Omori K.#TITLE:Comparison of enzymatic characterization and gene organization of cyclic nucleotide phosphodiesterase 8 family in humans.#JOURNAL:Cell Signal#YEAR:2003#PUBMED:12681444# AUTHORS:Hetman JM, Robas N, Baxendale R, Fidock M, Phillips SC, Soderling SH, Beavo JA.#TITLE:Cloning and characterization of two splice variants of human phosphodiesterase 11A.#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2000#PUBMED:11050148# AUTHORS:Fawcett L, Baxendale R, Stacey P, McGrouther C, Harrow I, Soderling S, Hetman J, Beavo JA, Phillips SC.#TITLE:Molecular cloning and characterization of a distinct human phosphodiesterase gene family: PDE11A. #JOURNAL:Proc Natl Acad Sci U S A#YEAR:2000#PUBMED:10725373# AUTHORS:Yuasa K, Kotera J, Fujishige K, Michibata H, Sasaki T, Omori K.#TITLE:Isolation and characterization of two novel phosphodiesterase PDE11A variants showing unique structure and tissue-specific expression.#JOURNAL:J Biol Chem.#YEAR:2000#PUBMED:10906126# GENE ASSOCIATION: (5137.1) or (5142.1) or (5138.1) or (5151.4) or (5150.2) or (5143.1) or (5150.1) or (5151.5) or (5144.1) or (5151.3) or (5151.1) or (5140.2) or (10846.1) or (5136.2) or (27115.1) or (5141.1) or (8622.1) or (5151.2) or (5136.1) or (5139.1) or (50940.1) or (5153.1) or (5140.1)
Abbreviation: R_TYRASE Synonyms: _0 EC Number: 1.14.18 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 56dihindlcrbxlt --> melanin Confidence Level: 2 SAB This reaction is probably supposed to be a polymerization, but the details are not known. genes: LOCUS:_7306#TRANSCRIPT:1#ABBREVIATION:TYRP1# proteins: NAME:tyrosinase-related protein 1#ABBREVIATION:Tyrp1.1# citations: AUTHORS:Box NF, Wyeth JR, Mayne CJ, O'Gorman LE, Martin NG, Sturm RA#TITLE:Complete sequence and polymorphism study of the human TYRP1 gene encoding tyrosinase-related protein 1#JOURNAL:Mamm Genome#YEAR:1998#PUBMED:9434945# GENE ASSOCIATION: (7306.1)
Abbreviation: R_PRAIS Synonyms: _0 EC Number: 6.3.3.1 SUBSYSTEM: IMP Biosynthesis Equation: [c] : atp + fpram --> adp + air + (2) h + pi Confidence Level: 4 genes: LOCUS:_2618#TRANSCRIPT:1#ABBREVIATION:GART#ECNUMBERS:2.1.2.2,,6.3.1.# LOCUS:_2618#TRANSCRIPT:2#ABBREVIATION:GART#ECNUMBERS:2.1.2.2,,6.3.1.# proteins: NAME:phosphoribosylglycinamide formyltransferase, phosphoribosylglycinamide synthetase, phosphoribosylaminoimidazole synthetase #ABBREVIATION:GarT.1# NAME:phosphoribosylglycinamide formyltransferase, phosphoribosylglycinamide synthetase, phosphoribosylaminoimidazole synthetase #ABBREVIATION:GarT.2# citations: AUTHORS:Brodsky G, Barnes T, Bleskan J, Becker L, Cox M, Patterson D.#TITLE:The human GARS-AIRS-GART gene encodes two proteins which are differentially expressed during human brain development and temporally overexpressed in cerebellum of individuals with Down syndrome. #JOURNAL:Hum Mol Genet. #YEAR:1997#PUBMED:9328467# AUTHORS:Zhang Y, Desharnais J, Greasley SE, Beardsley GP, Boger DL, Wilson IA.#TITLE:Crystal structures of human GAR Tfase at low and high pH and with substrate beta-GAR.#JOURNAL:Biochemistry.#YEAR:2002#PUBMED:12450384# AUTHORS:Schild D, Brake AJ, Kiefer MC, Young D, Barr PJ.#TITLE:Cloning of three human multifunctional de novo purine biosynthetic genes by functional complementation of yeast mutations. #JOURNAL:Proc Natl Acad Sci U S A. #YEAR:1990#PUBMED:2183217# AUTHORS:Poch MT, Qin W, Caperelli CA. #TITLE:The human trifunctional enzyme of de novo purine biosynthesis: heterologous expression, purification, and preliminary characterization.#JOURNAL:Protein Expr Purif.#YEAR:1998#PUBMED:9473452# GENE ASSOCIATION: (2618.1) or (2618.2)
Abbreviation: R_RTOT4 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : R4coa_hs --> Rtotalcoa Confidence Level: 0 User defined, tissue specific fluxes that can be customized w/ various ratios of R1 through R5 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CHOLATEt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cholate[e] + hco3[c] <==> cholate[c] + hco3[e] Confidence Level: 4 NJ genes: LOCUS:_6579#TRANSCRIPT:1#ABBREVIATION:SLCO1A2# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1A2#ABBREVIATION:Slco1a2.1# NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# citations: AUTHORS:Kullak-Ublick GA, Beuers U, Meier PJ, Domdey H, Paumgartner G.#TITLE:Assignment of the human organic anion transporting polypeptide (OATP) gene to chromosome 12p12 by fluorescence in situ hybridization.#JOURNAL:J Hepatol.#YEAR:1996#PUBMED:9007731# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (6579.1) or (10599.1)
cholate transport via bicarbonate countertransport
Abbreviation: R_EX_ebastineoh_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ebastineoh <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_S6TASE18ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg27 <==> h + ksi_deg28 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_3HBCOAHLm Synonyms: _2 EC Number: 3.1.2.4 SUBSYSTEM: Valine, Leucine, and Isoleucine Metabolism Equation: [m] : 3hibutcoa + h2o --> 3hmp + coa + h Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Hawes JW, Jaskiewicz J, Shimomura Y, Huang B, Bunting J, Harper ET, Harris RA. #TITLE:Primary structure and tissue-specific expression of human beta-hydroxyisobutyryl-coenzyme A hydrolase. #JOURNAL:J Biol Chem#YEAR:1996#PUBMED:8824301# GENE ASSOCIATION:
_3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
Abbreviation: R_FUT96g Synonyms: _3 EC Number: 2.4.1.152 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galacglcgal14acglcgalgluside_hs + gdpfuc --> fuc132galacglcgal14acglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_10690#TRANSCRIPT:1#ABBREVIATION:FUT9#ECNUMBERS:2.4.1.152# proteins: NAME:fucosyltransferase 9 (alpha(,13) fucosyltransferase)#ABBREVIATION:Fut9.1# citations: AUTHORS:Kaneko M, Kudo T, Iwasaki H, Ikehara Y, Nishihara S, Nakagawa S, Sasaki K, Shiina T, Inoko H, Saitou N, Narimatsu H.#TITLE:Alpha1,3-fucosyltransferase IX (Fuc-TIX) is very highly conserved between human and mouse; molecular cloning, characterization and tissue distribution of human Fuc-TIX.#JOURNAL:FEBS Lett.#YEAR:1999#PUBMED:10386598# GENE ASSOCIATION: (10690.1)
Abbreviation: R_VLCS2r Synonyms: _5 EC Number: 6.2.1.7 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : atp + coa + dhcholestanate --> amp + dhcholestancoa + ppi Confidence Level: 2 from uniprot and lit - see human recon lit refs divalent cation requirement - unclear which one, reaction inhibited in the presence of EDTA (may just be MgATP or MgATP) - see Kelley and Vessey Biochimica et Biophysica Acta (1994) 1209: 51-55 temporarily will have H+ balance reaction NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# proteins: NAME:bile acid CoA ligase#ABBREVIATION:Slc27a1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Steinberg SJ, Wang SJ, Kim DG, Mihalik J, Watkins PA#TITLE:Human very-long-chain-acyl-CoA synthetase#JOURNAL:Biochemical and biophysical research communications#YEAR:1999# AUTHORS:Steinberg SJ, Mihalik SJ, Kim DG, Cuebas DA, Watkins PA#TITLE:The human liver-specific homolog of very long-chain acyl-CoA synthetase is cholate: CoA ligase#JOURNAL:Journal of Biological Chemistry#YEAR:2000# AUTHORS:Mihalik SJ, Steinberg SJ, Pei Z, Park J, Kim DG, Heinzer AK, Dacremont G, Wanders JAR, Cuebas DA, Smith KD, Watkins PA#TITLE:Participation of two members of the veryl long-chain acyl-CoA synthetase family in bile acid synthesis and recycling#JOURNAL:Journal of Biological Chemistry#YEAR:2002# AUTHORS:Kelley M, Vessey DA#TITLE:Dual role of diavlent cations in the bile acid: CoA ligase catalyzed reaction#JOURNAL:Biochimica et Biophysica Acta#YEAR:1994# GENE ASSOCIATION: (11001.1)
Abbreviation: R_TRIODTHYt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hco3[c] + triodthy[e] <==> hco3[e] + triodthy[c] Confidence Level: 4 NJ genes: LOCUS:_53919#TRANSCRIPT:1#ABBREVIATION:SLCO1C1# LOCUS:_28234#TRANSCRIPT:1#ABBREVIATION:SLCO1B3# LOCUS:_6579#TRANSCRIPT:1#ABBREVIATION:SLCO1A2# LOCUS:_28231#TRANSCRIPT:1#ABBREVIATION:SLCO4A1# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1A2#ABBREVIATION:Slco1a2.1# NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# NAME:solute carrier organic anion transporter family, member 1B3#ABBREVIATION:Slco1b3.1# NAME:solute carrier organic anion transporter family, member 4A1#ABBREVIATION:Slco4a1.1# NAME:solute carrier organic anion transporter family, member 1C1#ABBREVIATION:Slco1c1.1# citations: AUTHORS:Kullak-Ublick GA, Beuers U, Meier PJ, Domdey H, Paumgartner G.#TITLE:Assignment of the human organic anion transporting polypeptide (OATP) gene to chromosome 12p12 by fluorescence in situ hybridization.#JOURNAL:J Hepatol.#YEAR:1996#PUBMED:9007731# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (28234.1) or (28231.1) or (10599.1) or (6579.1) or (53919.1)
T3 transport via bicarbonate countertransport
Abbreviation: R_NTD3l Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleic acid degradation Equation: [l] : dcmp + h2o --> dcyt + pi Confidence Level: 4 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 IT genes: LOCUS:_53#TRANSCRIPT:1#ABBREVIATION:ACP2#ECNUMBERS:3.1.3.2# proteins: NAME:acid phosphatase 2, lysosomal #ABBREVIATION:Acp2.1-l# citations: AUTHORS:R.L. Pisoni#TITLE:Lysosomal Nucleic Acid and Phosphate Metabolism and Related Metabolic Reactions, Chapter 9#YEAR:1996# GENE ASSOCIATION: (53.1)
Abbreviation: R_NAHCO3_HCLt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[c] + h[c] + hco3[e] + na1[e] <==> cl[e] + h[e] + hco3[c] + na1[c] Confidence Level: 4 MM genes: LOCUS:_57282#TRANSCRIPT:1#ABBREVIATION:SLC4A10# LOCUS:_9498#TRANSCRIPT:1#ABBREVIATION:SLC4A8# proteins: NAME:solute carrier family 4, sodium bicarbonate cotransporter, member 8, extracellular #ABBREVIATION:Slc4a8.1-e# NAME:solute carrier family 4, sodium bicarbonate transporter-like, member 10, extracellular #ABBREVIATION:Slc4a10.1-e# citations: AUTHORS:Romero MF, Fulton CM, Boron WF.#TITLE:The SLC4 family of HCO 3 - transporters.#YEAR:2004#PUBMED:14722772# GENE ASSOCIATION: (9498.1) or (57282.1)
bicarbonate transport (HCl/NaHCO3 exchange)
Abbreviation: R_T4HCINNMFM Synonyms: _0 SUBSYSTEM: Ubiquinone Biosynthesis Equation: [m] : 34hpl --> T4hcinnm + h2o Confidence Level: 2 IT reaction involved in formation of 4-hydroxybenzoate from tyrosine. genes: none proteins: none citations: AUTHORS:Meganathan R.#TITLE:Ubiquinone biosynthesis in microorganisms.#JOURNAL:FEMS Microbiol Lett. #YEAR:2001#PUBMED:11583838# AUTHORS:Loscher R, Heide L.#TITLE:Biosynthesis of p-Hydroxybenzoate from p-Coumarate and p-Coumaroyl-Coenzyme A in Cell-Free Extracts of Lithospermum erythrorhizon Cell Cultures. #JOURNAL:Plant Physiol.#YEAR:1994#PUBMED:12232327# AUTHORS:Booth, A. N., Masri, M. S., Robbins, D. J., Emerson, O. H., Jones, F. T. & DeEds, F.#TITLE:Urinary Phenolic Acid Metabolites of Tyrosine#JOURNAL:J. Biol. Chem#YEAR:1960# GENE ASSOCIATION:
Abbreviation: R_ACGALK Synonyms: _0 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acgal + atp --> acgal1p + adp + h Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_FAOXC2051843x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : coa + h2o + nad + tmndnccoa --> accoa + h + nadh + strdnccoa Confidence Level: 4 _1 round beta ox, 1 degree loss of sat -> no fad oxidation - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) peroxisomal (very long chain FA) c20:5,n-3 -> c18:4, n-3 - final '3' in abbreviation indicates omega3 FA 4 step FAO lumped for 2 rounds also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (30.1 and 51.2 and 1962.1) or (51.1 and 3295.1 and 30.1) or (51.1 and 30.1 and 1962.1) or (3295.1 and 30.1 and 51.2)
Abbreviation: R_34HPPOR Synonyms: _0 EC Number: 1.13.11.27 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 34hpp + o2 --> co2 + hgentis Confidence Level: 4 NCD genes: LOCUS:_3242#TRANSCRIPT:1#ABBREVIATION:HPD#ECNUMBERS:1.13.11.27# proteins: NAME:_4-hydroxyphenylpyruvate dioxygenase#ABBREVIATION:Hpd.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (3242.1)
_4-Hydroxyphenylpyruvate:oxygen oxidoreductase
Abbreviation: R_SERt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + ser-L[e] --> na1[c] + ser-L[c] Confidence Level: 4 JLR genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_54407#TRANSCRIPT:1#ABBREVIATION:SLC38A2# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# LOCUS:_81539#TRANSCRIPT:1#ABBREVIATION:SLC38A1# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 38, member 2#ABBREVIATION:Slc38a2.1# NAME:solute carrier family 38, member 1#ABBREVIATION:Slc38a1.1# NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Sugawara M, Nakanishi T, Fei YJ, Huang W, Ganapathy ME, Leibach FH, Ganapathy V#TITLE:Cloning of an amino acid transporter with functional characteristics and tissue expression pattern identical to that of system A#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747860# AUTHORS:Hatanaka T, Huang W, Wang H, Sugawara M, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, functional characteristics and tissue expression pattern of human ATA2, a subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10930503# AUTHORS:Wang H, Huang W, Sugawara M, Devoe LD, Leibach FH, Prasad PD, Ganapathy V#TITLE:Cloning and functional expression of ATA1, a subtype of amino acid transporter A, from human placenta#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10891391# AUTHORS:Gu S, Roderick HL, Camacho P, Jiang JX#TITLE:Characterization of an N-system amino acid transporter expressed in retina and its involvement in glutamine transport#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11325958# GENE ASSOCIATION: (81539.1) or (11254.1) or (54407.1) or (55089.1)
Abbreviation: R_VACCCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: vacccrn[c] --> vacccrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_HSAT3ly Synonyms: _1 EC Number: 2.3.1.78 SUBSYSTEM: Heparan sulfate degradation Equation: accoa[c] + hs_deg13[l] --> coa[c] + h[l] + hs_deg14[l] Confidence Level: 4 The proton is located in the lysosome since it comes from oxidation of the NH3+ group in glucosamine. This reaction assumes that the other proton liberated from glucosamine reduces coa in the cytosol. NCD genes: none proteins: none citations: AUTHORS:Ausseil J, Loredo-Osti JC, Verner A, Darmond-Zwaig C, Maire I, Poorthuis B, van Diggelen OP, Hudson TJ, Fujiwara TM, Morgan K, Pshezhetsky AV#TITLE:Localisation of a gene for mucopolysaccharidosis IIIC to the pericentromeric region of chromosome 8#JOURNAL:J Med Genet#YEAR:2004#PUBMED:15591281# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
Abbreviation: R_EX_fuc_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fuc-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HESTRATRIOLtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: hestratriol[c] <==> hestratriol[r] Confidence Level: 0 unknown mechanism, hydroxylated (inactivated) estrogen derivative needs to be transported out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
hydroxylated estrogen derivative transport
Abbreviation: R_DNDPt29m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[m] + dcdp[c] --> dadp[c] + dcdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_NH4t3r Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[e] + nh4[c] <==> h[c] + nh4[e] Confidence Level: 4 genes: LOCUS:_6005#TRANSCRIPT:1#ABBREVIATION:RHAG# LOCUS:_57127#TRANSCRIPT:1#ABBREVIATION:RHBG# proteins: NAME:Rhesus blood group-associated glycoprotein#ABBREVIATION:Rhag.1-e# NAME:Rhesus blood group, B glycoprotein#ABBREVIATION:Rhbg.1-e# citations: AUTHORS:Westhoff CM, Siegel DL, Burd CG, Foskett JK#TITLE:Mechanism of genetic complementation of ammonium transport in yeast by human erythrocyte Rh-associated glycoprotein#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14966114# AUTHORS:Ridgwell K, Spurr NK, Laguda B, MacGeoch C, Avent ND, Tanner MJ#TITLE:Isolation of cDNA clones for a 50 kDa glycoprotein of the human erythrocyte membrane associated with Rh (rhesus) blood-group antigen expression#JOURNAL:Biochem J#YEAR:1992#PUBMED:1417776# AUTHORS:Nakhoul NL, Hamm LL#TITLE:Non-erythroid Rh glycoproteins: a putative new family of mammalian ammonium transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12920597# AUTHORS:Ludewig U#TITLE:Electroneutral ammonium transport by basolateral rhesus B glycoprotein#JOURNAL:J Physiol#YEAR:2004#PUBMED:15284342# AUTHORS:Liu Z, Peng J, Mo R, Hui C, Huang CH#TITLE:Rh type B glycoprotein is a new member of the Rh superfamily and a putative ammonia transporter in mammals#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11024028# AUTHORS:Malakooti J, Dahdal RY, Schmidt L, Layden TJ, Dudeja PK, Ramaswamy K#TITLE:Molecular cloning, tissue distribution, and functional expression of the human Na(+)/H(+) exchanger NHE2#JOURNAL:Am J Physiol#YEAR:1999#PUBMED:10444453# AUTHORS:Ghishan FK, Knobel SM, Summar M#TITLE:Molecular cloning, sequencing, chromosomal localization, and tissue distribution of the human Na+/H+ exchanger (SLC9A2)#JOURNAL:Genomics#YEAR:1995#PUBMED:8595899# GENE ASSOCIATION: (6005.1) or (57127.1)
Abbreviation: R_CYTK3n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : ctp + dcmp <==> cdp + dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_S3TASE1ly Synonyms: _1 EC Number: 3.1.6.15 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg10 <==> h + hs_deg11 + so4 Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Leder IG#TITLE:A novel 3-O sulfatase from human urine acting on methyl-2-deoxy-2-sulfamino-alphs-D-glucopyranoside 3-sulfate#JOURNAL:Biochem Biophys Res Commun#YEAR:1980#PUBMED:7396957# GENE ASSOCIATION:
Abbreviation: R_GTHO Synonyms: _0 EC Number: 1.8.1.7 SUBSYSTEM: Glutamate metabolism Equation: [c] : gthox + h + nadph --> (2) gthrd + nadp Confidence Level: 2 NCD, TV This EC (1.6.4.2) is obsolete. The new EC is 1.8.1.7. http://www.genome.ad.jp/dbget-bin/www_bget?ec:1.6.4.2 genes: LOCUS:_2936#TRANSCRIPT:1#ABBREVIATION:GSR#ECNUMBERS:1.6.4.2# proteins: NAME:glutathione reductase (cytosolic)#ABBREVIATION:Gsr.2# citations: AUTHORS:Kelner MJ, Montoya MA.#TITLE:Structural organization of the human glutathione reductase gene: determination of correct cDNA sequence and identification of a mitochondrial leader sequence. #JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10708558# GENE ASSOCIATION: (2936.1)
Abbreviation: R_M16N6Tg Synonyms: _5 EC Number: 2.4.1.155 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : n3m2masn + uacgam --> h + n4m2masn + udp Confidence Level: 0 NCD genes: LOCUS:_4249#TRANSCRIPT:1#ABBREVIATION:MGAT5#ECNUMBERS:2.4.1.155# proteins: NAME:mannosyl (alpha-1,6-)-glycoprotein beta-1,6-N-acetyl-glucosaminyltransferase, Golgi apparatus#ABBREVIATION:Mgat5.1-g# citations: none GENE ASSOCIATION: (4249.1)
alpha-1,6-mannosyl-glycoprotein 6-beta-N-acetylglucosaminyltransferase
_3-Methoxy-4-hydroxyphenylglycolaldehyde:NAD+ oxidoreductase
Abbreviation: R_3MOX4HOXPGALDOX Synonyms: _0 EC Number: 1.2.1.5 SUBSYSTEM: Tyrosine metabolism Equation: [c] : 3m4hpga + h2o + nad --> 3mox4hoxm + (2) h + nadh Confidence Level: 2 SAB genes: LOCUS:_221#TRANSCRIPT:1#ABBREVIATION:ALDH3B1#ECNUMBERS:1.2.1.5# LOCUS:_222#TRANSCRIPT:1#ABBREVIATION:ALDH3B2#ECNUMBERS:1.2.1.5# LOCUS:_220#TRANSCRIPT:1#ABBREVIATION:ALDH1A3#ECNUMBERS:1.2.1.3# LOCUS:_218#TRANSCRIPT:1#ABBREVIATION:ALDH3A1#ECNUMBERS:1.2.1.5# proteins: NAME:aldehyde dehydrogenase 3 family, member A1#ABBREVIATION:Aldh3a1.1# NAME:aldehyde dehydrogenase 1 family, member A3#ABBREVIATION:Aldh1a3.1# NAME:aldehyde dehydrogenase 3 family, member B1#ABBREVIATION:Aldh3b1.1# NAME:aldehyde dehydrogenase 3 family, member B2#ABBREVIATION:Aldh3b2.1# citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# GENE ASSOCIATION: (222.1) or (220.1) or (221.1) or (218.1)
Abbreviation: R_ABUTt2rL Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _4abut[l] + h[l] <==> 4abut[c] + h[c] Confidence Level: 4 SAB r, lyso genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
_4-aminobutyrate reversible transport in via proton symport (lysosome)
Abbreviation: R_M13N2Tg Synonyms: _6 EC Number: 2.4.1.101 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : m4masn + uacgam --> h + nm4masn + udp Confidence Level: 0 NCD genes: LOCUS:_4245#TRANSCRIPT:1#ABBREVIATION:MGAT1#ECNUMBERS:2.4.1.101# proteins: NAME:mannosyl (alpha-1,3-)-glycoprotein beta-1,2-N-acetylglucosaminyltransferase, Golgi apparatus (liver, kidney)#ABBREVIATION:Mgat1.1-g# citations: AUTHORS:Yen CL, Farese RV Jr.#TITLE:MGAT2, a monoacylglycerol acyltransferase expressed in the small intestine#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12621063# GENE ASSOCIATION: (4245.1)
alpha-1,3-mannosyl-glycoprotein 2-beta-N-acetylglucosaminyltransferase
Abbreviation: R_P4508B13r Synonyms: _3 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : h + nadh + o2 + xol7aone --> h2o + nad + xoldiolone Confidence Level: 4 NJ - added, substrates by analogy to other xol hydroxylase reactions (NADH and NADPH can both be used) genes: LOCUS:_1582#TRANSCRIPT:1#ABBREVIATION:CYP8B1# proteins: NAME:cytochrome P450, family 8, subfamily B, polypeptide 1#ABBREVIATION:Cyp8b1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Gafvels M, Olin M, Chowdhary BP, Raudsepp T, Andersson U, Persson B, Jansson M, Bjorkhem I, Eggertsen G#TITLE:Structure and chromosomal assignment of the sterol 12-alpha-hydroxylase gene (cyp8b1) in human and mouse#JOURNAL:Genomics#YEAR:1999# GENE ASSOCIATION: (1582.1)
Abbreviation: R_10FTHF6GLUtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _10fthf6glu[m] --> 10fthf6glu[c] Confidence Level: 2 mechanism is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_6-glutamyl-10FTHF transport, mitochondrial
Abbreviation: R_ACACT6p Synonyms: _0 EC Number: 2.3.1.16 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : 3otdcoa + coa --> accoa + ddcacoa Confidence Level: 4 NCD genes: LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# proteins: NAME:acetyl-Coenzyme A acyltransferase 1#ABBREVIATION:Acaa1.1# citations: AUTHORS:Bout A, Franse MM, Collins J, Blonden L, Tager JM, Benne R.#TITLE:Characterization of the gene encoding human peroxisomal 3-oxoacyl-CoA thiolase (ACAA). No large DNA rearrangement in a thiolase-deficient patient.#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1679347# GENE ASSOCIATION: (30.1)
acetyl-CoA C-acetyltransferase (dodecanoyl), peroxisomal
fatty-acid--CoA ligase (butanoate), mitochondrial
Abbreviation: R_FACOAL40im Synonyms: _0 EC Number: 6.2.1.2 SUBSYSTEM: Fatty acid activation Equation: [m] : atp + but + coa --> amp + btcoa + ppi Confidence Level: 4 JLR changed EC from 6.2.1.3 (long chain fatty-acyl CoA ligase) to 6.2.1.2 (butyrate-CoA ligase) - NCD genes: LOCUS:_116285#TRANSCRIPT:1#ABBREVIATION:BUCS1#ECNUMBERS:6.2.1.2# proteins: NAME:butyryl Coenzyme A synthetase 1, mitochondrial#ABBREVIATION:Bucs1.1-m# citations: AUTHORS:Fujino T, Takei YA, Sone H, Ioka RX, Kamataki A, Magoori K, Takahashi S, Sakai J, Yamamoto TT#TITLE:Molecular identification and characterization of two medium-chain acyl-CoA synthetases, MACS1 and the Sa gene product#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11470804# AUTHORS:Bugaut M#TITLE:Occurrence, absorption and metabolism of short chain fatty acids in the digestive tract of mammals#JOURNAL:Comp Biochem Physiol B#YEAR:1987#PUBMED:3297476# GENE ASSOCIATION: (116285.1)
Abbreviation: R_SMS Synonyms: _2 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : crm_hs + pchol_hs --> dag_hs + sphmyln_hs Confidence Level: 4 NJ genes: LOCUS:_259230#TRANSCRIPT:1#ABBREVIATION:TMEM23# proteins: NAME:Phosphatidylcholine:ceramide cholinephosphotransferase 1#ABBREVIATION:Tmem23.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Huitema K, van den Dikkenberg J, Brouwers JF, Holthuis JC.#TITLE:Identification of a family of animal sphingomyelin synthases.#JOURNAL:EMBO J#YEAR:2003#PUBMED:14685263# GENE ASSOCIATION: (259230.1)
Abbreviation: R_DCYTDn Synonyms: _0 EC Number: 3.5.4.14 SUBSYSTEM: Nucleotides Equation: [n] : dcyt + h + h2o --> duri + nh4 Confidence Level: 2 IT genes: LOCUS:_57379#TRANSCRIPT:1#ABBREVIATION:AICDA# proteins: NAME:activation-induced cytidine deaminase #ABBREVIATION:Aicda.1-n# citations: AUTHORS:Brar SS, Watson M, Diaz M.#TITLE:Activation-induced cytosine deaminase (AID) is actively exported out of the nucleus but retained by the induction of DNA breaks.#JOURNAL:J Biol Chem.#YEAR:2004#PUBMED:15087440# GENE ASSOCIATION: (57379.1)
Abbreviation: R_P4504F121r Synonyms: _0 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : arachd + h + nadph + o2 --> h2o + nadp + wharachd Confidence Level: 4 omega hydroxylation of arachidonic acid NJ genes: LOCUS:_66002#TRANSCRIPT:1#ABBREVIATION:CYP4F12# proteins: NAME:cytochrome P450, family 4, subfamily F, polypeptide 12#ABBREVIATION:Cyp4f12.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# GENE ASSOCIATION: (66002.1)
Abbreviation: R_EX_galacglcgalgbside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : galacglcgalgbside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Gal-GlcNAc-Gal globoside (homo sapiens) exchange
Abbreviation: R_B3GNT31g Synonyms: _9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : thcrm_hs + udpgal --> galthcrm_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Zhou D, Dinter A, Gutierrez Gallego R, Kamerling JP, Vliegenthart JF, Berger EG, Hennet T. #TITLE:A beta-1,3-N-acetylglucosaminyltransferase with poly-N-acetyllactosamine synthase activity is structurally related to beta-1,3-galactosyltransferases. #JOURNAL:Proc Natl Acad Sci#YEAR:1999# GENE ASSOCIATION: (10331.1)
Abbreviation: R_2HBt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _2hb[e] + h[e] <==> 2hb[c] + h[c] Confidence Level: 4 NCD genes: LOCUS:_9194#TRANSCRIPT:1#ABBREVIATION:SLC16A7# LOCUS:_9123#TRANSCRIPT:1#ABBREVIATION:SLC16A3# LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 7#ABBREVIATION:Slc16a7.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1#ABBREVIATION:Slc16a1.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 3#ABBREVIATION:Slc16a3.1-e# citations: AUTHORS:Lin RY, Vera JC, Chaganti RS, Golde DW#TITLE:Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9786900# AUTHORS:Halestrap AP, Price NT#TITLE:The proton-linked monocarboxylate transporter (MCT) family: structure, function and regulation#JOURNAL:Biochem J#YEAR:1999#PUBMED:10510291# AUTHORS:Garcia CK, Li X, Luna J, Francke U#TITLE:cDNA cloning of the human monocarboxylate transporter 1 and chromosomal localization of the SLC16A1 locus to 1p13.2-p12#JOURNAL:Genomics#YEAR:1994#PUBMED:7835905# AUTHORS:Poole RC, Halestrap AP#TITLE:Transport of lactate and other monocarboxylates across mammalian plasma membranes#JOURNAL:Am J Physiol#YEAR:1993#PUBMED:8476015# AUTHORS:Ritzhaupt A, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:The characterization of butyrate transport across pig and human colonic luminal membrane#JOURNAL:J Physiol#YEAR:1998#PUBMED:9508842# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification of a monocarboxylate transporter isoform type 1 (MCT1) on the luminal membrane of human and pig colon#JOURNAL:Biochem Soc Trans#YEAR:1998#PUBMED:9649795# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification and characterization of a monocarboxylate transporter (MCT1) in pig and human colon: its potential to transport L-lactate as well as butyrate#JOURNAL:J Physiol#YEAR:1998#PUBMED:9824713# AUTHORS:Halestrap AP, Meredith D#TITLE:The SLC16 gene family-from monocarboxylate transporters (MCTs) to aromatic amino acid transporters and beyond#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739169# AUTHORS:Manning Fox JE, Meredith D, Halestrap AP#TITLE:Characterisation of human monocarboxylate transporter 4 substantiates its role in lactic acid efflux from skeletal muscle#JOURNAL:J Physiol#YEAR:2000#PUBMED:11101640# AUTHORS:Price NT, Jackson VN, Halestrap AP#TITLE:Cloning and sequencing of four new mammalian monocarboxylate transporter (MCT) homologues confirms the existence of a transporter family with an ancient past#JOURNAL:Biochem J#YEAR:1998#PUBMED:9425115# GENE ASSOCIATION: (6566.1) or (9123.1) or (9194.1)
_2-hydroxybutyrate cotransport with proton
Abbreviation: R_EX_ha_pre1_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ha_pre1 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
hyaluronan biosynthesis, precursor 1 exchange
Abbreviation: R_NNATm Synonyms: _0 EC Number: 2.7.7.18 SUBSYSTEM: NAD Metabolism Equation: [m] : atp + h + nicrnt --> dnad + ppi Confidence Level: 4 NCD genes: LOCUS:_349565#TRANSCRIPT:1#ABBREVIATION:NMNAT3# proteins: NAME:nicotinamide nucleotide adenylyltransferase 3 #ABBREVIATION:Nmnat3.1-m# citations: AUTHORS:Zhang X, Kurnasov OV, Karthikeyan S, Grishin NV, Osterman AL, Zhang H. #TITLE:Structural characterization of a human cytosolic NMN/NaMN adenylyltransferase and implication in human NAD biosynthesis.#JOURNAL:J Biol Chem. #YEAR:2003#PUBMED:12574164# GENE ASSOCIATION: (349565.1)
nicotinate-nucleotide adenylyltransferase, mitochondrial
Abbreviation: R_MALSO3tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: mal-L[c] + so3[m] <==> mal-L[m] + so3[c] Confidence Level: 4 MM genes: LOCUS:_1468#TRANSCRIPT:1#ABBREVIATION:SLC25A10# proteins: NAME:Solute carrier family 25 (dicarboxylate transporter) member 10, mitochondrial #ABBREVIATION:Slc25a10.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# AUTHORS:Crompton M, Palmieri F, Capano M, Quagliariello E.#TITLE:The transport of sulphate and sulphite in rat liver mitochondria. #YEAR:1974#PUBMED:4441366# GENE ASSOCIATION: (1468.1)
Abbreviation: R_ADNt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: adn[e] <==> adn[c] Confidence Level: 4 IT genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# LOCUS:_3177#TRANSCRIPT:1#ABBREVIATION:SLC29A2# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 2 #ABBREVIATION:Slc29a2.1-e# NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-e# citations: AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (3177.1) or (2030.1)
adenosine facilated transport in cytosol
Abbreviation: R_DURIt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: duri[e] <==> duri[c] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION:
Abbreviation: R_P4502C18 Synonyms: _2 SUBSYSTEM: CYP Metabolism Equation: [c] : antipyrene + h + nadph + (0.5) o2 --> dmantipyrine + meoh + nadp Confidence Level: 2 antipyrine N-demethylation assumed to take place on outer membrane stoichiometry inferred (methanol production vs methane production?) NJ genes: LOCUS:_1562#TRANSCRIPT:1#ABBREVIATION:CYP2C18#ECNUMBERS:1.14.14.1# proteins: NAME:cytochrome P450, family 2, subfamily C, polypeptide 18#ABBREVIATION:Cyp2c18.1# citations: AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# AUTHORS:Goldstein JA, Faletto MB, Romkes-Sparks M, Sullivan T, Kitareewan S, Raucy JL, Lasker JM, Ghanayem BI.#TITLE:Evidence that CYP2C19 is the major (S)-mephenytoin 4'-hydroxylase in humans.#JOURNAL:Biochemistry#YEAR:1994#PUBMED:8110777# GENE ASSOCIATION: (1562.1)
Abbreviation: R_SIAASEly Synonyms: _4 EC Number: 3.2.1.18 SUBSYSTEM: N-Glycan Degradation Equation: [l] : (2) h2o + s2l2n2m2mn --> (2) acnam + l2n2m2mn Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_DNDPt35m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dadp[m] + dgdp[c] --> dadp[c] + dgdp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_HSD3A2r Synonyms: _0 SUBSYSTEM: Steroid Metabolism Equation: [r] : andrstandn + h + nadph <==> andrstrn + nadp Confidence Level: 0 Microsomal rxn see PMID: 14643063 substrate specificity still not clear (nad vs nadp) NJ genes: LOCUS:_1109#TRANSCRIPT:1#ABBREVIATION:AKR1C4#ECNUMBERS:1.1.1.225# proteins: NAME:aldo-keto reductase family 1, member C4#ABBREVIATION:Akr1c4.1# citations: AUTHORS:Belanger A, Pelletier G, Labrie F, Barbier O, Chouinard S.#TITLE:Inactivation of androgens by UDP-glucuronosyltransferase enzymes in humans.#JOURNAL:Trends Endocrinol Metab.#YEAR:2003#PUBMED:14643063# AUTHORS:Khanna M, Qin KN, Wang RW, Cheng KC.#TITLE:Substrate specificity, gene structure, and tissue-specific distribution of multiple human 3 alpha-hydroxysteroid dehydrogenases.#JOURNAL:J Biol Chem. #YEAR:1995#PUBMED:7650035# GENE ASSOCIATION: (1109.1)
Abbreviation: R_SO4OXAtex2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: oxa[c] + (2) so4[e] --> oxa[e] + (2) so4[c] Confidence Level: 2 SAB genes: LOCUS:_65010#TRANSCRIPT:1#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:2#ABBREVIATION:SLC26A6# LOCUS:_65010#TRANSCRIPT:3#ABBREVIATION:SLC26A6# proteins: NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.1# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.2# NAME:solute carrier family 26, member 6#ABBREVIATION:Slc26a6.3# citations: AUTHORS:Mount DB, Romero MF#TITLE:The SLC26 gene family of multifunctional anion exchangers#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759755# GENE ASSOCIATION: (65010.1) or (65010.3) or (65010.2)
sulfate transport via oxalate countertransport (2:1)
Abbreviation: R_10FTHF5GLUtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _10fthf5glu[m] --> 10fthf5glu[c] Confidence Level: 2 mechanism is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
_5-glutamyl-10FTHF transport, mitochondrial
Abbreviation: R_C181CPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + odecoa --> coa + odecrn Confidence Level: 4 TDV genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: AUTHORS:Finocchiaro G, Taroni F, Rocchi M, Martin AL, Colombo I, Tarelli GT, DiDonato S. #TITLE:cDNA cloning, sequence analysis, and chromosomal localization of the gene for human carnitine palmitoyltransferase. #JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1991#PUBMED:1988962# GENE ASSOCIATION: (126129.1) or (1375.3) or (1374.1) or (1375.2) or (1375.1) or (1375.4)
Abbreviation: R_10FTHF6GLUtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _10fthf6glu[c] --> 10fthf6glu[l] Confidence Level: 2 carrier-mediated transport - but which and how is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
Abbreviation: R_EHGLATm Synonyms: _0 EC Number: 2.6.1.1 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : akg + e4hglu --> 4h2oglt + glu-L Confidence Level: 1 NCD genes: LOCUS:_2806#TRANSCRIPT:1#ABBREVIATION:GOT2#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase, mitochondrial#ABBREVIATION:Got2.1-m# citations: AUTHORS:Knight J, Holmes RP#TITLE:Mitochondrial hydroxyproline metabolism: implications for primary hyperoxaluria#JOURNAL:Am J Nephrol#YEAR:2005#PUBMED:15849464# GENE ASSOCIATION: (2806.1)
L-erythro-4-Hydroxyglutamate:2-oxoglutarate aminotransferase, mitochondrial
Abbreviation: R_DMATTx Synonyms: _0 EC Number: 2.5.1.1 SUBSYSTEM: Cholesterol Metabolism Equation: [x] : dmpp + ipdp --> grdp + ppi Confidence Level: 4 peroxisomal version - NJ genes: LOCUS:_2224#TRANSCRIPT:1#ABBREVIATION:FDPS#ECNUMBERS:2.5.1.1,,2.5.1.# proteins: NAME:farnesyl diphosphate synthase (farnesyl pyrophosphate synthetase, dimethylallyltranstransferase, geranyltranstransferase)#ABBREVIATION:Fdps.1# citations: AUTHORS:Biardi L, Krisans K#TITLE:Compartmentalization of cholesterol biosynthesis#JOURNAL:Journal of Biological Chemistry#YEAR:1996# AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# GENE ASSOCIATION: (2224.1)
Abbreviation: R_EX_ak2lgchol_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ak2lgchol_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_1-alkyl 2-lysoglycerol 3-phosphocholine exchange
Abbreviation: R_EX_oagd3_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : oagd3_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
_9-O-Acetylated GD3 (homo sapiens) exchange
Abbreviation: R_CITt4_2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cit[e] + (2) na1[e] <==> cit[c] + (2) na1[c] Confidence Level: 4 NCD genes: LOCUS:_9058#TRANSCRIPT:1#ABBREVIATION:SLC13A2# proteins: NAME:solute carrier family 13 (sodium-dependent dicarboxylate transporter), member 2#ABBREVIATION:Slc13a2.1-e# citations: AUTHORS:Pajor AM, Sun N#TITLE:Functional differences between rabbit and human Na(+)-dicarboxylate cotransporters, NaDC-1 and hNaDC-1.#JOURNAL:Am J Physiol#YEAR:1996#PUBMED:8946005# AUTHORS:Pajor AM#TITLE:Molecular cloning and functional expression of a sodium-dicarboxylate cotransporter from human kidney#JOURNAL:Am J Physiol#YEAR:1996#PUBMED:8967342# GENE ASSOCIATION: (9058.1)
Abbreviation: R_DOPAQNISO1 Synonyms: _1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : dopaqn <==> 2c23dh56dhoxin + h Confidence Level: 4 SAB genes: none proteins: none citations: AUTHORS:Metzler, David E#TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 2#YEAR:2001# AUTHORS:Aaron Bunsen Lerner, Thomas B. Fitzpatrick, Evan Calkins, and William H. Summerson#TITLE:MAMMALIAN TYROSINASE: PREPARATION AND PROPERTIES#JOURNAL:J. Biol. Chem#YEAR:1949# AUTHORS:Pomerantz SH#TITLE:The tyrosine hydroxylase activity of mammalian tyrosinase#JOURNAL:J Biol Chem#YEAR:1968#PUBMED:5294951# GENE ASSOCIATION:
Abbreviation: R_ANTIPYRENEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: antipyrene[e] <==> antipyrene[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ESTRIOLGLCtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: estriolglc[c] <==> estriolglc[r] Confidence Level: 2 unknown mechanism, however glucuronidated metabolites need to get out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_THYMDtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: thymd[c] <==> thymd[m] Confidence Level: 4 TDV genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-m# citations: AUTHORS:Lai Y, Tse CM, Unadkat JD.#TITLE:Mitochondrial expression of the human equilibrative nucleoside transporter 1 (hENT1) results in enhanced mitochondrial toxicity of antiviral drugs.#JOURNAL:J Biol Chem. #YEAR:2004#PUBMED:14607828# AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (2030.1)
Abbreviation: R_PFK Synonyms: _0 EC Number: 2.7.1.11 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : atp + f6p --> adp + fdp + h Confidence Level: 2 SMP genes: LOCUS:_5214#TRANSCRIPT:1#ABBREVIATION:PFKP#ECNUMBERS:2.7.1.11# LOCUS:_5213#TRANSCRIPT:1#ABBREVIATION:PFKM#ECNUMBERS:2.7.1.11# LOCUS:_5211#TRANSCRIPT:1#ABBREVIATION:PFKL#ECNUMBERS:2.7.1.11# LOCUS:_5211#TRANSCRIPT:2#ABBREVIATION:PFKL#ECNUMBERS:2.7.1.11# proteins: NAME:muscle phosphofructokinase (muscle, platelet)#ABBREVIATION:Pfkm.1# NAME:liver phosphofructokinase (liver, platelet)#ABBREVIATION:Pfkl.1# NAME:liver phosphofructokinase (liver, platelet)#ABBREVIATION:Pfkl.2# NAME:phosphofructokinase (platelet)#ABBREVIATION:Pfkl1m1# NAME:phosphofructokinase (platelet)#ABBREVIATION:Pfkl2m1# NAME:platelet phosphofructokinase (platelet)#ABBREVIATION:Pfkp.1# NAME:phosphofructokinase (platelet)#ABBREVIATION:Pfkp1l1# NAME:phosphofructokinase (platelet)#ABBREVIATION:Pfkp1l2# NAME:phosphofructokinase (platelet)#ABBREVIATION:Pfkp1m1# citations: AUTHORS:Eto K, Sakura H, Yasuda K, Hayakawa T, Kawasaki E, Moriuchi R, Nagataki S, Yazaki Y, Kadowaki T#TITLE:Cloning of a complete protein-coding sequence of human platelet-type phosphofructokinase isozyme from pancreatic islet#JOURNAL:Biochem Biophys Res Commun#YEAR:1994#PUBMED:8117307# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (5214.1) or (5211.2) or (5214.1 and 5211.1) or (5214.1 and 5213.1) or (5213.1) or (5214.1 and 5211.2) or (5211.1) or (5211.1 and 5213.1) or (5213.1 and 5211.2)
Abbreviation: R_NACHEX15ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg22 --> acgam + ksi_deg23 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3074.1) or (3073.1)
Abbreviation: R_THMMPtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: oh1[m] + thmmp[c] <==> oh1[c] + thmmp[m] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Song Q, Singleton CK. #TITLE:Mitochondria from cultured cells derived from normal and thiamine-responsive megaloblastic anemia individuals efficiently import thiamine diphosphate.#JOURNAL:BMC Biochem.#YEAR:2005#PUBMED:12014993# AUTHORS:Barile M, Valenti D, Brizio C, Quagliariello E, Passarella S. #TITLE:Rat liver mitochondria can hydrolyse thiamine pyrophosphate to thiamine monophosphate which can cross the mitochondrial membrane in a carrier-mediated process.#JOURNAL:FEBS Lett.#YEAR:1998#PUBMED:9755848# GENE ASSOCIATION:
Thiamine monophosphate transport, mitochondrial
Abbreviation: R_NCCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cl[e] + na1[e] --> cl[c] + na1[c] Confidence Level: 4 NCD genes: LOCUS:_6559#TRANSCRIPT:1#ABBREVIATION:SLC12A3# proteins: NAME:solute carrier family 12 (sodium/chloride transporters), member 3#ABBREVIATION:Slc12a3.1-e# citations: AUTHORS:Hebert SC, Mount DB, Gamba G#TITLE:Molecular physiology of cation-coupled Cl- cotransport: the SLC12 family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739168# AUTHORS:Simon DB, Nelson-Williams C, Bia MJ, Ellison D, Karet FE, Molina AM, Vaara I, Iwata F, Cushner HM, Koolen M, Gainza FJ, Gitleman HJ, Lifton RP#TITLE:Gitelman's variant of Bartter's syndrome, inherited hypokalaemic alkalosis, is caused by mutations in the thiazide-sensitive Na-Cl cotransporter#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8528245# AUTHORS:Mastroianni N, De Fusco M, Zollo M, Arrigo G, Zuffardi O, Bettinelli A, Ballabio A, Casari G#TITLE:Molecular cloning, expression pattern, and chromosomal localization of the human Na-Cl thiazide-sensitive cotransporter (SLC12A3).#JOURNAL:Genomics#YEAR:1996#PUBMED:8812482# AUTHORS:Chang H, Tashiro K, Hirai M, Ikeda K, Kurokawa K, Fujita T#TITLE:Identification of a cDNA encoding a thiazide-sensitive sodium-chloride cotransporter from the human and its mRNA expression in various tissues#JOURNAL:Biochem Biophys Res Commun#YEAR:1996#PUBMED:8670281# GENE ASSOCIATION: (6559.1)
Abbreviation: R_FUT34g Synonyms: _5 EC Number: 2.4.1.65 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucgalacglcgalgluside_hs + gdpfuc --> fucfucgalacglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2525#TRANSCRIPT:1#ABBREVIATION:FUT3#ECNUMBERS:2.4.1.65# proteins: NAME:fucosyltransferase 3 (galactoside 3(4)-L-fucosyltransferase, Lewis blood group)#ABBREVIATION:Fut3.1# citations: AUTHORS:Cakir B, Pankow JS, Salomaa V, Couper D, Morris TL, Brantley KR, Hiller KM, Heiss G, Weston BW.#TITLE:Distribution of Lewis (FUT3)genotype and allele: frequencies in a biethnic United States population.#JOURNAL:Ann Hematol#YEAR:2002#PUBMED:12424536# AUTHORS:Sousa VL, Brito C, Costa T, Lanoix J, Nilsson T, Costa J.#TITLE:Importance of Cys, Gln, and Tyr from the transmembrane domain of human alpha 3/4 fucosyltransferase III for its localization and sorting in the Golgi of baby hamster kidney cells.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12493760# AUTHORS:Kukowska-Latallo JF, Larsen RD, Nair RP, Lowe JB.#TITLE:A cloned human cDNA determines expression of a mouse stage-specific embryonic antigen and the Lewis blood group alpha(1,3/1,4)fucosyltransferase.#JOURNAL:Genes Dev#YEAR:1990#PUBMED:1977660# GENE ASSOCIATION: (2525.1)
Abbreviation: R_LEUKTRB4tr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: leuktrB4[c] <==> leuktrB4[r] Confidence Level: 1 unknown mechanism, presumed diffusion NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PGPP_hs Synonyms: _0 EC Number: 3.1.3.27 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : h2o + pgp_hs --> pglyc_hs + pi Confidence Level: 0 NJ genes: none proteins: none citations: AUTHORS:Schlame M, Rua D, Greenberg ML.#TITLE:The biosynthesis and functional role of cardiolipin.#JOURNAL:Prog Lipid Res.#YEAR:2000#PUBMED:10799718# GENE ASSOCIATION:
Phosphatidylglycerol phosphate phosphatase (homo sapiens)
Abbreviation: R_GALGT2 Synonyms: _3 EC Number: 2.4.1.92 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gm3_hs + udpacgal --> gm2_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_2583#TRANSCRIPT:1#ABBREVIATION:GALGT#ECNUMBERS:2.4.1.92# proteins: NAME:UDP-N-acetyl-alpha-D-galactosamine:(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase (GalNAc-T)#ABBREVIATION:Galgt.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Nagata Y, Yamashiro S, Yodoi J, Lloyd KO, Shiku H, Furukawa K.#TITLE:Expression cloning of beta 1,4 N-acetylgalactosaminyltransferase cDNAs that determine the expression of GM2 and GD2 gangliosides.#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1601877# GENE ASSOCIATION: (2583.1)
Abbreviation: R_ACS Synonyms: _0 EC Number: 6.2.1.1 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : ac + atp + coa --> accoa + amp + ppi Confidence Level: 4 genes: LOCUS:_65985#TRANSCRIPT:1#ABBREVIATION:AACS#ECNUMBERS:6.2.1.16# LOCUS:_55902#TRANSCRIPT:1#ABBREVIATION:ACAS2# LOCUS:_55902#TRANSCRIPT:2#ABBREVIATION:ACAS2# proteins: NAME:acetly-CoA synthetase 2 (ADP forming)#ABBREVIATION:Acas2.1# NAME:acetyl-CoA synthetase 2 (ADP forming)#ABBREVIATION:Acas2.2# NAME:acetoacetyl-CoA synthetase#ABBREVIATION:Aacs.1# citations: AUTHORS:Ohgami M, Takahashi N, Yamasaki M, Fukui T#TITLE:Expression of acetoacetyl-CoA synthetase, a novel cytosolic ketone body-utilizing enzyme, in human brain#JOURNAL:Biochem Pharmacol#YEAR:2003#PUBMED:12623130# AUTHORS:Luong A, Hannah VC, Brown MS, Goldstein JL#TITLE:Molecular characterization of human acetyl-CoA synthetase, an enzyme regulated by sterol regulatory element-binding proteins#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10843999# GENE ASSOCIATION: (65985.1) or (55902.2) or (55902.1)
Abbreviation: R_CSPG_Et Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cspg_e[g] --> cspg_e[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
chondroitin sulfate E transport, golgi to extracellular
Abbreviation: R_RBFK Synonyms: _0 EC Number: 2.7.1.26 SUBSYSTEM: Riboflavin Metabolism Equation: [c] : atp + ribflv --> adp + fmn + h Confidence Level: 4 genes: LOCUS:_55312#TRANSCRIPT:1#ABBREVIATION:RFK#ECNUMBERS:2.7.1.26# proteins: NAME:Riboflavin kinase#ABBREVIATION:Rfk.1# citations: AUTHORS:Karthikeyan S, Zhou Q, Mseeh F, Grishin NV, Osterman AL, Zhang H.#TITLE:Crystal structure of human riboflavin kinase reveals a beta barrel fold and a novel active site arch.#JOURNAL:Structure (Camb). #YEAR:2003#PUBMED:12623014# AUTHORS:Karthikeyan S, Zhou Q, Osterman AL, Zhang H. #TITLE:Ligand binding-induced conformational changes in riboflavin kinase: structural basis for the ordered mechanism.#JOURNAL:Biochemistry. #YEAR:2003#PUBMED:14580199# GENE ASSOCIATION: (55312.1)
Abbreviation: R_ACNAM9PL Synonyms: _0 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acmanap + h2o + pep --> acnamp + pi Confidence Level: 4 EC-2.5.1.56 NCD genes: LOCUS:_54187#TRANSCRIPT:1#ABBREVIATION:NANS# proteins: NAME:N-acetylneuraminic acid synthase (sialic acid synthase)#ABBREVIATION:Nans.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Lawrence SM, Huddleston KA, Pitts LR, Nguyen N, Lee YC, Vann WF, Coleman TA, Betenbaugh MJ#TITLE:Cloning and expression of the human N-acetylneuraminic acid phosphate synthase gene with 2-keto-3-deoxy-D-glycero- D-galacto-nononic acid biosynthetic ability#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10749855# GENE ASSOCIATION: (54187.1)
Abbreviation: R_B3GNT33g Synonyms: _9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : galgalthcrm_hs + udpgal --> galgalgalthcrm_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Zhou D, Dinter A, Gutierrez Gallego R, Kamerling JP, Vliegenthart JF, Berger EG, Hennet T. #TITLE:A beta-1,3-N-acetylglucosaminyltransferase with poly-N-acetyllactosamine synthase activity is structurally related to beta-1,3-galactosyltransferases. #JOURNAL:Proc Natl Acad Sci#YEAR:1999# GENE ASSOCIATION: (10331.1)
Abbreviation: R_3MLDAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _3mlda[c] <==> 3mlda[e] Confidence Level: 2 SAB genes: none proteins: none citations: AUTHORS:Johansson AC, Lonnqvist B, Granerus G#TITLE:The relationship between body size and the urinary excretion of the main histamine metabolite tele-methylimidazoleacetic acid in man#JOURNAL:Inflamm Res#YEAR:2001#PUBMED:11411609# GENE ASSOCIATION:
_3MLDA extracellular transport via diffusion
Abbreviation: R_AKGDm Synonyms: _0 EC Number: 1.2.4.2 SUBSYSTEM: Citric Acid Cycle Equation: [m] : akg + coa + nad --> co2 + nadh + succoa Confidence Level: 0 EC taken from Ligand TV genes: LOCUS:_4967#TRANSCRIPT:1#ABBREVIATION:OGDH#ECNUMBERS:1.2.4.2# LOCUS:_8050#TRANSCRIPT:1#ABBREVIATION:PDHX#ECNUMBERS:2.3.1.12# LOCUS:_1738#TRANSCRIPT:1#ABBREVIATION:DLD#ECNUMBERS:1.8.1.4# LOCUS:_1743#TRANSCRIPT:1#ABBREVIATION:DLST#ECNUMBERS:2.3.1.61# LOCUS:_4967#TRANSCRIPT:2#ABBREVIATION:OGDH# proteins: NAME:dihydrolipoamide dehydrogenase (pyruvate dehydrogenase, E3 component), mitochondrial#ABBREVIATION:Dld/Pdhx.1-m# NAME: oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide), mitochondrial#ABBREVIATION:Ogdh.1-m# NAME:oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide), mitochondrial#ABBREVIATION:Ogdh.2-m# NAME:dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex), mitochondrial#ABBREVIATION:Dlst.1-m# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (4967.1) and (1738.1 and 8050.1) and (1743.1) or (4967.2) and (1738.1 and 8050.1) and (1743.1)
Abbreviation: R_ALAtN1 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[e] + h[c] + (2) na1[e] <==> ala-L[c] + h[e] + (2) na1[c] Confidence Level: 4 SAB based om PMID 10823827 genes: LOCUS:_92745#TRANSCRIPT:1#ABBREVIATION:SLC38A5# LOCUS:_10991#TRANSCRIPT:1#ABBREVIATION:SLC38A3# proteins: NAME:solute carrier family 38, member 3#ABBREVIATION:Slc38a3.1# NAME:solute carrier family 38, member 5#ABBREVIATION:Slc38a5.1# citations: AUTHORS:Fei YJ, Sugawara M, Nakanishi T, Huang W, Wang H, Prasad PD, Leibach FH, Ganapathy V#TITLE:Primary structure, genomic organization, and functional and electrogenic characteristics of human system N 1, a Na+- and H+-coupled glutamine transporter#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10823827# AUTHORS:Mackenzie B, Erickson JD#TITLE:Sodium-coupled neutral amino acid (System N/A) transporters of the SLC38 gene family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12845534# GENE ASSOCIATION: (92745.1) or (10991.1)
Abbreviation: R_PTDCAt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ptdca[e] <==> ptdca[c] Confidence Level: 2 NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_XOLTRI25tc Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: xoltri25[r] <==> xoltri25[c] Confidence Level: 1 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ELAIDCRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: elaidcrn[c] --> elaidcrn[m] Confidence Level: 4 NJ genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_NACHEX9ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_e_deg6 + h2o --> acgal + cs_e_deg7 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1 and 3074.1) or (3074.1) or (3073.1)
Abbreviation: R_FUCtly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: fuc-L[l] --> fuc-L[c] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Pisoni RL, Thoene JG#TITLE:The transport systems of mammalian lysosomes#JOURNAL:Biochim Biophys Acta#YEAR:1991#PUBMED:1751541# AUTHORS:Lloyd JB#TITLE:Metabolite efflux and influx across the lysosome membrane#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993166# GENE ASSOCIATION:
Abbreviation: R_PFK26 Synonyms: _0 EC Number: 2.7.1.105 SUBSYSTEM: Fructose and Mannose Metabolism Equation: [c] : atp + f6p --> adp + f26bp + h Confidence Level: 0 IF genes: LOCUS:_5207#TRANSCRIPT:1#ABBREVIATION:PFKFB1#ECNUMBERS:2.7.1.105,,3.1.# LOCUS:_5210#TRANSCRIPT:1#ABBREVIATION:PFKFB4#ECNUMBERS:2.7.1.105,3.1.3.46# LOCUS:_5209#TRANSCRIPT:1#ABBREVIATION:PFKFB3#ECNUMBERS:2.7.1.105,3.1.3.46# LOCUS:_5208#TRANSCRIPT:1#ABBREVIATION:PFKFB2#ECNUMBERS:2.7.1.105,,3.1.# proteins: NAME:_6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1#ABBREVIATION:Pfkfb1.1# NAME:_6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 2#ABBREVIATION:Pfkfb2.1# NAME:_6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 3#ABBREVIATION:Pfkfb3.1# NAME:_6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 4#ABBREVIATION:Pfkfb4.1# citations: none GENE ASSOCIATION: (5210.1) or (5207.1) or (5209.1) or (5208.1)
Abbreviation: R_BVITEt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: bvite[e] --> bvite[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_STS3 Synonyms: _0 EC Number: 3.1.6.2 SUBSYSTEM: Steroid Metabolism Equation: [c] : chsterols + h2o --> chsterol + h + so4 Confidence Level: 4 NJ genes: LOCUS:_412#TRANSCRIPT:1#ABBREVIATION:STS#ECNUMBERS:3.1.6.2# proteins: NAME:steroid sulfatase (microsomal), arylsulfatase C#ABBREVIATION:Sts.1# citations: AUTHORS:Stein C, Hille A, Seidel J, Rijnbout S, Waheed A, Schmidt B, Geuze H, von Figura K.#TITLE:Cloning and expression of human steroid-sulfatase. Membrane topology, glycosylation, and subcellular distribution in BHK-21 cells.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2668275# AUTHORS:Migeon BR, Shapiro LJ, Norum RA, Mohandas T, Axelman J, Dabora RL.#TITLE:Abstract Differential expression of steroid sulphatase locus on active and inactive human X chromosome.#JOURNAL:Nature#YEAR:1982#PUBMED:6957717# GENE ASSOCIATION: (412.1)
Abbreviation: R_TETPENT6t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tetpent6[e] <==> tetpent6[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_AACOAT Synonyms: _0 EC Number: 6.2.1.16 SUBSYSTEM: Propanoate Metabolism Equation: [c] : acac + atp + coa <==> aacoa + amp + ppi Confidence Level: 4 also EC 2.8.3.8 NCD genes: LOCUS:_65985#TRANSCRIPT:1#ABBREVIATION:AACS#ECNUMBERS:6.2.1.16# proteins: NAME:acetoacetyl-CoA synthetase#ABBREVIATION:Aacs.1# citations: AUTHORS:Ohgami M, Takahashi N, Yamasaki M, Fukui T#TITLE:Expression of acetoacetyl-CoA synthetase, a novel cytosolic ketone body-utilizing enzyme, in human brain#JOURNAL:Biochem Pharmacol#YEAR:2003#PUBMED:12623130# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (65985.1)
Abbreviation: R_EX_dopa_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : dopa <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FPGS5m Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [m] : 5dhf + atp + glu-L --> 6dhf + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_PTE4x Synonyms: _1 SUBSYSTEM: Fatty Acid Metabolism Equation: [x] : h2o + phytcoa --> coa + h + phyt Confidence Level: 4 NJ genes: LOCUS:_10965#TRANSCRIPT:1#ABBREVIATION:ZAP128# proteins: NAME:peroxisomal long-chain acyl-CoA thioesterase#ABBREVIATION:Zap128.1# citations: AUTHORS:Jones JM, Gould SJ.#TITLE:Identification of PTE2, a human peroxisomal long-chain acyl-CoA thioesterase.#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10944470# GENE ASSOCIATION: (10965.1)
Abbreviation: R_FACOAL181i Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + ocdcea --> amp + odecoa + ppi Confidence Level: 4 TV catalyzed by acyl-CoA synthetases genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# LOCUS:_2181#TRANSCRIPT:1#ABBREVIATION:ACSL3# LOCUS:_2181#TRANSCRIPT:2#ABBREVIATION:ACSL3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.2# citations: none GENE ASSOCIATION: (2180.1) or (2181.2) or (2181.1)
Abbreviation: R_ENGASEly Synonyms: _15 EC Number: 3.2.1.96 SUBSYSTEM: N-Glycan Degradation Equation: [l] : h2o + s2l2n2m2m --> acgam + s2l2n2m2mn Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
_5-Oxoproline amidohydrolase (ATP-hydrolysing) (ir)
Abbreviation: R_OPAHir Synonyms: _0 EC Number: 3.5.2.9 SUBSYSTEM: Glutathione Metabolism Equation: [c] : 5oxpro + atp + (2) h2o --> adp + glu-L + h + pi Confidence Level: 4 AF Added from KEGG genes: LOCUS:_26873#TRANSCRIPT:1#ABBREVIATION:OPLAH# proteins: NAME:_5-oxoprolinase (ATP-hydrolysing)#ABBREVIATION:Oplah.1# citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# GENE ASSOCIATION: (26873.1)
Abbreviation: R_DNDPt18m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[m] + dudp[c] --> adp[c] + dudp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_gp1c_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : gp1c_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TS3 Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [c] : pd3 <==> ts3 Confidence Level: 4 This reaction takes place in skin and needs UV 290-305. based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.194 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_AKGtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: akg[c] <==> akg[x] Confidence Level: 0 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_STS1r Synonyms: _4 EC Number: 3.1.6.2 SUBSYSTEM: Steroid Metabolism Equation: [r] : dheas + h2o --> dhea + h + so4 Confidence Level: 4 NJ genes: LOCUS:_412#TRANSCRIPT:1#ABBREVIATION:STS#ECNUMBERS:3.1.6.2# proteins: NAME:steroid sulfatase (microsomal), arylsulfatase C#ABBREVIATION:Sts.1# citations: AUTHORS:Stein C, Hille A, Seidel J, Rijnbout S, Waheed A, Schmidt B, Geuze H, von Figura K.#TITLE:Cloning and expression of human steroid-sulfatase. Membrane topology, glycosylation, and subcellular distribution in BHK-21 cells.#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2668275# AUTHORS:Migeon BR, Shapiro LJ, Norum RA, Mohandas T, Axelman J, Dabora RL.#TITLE:Abstract Differential expression of steroid sulphatase locus on active and inactive human X chromosome.#JOURNAL:Nature#YEAR:1982#PUBMED:6957717# GENE ASSOCIATION: (412.1)
Abbreviation: R_TTDCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + tdcoa --> coa + ttdcrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1374.1) or (1375.2) or (126129.1) or (1375.4)
Abbreviation: R_AG13T6g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre6 + uacgam --> h + ksi_pre7 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
Abbreviation: R_MTAP Synonyms: _0 EC Number: 2.4.2.28 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : 5mta + pi --> 5mdr1p + ade Confidence Level: 4 genes: LOCUS:_4507#TRANSCRIPT:1#ABBREVIATION:MTAP#ECNUMBERS:2.4.2.28# proteins: NAME:methylthioadenosine phosphorylase#ABBREVIATION:Mtap.1# citations: AUTHORS:Evans GB, Furneaux RH, Lenz DH, Painter GF, Schramm VL, Singh V, Tyler PC#TITLE:Second generation transition state analogue inhibitors of human 5'-methylthioadenosine phosphorylase#JOURNAL:J Med Chem#YEAR:2005#PUBMED:16000004# AUTHORS:Della Ragione F, Carteni-Farina M, Gragnaniello V, Schettino MI, Zappia V#TITLE:Purification and characterization of 5'-deoxy-5'-methylthioadenosine phosphorylase from human placenta#JOURNAL:J Biol Chem#YEAR:1986#PUBMED:3091600# AUTHORS:Myers RW, Abeles RH#TITLE:Conversion of 5-S-ethyl-5-thio-D-ribose to ethionine in Klebsiella pneumoniae. Basis for the selective toxicity of 5-S-ethyl-5-thio-D-ribose#JOURNAL:J Biol Chem#YEAR:1989#PUBMED:2543672# GENE ASSOCIATION: (4507.1)
Abbreviation: R_EX_retinol_DASH_9_DASH_cis_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : retinol-9-cis <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ASP1DC Synonyms: _0 EC Number: 4.1.1.11 SUBSYSTEM: beta-Alanine metabolism Equation: [c] : asp-L + h --> ala-B + co2 Confidence Level: 1 The E. coli enzyme contains a pyruvoyl group. genes: LOCUS:_2571#TRANSCRIPT:1#ABBREVIATION:GAD1#ECNUMBERS:4.1.1.15# LOCUS:_2571#TRANSCRIPT:2#ABBREVIATION:GAD1#ECNUMBERS:4.1.1.15# proteins: NAME:glutamate decarboxylase 1 (brain, 67kDa)#ABBREVIATION:Gad1.1# NAME:glutamate decarboxylase 1 (brain, 67kDa)#ABBREVIATION:Gad1.2# citations: none GENE ASSOCIATION: (2571.1) or (2571.2)
Abbreviation: R_GLCNACASE4ly Synonyms: _4 EC Number: 3.2.1.50 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg20 --> acgam + hs_deg21 Confidence Level: 4 NCD genes: LOCUS:_4669#TRANSCRIPT:1#ABBREVIATION:NAGLU#ECNUMBERS:3.2.1.50# proteins: NAME:N-acetylglucosaminidase, alpha- (Sanfilippo disease IIIB), lysosome#ABBREVIATION:Naglu.1-l# citations: AUTHORS:Weber B, Blanch L, Clements PR, Scott HS, Hopwood JJ#TITLE:Cloning and expression of the gene involved in Sanfilippo B syndrome (mucopolysaccharidosis III B)#JOURNAL:Hum Mol Genet#YEAR:1996#PUBMED:8776591# AUTHORS:Sasaki T, Sukegawa K, Masue M, Fukuda S, Tomatsu S, Orii T#TITLE:Purification and partial characterization of alpha-N-acetylglucosaminidase from human liver#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1783617# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION: (4669.1)
Abbreviation: R_GPIDA2er Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : em2emgacpail_prot_hs + h2o --> dem2emgacpail_prot_hs + h + hdca Confidence Level: 4 NCD genes: LOCUS:_80055#TRANSCRIPT:1#ABBREVIATION:PGAP1# proteins: NAME:GPI deacylase, endoplasmic reticulum#ABBREVIATION:Pgap1.1-r# citations: AUTHORS:Tanaka S, Maeda Y, Tashima Y, Kinoshita T#TITLE:Inositol deacylation of glycosylphosphatidylinositol-anchored proteins is mediated by mammalian PGAP1 and yeast Bst1p#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:14734546# GENE ASSOCIATION: (80055.1)
Abbreviation: R_CYTK2n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : atp + dcmp <==> adp + dcdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_PEFLIPm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[c] + h2o[c] + pe_hs[c] --> adp[c] + h[c] + pe_hs[m] + pi[c] Confidence Level: 4 See PMID: 10856717 - flippase activity no detailed data on which compartment ATP hydrolysis occurs, so for the time being assumed to be cytosolic NJ genes: LOCUS:_10396#TRANSCRIPT:1#ABBREVIATION:ATP8A1#ECNUMBERS:3.6.3.1# LOCUS:_57194#TRANSCRIPT:1#ABBREVIATION:ATP10A#ECNUMBERS:3.6.3.1# proteins: NAME:ATPase, aminophospholipid transporter (APLT), Class I, type 8A, member 1#ABBREVIATION:Atp8a1.1# NAME:ATPase, Class V, type 10A#ABBREVIATION:Atp10a.1# citations: AUTHORS:Daleke DL, Lyles JV.#TITLE:Identification and purification of aminophospholipid flippases.#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:10856717# AUTHORS:Herzing LB, Kim SJ, Cook EH Jr, Ledbetter DH.#TITLE:The human aminophospholipid-transporting ATPase gene ATP10C maps adjacent to UBE3A and exhibits similar imprinted expression.#JOURNAL:Am J Hum Genet#YEAR:2001#PUBMED:11353404# AUTHORS:Mouro I, Halleck MS, Schlegel RA, Mattei MG, Williamson P, Zachowski A, Devaux P, Cartron JP, Colin Y.#TITLE:Cloning, expression, and chromosomal mapping of a human ATPase II gene, member of the third subfamily of P-type ATPases and orthologous to the presumed bovine and murine aminophospholipid translocase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10198212# GENE ASSOCIATION: (10396.1) or (57194.1)
Abbreviation: R_FUCGAL14ACGLCGALGLUSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: fucgal14acglcgalgluside_hs[g] <==> fucgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_7THFtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _7thf[c] --> 7thf[l] Confidence Level: 2 carrier-mediated transport - but which and how is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
Abbreviation: R_O2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: o2[e] <==> o2[c] Confidence Level: 1 tv renamed from O2TP to O2t to avoid confusion with transport reaction from cytosol to peroxisome NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_galfuc12gal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : galfuc12gal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
(Gal)3 (Glc)1 (GlcNAc)1 (LFuc)1 (Cer)1 exchange
Abbreviation: R_PRO1x Synonyms: _0 EC Number: 1.5.1.2 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : nad + pro-L --> 1pyr5c + (2) h + nadh Confidence Level: 2 genes: LOCUS:_58510#TRANSCRIPT:1#ABBREVIATION:PRODH2# proteins: NAME:proline dehydrogenase (oxidase) 2#ABBREVIATION:Prodh2.1# citations: AUTHORS:Gogos JA, Santha M, Takacs Z, Beck KD, Luine V, Lucas LR, Nadler JV, Karayiorgou M.#TITLE:The gene encoding proline dehydrogenase modulates sensorimotor gating in mice. #JOURNAL:Nat Genet#YEAR:1999#PUBMED:10192398# GENE ASSOCIATION: (58510.1)
Abbreviation: R_NTPP9 Synonyms: _0 EC Number: 3.6.1.19 SUBSYSTEM: Nucleotides Equation: [c] : h2o + itp --> h + imp + ppi Confidence Level: 4 JLR; IT genes: LOCUS:_3704#TRANSCRIPT:1#ABBREVIATION:ITPA#ECNUMBERS:3.6.1.19# LOCUS:_3704#TRANSCRIPT:2#ABBREVIATION:ITPA#ECNUMBERS:3.6.1.19# proteins: NAME:inosine triphosphatase (nucleoside triphosphate pyrophosphatase) #ABBREVIATION:ItpA.1# NAME:inosine triphosphatase (nucleoside triphosphate pyrophosphatase) #ABBREVIATION:ItpA.2# citations: AUTHORS:Lin S, McLennan AG, Ying K, Wang Z, Gu S, Jin H, Wu C, Liu W, Yuan Y, Tang R, Xie Y, Mao Y. #TITLE:Cloning, expression, and characterization of a human inosine triphosphate pyrophosphatase encoded by the itpa gene. #JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11278832# GENE ASSOCIATION: (3704.1) or (3704.2)
Nucleoside triphosphate pyrophosphorylase (itp)
Abbreviation: R_NDPK4m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + dtdp <==> adp + dttp Confidence Level: 2 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:dTDP), mitochondrial
Abbreviation: R_BACCL Synonyms: _7 EC Number: 6.3.4.15 SUBSYSTEM: Biotin Metabolism Equation: [c] : atp + btn + h --> btamp + ppi Confidence Level: 4 IT genes: LOCUS:_3141#TRANSCRIPT:1#ABBREVIATION:HLCS#ECNUMBERS:6.3.4.10# proteins: NAME:holocarboxylase synthetase (biotin-[proprionyl-Coenzyme A-carboxylase (ATP-hydrolysing)] ligase) #ABBREVIATION:Hcls.1# citations: AUTHORS:Suzuki Y, Aoki Y, Ishida Y, Chiba Y, Iwamatsu A, Kishino T, Niikawa N, Matsubara Y, Narisawa K.#TITLE:Isolation and characterization of mutations in the human holocarboxylase synthetase cDNA.#JOURNAL:Nat Genet. #YEAR:1994#PUBMED:7842009# AUTHORS:Pacheco-Alvarez D, Solorzano-Vargas RS, Del Rio AL.#TITLE:Biotin in metabolism and its relationship to human disease.#JOURNAL:Arch Med Res. #YEAR:2002#PUBMED:12459313# GENE ASSOCIATION: (3141.1)
Abbreviation: R_S6TASE26ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksii_core4_deg3 <==> h + ksii_core4_deg4 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_B3GNT314g Synonyms: _9 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acglcgalacglcgal14acglcgalgluside_hs + udpgal --> galacglcgalacglcgal14acglcgalgluside_hs + h + udp Confidence Level: 2 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3#ABBREVIATION:B3gnt3.1# citations: AUTHORS:Yokoyama-Kobayashi M, Yamaguchi T, Sekine S, Kato S.#TITLE:Selection of cDNAs encoding putative type II membrane proteins on the cell surface from a human full-length cDNA bank.#JOURNAL:Gene#YEAR:1999#PUBMED:10072769# GENE ASSOCIATION: (10331.1)
Abbreviation: R_DOLPMT4_Uer Synonyms: _2 EC Number: 2.4.1.130 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : (0.1) dolmanp_U + (0.1) m7mpdol_U --> (0.1) dolp_U + h + (0.1) m8mpdol_U Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_MANter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: man[r] --> man[c] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
mannose efflux from endoplasmic reticulum
Abbreviation: R_FUCACGALFUCGALACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucacgalfucgalacglcgalgluside_hs[e] <==> fucacgalfucgalacglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PGL Synonyms: _0 EC Number: 3.1.1.31 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : 6pgl + h2o --> 6pgc + h Confidence Level: 0 SMP Net reaction same as described in Lehringer 334 genes: LOCUS:_25796#TRANSCRIPT:1#ABBREVIATION:PGLS#ECNUMBERS:3.1.1.31# proteins: NAME:_6-phosphogluconolactonase#ABBREVIATION:Pgls.1# citations: none GENE ASSOCIATION: (25796.1)
Abbreviation: R_PETOHMr_hs Synonyms: _0 EC Number: 2.1.1.17 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [r] : (3) amet + pe_hs --> (3) ahcys + (3) h + pchol_hs Confidence Level: 2 for human Catalyzes three sequential methylation of phosphatidylethanolamine (PE) by AdoMet, thus producing phosphatidylcholine (PC). [Swiss-Prot] NJ genes: LOCUS:_10400#TRANSCRIPT:1#ABBREVIATION:PEMT#ECNUMBERS:2.1.1.17# LOCUS:_10400#TRANSCRIPT:2#ABBREVIATION:PEMT#ECNUMBERS:2.1.1.17# LOCUS:_10400#TRANSCRIPT:3#ABBREVIATION:PEMT#ECNUMBERS:2.1.1.17# proteins: NAME:phosphatidylethanolamine N-methyltransferase #ABBREVIATION:Pemt.1# NAME:phosphatidylethanolamine N-methyltransferase #ABBREVIATION:Pemt.2# NAME:phosphatidylethanolamine N-methyltransferase #ABBREVIATION:Pemt.3# citations: AUTHORS:Walkey CJ, Shields DJ, Vance DE.#TITLE:Identification of three novel cDNAs for human phosphatidylethanolamine N-methyltransferase and localization of the human gene on chromosome 17p11.2.#JOURNAL:Biochim Biophys Acta.#YEAR:1999#PUBMED:9989271# GENE ASSOCIATION: (10400.2) or (10400.1) or (10400.3)
Abbreviation: R_EX_rbt_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : rbt <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SERGLNNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: gln-L[c] + na1[e] + ser-L[e] --> gln-L[e] + na1[c] + ser-L[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-serine/L-glutamine Na-dependent exchange (Ser-L in)
Abbreviation: R_EX_sel_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : sel <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PEPCKm Synonyms: _5 EC Number: 4.1.1.32 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [m] : gtp + oaa --> co2 + gdp + pep Confidence Level: 0 genes: LOCUS:_5106#TRANSCRIPT:1#ABBREVIATION:PCK2#ECNUMBERS:4.1.1.32# proteins: NAME:phosphoenolpyruvate carboxykinase 2, mitochondrial (liver, kidney, intestine)#ABBREVIATION:Pck2.1-m# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Modaressi S, Christ B, Bratke J, Zahn S, Heise T, Jungermann K.#TITLE:Molecular cloning, sequencing and expression of the cDNA of the mitochondrial form of phosphoenolpyruvate carboxykinase from human liver.#JOURNAL:Biochem J#YEAR:1996#PUBMED:8645161# GENE ASSOCIATION: (5106.1)
Abbreviation: R_CYTK6 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : cmp + ctp <==> (2) cdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_GASNASEly Synonyms: _8 EC Number: 3.5.1.26 SUBSYSTEM: N-Glycan Degradation Equation: [l] : h2o + s2l2n2m2masn --> Asn-X-Ser/Thr + s2l2n2m2m Confidence Level: 0 NCD genes: LOCUS:_175#TRANSCRIPT:1#ABBREVIATION:AGA#ECNUMBERS:3.5.1.26# proteins: NAME:aspartylglucosaminidase, lysosomal#ABBREVIATION:Aga.1-l# citations: none GENE ASSOCIATION: (175.1)
Abbreviation: R_OMEPRAZOLEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: omeprazole[e] <==> omeprazole[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TRDR Synonyms: _0 EC Number: 1.8.1.9 SUBSYSTEM: Nucleotides Equation: [c] : h + nadph + trdox --> nadp + trdrd Confidence Level: 4 genes: LOCUS:_7296#TRANSCRIPT:1#ABBREVIATION:TXNRD1# LOCUS:_7296#TRANSCRIPT:4#ABBREVIATION:TXNRD1# LOCUS:_7296#TRANSCRIPT:2#ABBREVIATION:TXNRD1# LOCUS:_7296#TRANSCRIPT:3#ABBREVIATION:TXNRD1# proteins: NAME:thioredoxin reductase 1 #ABBREVIATION:Txnrd1.1# NAME:thioredoxin reductase 1#ABBREVIATION:Txnrd1.2# NAME:thioredoxin reductase 1#ABBREVIATION:Txnrd1.3# NAME:thioredoxin reductase 1#ABBREVIATION:Txnrd1.4# citations: AUTHORS:Tamura T, Stadtman TC.#TITLE:A new selenoprotein from human lung adenocarcinoma cells: purification, properties, and thioredoxin reductase activity. #JOURNAL:Proc Natl Acad Sci U S A. #YEAR:1996#PUBMED:8577704# AUTHORS:Koishi R, Kawashima I, Yoshimura C, Sugawara M, Serizawa N.#TITLE:Cloning and characterization of a novel oxidoreductase KDRF from a human bone marrow-derived stromal cell line KM-102. #JOURNAL:J Biol Chem. #YEAR:1997#PUBMED:8999974# GENE ASSOCIATION: (7296.3) or (7296.1) or (7296.4) or (7296.2)
Abbreviation: R_CYTDt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cytd[e] + na1[e] --> cytd[c] + na1[c] Confidence Level: 4 SAB genes: LOCUS:_9154#TRANSCRIPT:1#ABBREVIATION:SLC28A1# LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# LOCUS:_9154#TRANSCRIPT:2#ABBREVIATION:SLC28A1# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 1, extracellular #ABBREVIATION:Slc28a1.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 1 #ABBREVIATION:Slc28a1.2-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (9154.1) or (9154.2) or (64078.1)
cytidine transport in via sodium symport
Abbreviation: R_FPGS8m Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [m] : 10fthf5glu + atp + glu-L --> 10fthf6glu + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_EX_25hvitd2_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 25hvitd2 <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
L-aminoadipate-semialdehyde dehydrogenase (NADH), mitochondrial
Abbreviation: R_AASAD3m Synonyms: _0 EC Number: 1.2.1.31 SUBSYSTEM: Lysine Metabolism Equation: [m] : L2aadp6sa + h2o + nad --> L2aadp + (2) h + nadh Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_EX_fucacngal14acglcgalgluside_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fucacngal14acglcgalgluside_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_TYMSULT Synonyms: _2 EC Number: 2.8.2.1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : paps + tym --> h + pap + tymsf Confidence Level: 4 TV/RS (6/14/05) genes: LOCUS:_6818#TRANSCRIPT:1#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:1#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:2#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:3#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:4#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:5#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6818#TRANSCRIPT:2#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# LOCUS:_6818#TRANSCRIPT:3#ABBREVIATION:SULT1A3#ECNUMBERS:2.8.2.1# proteins: NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.2# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.3# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.4# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.5# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.2# NAME:sulfotransferase family, cytosolic, 1A, phenol-preferring, member 3 #ABBREVIATION:Sult1a3.3# citations: AUTHORS:Chapman E, Best MD, Hanson SR, Wong CH.#TITLE:Sulfotransferases: structure, mechanism, biological activity, inhibition, and synthetic utility.#JOURNAL:Angew Chem Int Ed Engl#YEAR:2004#PUBMED:15293241# AUTHORS:Glatt H, Boeing H, Engelke CE, Ma L, Kuhlow A, Pabel U, Pomplun D, Teubner W, Meinl W.#TITLE:Human cytosolic sulphotransferases: genetics, characteristics, toxicological aspects.#JOURNAL:Mutat Res#YEAR:2001#PUBMED:11535246# GENE ASSOCIATION: (6818.3) or (6818.1) or (6817.4) or (6817.1) or (6817.3) or (6818.2) or (6817.2) or (6817.5)
Abbreviation: R_RBK Synonyms: _0 EC Number: 2.7.1.15 SUBSYSTEM: Pentose Phosphate Pathway Equation: [c] : atp + rib-D --> adp + h + r5p Confidence Level: 2 genes: LOCUS:_64080#TRANSCRIPT:1#ABBREVIATION:RBKS#ECNUMBERS:2.7.1.15# proteins: NAME:ribokinase#ABBREVIATION:Rbks.1# citations: AUTHORS:Agranoff BW, Brady RO#TITLE:Purification and properties of calf liver ribokinase#JOURNAL:J Biol Chem#YEAR:1956#PUBMED:13295274# AUTHORS:Segal S, Foley J#TITLE:The metabolism of D-ribose in man.#JOURNAL:J Clin Invest#YEAR:1958#PUBMED:13539215# GENE ASSOCIATION: (64080.1)
Abbreviation: R_GLNtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: gln-L[c] --> gln-L[m] Confidence Level: 2 TV (6/7/2005) Study in rat liver (Soboll, 1991) genes: none proteins: none citations: none GENE ASSOCIATION:
L-glutamine transport via electroneutral transporter
Abbreviation: R_S6TASE19ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg30 <==> h + ksi_deg31 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
N-Methylputrescine:oxygen oxidoreductase (deaminating)
Abbreviation: R_NMPTRCOX Synonyms: _0 EC Number: 1.4.3.6 SUBSYSTEM: Alkaloid biosynthesis II Equation: [c] : nmptrc + o2 --> 1mpyr + h2o2 + nh4 Confidence Level: 2 SAB genes: LOCUS:_314#TRANSCRIPT:1#ABBREVIATION:AOC2#ECNUMBERS:1.4.3.6# LOCUS:_8639#TRANSCRIPT:1#ABBREVIATION:AOC3#ECNUMBERS:1.4.3.6# LOCUS:_26#TRANSCRIPT:1#ABBREVIATION:ABP1#ECNUMBERS:1.4.3.6# LOCUS:_314#TRANSCRIPT:2#ABBREVIATION:AOC2# proteins: NAME:amine oxidase, copper containing 3#ABBREVIATION:Aoc3.1# NAME:amiloride binding protein 1 (amine oxidase (copper-containing))#ABBREVIATION:Abp1.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.1# NAME:amine oxidase, copper containing 2#ABBREVIATION:Aoc2.2# citations: AUTHORS:Frydman J, Ruiz O, Robetto E, Dellacha JM, Frydman RB#TITLE:Modulation of insulin induced ornithine decarboxylase by putrescine and methylputrescines in H-35 hepatoma cells#JOURNAL:Mol Cell Biochem#YEAR:1991#PUBMED:2051998# AUTHORS:Frydman RB, Ruiz O, Kreisel M, Bachrach U#TITLE:Oxidation of N-alkyl and C-alkylputrescines by diamine oxidases#JOURNAL:FEBS Lett#YEAR:1987#PUBMED:3111885# GENE ASSOCIATION: (26.1) or (314.1) or (8639.1) or (314.2)
Abbreviation: R_DLNLCGCPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + dlnlcgcrn --> crn + dlnlcgcoa Confidence Level: 4 NJ genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_EX_arab_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : arab-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
GDPmannose:chitobiosyldiphosphodolichol beta-D-mannosyltransferase (liver)
Abbreviation: R_BDMT_L Synonyms: _0 EC Number: 2.4.1.142 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : (0.1) chito2pdol_L + gdpmann --> gdp + h + (0.1) mpdol_L Confidence Level: 0 NCD genes: LOCUS:_56052#TRANSCRIPT:1#ABBREVIATION:ALG1# proteins: NAME:beta-1,4-mannosyltransferase#ABBREVIATION:Alg1.1# citations: none GENE ASSOCIATION: (56052.1)
Abbreviation: R_UDPGLCtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: udpg[c] + ump[g] <==> udpg[g] + ump[c] Confidence Level: 4 NCD genes: LOCUS:_84912#TRANSCRIPT:1#ABBREVIATION:SLC35B4# LOCUS:_23443#TRANSCRIPT:1#ABBREVIATION:SLC35A3# LOCUS:_11046#TRANSCRIPT:1#ABBREVIATION:SLC35D2# proteins: NAME:solute carrier family 35 (UDP-N-acetylglucosamine (UDP-GlcNAc) transporter), member A3#ABBREVIATION:Slc35a3.1-g# NAME:solute carrier family 35, member D2#ABBREVIATION:Slc35d2.1-g# NAME:solute carrier family 35, member B4, Golgi apparatus#ABBREVIATION:Slc35b4.1-g# citations: AUTHORS:Ishida N, Yoshioka S, Chiba Y, Takeuchi M, Kawakita M#TITLE:Molecular cloning and functional expression of the human Golgi UDP-N-acetylglucosamine transporter#JOURNAL:J Biochem (Tokyo)#YEAR:1999#PUBMED:10393322# AUTHORS:Suda T, Kamiyama S, Suzuki M, Kikuchi N, Nakayama K, Narimatsu H, Jigami Y, Aoki T, Nishihara S#TITLE:Molecular cloning and characterization of a human multisubstrate specific nucleotide-sugar transporter homologous to Drosophila fringe connection#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15082721# AUTHORS:Ishida N, Kuba T, Aoki K, Miyatake S, Kawakita M, Sanai Y#TITLE:Identification and characterization of human Golgi nucleotide sugar transporter SLC35D2, a novel member of the SLC35 nucleotide sugar transporter family#JOURNAL:Genomics#YEAR:2005#PUBMED:15607426# AUTHORS:Ashikov A, Routier F, Fuhlrott J, Helmus Y, Wild M, Gerardy-Schahn R, Bakker H#TITLE:The Human Solute Carrier Gene SLC35B4 Encodes a Bifunctional Nucleotide Sugar Transporter with Specificity for UDP-Xylose and UDP-N-Acetylglucosamine#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15911612# AUTHORS:Nuwayhid N, Glaser JH, Johnson JC, Conrad HE, Hauser SC, Hirschberg CB#TITLE:Xylosylation and glucuronosylation reactions in rat liver Golgi apparatus and endoplasmic reticulum#JOURNAL:J Biol Chem#YEAR:1986#PUBMED:3093474# AUTHORS:Milla ME, Clairmont CA, Hirschberg CB#TITLE:Reconstitution into proteoliposomes and partial purification of the Golgi apparatus membrane UDP-galactose, UDP-xylose, and UDP-glucuronic acid transport activities#JOURNAL:J Biol Chem#YEAR:1992#PUBMED:1730575# GENE ASSOCIATION: (23443.1) or (84912.1) or (11046.1)
UDP-Glc Golgi transport via CMP antiport
N-acetyl-g-glutamyl-phosphate reductase, irreversible, mitochondrial
Abbreviation: R_AGPRim Synonyms: _0 EC Number: 1.2.1.38 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : acg5p + h + nadph --> acg5sa + nadp + pi Confidence Level: 1 NCD genes: LOCUS:_5832#TRANSCRIPT:1#ABBREVIATION:ALDH18A1# proteins: NAME:aldehyde dehydrogenase 18 family, member A1#ABBREVIATION:Aldh18a1.1-m# citations: none GENE ASSOCIATION: (5832.1)
Abbreviation: R_PAN4PP Synonyms: _0 SUBSYSTEM: CoA Catabolism Equation: [c] : h2o + pan4p --> pi + ptth Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# GENE ASSOCIATION:
Abbreviation: R_HS3ly Synonyms: _4 EC Number: 3.10.1.1 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg12 --> h + hs_deg13 + so4 Confidence Level: 4 NCD genes: LOCUS:_6448#TRANSCRIPT:1#ABBREVIATION:SGSH# proteins: NAME:N-sulfoglucosamine sulfohydrolase (sulfamidase), lysosomal#ABBREVIATION:Sgsh.1-l# citations: AUTHORS:Scott HS, Blanch L, Guo XH, Freeman C, Orsborn A, Baker E, Sutherland GR, Morris CP, Hopwood JJ#TITLE:Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7493035# AUTHORS:Bielicki J, Hopwood JJ, Melville EL, Anson DS#TITLE:Recombinant human sulphamidase: expression, amplification, purification and characterization#JOURNAL:Biochem J#YEAR:1998#PUBMED:9405287# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION: (6448.1)
Abbreviation: R_EX_thyox_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : thyox-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_tymsf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tymsf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PCHOLPm_hs Synonyms: _0 EC Number: 3.1.4.4 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [m] : h2o + pchol_hs --> chol + h + pa_hs Confidence Level: 4 NJ genes: LOCUS:_5337#TRANSCRIPT:1#ABBREVIATION:PLD1#ECNUMBERS:3.1.4.4# proteins: NAME:phospholipase D1, phosphatidylcholine-specific#ABBREVIATION:Pld1.1# citations: AUTHORS:Cases S, Stone SJ, Zhou P, Yen E, Tow B, Lardizabal KD, Voelker T, Farese RV Jr.#TITLE:Cloning of DGAT2, a second mammalian diacylglycerol acyltransferase, and related family members.#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11481335# AUTHORS:Hammond SM, Altshuller YM, Sung TC, Rudge SA, Rose K, Engebrecht J, Morris AJ, Frohman MA.#TITLE:Human ADP-ribosylation factor-activated phosphatidylcholine-specific phospholipase D defines a new and highly conserved gene family.#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:8530346# GENE ASSOCIATION: (5337.1)
Abbreviation: R_DMGDHm Synonyms: _0 EC Number: 1.5.99.2 SUBSYSTEM: Glycine, Serine, and Threonine Metabolism Equation: [m] : dmgly + fad + h2o --> fadh2 + fald + sarcs Confidence Level: 4 MM genes: LOCUS:_29958#TRANSCRIPT:1#ABBREVIATION:DMGDH#ECNUMBERS:1.5.99.2# proteins: NAME:dimethylglycine dehydrogenase precursor, mitochondrial #ABBREVIATION:Dmgdh.1-m# citations: AUTHORS:Binzak BA, Wevers RA, Moolenaar SH#TITLE:Cloning of dimethylglycine dehydrogenase and a new human inborn error of metabolism, dimethylglycine dehydrogenase deficiency.#YEAR:2001#PUBMED:1123190# AUTHORS:Binzak,B.A. , Vockley,J.G. , Jenkins,R.B. , Vockley,J. #TITLE:Structure and analysis of the human dimethylglycine dehydrogenase gene.#YEAR:2000#PUBMED:10767172# GENE ASSOCIATION: (29958.1)
dimethylglycine dehydrogenase, mitochondrial
Abbreviation: R_ARAB_DASH_Lt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: arab-L[e] <==> arab-L[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Onkenhout W, Groener JE, Verhoeven NM, Yin C, Laan LA#TITLE:L-Arabinosuria: a new defect in human pentose metabolism#JOURNAL:Mol Genet Metab#YEAR:2002#PUBMED:12359133# AUTHORS:Huck JH, Roos B, Jakobs C, van der Knaap MS, Verhoeven NM#TITLE:Evaluation of pentitol metabolism in mammalian tissues provides new insight into disorders of human sugar metabolism#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15234337# AUTHORS:Lobley RW, Burrows PC, Warwick R, Dawson DJ, Holmes R#TITLE:Simultaneous assessment of intestinal permeability and lactose tolerance with orally administered raffinose, lactose and L-arabinose#JOURNAL:Clin Sci (Lond).#YEAR:1990#PUBMED:2167807# GENE ASSOCIATION:
Abbreviation: R_ALOX12 Synonyms: _2 EC Number: 1.13.11.31 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : arachd + o2 --> 12HPET Confidence Level: 4 NJ genes: LOCUS:_239#TRANSCRIPT:1#ABBREVIATION:ALOX12#ECNUMBERS:1.13.11.31# proteins: NAME:arachidonate 12-lipoxygenase#ABBREVIATION:Alox12.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Izumi T, Hoshiko S, Radmark O, Samuelsson B.#TITLE:Cloning of the cDNA for human 12-lipoxygenase.#JOURNAL:Proc Natl Acad Sci #YEAR:1990#PUBMED:2217179# AUTHORS:Funk CD, Furci L, FitzGerald GA.#TITLE:Molecular cloning, primary structure, and expression of the human platelet/erythroleukemia cell 12-lipoxygenase.#JOURNAL:Proc Natl Acad Sci #YEAR:1990#PUBMED:2377602# GENE ASSOCIATION: (239.1)
Abbreviation: R_RETNGLCtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: retnglc[r] --> retnglc[c] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Barua AB, Sidell N.#TITLE:Retinoyl beta-glucuronide: a biologically active interesting retinoid.#JOURNAL:J Nutr.#YEAR:2004#PUBMED:14704335# AUTHORS:Tang GW, Russell RM.#TITLE:_13-cis-retinoic acid is an endogenous compound in human serum.#JOURNAL:J Lipid Res.#YEAR:1990#PUBMED:2324641# GENE ASSOCIATION:
Abbreviation: R_AASAD3 Synonyms: _0 EC Number: 1.2.1.31 SUBSYSTEM: Lysine Metabolism Equation: [c] : L2aadp6sa + h2o + nad --> L2aadp + (2) h + nadh Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:Cox, RP#TITLE:Errors of lysine metabolism#JOURNAL:The Metabolic and Molecular Bases of Inherited Disease, 8th ed#YEAR:2001# GENE ASSOCIATION:
L-aminoadipate-semialdehyde dehydrogenase (NADH)
Abbreviation: R_EX_taur_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : taur <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ST8SIA12 Synonyms: _13 EC Number: 2.4.99.8 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gm3_hs --> cmp + gd3_hs + h Confidence Level: 4 NJ genes: LOCUS:_6489#TRANSCRIPT:1#ABBREVIATION:SIAT8A#ECNUMBERS:2.4.99.8# proteins: NAME:ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase 1#ABBREVIATION:St8sia1.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Sasaki K, Kurata K, Kojima N, Kurosawa N, Ohta S, Hanai N, Tsuji S, Nishi T#TITLE:Expression cloning of a Gm3-specific alpha-2,8-sialyltransferase (GD3 synthase)#JOURNAL:Journal of Biological Chemistry#YEAR:1994# AUTHORS:Kim YJ, Kim KS, Do S, Kim CH, Kim SK, Lee YC.#TITLE:Molecular cloning and expression of human alpha2,8-sialyltransferase (hST8Sia V).#JOURNAL:Biochem Biophys Res Commun.#YEAR:1997#PUBMED:9199191# GENE ASSOCIATION: (6489.1)
Abbreviation: R_C161CPT2 Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: [m] : coa + hdcecrn --> crn + hdcoa Confidence Level: 4 TDV genes: LOCUS:_1376#TRANSCRIPT:1#ABBREVIATION:CPT2#ECNUMBERS:2.3.1.21# proteins: NAME:Carnitine palmitoyltransferase II, mitochondrial #ABBREVIATION:Cpt2.1-m# citations: none GENE ASSOCIATION: (1376.1)
Abbreviation: R_PI45PLCn Synonyms: _6 EC Number: 3.1.4.11 SUBSYSTEM: Inositol Phosphate Metabolism Equation: [n] : h2o + pail45p_hs --> dag_hs + h + mi145p Confidence Level: 4 NCD genes: LOCUS:_23236#TRANSCRIPT:1#ABBREVIATION:PLCB1#ECNUMBERS:3.1.4.11# LOCUS:_23236#TRANSCRIPT:2#ABBREVIATION:PLCB1# proteins: NAME:phospholipase C, beta 1 (phosphoinositide-specific), nucleus#ABBREVIATION:Plcb1.2-n# NAME:phospholipase C, beta 1 (phosphoinositide-specific), nucleus#ABBREVIATION:Plcb1.1-n# citations: AUTHORS:Peruzzi D, Aluigi M, Manzoli L, Billi AM, Di Giorgio FP, Morleo M, Martelli AM, Cocco L#TITLE:Molecular characterization of the human PLC beta1 gene#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12213492# AUTHORS:Caricasole A, Sala C, Roncarati R, Formenti E, Terstappen GC#TITLE:Cloning and characterization of the human phosphoinositide-specific phospholipase C-beta 1 (PLC beta 1)#JOURNAL:Biochim Biophys Acta#YEAR:2000#PUBMED:11118617# AUTHORS:Peruzzi D, Calabrese G, Faenza I, Manzoli L, Matteucci A, Gianfrancesco F, Billi AM, Stuppia L, Palka G, Cocco L#TITLE:Identification and chromosomal localisation by fluorescence in situ hybridisation of human gene of phosphoinositide-specific phospholipase C beta(1)#JOURNAL:Biochim Biophy Acta#YEAR:2000#PUBMED:10760467# AUTHORS:Irvine RF#TITLE:Nuclear lipid signalling#JOURNAL:Nat Rev Mol Cell Biol#YEAR:2003#PUBMED:12728269# GENE ASSOCIATION: (23236.1) or (23236.2)
phosphatidylinositol 4,5-bisphosphate phospholipase C, nucleus
Abbreviation: R_EX_fol_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : fol <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_BILGLCURt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: bilglcur[e] + hco3[c] <==> bilglcur[c] + hco3[e] Confidence Level: 4 NJ genes: LOCUS:_28234#TRANSCRIPT:1#ABBREVIATION:SLCO1B3# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# NAME:solute carrier organic anion transporter family, member 1B3#ABBREVIATION:Slco1b3.1# citations: AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# AUTHORS:Abe T, Kakyo M, Tokui T, Nakagomi R, Nishio T, Nakai D, Nomura H, Unno M, Suzuki M, Naitoh T, Matsuno S, Yawo H.#TITLE:Identification of a novel gene family encoding human liver-specific organic anion transporter LST-1.#JOURNAL:J Biol Chem.#YEAR:1999#PUBMED:10358072# GENE ASSOCIATION: (10599.1) or (28234.1)
bilirubin monoglucuronide transport via bicarbonate countertransport
Abbreviation: R_B3GNT34g Synonyms: _9 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : galgbside_hs + uacgam --> acglcgalgbside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10331.1)
Abbreviation: R_LYSOXp Synonyms: _0 EC Number: 1.4.3.14 SUBSYSTEM: Lysine Metabolism Equation: [x] : h2o + lys-L + o2 --> 6a2ohxnt + h2o2 + nh4 Confidence Level: 2 MM genes: none proteins: none citations: AUTHORS:IJlst L, de Kromme I, Oostheim W, Wanders RJ.#TITLE:Molecular cloning and expression of human L-pipecolate oxidase.#YEAR:2000#PUBMED:10772957# AUTHORS:Murthy SN, Janardanasarma MK.#TITLE:Identification of L-amino acid/L-lysine alpha-amino oxidase in mouse brain.#YEAR:1999#PUBMED:10485319# AUTHORS:Yung-Feng Chang#TITLE:Lysine metabolism in the human and the monkey: Demonstration of pipecolic acid formation in the brain and other organs#YEAR:1982#PUBMED:6811962# GENE ASSOCIATION:
Abbreviation: R_NACHEXA11ly Synonyms: _0 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : (2) h2o + ksi_deg12 --> acgam + h + ksi_deg14 + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_FORt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: for[m] <==> for[c] Confidence Level: 1 NCD,IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ACNAMPH Synonyms: _0 EC Number: 3.1.3.29 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acnamp + h2o --> acnam + pi Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
N-Acetylneuraminate 9-phosphate phosphohydrolase
Abbreviation: R_EX_lac_DASH_D_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : lac-D <==> Confidence Level: JLR genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_BTNt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: btn[e] + h[e] <==> btn[c] + h[c] Confidence Level: 4 NCD genes: LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1#ABBREVIATION:Slc16a1.1# citations: AUTHORS:Daberkow RL, White BR, Cederberg RA, Griffin JB, Zempleni J.#TITLE:Monocarboxylate transporter 1 mediates biotin uptake in human peripheral blood mononuclear cells.#JOURNAL:J Nutr. #YEAR:2003# AUTHORS:Halestrap AP, Price NT#TITLE:The proton-linked monocarboxylate transporter (MCT) family: structure, function and regulation#JOURNAL:Biochem J#YEAR:1999#PUBMED:10510291# AUTHORS:Garcia CK, Li X, Luna J, Francke U#TITLE:cDNA cloning of the human monocarboxylate transporter 1 and chromosomal localization of the SLC16A1 locus to 1p13.2-p12#JOURNAL:Genomics#YEAR:1994#PUBMED:7835905# AUTHORS:Poole RC, Halestrap AP#TITLE:Transport of lactate and other monocarboxylates across mammalian plasma membranes#JOURNAL:Am J Physiol#YEAR:1993#PUBMED:8476015# AUTHORS:Ritzhaupt A, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:The characterization of butyrate transport across pig and human colonic luminal membrane#JOURNAL:J Physiol#YEAR:1998#PUBMED:9508842# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification of a monocarboxylate transporter isoform type 1 (MCT1) on the luminal membrane of human and pig colon#JOURNAL:Biochem Soc Trans#YEAR:1998#PUBMED:9649795# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification and characterization of a monocarboxylate transporter (MCT1) in pig and human colon: its potential to transport L-lactate as well as butyrate#JOURNAL:J Physiol#YEAR:1998#PUBMED:9824713# AUTHORS:Halestrap AP, Meredith D#TITLE:The SLC16 gene family-from monocarboxylate transporters (MCTs) to aromatic amino acid transporters and beyond#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739169# GENE ASSOCIATION: (6566.1)
Biotin reversible transport via proton symport
Abbreviation: R_GLCAT7g Synonyms: _2 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre3 + udpglcur --> h + hs_pre4 + udp Confidence Level: 4 EC 2.4.1.225 NCD genes: LOCUS:_2131#TRANSCRIPT:1#ABBREVIATION:EXT1# LOCUS:_2132#TRANSCRIPT:1#ABBREVIATION:EXT2#ECNUMBERS:2.4.1.-# LOCUS:_2132#TRANSCRIPT:2#ABBREVIATION:EXT2# proteins: NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.1-g# NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.2-g# citations: AUTHORS:McCormick C, Duncan G, Goutsos KT, Tufaro F#TITLE:The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10639137# AUTHORS:Kobayashi S, Morimoto K, Shimizu T, Takahashi M, Kurosawa H, Shirasawa T#TITLE:Association of EXT1 and EXT2, hereditary multiple exostoses gene products, in Golgi apparatus#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679296# AUTHORS:Ahn J, Ludecke HJ, Lindow S, Horton WA, Lee B, Wagner MJ, Horsthemke B, Wells DE#TITLE:Cloning of the putative tumour suppressor gene for hereditary multiple exostoses (EXT1)#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7550340# AUTHORS:McCormick C, Leduc Y, Martindale D, Mattison K, Esford LE, Dyer AP, Tufaro F#TITLE:The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate#JOURNAL:Nat Genet#YEAR:1998#PUBMED:9620772# AUTHORS:Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K#TITLE:The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9756849# AUTHORS:Stickens D, Clines G, Burbee D, Ramos P, Thomas S, Hogue D, Hecht JT, Lovett M, Evans GA#TITLE:The EXT2 multiple exostoses gene defines a family of putative tumour suppressor genes#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8782816# GENE ASSOCIATION: (2131.1 and 2132.1) or (2131.1 and 2132.2)
Abbreviation: R_EX_triodthysuf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : triodthysuf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DNAMTSEn Synonyms: _30 EC Number: 2.1.1.37 SUBSYSTEM: Selenoamino acid metabolism Equation: [n] : dna + seasmet --> dna5mtc + h + seahcys Confidence Level: 1 SAB copied from DNAMTn genes: LOCUS:_1786#TRANSCRIPT:1#ABBREVIATION:DNMT1#ECNUMBERS:2.1.1.37# LOCUS:_1789#TRANSCRIPT:1#ABBREVIATION:DNMT3B#ECNUMBERS:2.1.1.37# LOCUS:_1789#TRANSCRIPT:2#ABBREVIATION:DNMT3B#ECNUMBERS:2.1.1.37# LOCUS:_1789#TRANSCRIPT:3#ABBREVIATION:DNMT3B#ECNUMBERS:2.1.1.37# LOCUS:_1789#TRANSCRIPT:4#ABBREVIATION:DNMT3B#ECNUMBERS:2.1.1.37# LOCUS:_1788#TRANSCRIPT:1#ABBREVIATION:DNMT3A#ECNUMBERS:2.1.1.37# LOCUS:_1788#TRANSCRIPT:2#ABBREVIATION:DNMT3A#ECNUMBERS:2.1.1.37# LOCUS:_1788#TRANSCRIPT:3#ABBREVIATION:DNMT3A#ECNUMBERS:2.1.1.37# LOCUS:_1788#TRANSCRIPT:4#ABBREVIATION:DNMT3A#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:1#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:2#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:3#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:4#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:5#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# LOCUS:_1787#TRANSCRIPT:6#ABBREVIATION:DNMT2#ECNUMBERS:2.1.1.37# proteins: NAME:DNA (cytosine-5-)-methyltransferase 1, nuclear #ABBREVIATION:Dnmt1.1-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.1-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.2-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.3-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.4-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.5-n# NAME:DNA (cytosine-5-)-methyltransferase 2, nuclear #ABBREVIATION:Dnmt2.6-n# NAME:DNA (cytosine-5-)-methyltransferase 3, nuclear #ABBREVIATION:Dnmt3a.1-n# NAME:DNA (cytosine-5-)-methyltransferase 3, nuclear #ABBREVIATION:Dnmt3a.2-n# NAME:DNA (cytosine-5-)-methyltransferase 3, nuclear #ABBREVIATION:Dnmt3a.3-n# NAME:DNA (cytosine-5-)-methyltransferase 3, nuclear #ABBREVIATION:Dnmt3a.4-n# NAME:DNA (cytosine-5-)-methyltransferase 3 beta, nuclear #ABBREVIATION:Dnmt3b.1-n# NAME:DNA (cytosine-5-)-methyltransferase 3 beta, nuclear #ABBREVIATION:Dnmt3b.2-n# NAME:DNA (cytosine-5-)-methyltransferase 3 beta, nuclear #ABBREVIATION:Dnmt3b.3-n# NAME:DNA (cytosine-5-)-methyltransferase 3 beta, nuclear #ABBREVIATION:Dnmt3b.4-n# citations: AUTHORS:Katsuhiko Nakamuro, Tomofumi Okuno, and Tatsuya Hasegawa#TITLE:Metabolism of Selenoamino Acids and Contribution of Selenium Methylation to Their Toxicity#JOURNAL:Journal of Health Science#YEAR:2000#PUBMED:?# GENE ASSOCIATION: (1787.5) or (1788.4) or (1787.2) or (1787.6) or (1788.2) or (1789.1) or (1787.1) or (1789.4) or (1789.3) or (1788.1) or (1786.1) or (1787.3) or (1789.2) or (1788.3) or (1787.4)
Abbreviation: R_PLA2_2e Synonyms: _1 EC Number: 3.1.1.4 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [e] : h2o + pchol_hs --> Rtotal2 + h + lpchol_hs Confidence Level: 4 phosphatidyl choline degradation NJ genes: LOCUS:_26279#TRANSCRIPT:1#ABBREVIATION:PLA2G2D# LOCUS:_5320#TRANSCRIPT:1#ABBREVIATION:PLA2G2A# LOCUS:_5322#TRANSCRIPT:1#ABBREVIATION:PLA2G5#ECNUMBERS:3.1.1.4# LOCUS:_30814#TRANSCRIPT:1#ABBREVIATION:PLA2G2E#ECNUMBERS:3.1.1.4# LOCUS:_5319#TRANSCRIPT:1#ABBREVIATION:PLA2G1B#ECNUMBERS:3.1.1.4# LOCUS:_8399#TRANSCRIPT:1#ABBREVIATION:PLA2G10# LOCUS:_81579#TRANSCRIPT:1#ABBREVIATION:PLA2G12A# LOCUS:_64600#TRANSCRIPT:1#ABBREVIATION:PLA2G2F# proteins: NAME:phospholipase A2, group 1B (pancreas)#ABBREVIATION:Pla2g1b.1# NAME:phospholipase A2, group XIIA#ABBREVIATION:Pla2g12a.1# NAME:phospholipase A2, group IIE#ABBREVIATION:Pla2g2e.1# NAME:phospholipase A2, groupIIA (platelets, synovial fluid)#ABBREVIATION:Pla2g2a.1# NAME:phospholipase A2, group V#ABBREVIATION:Pla2g5.1# NAME:phosphlipase A2, group IIF#ABBREVIATION:Pla2g2f.1# NAME:phospholipase A2, goup X#ABBREVIATION:Pla2g10.1# NAME:phospholipase A2, group IID#ABBREVIATION:Pla2g2d.1# citations: AUTHORS: Nardelli B, Tiffany HL, Bong GW, Yourey PA, Morahan DK, Li Y, Murphy PM, Alderson RF.#TITLE:Characterization of the signal transduction pathway activated in human monocytes and dendritic cells by MPIF-1, a specific ligand for CC chemokine receptor 1.#JOURNAL:J Immunol#YEAR:1999#PUBMED:9886417# AUTHORS:Gelb MH, Valentin E, Ghomashchi F, Lazdunski M, Lambeau G.#TITLE:Cloning and recombinant expression of a structurally novel human secreted phospholipase A2.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:11031251# AUTHORS:Suzuki N, Ishizaki J, Yokota Y, Higashino K, Ono T, Ikeda M, Fujii N, Kawamoto K, Hanasaki K.#TITLE:Structures, enzymatic properties, and expression of novel human and mouse secretory phospholipase A(2)s.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10681567# AUTHORS:Nimmrich I, Friedl W, Kruse R, Pietsch S, Hentsch S, Deuter R, Winde G, Muller O.#TITLE:Loss of the PLA2G2A gene in a sporadic colorectal tumor of a patient with a PLA2G2A germline mutation and absence of PLA2G2A germline alterations in patients with FAP.#JOURNAL:Hum Genet#YEAR:1997#PUBMED:9272153# AUTHORS:Chen J, Engle SJ, Seilhamer JJ, Tischfield JA.#TITLE:Cloning and recombinant expression of a novel human low molecular weight Ca(2+)-dependent phospholipase A2.#JOURNAL:J Biol Chem#YEAR:1994#PUBMED:8300559 [# AUTHORS:Valentin E, Singer AG, Ghomashchi F, Lazdunski M, Gelb MH, Lambeau G.#TITLE:Cloning and recombinant expression of human group IIF-secreted phospholipase A(2).#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:11112443# AUTHORS:Cupillard L, Koumanov K, Mattei MG, Lazdunski M, Lambeau G.#TITLE:Cloning, chromosomal mapping, and expression of a novel human secretory phospholipase A2.#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9188469# AUTHORS:Ishizaki J, Suzuki N, Higashino K, Yokota Y, Ono T, Kawamoto K, Fujii N, Arita H, Hanasaki K.#TITLE:Cloning and characterization of novel mouse and human secretory phospholipase A(2)s.#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10455175# GENE ASSOCIATION: (5322.1) or (30814.1) or (5319.1) or (64600.1) or (26279.1) or (5320.1) or (8399.1) or (81579.1)
Abbreviation: R_RNDR2 Synonyms: _0 EC Number: 1.17.4.1 SUBSYSTEM: Nucleotides Equation: [c] : gdp + trdrd --> dgdp + h2o + trdox Confidence Level: 2 genes: LOCUS:_6241#TRANSCRIPT:1#ABBREVIATION:RRM2#ECNUMBERS:1.17.4.1# LOCUS:_6240#TRANSCRIPT:1#ABBREVIATION:RRM1#ECNUMBERS:1.7.14.1# proteins: NAME:ribonucleotide reductase #ABBREVIATION:Rrm12.1# citations: AUTHORS:Shao J, Zhou B, Zhu L, Qiu W, Yuan YC, Xi B, Yen Y.#TITLE: In vitro characterization of enzymatic properties and inhibition of the p53R2 subunit of human ribonucleotide reductase. #JOURNAL:Cancer Res. #YEAR:2004#PUBMED:14729598# GENE ASSOCIATION: (6240.1 and 6241.1)
ribonucleoside-diphosphate reductase (GDP)
Abbreviation: R_HPACtr Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _4hphac[c] <==> 4hphac[e] Confidence Level: 2 SAB genes: none proteins: none citations: AUTHORS:Shimamura M, Kamada S, Hayashi T, Naruse H, Iida Y.#TITLE:Sensitive determination of tyrosine metabolites, p-hydroxyphenylacetic acid, 4-hydroxy-3-methoxyphenyl-acetic acid and 4-hydroxy-3-methoxymandelic acid, by gas chromatography-negative-ion chemical-ionization mass spectrometry.#JOURNAL:J Chromatogr#YEAR:1986#PUBMED:3753983# GENE ASSOCIATION:
hydroxyphenylacetate transport via diffusion
Abbreviation: R_SFGTH Synonyms: _0 EC Number: 3.1.2.12 SUBSYSTEM: Tyrosine metabolism Equation: [c] : Sfglutth + h2o <==> for + gthrd + h Confidence Level: 4 NCD genes: LOCUS:_2098#TRANSCRIPT:1#ABBREVIATION:ESD#ECNUMBERS:3.1.1.1# proteins: NAME:esterase D/formylglutathione hydrolase#ABBREVIATION:Esd.1# citations: AUTHORS:Engeland K, Hoog JO, Holmquist B, Estonius M, Jornvall H, Vallee BL#TITLE:Mutation of Arg-115 of human class III alcohol dehydrogenase: a binding site required for formaldehyde dehydrogenase activity and fatty acid activation#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1993#PUBMED:8460164# AUTHORS:Uotila L, Koivusalo M#TITLE:Purification and properties of S-formylglutathione hydrolase from human liver#JOURNAL:J Biol Chem#YEAR:1974#PUBMED:4436331# GENE ASSOCIATION: (2098.1)
Abbreviation: R_CO2tn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: co2[n] <==> co2[c] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_KSII_CORE2tly Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: ksii_core2[e] --> ksii_core2[l] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
keratan sulfate II (core 2) transport, extracellular to lysosome
Abbreviation: R_CYTK7 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : cmp + utp <==> cdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_H2O2tp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: h2o2[c] <==> h2o2[x] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Ohno Y, Gallin JI. #TITLE:Diffusion of extracellular hydrogen peroxide into intracellular compartments of human neutrophils. Studies utilizing the inactivation of myeloperoxidase by hydrogen peroxide and azide.#JOURNAL:J Biol Chem#YEAR:1985#PUBMED:2989289# GENE ASSOCIATION:
hydrogen peroxide peroxisomal transport via diffusion
Abbreviation: R_DMHPTCRNte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dmhptcrn[c] <==> dmhptcrn[e] Confidence Level: 2 unknown mechanism for transport -see PMID 9299016, identifies 2,6-dimethylheptanoyl carnitine in the urine! NJ genes: none proteins: none citations: AUTHORS:Verhoeven NM, Wanders RJ, Poll-The BT, Saudubray JM, Jakobs C.#TITLE:The metabolism of phytanic acid and pristanic acid in man: a review.#JOURNAL:J Inherit Metab Dis#YEAR:1998#PUBMED:9819701# AUTHORS:Libert R, Van Hoof F, Thillaye M, Vincent MF, Nassogne MC, Stroobant V, de Hoffmann E, Schanck A.#TITLE:Identification of new medium-chain acylcarnitines present in normal human urine#JOURNAL:Anal Biochem.#YEAR:1997#PUBMED:9299016# GENE ASSOCIATION:
_2,6 dimethylheptanoyl carnitine transport
Abbreviation: R_5ADTSTSTERONEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _5adtststerone[e] <==> 5adtststerone[c] Confidence Level: 1 specific mechanism not known, proton dependence for trans-mit transport has been suggested, however directionality and stoichiometry has not yet been elucidated. see PMID: 12770731 and others NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SERCYSNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[c] + na1[e] + ser-L[e] --> cys-L[e] + na1[c] + ser-L[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
L-serine/L-cysteine Na-dependent exchange (Ser-L in)
Abbreviation: R_FACOAL2251 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + dcsptn1 <==> amp + dcsptn1coa + ppi Confidence Level: 4 NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_EX_val_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : val-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CMPACNAtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: cmp[g] + cmpacna[c] <==> cmp[c] + cmpacna[g] Confidence Level: 4 NCD genes: LOCUS:_10559#TRANSCRIPT:1#ABBREVIATION:SLC35A1# proteins: NAME:solute carrier family 35 (CMP-sialic acid transporter), member A1, Golgi#ABBREVIATION:Slc35a1.1-g# citations: AUTHORS:Ishida N, Ito M, Yoshioka S, Sun-Wada GH, Kawakita M#TITLE:Functional expression of human golgi CMP-sialic acid transporter in the Golgi complex of a transporter-deficient Chinese hamster ovary cell mutant#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9644260# AUTHORS:Ishida N, Miura N, Yoshioka S, Kawakita M#TITLE:Molecular cloning and characterization of a novel isoform of the human UDP-galactose transporter, and of related complementary DNAs belonging to the nucleotide-sugar transporter gene family#JOURNAL:J Biochem (Tokyo)#YEAR:1996#PUBMED:9010752# AUTHORS:Ishida N, Kawakita M#TITLE:Molecular physiology and pathology of the nucleotide sugar transporter family (SLC35)#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12759756# GENE ASSOCIATION: (10559.1)
CMP-Sia Golgi transport via CMP antiport
Abbreviation: R_RETFAt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _11-cis-retfa[c] --> 11-cis-retfa[e] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_B3GNT51g Synonyms: _5 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : galgluside_hs + uacgam --> acglcgalgluside_hs + h + udp Confidence Level: 2 NJ genes: LOCUS:_84002#TRANSCRIPT:1#ABBREVIATION:B3GNT5# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 5#ABBREVIATION:B3gnt5.1# citations: none GENE ASSOCIATION: (84002.1)
UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 5
Abbreviation: R_TMDK1m Synonyms: _0 EC Number: 2.7.1.21 SUBSYSTEM: Nucleotides Equation: [m] : atp + thymd --> adp + dtmp + h Confidence Level: 4 TV genes: LOCUS:_7084#TRANSCRIPT:1#ABBREVIATION:TK2#ECNUMBERS:2.7.1.21# proteins: NAME:thymidine kinase 2, mitochondrial #ABBREVIATION:Tk2.1-m# citations: AUTHORS:Johansson M, Brismar S, Karlsson A.#TITLE:Human deoxycytidine kinase is located in the cell nucleus.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9342341# AUTHORS:Krawiec K, Kierdaszuk B, Shugar D.#TITLE:Inorganic tripolyphosphate (PPP(i)) as a phosphate donor for human deoxyribonucleoside kinases. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12535661# GENE ASSOCIATION: (7084.1)
Abbreviation: R_ASCBOX Synonyms: _0 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [c] : ascb-L + (2) h + (2) o2s --> dhdascb + (2) h2o2 Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# GENE ASSOCIATION:
Abbreviation: R_FCOAH Synonyms: _0 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : formcoa + h2o --> coa + for + (5) h Confidence Level: 2 Note - formcoa formula is uncharged - the correct is FACOAH2 w/ forcoa NJ genes: none proteins: none citations: AUTHORS:Verhoeven NM, Jakobs C.#TITLE:Human metabolism of phytanic acid and pristanic acid.#JOURNAL:Prog Lipid Res#YEAR:2001#PUBMED:11591435# AUTHORS:Jansen GA, van den Brink DM, Ofman R, Draghici O, Dacremont G, Wanders RJ.#TITLE:Identification of pristanal dehydrogenase activity in peroxisomes: conclusive evidence that the complete phytanic acid alpha-oxidation pathway is localized in peroxisomes.#JOURNAL:Biochem Biophys Res Commun#YEAR:2001#PUBMED:11341778# GENE ASSOCIATION:
Abbreviation: R_SERPT Synonyms: _0 EC Number: 2.3.1.50 SUBSYSTEM: Sphingolipid Metabolism Equation: [c] : h + pmtcoa + ser-L --> 3dsphgn + co2 + coa Confidence Level: 4 NCD genes: LOCUS:_9517#TRANSCRIPT:1#ABBREVIATION:SPTLC2#ECNUMBERS:2.3.1.50# LOCUS:_10558#TRANSCRIPT:1#ABBREVIATION:SPTLC1# proteins: NAME:serine palmitoyltransferase#ABBREVIATION:Sptlc.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Weiss B, Stoffel W#TITLE:Human and murine serine-palmitoyl-CoA transferase#JOURNAL:Eur J Biochem#YEAR:1997# GENE ASSOCIATION: (10558.1 and 9517.1)
Abbreviation: R_RBTt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: rbt[c] --> rbt[e] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Huck JH, Roos B, Jakobs C, van der Knaap MS, Verhoeven NM#TITLE:Evaluation of pentitol metabolism in mammalian tissues provides new insight into disorders of human sugar metabolism#JOURNAL:Mol Genet Metab#YEAR:2004#PUBMED:15234337# GENE ASSOCIATION:
Abbreviation: R_PCt Synonyms: _10 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + h2o[c] + pchol_hs[c] <==> adp[c] + h[c] + pchol_hs[e] + pi[c] Confidence Level: 4 for PC secretion into bile NJ genes: LOCUS:_5244#TRANSCRIPT:1#ABBREVIATION:ABCB4# LOCUS:_5244#TRANSCRIPT:3#ABBREVIATION:ABCB4# LOCUS:_5244#TRANSCRIPT:2#ABBREVIATION:ABCB4# proteins: NAME:ABC, sub-family (MDR/TAP), member 4#ABBREVIATION:Abcb4.1# NAME:ABC, sub-family (MDR/TAP), member 4#ABBREVIATION:Abcb4.2# NAME:ABC, sub-family (MDR/TAP), member 4#ABBREVIATION:Abcb4.3# citations: AUTHORS:Ruetz S, Gros P.#TITLE:Phosphatidylcholine translocase: a physiological role for the mdr2 gene.#JOURNAL:Cell#YEAR:1994#PUBMED:7912658# AUTHORS:Meier PJ, Stieger B.#TITLE:Bile salt transporters.#JOURNAL:Annu Rev Physiol.#YEAR:2002#PUBMED:11826283# GENE ASSOCIATION: (5244.1) or (5244.3) or (5244.2)
Abbreviation: R_IDOAASE1ly Synonyms: _1 EC Number: 3.2.1.76 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : h2o + hs_deg4 --> hs_deg5 + idour Confidence Level: 4 NCD genes: LOCUS:_3425#TRANSCRIPT:1#ABBREVIATION:IDUA#ECNUMBERS:3.2.1.76# proteins: NAME:iduronidase, alpha-L-, lysosomal#ABBREVIATION:Idua.1-l# citations: AUTHORS:Scott HS, Anson DS, Orsborn AM, Nelson PV, Clements PR, Morris CP, Hopwood JJ#TITLE:Human alpha-L-iduronidase: cDNA isolation and expression#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1991#PUBMED:1946389# AUTHORS:Clements PR, Brooks DA, McCourt PA, Hopwood JJ#TITLE:Immunopurification and characterization of human alpha-L-iduronidase with the use of monoclonal antibodies#JOURNAL:Biochem J#YEAR:1989#PUBMED:2470345# GENE ASSOCIATION: (3425.1)
Abbreviation: R_AG13T4g Synonyms: _6 EC Number: 2.4.1.149 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksi_pre1 + uacgam --> h + ksi_pre2 + udp Confidence Level: 4 NCD genes: LOCUS:_10331#TRANSCRIPT:1#ABBREVIATION:B3GNT3#ECNUMBERS:2.4.1.-# LOCUS:_10678#TRANSCRIPT:1#ABBREVIATION:B3GNT1#ECNUMBERS:2.4.1.-# LOCUS:_11041#TRANSCRIPT:1#ABBREVIATION:B3GNT6# LOCUS:_374907#TRANSCRIPT:1#ABBREVIATION:B3GALT7# LOCUS:_284208#TRANSCRIPT:1#ABBREVIATION:B3GNTL1# LOCUS:_93010#TRANSCRIPT:1#ABBREVIATION:B3GNT7# LOCUS:_79369#TRANSCRIPT:1#ABBREVIATION:B3GNT4# LOCUS:_374907#TRANSCRIPT:2#ABBREVIATION:B3GALT7# LOCUS:_10678#TRANSCRIPT:2#ABBREVIATION:B3GNT1# proteins: NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 3, Golgi apparatus#ABBREVIATION:B3gnt3.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1, Golgi apparatus#ABBREVIATION:B3gnt1.2-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 7, Golgi apparatus#ABBREVIATION:B3gnt7.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 6, Golgi apparatus#ABBREVIATION:B3gnt6.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase-like 1#ABBREVIATION:B3gntl1.1-g# NAME:UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 4, Golgi apparatus#ABBREVIATION:B3gnt4.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.1-g# NAME:UDP-Gal:betaGal beta 1,3-galactosyltransferase polypeptide 7#ABBREVIATION:B3galt7.2-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shiraishi N, Natsume A, Togayachi A, Endo T, Akashima T, Yamada Y, Imai N, Nakagawa S, Koizumi S, Sekine S, Narimatsu H, Sasaki K#TITLE:Identification and characterization of three novel beta 1,3-N-acetylglucosaminyltransferases structurally related to the beta 1,3-galactosyltransferase family#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11042166# AUTHORS:Kataoka K, Huh NH#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase involved in invasion of cancer cells as assayed in vitro#JOURNAL:Biochem Biophys Res Commun#YEAR:2002#PUBMED:12061784# AUTHORS:Sasaki K, Kurata-Miura K, Ujita M, Angata K, Nakagawa S, Sekine S, Nishi T, Fukuda M#TITLE:Expression cloning of cDNA encoding a human beta-1,3-N-acetylglucosaminyltransferase that is essential for poly-N-acetyllactosamine synthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9405606# AUTHORS:Ujita M, Misra AK, McAuliffe J, Hindsgaul O, Fukuda M#TITLE:Poly-N-acetyllactosamine extension in N-glycans and core 2- and core 4-branched O-glycans is differentially controlled by i-extension enzyme and different members of the beta 1,4-galactosyltransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10747980# AUTHORS:Zheng H, Li Y, Ji C, Li J, Zhang J, Yin G, Xu J, Ye X, Wu M, Zou X, Gu S, Xie Y, Mao Y#TITLE:Characterization of a cDNA encoding a protein with limited similarity to beta1, 3-N-acetylglucosaminyltransferase#JOURNAL:Mol Biol Rep#YEAR:2004#PUBMED:15560372# AUTHORS:Ishida H, Togayachi A, Sakai T, Iwai T, Hiruma T, Sato T, Okubo R, Inaba N, Kudo T, Gotoh M, Shoda J, Tanaka N, Narimatsu H#TITLE:A novel beta1,3-N-acetylglucosaminyltransferase (beta3Gn-T8), which synthesizes poly-N-acetyllactosamine, is dramatically upregulated in colon cancer#JOURNAL:FEBS Lett#YEAR:2005#PUBMED:15620693# AUTHORS:Funderburgh JL#TITLE:Keratan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512857# GENE ASSOCIATION: (11041.1) or (10678.1) or (93010.1) or (10331.1) or (374907.1) or (284208.1) or (374907.2) or (10678.2) or (79369.1)
Abbreviation: R_FA1821ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + lnlcACP <==> ACP + h + lnlc Confidence Level: 4 NJ genes: none proteins: none citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION:
Abbreviation: R_ACCOAgt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: accoa[c] <==> accoa[g] Confidence Level: 4 transport between cytosol and Golgi/ER presumed presumed facilitated transport, but unknown co-substrates, see PMID: 12739170, Hirabayashi et al NJ genes: LOCUS:_9197#TRANSCRIPT:1#ABBREVIATION:SLC33A1# proteins: NAME:acetyl-CoA transporter/O-acetylator#ABBREVIATION:Slc33a1.1# citations: AUTHORS:Hirabayashi Y, Kanamori A, Nomura KH, Nomura K.#TITLE:The acetyl-CoA transporter family SLC33.#JOURNAL:Pflugers Arch.#YEAR:2004#PUBMED:12739170# GENE ASSOCIATION: (9197.1)
Abbreviation: R_1MNCAMti Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: _1mncam[c] + atp[c] + h2o[c] --> 1mncam[e] + adp[c] + h[c] + pi[c] Confidence Level: 2 IT based on information in Bender, 2003, 2nd Ed., Nutritional Biochemistry of the Vitamins, p. 206 genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# GENE ASSOCIATION:
Abbreviation: R_INSt5 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ins[e] + (2) na1[e] --> ins[c] + (2) na1[c] Confidence Level: 4 MM genes: LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (64078.1)
inosine transport in via sodium (1:2) symport
Abbreviation: R_LINKDEG1ly Synonyms: _0 SUBSYSTEM: Heparan sulfate degradation Equation: [l] : (3) h2o + hs_deg25 --> (2) gal + glcur + xyl-D Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# GENE ASSOCIATION:
Abbreviation: R_HEXCCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + hexccoa --> coa + hexccrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_BALAtmr Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: ala-B[c] <==> ala-B[m] Confidence Level: 1 SAB genes: none proteins: none citations: AUTHORS:Ohyama T, Matsuda K, Tachibana H, Fujimoto Sakata S, Mori M, Horiuchi M, Tamaki N#TITLE:Purification and expression of a processing protease on beta-alanine-oxoglutarate aminotransferase from rat liver mitochondria#JOURNAL:FEBS Lett#YEAR:2004#PUBMED:15304357# GENE ASSOCIATION:
Beta-alanine reversible mitochondrial transport (diffusion)
Abbreviation: R_NADPtxu Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: nadp[x] --> nadp[c] Confidence Level: 1 diffusion - unidirectional to avoid NADP/NAD loops w/ potential for free H NJ genes: none proteins: none citations: AUTHORS:Geisbrecht BV, Gould SJ#TITLE:The human PICD gene encodes a cytoplasmic and peroxisomal NADP(+)-dependent isocitrate dehydrogenase#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10521434# GENE ASSOCIATION:
Abbreviation: R_ALKP Synonyms: _0 EC Number: 3.1.3.1 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : dhap + h2o --> dha + pi Confidence Level: 4 added for staph (SAB) genes: LOCUS:_249#TRANSCRIPT:1#ABBREVIATION:ALPL#ECNUMBERS:3.1.3.1# LOCUS:_248#TRANSCRIPT:1#ABBREVIATION:ALPI#ECNUMBERS:3.1.3.1# LOCUS:_250#TRANSCRIPT:1#ABBREVIATION:ALPP#ECNUMBERS:3.1.3.1# LOCUS:_251#TRANSCRIPT:1#ABBREVIATION:ALPPL2# proteins: NAME:alkapline phosphatase, intestinal#ABBREVIATION:Alpi.1# NAME:alkaline phosphatase, liver/bone/kidney#ABBREVIATION:Alpl.1# NAME:alkaline phosphatase, placental (Regan isozyme)#ABBREVIATION:Alpp.1# NAME:alkaline phosphatase, placental-like 2#ABBREVIATION:Alppl2.1# citations: AUTHORS:Knoll BJ, Rothblum KN, Longley M.#TITLE:Nucleotide sequence of the human placental alkaline phosphatase gene. Evolution of the 5' flanking region by deletion/substitution.#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3042787# GENE ASSOCIATION: (248.1) or (251.1) or (249.1) or (250.1)
Abbreviation: R_HESTRATRIOLte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hestratriol[c] <==> hestratriol[e] Confidence Level: 1 unknown mechanism, hydroxylated (inactivated) estrogen derivative needs to be exported NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NTD2e Synonyms: _0 EC Number: 3.1.3.5 SUBSYSTEM: Nucleotides Equation: [e] : h2o + ump --> pi + uri Confidence Level: 2 Wide specificity for 5'-nucleotides Also see EC 3.1.3.31 genes: LOCUS:_4907#TRANSCRIPT:1#ABBREVIATION:NT5E#ECNUMBERS:3.1.3.5# proteins: NAME:_5'-nucleotidase, ecto (CD73) #ABBREVIATION:Nt5E.1-e# citations: none GENE ASSOCIATION: (4907.1)
Abbreviation: R_PGS Synonyms: _9 EC Number: 1.14.99.1 SUBSYSTEM: Eicosanoid Metabolism Equation: [c] : arachd + h + nadph + (2) o2 --> h2o + nadp + prostgh2 Confidence Level: 4 NJ genes: LOCUS:_5742#TRANSCRIPT:1#ABBREVIATION:PTGS1#ECNUMBERS:1.14.99.1# LOCUS:_5743#TRANSCRIPT:1#ABBREVIATION:PTGS2#ECNUMBERS:1.14.99.1# proteins: NAME:prostaglandin-endoperoxide synthase 2#ABBREVIATION:Ptgs2.1# NAME:prostaglandin-endoperoxide synthase 1#ABBREVIATION:Ptgs1.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Maihofner C, Probst-Cousin S, Bergmann M, Neuhuber W, Neundorfer B, Heuss D.#TITLE:Expression and localization of cyclooxygenase-1 and -2 in human sporadic amyotrophic lateral sclerosis.#JOURNAL:Eur J Neurosci#YEAR:2003#PUBMED:14511332# AUTHORS:Funk CD, Funk LB, Kennedy ME, Pong AS, Fitzgerald GA.#TITLE:Human platelet/erythroleukemia cell prostaglandin G/H synthase: cDNA cloning, expression, and gene chromosomal assignment.#JOURNAL:FASEB J#YEAR:1991#PUBMED:1907252# GENE ASSOCIATION: (5743.1) or (5742.1)
Abbreviation: R_CKc Synonyms: _0 EC Number: 2.7.3.2 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : atp + creat <==> adp + pcreat Confidence Level: 4 kegg C02305 MJC SAB c genes: LOCUS:_1158#TRANSCRIPT:1#ABBREVIATION:CKM#ECNUMBERS:2.7.3.2# LOCUS:_1152#TRANSCRIPT:1#ABBREVIATION:CKB#ECNUMBERS:2.7.3.2# proteins: NAME:creatine kinase, brain#ABBREVIATION:Ckb.1# NAME:creatine kinase, muscle#ABBREVIATION:Ckm.1# citations: AUTHORS:Villarreal-Levy G, Ma TS, Kerner SA, Roberts R, Perryman MB#TITLE:Human creatine kinase: isolation and sequence analysis of cDNA clones for the B subunit, development of subunit specific probes and determination of gene copy number#JOURNAL:Biochem Biophys Res Commun#YEAR:1987#PUBMED:3034271# AUTHORS:Kaye FJ, McBride OW, Battey JF, Gazdar AF, Sausville EA#TITLE:Human creatine kinase-B complementary DNA. Nucleotide sequence, gene expression in lung cancer, and chromosomal assignment to two distinct loci#JOURNAL:J Clin Invest#YEAR:1987#PUBMED:2883200# GENE ASSOCIATION: (1152.1) or (1158.1) and (1152.1) or (1158.1)
Abbreviation: R_BAMPPALDOX Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: beta-Alanine metabolism Equation: [c] : bamppald + h2o + nad --> ala-B + (2) h + nadh Confidence Level: 1 SAB genes: LOCUS:_223#TRANSCRIPT:1#ABBREVIATION:ALDH9A1#ECNUMBERS:1.2.1.3,,1.2.1.19# LOCUS:_501#TRANSCRIPT:1#ABBREVIATION:ALDH7A1# LOCUS:_224#TRANSCRIPT:1#ABBREVIATION:ALDH3A2# LOCUS:_216#TRANSCRIPT:1#ABBREVIATION:ALDH1A1#ECNUMBERS:1.2.1.36# LOCUS:_8854#TRANSCRIPT:1#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:3#ABBREVIATION:ALDH1A2# LOCUS:_8854#TRANSCRIPT:2#ABBREVIATION:ALDH1A2# proteins: NAME:aldehyde dehydrogenase 1 family, member A1#ABBREVIATION:Aldh1a1.1# NAME:aldehyde dehydrogenase 9 family, member A1#ABBREVIATION:Aldh9a1.1# NAME:aldehyde dehydrogenase 3 family, member A2#ABBREVIATION:Aldh3a2.1# NAME:aldehyde dehydrogenase 7 family, member A1#ABBREVIATION:Aldh7a1.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.1# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.2# NAME:aldehyde dehydrogenase 1 family, member A2#ABBREVIATION:Aldh1a2.3# citations: AUTHORS:White WH, Skatrud PL, Xue Z, Toyn JH#TITLE:Specialization of function among aldehyde dehydrogenases: the ALD2 and ALD3 genes are required for beta-alanine biosynthesis in Saccharomyces cerevisiae#JOURNAL:Genetics#YEAR:2003#PUBMED:12586697# GENE ASSOCIATION: (8854.2) or (501.1) or (216.1) or (224.1) or (8854.3) or (223.1) or (8854.1)
beta-Aminopropion aldehyde:NAD+ oxidoreductase
Abbreviation: R_SARDHm Synonyms: _0 EC Number: 1.5.99.1 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [m] : fad + sarcs + thf --> fadh2 + gly + mlthf Confidence Level: 4 SAB EC # is questionable overall reaction is questionable genes: LOCUS:_1757#TRANSCRIPT:1#ABBREVIATION:SARDH#ECNUMBERS:1.5.99.2# proteins: NAME:sarcosine dehydrogenase#ABBREVIATION:Sardh.1-m# citations: AUTHORS:Eschenbrenner M, Jorns MS. #TITLE:Cloning and mapping of the cDNA for human sarcosine dehydrogenase, a flavoenzyme defective in patients with sarcosinemia. #JOURNAL:Genomics#YEAR:1999#PUBMED:10444331# GENE ASSOCIATION: (1757.1)
Abbreviation: R_CYTK14n Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [n] : dcmp + utp <==> dcdp + udp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1-n# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_2AMADPTm Synonyms: _0 SUBSYSTEM: Lysine Metabolism Equation: L2aadp[c] + akg[m] <==> L2aadp[m] + akg[c] Confidence Level: 4 SAB PMID 11083877 genes: LOCUS:_89874#TRANSCRIPT:1#ABBREVIATION:SLC25A21# proteins: NAME:solute carrier family 25 (mitochondrial oxodicarboxylate carrier), member 21#ABBREVIATION:Slc25a21.1-m# citations: AUTHORS:Fiermonte G, Dolce V, Palmieri L, Ventura M, Runswick MJ, Palmieri F, Walker JE#TITLE:Identification of the human mitochondrial oxodicarboxylate carrier. Bacterial expression, reconstitution, functional characterization, tissue distribution, and chromosomal location#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11083877# GENE ASSOCIATION: (89874.1)
L-2-aminoadipate shuttle (cytosol/mitochondria)
Abbreviation: R_P4508B11r Synonyms: _3 SUBSYSTEM: Bile Acid Biosynthesis Equation: [r] : h + nadph + o2 + xol7aone --> h2o + nadp + xoldiolone Confidence Level: 4 NJ - added, substrates by analogy to other xol hydroxylase reactions (NADH and NADPH can both be used) genes: LOCUS:_1582#TRANSCRIPT:1#ABBREVIATION:CYP8B1# proteins: NAME:cytochrome P450, family 8, subfamily B, polypeptide 1#ABBREVIATION:Cyp8b1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# AUTHORS:Gafvels M, Olin M, Chowdhary BP, Raudsepp T, Andersson U, Persson B, Jansson M, Bjorkhem I, Eggertsen G#TITLE:Structure and chromosomal assignment of the sterol 12-alpha-hydroxylase gene (cyp8b1) in human and mouse#JOURNAL:Genomics#YEAR:1999# GENE ASSOCIATION: (1582.1)
Abbreviation: R_D_DASH_LACt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[e] + lac-D[e] <==> h[c] + lac-D[c] Confidence Level: 4 JLR genes: LOCUS:_23539#TRANSCRIPT:1#ABBREVIATION:SLC16A8# LOCUS:_9194#TRANSCRIPT:1#ABBREVIATION:SLC16A7# LOCUS:_9123#TRANSCRIPT:1#ABBREVIATION:SLC16A3# LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 7#ABBREVIATION:Slc16a7.1# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1#ABBREVIATION:Slc16a1.1# NAME:solute carrier 16 (monocarboxylic acid transporters), member 8#ABBREVIATION:Slc16a8.1-e# NAME:solute carrier family 16 (monocarboxylic acid transporters), member 3#ABBREVIATION:Slc16a3.1-e# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Lin RY, Vera JC, Chaganti RS, Golde DW#TITLE:Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9786900# AUTHORS:Champe, P.C., Harvey, R.A., Ferrier D.R.#TITLE:Biochemistry, 3rd edition#YEAR:2005# AUTHORS:Halestrap AP, Price NT#TITLE:The proton-linked monocarboxylate transporter (MCT) family: structure, function and regulation#JOURNAL:Biochem J#YEAR:1999#PUBMED:10510291# AUTHORS:Garcia CK, Li X, Luna J, Francke U#TITLE:cDNA cloning of the human monocarboxylate transporter 1 and chromosomal localization of the SLC16A1 locus to 1p13.2-p12#JOURNAL:Genomics#YEAR:1994#PUBMED:7835905# AUTHORS:Poole RC, Halestrap AP#TITLE:Transport of lactate and other monocarboxylates across mammalian plasma membranes#JOURNAL:Am J Physiol#YEAR:1993#PUBMED:8476015# AUTHORS:Ritzhaupt A, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:The characterization of butyrate transport across pig and human colonic luminal membrane#JOURNAL:J Physiol#YEAR:1998#PUBMED:9508842# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification of a monocarboxylate transporter isoform type 1 (MCT1) on the luminal membrane of human and pig colon#JOURNAL:Biochem Soc Trans#YEAR:1998#PUBMED:9649795# AUTHORS:Ritzhaupt A, Wood IS, Ellis A, Hosie KB, Shirazi-Beechey SP#TITLE:Identification and characterization of a monocarboxylate transporter (MCT1) in pig and human colon: its potential to transport L-lactate as well as butyrate#JOURNAL:J Physiol#YEAR:1998#PUBMED:9824713# AUTHORS:Halestrap AP, Meredith D#TITLE:The SLC16 gene family-from monocarboxylate transporters (MCTs) to aromatic amino acid transporters and beyond#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739169# AUTHORS:Yoon H, Donoso LA, Philp NJ#TITLE:Cloning of the human monocarboxylate transporter MCT3 gene: localization to chromosome 22q12.3-q13.2.#JOURNAL:Genomics#YEAR:1999#PUBMED:10493836# AUTHORS:Manning Fox JE, Meredith D, Halestrap AP#TITLE:Characterisation of human monocarboxylate transporter 4 substantiates its role in lactic acid efflux from skeletal muscle#JOURNAL:J Physiol#YEAR:2000#PUBMED:11101640# AUTHORS:Price NT, Jackson VN, Halestrap AP#TITLE:Cloning and sequencing of four new mammalian monocarboxylate transporter (MCT) homologues confirms the existence of a transporter family with an ancient past#JOURNAL:Biochem J#YEAR:1998#PUBMED:9425115# GENE ASSOCIATION: (9123.1) or (6566.1) or (23539.1) or (9194.1)
Abbreviation: R_TCYNTt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tcynt[c] --> tcynt[e] Confidence Level: 2 thiocyanate is found excreted in urine (PMID: 6351723) MM genes: none proteins: none citations: AUTHORS:Cooper AJ#TITLE:Biochemistry of sulfur-containing amino acids.#YEAR:1983#PUBMED:6351723# GENE ASSOCIATION:
Thiocyanate transport via diffusion (cytosol to extracellular)
Abbreviation: R_EX_sphs1p_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : sphs1p <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PTRCAT1 Synonyms: _0 EC Number: 2.3.1.57 SUBSYSTEM: Arginine and Proline Metabolism Equation: [c] : accoa + ptrc --> aprut + coa + h Confidence Level: 4 SAB genes: LOCUS:_6303#TRANSCRIPT:1#ABBREVIATION:SAT#ECNUMBERS:2.3.1.57# LOCUS:_112483#TRANSCRIPT:1#ABBREVIATION:SAT2# proteins: NAME:spermidine/spermine N1-acetyltransferase#ABBREVIATION:Sat.1# NAME:spermidine/spermine N1-acetyltransferase 2#ABBREVIATION:Sat2.1# citations: AUTHORS:Chen Y, Vujcic S, Liang P, Diegelman P, Kramer DL, Porter CW.#TITLE:Genomic identification and biochemical characterization of a second spermidine/spermine N1-acetyltransferase#JOURNAL:Biochem J#YEAR:2003#PUBMED:12803540# GENE ASSOCIATION: (6303.1) or (112483.1)
Abbreviation: R_DMATT Synonyms: _0 EC Number: 2.5.1.1 SUBSYSTEM: Cholesterol Metabolism Equation: [c] : dmpp + ipdp --> grdp + ppi Confidence Level: 2 genes: LOCUS:_9453#TRANSCRIPT:1#ABBREVIATION:GGPS1#ECNUMBERS:2.5.1.1,2.5.1.10,,2.5.1.29# proteins: NAME:geranylgeranyl diphosphate synthase 1#ABBREVIATION:Ggps1.1# citations: none GENE ASSOCIATION: (9453.1)
Abbreviation: R_CMPSAS Synonyms: _0 EC Number: 2.7.7.43 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : acnam + ctp --> cmpacna + ppi Confidence Level: 2 NCD genes: LOCUS:_55907#TRANSCRIPT:1#ABBREVIATION:CMAS#ECNUMBERS:2.7.7.43# proteins: NAME:cytidine monophosphate N-acetylneuraminic acid synthetase#ABBREVIATION:Cmas.1# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Munster AK, Eckhardt M, Potvin B, Muhlenhoff M, Stanley P, Gerardy-Schahn R#TITLE:Mammalian cytidine 5'-monophosphate N-acetylneuraminic acid synthetase: a nuclear protein with evolutionarily conserved structural motifs#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1998#PUBMED:9689047# GENE ASSOCIATION: (55907.1)
Abbreviation: R_SR5AR2r Synonyms: _4 EC Number: 1.3.99.5 SUBSYSTEM: Steroid Metabolism Equation: [r] : andrstndn + h + nadph --> andrstandn + nadp Confidence Level: 2 see PMID: 14643063 there are actually two types, but the difference between them lies in optimal pH function - grouped as one reaction at his point to eliminate redundancy. NADP known to be cofactor NJ genes: LOCUS:_6716#TRANSCRIPT:1#ABBREVIATION:SRD5A2#ECNUMBERS:1.3.99.5# LOCUS:_6715#TRANSCRIPT:1#ABBREVIATION:SRD5A1#ECNUMBERS:1.3.99.5# proteins: NAME:steroid-5-alpha-reductase, alpha polypeptide 1#ABBREVIATION:Srd5a1.1# NAME:steroid-5-alpha-reductase, alpha polypeptide 2#ABBREVIATION:Srd5a2.1# citations: AUTHORS:Belanger A, Pelletier G, Labrie F, Barbier O, Chouinard S.#TITLE:Inactivation of androgens by UDP-glucuronosyltransferase enzymes in humans.#JOURNAL:Trends Endocrinol Metab.#YEAR:2003#PUBMED:14643063# GENE ASSOCIATION: (6715.1) or (6716.1)
Abbreviation: R_FAOXC80 Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation Equation: [m] : (3) coa + (3) fad + (3) h2o + (3) nad + occoa --> (4) accoa + (3) fadh2 + (3) h + (3) nadh Confidence Level: 4 TDV (9/7/2004) Other enzymes: 1.3.3.6, 4.2.1.74, 4.2.1.17, 1.1.1.35, 1.1.1.211 genes: LOCUS:_34#TRANSCRIPT:1#ABBREVIATION:ACADM#ECNUMBERS:1.3.99.3# LOCUS:_35#TRANSCRIPT:1#ABBREVIATION:ACADS#ECNUMBERS:1.3.99.2# proteins: NAME:acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain #ABBREVIATION:Acads.1-m# NAME:acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, mitochondrial#ABBREVIATION:Acadm.1-m# citations: AUTHORS:Kelly DP, Kim JJ, Billadello JJ, Hainline BE, Chu TW, Strauss AW#TITLE:Nucleotide sequence of medium-chain acyl-CoA dehydrogenase mRNA and its expression in enzyme-deficient human tissue.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3035565# AUTHORS:Naito E, Ozasa H, Ikeda Y, Tanaka K.#TITLE:Molecular cloning and nucleotide sequence of complementary DNAs encoding human short chain acyl-coenzyme A dehydrogenase and the study of the molecular basis of human short chain acyl-coenzyme A dehydrogenase deficiency.#JOURNAL:J Clin Invest#YEAR:1985#PUBMED:2565344# GENE ASSOCIATION: (35.1) or (34.1)
Beta oxidation of med/long chain fatty acid
Abbreviation: R_P45027A14m Synonyms: _5 EC Number: 1.14.13.15 SUBSYSTEM: Bile Acid Biosynthesis Equation: [m] : h + nadph + o2 + xol7ah2 --> h2o + nadp + xol7ah3 Confidence Level: 4 from uniprot NJ genes: LOCUS:_1593#TRANSCRIPT:1#ABBREVIATION:CYP27A1# proteins: NAME:cytochrome P450, family 27, subfamily A, polypeptide 1#ABBREVIATION:Cyp27a1.1-m# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (1593.1)
Abbreviation: R_ASPCTr Synonyms: _0 EC Number: 2.1.3.2 SUBSYSTEM: Pyrimidine Biosynthesis Equation: [c] : asp-L + cbp <==> cbasp + h + pi Confidence Level: 4 genes: LOCUS:_790#TRANSCRIPT:1#ABBREVIATION:CAD1#ECNUMBERS:6.3.5.5,2.1.3.2,3.5.2.3# proteins: NAME:CAD protein (3.5.2.3, 2.1.3.2, 6.3.5.5)#ABBREVIATION:Cad1.1# citations: AUTHORS:Iwahana H, Fujimura M, Ii S, Kondo M, Moritani M, Takahashi Y, Yamaoka T, Yoshimoto K, Itakura M.#TITLE:Molecular cloning of a human cDNA encoding a trifunctional enzyme of carbamoyl-phosphate synthetase-aspartate transcarbamoylase-dihydroorotase in de Novo pyrimidine synthesis. #JOURNAL:Biochem Biophys Res Commun.#YEAR:1996#PUBMED:8619816# AUTHORS:Chen KC, Vannais DB, Jones C, Patterson D, Davidson JN.#TITLE:Mapping of the gene encoding the multifunctional protein carrying out the first three steps of pyrimidine biosynthesis to human chromosome 2. #JOURNAL: Hum Genet.#YEAR:1989#PUBMED:2565865# GENE ASSOCIATION: (790.1)
aspartate carbamoyltransferase (reversible)
Abbreviation: R_G12MT2_U Synonyms: _0 EC Number: 2.4.1.131 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : gdpmann + (0.1) m3mpdol_U --> gdp + h + (0.1) m4mpdol_U Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_DPMVDx Synonyms: _0 EC Number: 4.1.1.33 SUBSYSTEM: Cholesterol Metabolism Equation: [x] : 5dpmev + atp --> adp + co2 + ipdp + pi Confidence Level: 4 peroxisomal version - NJ genes: LOCUS:_4597#TRANSCRIPT:1#ABBREVIATION:MVD# proteins: NAME:mevaloante (diphospho) decarboxylase#ABBREVIATION:Mvd.1# citations: AUTHORS:Iglesias J, Gonzalez-Pacanowska D, Caamano G, Garcia-Peregrin E#TITLE:Mevalonate 5-pyrophosphate decarboxylase in isolated villus and crypt cells of chick intestine#JOURNAL:Lipids#YEAR:1988# AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Olivier LM, Krisans SK#TITLE:Peroxisomal protein targeting and identification of peroxisomal targeting signals in cholesterol biosynthetic enzymes#JOURNAL:Biochimica et Biophysica Acta#YEAR:2000# AUTHORS:Bonanno JB, Edo C, Eswar N, Pieper U, Romanowski MJ, Ilyin V, Gerchman SE, Kycia H, Studier W, Sali A, Burley SK#TITLE:Structural genomics of enzymes involved in sterol/isoprenoid biosynthesis#JOURNAL:Proceedings of the National Academy of Sciences#YEAR:2001# GENE ASSOCIATION: (4597.1)
Abbreviation: R_GLCURter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: glcur[c] --> glcur[r] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
glucuronate endoplasmic reticular transport
Abbreviation: R_M4BTAer Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : mem2emgacpail_hs + pre_prot --> gpi_sig + mem2emgacpail_prot_hs Confidence Level: 4 EC 2.6.-.- NCD genes: LOCUS:_10026#TRANSCRIPT:1#ABBREVIATION:PIGK# LOCUS:_8733#TRANSCRIPT:1#ABBREVIATION:GPAA1# LOCUS:_94005#TRANSCRIPT:1#ABBREVIATION:PIGS# LOCUS:_128869#TRANSCRIPT:1#ABBREVIATION:CDC91L1# LOCUS:_51604#TRANSCRIPT:1#ABBREVIATION:PIGT# proteins: NAME:GPAA1P anchor attachment protein 1 homolog (yeast), endoplasmic reticulum#ABBREVIATION:Gpaa1.1-r# NAME:phosphatidylinositol glycan, class K, endoplasmic reticulum#ABBREVIATION:Pigk.1-r# NAME:phosphatidylinositol glycan, class S, endoplasmic reticulum#ABBREVIATION:Pigs.1-r# NAME:phosphatidylinositol glycan, class T, endoplasmic reticulum#ABBREVIATION:Pigt.1-r# NAME:CDC91 cell division cycle 91-like 1 (S. cerevisiae), endoplasmic reticulum#ABBREVIATION:Cdc91l1.1-r# citations: AUTHORS:Hiroi Y, Komuro I, Chen R, Hosoda T, Mizuno T, Kudoh S, Georgescu SP, Medof ME, Yazaki Y#TITLE:Molecular cloning of human homolog of yeast GAA1 which is required for attachment of glycosylphosphatidylinositols to proteins#JOURNAL:FEBS Lett#YEAR:1998#PUBMED:9468317# AUTHORS:Yu J, Nagarajan S, Knez JJ, Udenfriend S, Chen R, Medof ME#TITLE:The affected gene underlying the class K glycosylphosphatidylinositol (GPI) surface protein defect codes for the GPI transamidase#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1997#PUBMED:9356492# AUTHORS:Meyer U, Benghezal M, Imhof I, Conzelmann A#TITLE:Active site determination of Gpi8p, a caspase-related enzyme required for glycosylphosphatidylinositol anchor addition to proteins#JOURNAL:Biochemistry#YEAR:2000#PUBMED:10727241# AUTHORS:Ohishi K, Inoue N, Kinoshita T#TITLE:PIG-S and PIG-T, essential for GPI anchor attachment to proteins, form a complex with GAA1 and GPI8#JOURNAL:EMBO J#YEAR:2001#PUBMED:11483512# AUTHORS:Hong Y, Ohishi K, Kang JY, Tanaka S, Inoue N, Nishimura J, Maeda Y, Kinoshita T#TITLE:Human PIG-U and yeast Cdc91p are the fifth subunit of GPI transamidase that attaches GPI-anchors to proteins#JOURNAL:Mol Biol Cell#YEAR:2003#PUBMED:12802054# AUTHORS:Taron BW, Colussi PA, Wiedman JM, Orlean P, Taron CH#TITLE:Human Smp3p adds a fourth mannose to yeast and human glycosylphosphatidylinositol precursors in vivo#JOURNAL:J Biol Chem#YEAR:2004#PUBMED:15208306# GENE ASSOCIATION: (10026.1) and (128869.1) and (8733.1) and (94005.1) and (51604.1)
Abbreviation: R_ACGAGBSIDEtg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: acgagbside_hs[c] <==> acgagbside_hs[g] Confidence Level: 1 unknown mechanism for transport - however must get to other intracellular compartments (e.g. lysosome for degradation) NJ genes: none proteins: none citations: AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# GENE ASSOCIATION:
Abbreviation: R_DNDPt17m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dcdp[m] + dudp[c] --> dcdp[c] + dudp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_ASCBt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ascb-L[e] <==> ascb-L[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Bianchi J, Rose RC#TITLE:Dehydroascorbic acid and cell membranes: possible disruptive effects#JOURNAL:Toxicology#YEAR:1986#PUBMED:3715892# AUTHORS:Wilson JX#TITLE:Regulation of Vitamin C Transport#JOURNAL:Annu Rev Nutr#YEAR:2004#PUBMED:15705056# GENE ASSOCIATION:
L-ascorbate transport via facilitated diffusion
Abbreviation: R_INStm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: ins[c] <==> ins[m] Confidence Level: 4 IT genes: LOCUS:_2030#TRANSCRIPT:1#ABBREVIATION:SLC29A1# proteins: NAME:solute carrier family 29 (nucleoside transporters), member 1 #ABBREVIATION:Slc29a1.1-m# citations: AUTHORS:Lai Y, Tse CM, Unadkat JD.#TITLE:Mitochondrial expression of the human equilibrative nucleoside transporter 1 (hENT1) results in enhanced mitochondrial toxicity of antiviral drugs.#JOURNAL:J Biol Chem. #YEAR:2004#PUBMED:14607828# AUTHORS:Baldwin SA, Beal PR, Yao SY, King AE, Cass CE, Young JD.#TITLE:The equilibrative nucleoside transporter family, SLC29.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12838422# AUTHORS:Ward JL, Sherali A, Mo ZP, Tse CM.#TITLE:Kinetic and pharmacological properties of cloned human equilibrative nucleoside transporters, ENT1 and ENT2, stably expressed in nucleoside transporter-deficient PK15 cells.#JOURNAL:J Biol Chem. #YEAR:2000#PUBMED:10722669# GENE ASSOCIATION: (2030.1)
inosine facilated transport in mitochondria
Abbreviation: R_GLNCYSNaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cys-L[c] + gln-L[e] + na1[e] --> cys-L[e] + gln-L[c] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6510.1)
L-cysteine/L-glutamine Na-dependent exchange (Gln-L in)
Abbreviation: R_PYNP2r Synonyms: _0 EC Number: 2.4.2.2 SUBSYSTEM: Pyrimidine Catabolism Equation: [c] : pi + uri <==> r1p + ura Confidence Level: 4 JLR- added reversible form of PYNP2 genes: LOCUS:_7378#TRANSCRIPT:1#ABBREVIATION:UPP1#ECNUMBERS:2.4.2.3# LOCUS:_151531#TRANSCRIPT:1#ABBREVIATION:UPP2# LOCUS:_7378#TRANSCRIPT:2#ABBREVIATION:UPP1#ECNUMBERS:2.4.2.3# proteins: NAME:uridine phosphorylase #ABBREVIATION:Upp1.1# NAME:uridine phosphorylase 1 #ABBREVIATION:Upp1.2# NAME:uridine phosphorylase 2 #ABBREVIATION:Upp2.1# citations: AUTHORS:Pizzorno G, Cao D, Leffert JJ, Russell RL, Zhang D, Handschumacher RE.#TITLE:Homeostatic control of uridine and the role of uridine phosphorylase: a biological and clinical update.#JOURNAL:Biochim Biophys Acta#YEAR:2002#PUBMED:12084455# AUTHORS:Watanabe S, Uchida T.#TITLE:Cloning and expression of human uridine phosphorylase.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1995#PUBMED:7488099# AUTHORS:Russell RL, Cao D, Zhang D, Handschumacher RE, Pizzorno G.#TITLE:Uridine phosphorylase association with vimentin. Intracellular distribution and localization.#JOURNAL:J Biol Chem. #YEAR:2001#PUBMED:11278417# AUTHORS:Johansson M.#TITLE:Identification of a novel human uridine phosphorylase. #JOURNAL:Biochem Biophys Res Commun. #YEAR:2003#PUBMED:12849978# GENE ASSOCIATION: (7378.2) or (151531.1) or (7378.1)
pyrimidine-nucleoside phosphorylase (uracil)
Abbreviation: R_VALt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: na1[e] + val-L[e] --> na1[c] + val-L[c] Confidence Level: 2 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# proteins: NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# AUTHORS:Van Winkle LJ#TITLE:Amino acid transport regulation and early embryo development#JOURNAL:Biol Reprod#YEAR:2001#PUBMED:11133652# GENE ASSOCIATION: (11254.1)
L-valine transport in via sodium symport
Abbreviation: R_C161CRNt Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: hdcecrn[c] --> hdcecrn[m] Confidence Level: 4 TDV genes: LOCUS:_788#TRANSCRIPT:1#ABBREVIATION:SLC25A20# proteins: NAME:Solute carrier family 25, carnitine/acylcarnitine translocase, member 20, mitochondrial#ABBREVIATION:Slc25a20.1-m# citations: none GENE ASSOCIATION: (788.1)
Abbreviation: R_FUT11g Synonyms: _4 EC Number: 2.4.1.69 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : galgbside_hs + gdpfuc --> fucgalgbside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2523#TRANSCRIPT:1#ABBREVIATION:FUT1#ECNUMBERS:2.4.1.69# proteins: NAME:fucosyltransferase 1#ABBREVIATION:Fut1.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (2523.1)
Abbreviation: R_UGCG Synonyms: _5 EC Number: 2.4.1.80 SUBSYSTEM: Sphingolipid Metabolism Equation: [r] : crm_hs + udpg --> gluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_7357#TRANSCRIPT:1#ABBREVIATION:UGCG#ECNUMBERS:2.4.1.80# proteins: NAME:UDP-glucose ceramide glucosyltransferase#ABBREVIATION:Ugcg.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Ichikawa S, Sakiyama H, Suzuki G, Hidari KI, Hirabayashi Y.#TITLE:Expression cloning of a cDNA for human ceramide glucosyltransferase that catalyzes the first glycosylation step of glycosphingolipid synthesis.#JOURNAL:Proc Natl Acad Sci#YEAR:1996#PUBMED:8643456# GENE ASSOCIATION: (7357.1)
beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase
Abbreviation: R_G14Tg Synonyms: _7 EC Number: 2.4.1.38 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : fn2m2masn + (2) udpgal --> (2) h + l2fn2m2masn + (2) udp Confidence Level: 4 NCD genes: LOCUS:_9334#TRANSCRIPT:1#ABBREVIATION:B4GALT5#ECNUMBERS:2.4.1.38# LOCUS:_2683#TRANSCRIPT:1#ABBREVIATION:B4GALT1#ECNUMBERS:2.4.1.22# LOCUS:_8704#TRANSCRIPT:1#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.-,2.4.1.22,2.4.1.90,2.4.1.38# LOCUS:_8703#TRANSCRIPT:1#ABBREVIATION:B4GALT3#ECNUMBERS:2.4.1.-,2.4.1.90,2.4.1.38# LOCUS:_8704#TRANSCRIPT:2#ABBREVIATION:B4GALT2#ECNUMBERS:2.4.1.90# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1, Golgi apparatus (all except brain)#ABBREVIATION:B4galt1.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt3.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 5, Golgi apparatus (ubiquitous)#ABBREVIATION:B4galt5.1-g# NAME:UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 2, Golgi apparatus (heart, muscle, pancreas)#ABBREVIATION:B4galt2.2-g# citations: AUTHORS:Lo NW, Shaper JH, Pevsner J, Shaper NL.#TITLE:The expanding beta 4-galactosyltransferase gene family: messages from the databanks#JOURNAL:Glycobiology#YEAR:1998#PUBMED:9597550# AUTHORS:Amado M, Almeida R, Carneiro F, Levery ST, Holmes EH, Nomoto M, Hollingsworth MA, Hassan H, Schwientek T, Nielsen PA, Bennett EP, Clausen H#TITLE:A family of human beta3-galactosyltransferases#JOURNAL:The Journal of Biological Chemistry#YEAR:1998# GENE ASSOCIATION: (9334.1) or (2683.1) or (8704.1) or (8703.1) or (8704.2)
Abbreviation: R_CYTDt5 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cytd[e] + (2) na1[e] --> cytd[c] + (2) na1[c] Confidence Level: 4 IT genes: LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (64078.1)
cytidine transport (1:2 Na/cytd cotransport)
Abbreviation: R_ASPTA Synonyms: _29 EC Number: 2.6.1.1 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [c] : akg + asp-L <==> glu-L + oaa Confidence Level: 4 genes: LOCUS:_2805#TRANSCRIPT:1#ABBREVIATION:GOT1#ECNUMBERS:2.6.1.1# proteins: NAME:aspartate aminotransferase #ABBREVIATION:Got1.1# citations: AUTHORS:Panteghini M.#TITLE:Aspartate aminotransferase isoenzymes.#JOURNAL:Clin Biochem#YEAR:1990#PUBMED:2225456# AUTHORS:Ford GC, Eichele G, Jansonius JN.#TITLE:Three-dimensional structure of a pyridoxal-phosphate-dependent enzyme, mitochondrial aspartate aminotransferase.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1980#PUBMED:6930651# GENE ASSOCIATION: (2805.1)
Abbreviation: R_NACHEX13ly Synonyms: _12 EC Number: 3.2.1.52 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg16 --> acgam + ksi_deg17 Confidence Level: 4 NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase B, lysosomal#ABBREVIATION:HexB-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3074.1) or (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_NS26T2g Synonyms: _0 EC Number: 2.4.99.3 SUBSYSTEM: O-Glycan Biosynthesis Equation: [g] : cmpacna + sT_antigen --> cmp + dsT_antigen + h Confidence Level: 0 NCD genes: LOCUS:_55808#TRANSCRIPT:1#ABBREVIATION:SIAT7A# proteins: NAME:sialyltransferase 7, Golgi apparatus (intestine)#ABBREVIATION:Siat7a.1-g# citations: AUTHORS:Ikehara Y, Kojima N, Kurosawa N, Kudo T, Kono M, Nishihara S, Issiki S, Morozumi K, Itzkowitz S, Tsuda T, Nishimura SI, Tsuji S, Narimatsu H#TITLE:Cloning and expression of a human gene encoding an N-acetylgalactosamine-alpha2,6-sialyltransferase (ST6GalNAc I): a candidate for synthesis of cancer-associated sialyl-Tn antigens#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10536037# GENE ASSOCIATION: (55808.1)
Abbreviation: R_S6TASE12ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg9 <==> h + ksi_deg10 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_FATP7t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: crvnc[c] + na1[c] <==> crvnc[e] + na1[e] Confidence Level: 4 electroneutral transport NJ genes: LOCUS:_11001#TRANSCRIPT:1#ABBREVIATION:SLC27A2# LOCUS:_10998#TRANSCRIPT:1#ABBREVIATION:SLC27A5# proteins: NAME:solute carrier family 27 (fatty acid transporter), member 5#ABBREVIATION:Slc27a5.1# NAME:solute carrier family 27 (fatty acid transporter), member 2#ABBREVIATION:Slc27a2.1# citations: AUTHORS:Steinberg SJ, Wang SJ, McGuinness MC, Watkins PA.#TITLE:Human liver-specific very-long-chain acyl-coenzyme A synthetase: cDNA cloning and characterization of a second enzymatically active protein.#JOURNAL:Mol Genet Metab.#YEAR:1999#PUBMED:10479480# AUTHORS:Stahl A.#TITLE:A current review of fatty acid transport proteins (SLC27).#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:12856180# AUTHORS:Pohl J, Ring A, Hermann T, Stremmel W.#TITLE:Role of FATP in parenchymal cell fatty acid uptake.#JOURNAL:Biochim Biophys Acta#YEAR:2004#PUBMED:15522816# GENE ASSOCIATION: (10998.1) or (11001.1)
alpha-1,6-mannosyl-glycoprotein 4-beta-N-acetylglucosaminyltransferase
Abbreviation: R_M16N4Tg Synonyms: _4 EC Number: 2.4.1.201 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : n4m2masn + uacgam --> h + n5m2masn + udp Confidence Level: 0 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ABO2g Synonyms: _11 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucgalacglcgalgluside_hs + udpgal --> galfucgalacglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_28#TRANSCRIPT:1#ABBREVIATION:ABO# proteins: NAME:ABO blood group#ABBREVIATION:Abo.1# citations: AUTHORS:Lloyd KO.#TITLE:The chemistry and immunochemistry of blood group A, B, H, and Lewis antigens: past, present and future.#JOURNAL:Glycoconj J#YEAR:2000#PUBMED:11421346# AUTHORS:Yamamoto F.-I., Hakomori S.-I.;#TITLE:Sugar-nucleotide donor specificity of histo-blood group A and B transferases is based on amino acid substitutions#JOURNAL:J. Biol. Chem#YEAR:1990#PUBMED:2121736# AUTHORS:Patenaude S.I., Seto N.O.L., Borisova S.N., Szpacenko A., Marcus S.L., Palcic M.M., Evans S.V.;#TITLE:The structural basis for specificity in human ABO(H) blood group biosynthesis#JOURNAL:Nat. Struct. Biol#YEAR:2002#PUBMED:12198488# GENE ASSOCIATION: (28.1)
Abbreviation: R_DHCR243r Synonyms: _3 SUBSYSTEM: Cholesterol Metabolism Equation: [r] : dsmsterol + fadh2 --> chsterol + fad Confidence Level: 4 lanosterol -> cholesterol pathway FAD known to be cofactor, other details missing NJ genes: LOCUS:_1718#TRANSCRIPT:1#ABBREVIATION:DHCR24# proteins: NAME:_24-dehydrocholesterol reductase#ABBREVIATION:Dhcr24.1# citations: AUTHORS:Marijanovic Z, Laubner D, Moller G, Gege C, Husenn B, Adamski J, Breitling R#TITLE:Closing the gap: Identification of human 3-ketosteroid reductase, the last unknown enzyme of mammalian cholesterol biosynthesis#JOURNAL:Molecular Endocrinology#YEAR:2005# GENE ASSOCIATION: (1718.1)
Abbreviation: R_ADNt5 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: adn[e] + (2) na1[e] --> adn[c] + (2) na1[c] Confidence Level: 4 MM genes: LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (64078.1)
adenosine transport (1:2 Na/Adn cotransport)
Abbreviation: R_NDP8 Synonyms: _0 EC Number: 3.6.1.6 SUBSYSTEM: Nucleotides Equation: [c] : dudp + h2o --> dump + h + pi Confidence Level: 4 genes: LOCUS:_1841#TRANSCRIPT:1#ABBREVIATION:DTYMK#ECNUMBERS:2.7.4.9# proteins: NAME:deoxythymidylate kinase (thymidylate kinase) #ABBREVIATION:Dtymk.1# citations: AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# GENE ASSOCIATION: (1841.1)
Abbreviation: R_GRTT Synonyms: _0 EC Number: 2.5.1.10 SUBSYSTEM: Cholesterol Metabolism Equation: [c] : grdp + ipdp --> frdp + ppi Confidence Level: 2 genes: LOCUS:_9453#TRANSCRIPT:1#ABBREVIATION:GGPS1#ECNUMBERS:2.5.1.1,2.5.1.10,,2.5.1.29# proteins: NAME:geranylgeranyl diphosphate synthase 1#ABBREVIATION:Ggps1.1# citations: none GENE ASSOCIATION: (9453.1)
Abbreviation: R_GK1m Synonyms: _0 EC Number: 2.7.4.8 SUBSYSTEM: Nucleotides Equation: [m] : atp + gmp <==> adp + gdp Confidence Level: 1 it genes: none proteins: none citations: none GENE ASSOCIATION:
guanylate kinase (GMP:ATP), mitochondrial
Abbreviation: R_GLCNACT5g Synonyms: _3 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre8 + uacgam --> h + hs_pre9 + udp Confidence Level: 4 EC 2.4.1.224 NCD genes: LOCUS:_2131#TRANSCRIPT:1#ABBREVIATION:EXT1# LOCUS:_2132#TRANSCRIPT:1#ABBREVIATION:EXT2#ECNUMBERS:2.4.1.-# LOCUS:_2137#TRANSCRIPT:1#ABBREVIATION:EXTL3#ECNUMBERS:2.4.1.-# LOCUS:_2134#TRANSCRIPT:1#ABBREVIATION:EXTL1# LOCUS:_2132#TRANSCRIPT:2#ABBREVIATION:EXT2# proteins: NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.1-g# NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.2-g# NAME:exostoses (multiple)-like 1, Golgi apparatus#ABBREVIATION:Extl1.1-g# NAME:exostoses (multiple)-like 3, Golgi apparatus#ABBREVIATION:Extl3.1-g# citations: AUTHORS:Saito T, Seki N, Yamauchi M, Tsuji S, Hayashi A, Kozuma S, Hori T#TITLE:Structure, chromosomal location, and expression profile of EXTR1 and EXTR2, new members of the multiple exostoses gene family#JOURNAL:Biochem Biophys Res Commun#YEAR:1998#PUBMED:9473480# AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Kim BT, Kitagawa H, Tamura J, Saito T, Kusche-Gullberg M, Lindahl U, Sugahara K#TITLE:Human tumor suppressor EXT gene family members EXTL1 and EXTL3 encode alpha 1,4- N-acetylglucosaminyltransferases that likely are involved in heparan sulfate/ heparin biosynthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390981# AUTHORS:Van Hul W, Wuyts W, Hendrickx J, Speleman F, Wauters J, De Boulle K, Van Roy N, Bossuyt P, Willems PJ#TITLE:Identification of a third EXT-like gene (EXTL3) belonging to the EXT gene family#JOURNAL:Genomics#YEAR:1998#PUBMED:9479495# AUTHORS:McCormick C, Duncan G, Goutsos KT, Tufaro F#TITLE:The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10639137# AUTHORS:Kobayashi S, Morimoto K, Shimizu T, Takahashi M, Kurosawa H, Shirasawa T#TITLE:Association of EXT1 and EXT2, hereditary multiple exostoses gene products, in Golgi apparatus#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679296# AUTHORS:Ahn J, Ludecke HJ, Lindow S, Horton WA, Lee B, Wagner MJ, Horsthemke B, Wells DE#TITLE:Cloning of the putative tumour suppressor gene for hereditary multiple exostoses (EXT1)#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7550340# AUTHORS:McCormick C, Leduc Y, Martindale D, Mattison K, Esford LE, Dyer AP, Tufaro F#TITLE:The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate#JOURNAL:Nat Genet#YEAR:1998#PUBMED:9620772# AUTHORS:Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K#TITLE:The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9756849# AUTHORS:Stickens D, Clines G, Burbee D, Ramos P, Thomas S, Hogue D, Hecht JT, Lovett M, Evans GA#TITLE:The EXT2 multiple exostoses gene defines a family of putative tumour suppressor genes#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8782816# AUTHORS:Wise CA, Clines GA, Massa H, Trask BJ, Lovett M#TITLE:Identification and localization of the gene for EXTL, a third member of the multiple exostoses gene family#JOURNAL:Genome Res#YEAR:1997#PUBMED:9037597# GENE ASSOCIATION: (2131.1 and 2132.2) or (2137.1) or (2131.1 and 2132.1) or (2134.1)
Abbreviation: R_AGTim Synonyms: _0 EC Number: 2.6.1.44 SUBSYSTEM: Alanine and Aspartate Metabolism Equation: [m] : ala-L + glx --> gly + pyr Confidence Level: 4 NCD genes: LOCUS:_64902#TRANSCRIPT:1#ABBREVIATION:AGXT2# LOCUS:_64850#TRANSCRIPT:1#ABBREVIATION:AGXT2L1# proteins: NAME:alanine-glyoxylate aminotransferase 2, mitochondrial #ABBREVIATION:Agxt2.1-m# NAME:alanine-glyoxylate aminotransferase 2-like 1, mitochondrial#ABBREVIATION:Agxt2l1.1-m# citations: AUTHORS:Holmes RP, Assimos DG#TITLE:Glyoxylate synthesis, and its modulation and influence on oxalate synthesis#JOURNAL:J Urol#YEAR:1998#PUBMED:9783918# AUTHORS:Baker PR, Cramer SD, Kennedy M, Assimos DG, Holmes RP.#TITLE:Glycolate and glyoxylate metabolism in HepG2 cells.#YEAR:2004#PUBMED:15240345# AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# GENE ASSOCIATION: (64902.1) or (64850.1)
alanine-glyoxylate transaminase (irreversible), mitochondrial
Abbreviation: R_FOLt2 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fol[e] + oh1[c] <==> fol[c] + oh1[e] Confidence Level: 4 IT genes: LOCUS:_6573#TRANSCRIPT:1#ABBREVIATION:SLC19A1# LOCUS:_6573#TRANSCRIPT:2#ABBREVIATION:SLC19A1# proteins: NAME:solute carrier family 19 (folate transporter), member 1 #ABBREVIATION:Slc19a1.1# NAME:solute carrier family 19 (folate transporter), member 1#ABBREVIATION:Slc19a1.2# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Ganapathy V, Smith SB, Prasad PD.#TITLE:SLC19: the folate/thiamine transporter family.#JOURNAL:Pflugers Arch. #YEAR:2004#PUBMED:14770311# AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION: (6573.1) or (6573.2)
Abbreviation: R_3HPPD Synonyms: _0 EC Number: 1.1.1.59 SUBSYSTEM: Propanoate Metabolism Equation: [c] : 3hpp + nad <==> h + msa + nadh Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Ando T, Rasmussen K, Nyhan WL, Hull D. #TITLE:_3-hydroxypropionate: significance of -oxidation of propionate in patients with propionic acidemia and methylmalonic acidemia#JOURNAL:Proc Natl Acad Sci U S A#YEAR:1972#PUBMED:4507604# GENE ASSOCIATION:
Abbreviation: R_EX_ps_hs_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : ps_hs <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
phosphatidylserine (homo sapiens) exchange
Abbreviation: R_DM_avite2_LPAREN_c_RPAREN_ Synonyms: _0 Equation: [c] : avite2 --> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLACOm Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Ascorbate and Aldarate Metabolism Equation: [m] : glac + (2) h2o + nad --> glcr + (3) h + nadh Confidence Level: 1 NCD genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: AUTHORS:Marsh CA#TITLE:Biosynthesis of D-glucaric acid in mammals: a free-radical mechanism?#JOURNAL:Carbohydr Res#YEAR:1986#PUBMED:3779687# GENE ASSOCIATION: (8659.2) or (217.1) or (8659.1) or (219.1)
D-Glucuronolactone:NAD+ oxidoreductase, mitochondrial
Abbreviation: R_AKR1D2 Synonyms: _4 SUBSYSTEM: Bile Acid Biosynthesis Equation: [c] : h + nadph + xoldiolone --> nadp + xoldioloneh Confidence Level: 4 refs: uniprot, KEGG DW Russell bile acid synthesis review paper (see human recon journal citations) NJ genes: LOCUS:_6718#TRANSCRIPT:1#ABBREVIATION:AKR1D1#ECNUMBERS:1.3.99.6# proteins: NAME:aldo-keto reductase (delta 4-3-ketosteroid-5-beta-reductase)#ABBREVIATION:Akr1d1.1# citations: AUTHORS:Russell DW#TITLE:The enzymes, regulation, and genetics of bile acid synthesis#JOURNAL:Annu Rev Biochem#YEAR:2003# GENE ASSOCIATION: (6718.1)
Abbreviation: R_ADPGLC Synonyms: _0 EC Number: 3.6.1.21 SUBSYSTEM: Nucleotides Equation: [c] : adpglc + h2o --> amp + g1p + (2) h Confidence Level: 4 Based on Yang et al, JBC, 2000, 275, 8844-8853 genes: LOCUS:_11164#TRANSCRIPT:1#ABBREVIATION:NUDT5# proteins: NAME:nudix (nucleoside diphosphate linked moiety X)-type motif 5 #ABBREVIATION:NudT5.1# citations: AUTHORS:Yang H, Slupska MM, Wei YF, Tai JH, Luther WM, Xia YR, Shih DM, Chiang JH, Baikalov C, Fitz-Gibbon S, Phan IT, Conrad A, Miller JH.#TITLE:Cloning and characterization of a new member of the Nudix hydrolases from human and mouse.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10722730# AUTHORS:Ishibashi T, Hayakawa H, Sekiguchi M.#TITLE:A novel mechanism for preventing mutations caused by oxidation of guanine nucleotides.#JOURNAL:EMBO Rep.#YEAR:2003#PUBMED:12717453# AUTHORS:Gasmi L, Cartwright JL, McLennan AG. #TITLE:Cloning, expression and characterization of YSA1H, a human adenosine 5'-diphosphosugar pyrophosphatase possessing a MutT motif.#JOURNAL:Biochem J.#YEAR:1999#PUBMED:10567213# GENE ASSOCIATION: (11164.1)
Abbreviation: R_DNDPt22m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dtdp[c] + dudp[m] --> dtdp[m] + dudp[c] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_EX_mepi_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : mepi <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HEX10 Synonyms: _0 EC Number: 2.7.1.1 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : atp + gam --> adp + gam6p + h Confidence Level: 1 D-Glucose, D-mannose, D-fructose, sorbitol and D-glucosamine can act as acceptors; ITP and dATP can act as donors. The liver isoenzyme has sometimes been called glucokinase. genes: LOCUS:_3098#TRANSCRIPT:1#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3101#TRANSCRIPT:1#ABBREVIATION:HK3#ECNUMBERS:2.7.1.1# LOCUS:_3099#TRANSCRIPT:1#ABBREVIATION:HK2#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:1#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# LOCUS:_80201#TRANSCRIPT:1#ABBREVIATION:FLJ22761# LOCUS:_3098#TRANSCRIPT:4#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:3#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:5#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_3098#TRANSCRIPT:2#ABBREVIATION:HK1#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:2#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# LOCUS:_2645#TRANSCRIPT:3#ABBREVIATION:GCK#ECNUMBERS:2.7.1.1# proteins: NAME:glucokinase (hexokinase 4) (pancreatic beta islet)#ABBREVIATION:Gck.1# NAME:glucokinase (hexokinase 4) (major liver isoform)#ABBREVIATION:Gck.2# NAME:glucokinase (hexokinase 4) (minor liver isoform)#ABBREVIATION:Gck.3# NAME:hexokinase 1 (ubiquitous)#ABBREVIATION:Hk1.1# NAME:hexokinase 1 (erythrocyte)#ABBREVIATION:Hk1.2# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.3# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.4# NAME:hexokinase 1 (testis)#ABBREVIATION:Hk1.5# NAME:hexokinase 2 (sk muscle)#ABBREVIATION:Hk2.1# NAME:hexokinase 3 (liver, lung, placenta)#ABBREVIATION:Hk3.1# NAME:hypothetical protein FLJ22761#ABBREVIATION:Flj22761.1# citations: none GENE ASSOCIATION: (2645.1) or (3099.1) or (3098.3) or (2645.2) or (80201.1) or (3098.2) or (2645.3) or (3098.1) or (3098.4) or (3101.1) or (3098.5)
Abbreviation: R_FAOXC180x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : coa + h2o + nad + o2 + stcoa --> accoa + h + h2o2 + nadh + pmtcoa Confidence Level: 4 - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (51.1 and 3295.1 and 30.1) or (51.1 and 30.1 and 1962.1) or (30.1 and 51.2 and 1962.1) or (3295.1 and 30.1 and 51.2)
aldo-keto reductase family 1, member C1 (chlordecone reductase; 3-alpha hydroxysteroid dehydrogenase, type I; dihydrodiol dehydrogenase 4)
Abbreviation: R_AKR1C1 Synonyms: _3 SUBSYSTEM: Steroid Metabolism Equation: [c] : h + nadph + prgstrn --> aprgstrn + nadp Confidence Level: 4 NJ genes: LOCUS:_1645#TRANSCRIPT:1#ABBREVIATION:AKR1C1#ECNUMBERS:1.3.1.20# proteins: NAME:aldo-keto reductase family 1, member C1#ABBREVIATION:Akr1c1.1# citations: AUTHORS:Stolz A, Hammond L, Lou H, Takikawa H, Ronk M, Shively JE.#TITLE:cDNA cloning and expression of the human hepatic bile acid-binding protein. A member of the monomeric reductase gene family.#JOURNAL:J Biol Chem#YEAR:1993#PUBMED:8486699# GENE ASSOCIATION: (1645.1)
Abbreviation: R_B3GALT43g Synonyms: _7 EC Number: 2.4.1.62 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : gd2_hs + udpgal --> gd1b_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_8705#TRANSCRIPT:1#ABBREVIATION:B3GALT4# proteins: NAME:UDP-Gal:betaGlcNAc beta 1,3-galactosyltransferase, polypeptide 4#ABBREVIATION:B3galt4.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# AUTHORS:Amado M, Almeida R, Carneiro F, Levery ST, Holmes EH, Nomoto M, Hollingsworth MA, Hassan H, Schwientek T, Nielsen PA, Bennett EP, Clausen H#TITLE:A family of human beta3-galactosyltransferases#JOURNAL:The Journal of Biological Chemistry#YEAR:1998# GENE ASSOCIATION: (8705.1)
Abbreviation: R_S6TASE7ly Synonyms: _7 EC Number: 3.1.6.4 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_d_deg4 + h2o <==> cs_d_deg5 + h + so4 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1)
Abbreviation: R_ADPMAN Synonyms: _0 EC Number: 3.6.1.21 SUBSYSTEM: Nucleotides Equation: [c] : adpman + h2o --> amp + (2) h + man1p Confidence Level: 4 Based on Yang et al, JBC, 2000, 275, 8844-8853 IT genes: LOCUS:_11164#TRANSCRIPT:1#ABBREVIATION:NUDT5# proteins: NAME:nudix (nucleoside diphosphate linked moiety X)-type motif 5 #ABBREVIATION:NudT5.1# citations: AUTHORS:Yang H, Slupska MM, Wei YF, Tai JH, Luther WM, Xia YR, Shih DM, Chiang JH, Baikalov C, Fitz-Gibbon S, Phan IT, Conrad A, Miller JH.#TITLE:Cloning and characterization of a new member of the Nudix hydrolases from human and mouse.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10722730# AUTHORS:Ishibashi T, Hayakawa H, Sekiguchi M.#TITLE:A novel mechanism for preventing mutations caused by oxidation of guanine nucleotides.#JOURNAL:EMBO Rep.#YEAR:2003#PUBMED:12717453# AUTHORS:Gasmi L, Cartwright JL, McLennan AG. #TITLE:Cloning, expression and characterization of YSA1H, a human adenosine 5'-diphosphosugar pyrophosphatase possessing a MutT motif.#JOURNAL:Biochem J.#YEAR:1999#PUBMED:10567213# GENE ASSOCIATION: (11164.1)
Abbreviation: R_5AOPtm Synonyms: _0 SUBSYSTEM: Heme Biosynthesis Equation: _5aop[c] <==> 5aop[m] Confidence Level: 2 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
_5-Aminolevulinate mitochondrial transport
Abbreviation: R_FA181ACPH Synonyms: _0 EC Number: 3.1.2.14 SUBSYSTEM: Fatty Acid Metabolism Equation: [c] : h2o + octeACP <==> ACP + h + ocdcea Confidence Level: 4 NCD genes: LOCUS:_55301#TRANSCRIPT:1#ABBREVIATION:THEDC1# proteins: NAME:s-acyl fatty acid synthase thioesterase#ABBREVIATION:Thedc1.1# citations: AUTHORS:Chirala SS, Huang WY, Jayakumar A, Sakai K, Wakil SJ.#TITLE:Animal fatty acid synthase: functional mapping and cloning and expression of the domain I constituent activities.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1997#PUBMED:9159116# GENE ASSOCIATION: (55301.1)
Abbreviation: R_TMLYSOX Synonyms: _3 EC Number: 1.14.11.8 SUBSYSTEM: Lysine Metabolism Equation: [c] : akg + o2 + tmlys --> 3htmelys + co2 + succ Confidence Level: 4 MM genes: LOCUS:_55217#TRANSCRIPT:1#ABBREVIATION:TMLHE# proteins: NAME:trimethyllysine hydroxylase#ABBREVIATION:Tmlhe.1# citations: AUTHORS:Vaz,F.M. , Ofman,R. , Westinga,K. , Back,J.W. , Wanders,R.J. #TITLE:Molecular and Biochemical Characterization of Rat epsilon -N-Trimethyllysine Hydroxylase, the First Enzyme of Carnitine Biosynthesis.#YEAR:2001#PUBMED:11431483# AUTHORS:Hulse JD, Ellis SR, Henderson LM.#TITLE:Carnitine biosynthesis. beta-Hydroxylation of trimethyllysine by an alpha-ketoglutarate-dependent mitochondrial dioxygenase. #YEAR:1978#PUBMED:627563# AUTHORS:Vaz FM, Wanders RJ.#TITLE:Carnitine biosynthesis in mammals.#YEAR:2002#PUBMED:11802770# GENE ASSOCIATION: (55217.1)
Abbreviation: R_ALCD1 Synonyms: _0 SUBSYSTEM: Miscellaneous Equation: [c] : meoh + nad <==> fald + h + nadh Confidence Level: 4 genes: LOCUS:_130#TRANSCRIPT:1#ABBREVIATION:ADH6#ECNUMBERS:1.1.1.1# LOCUS:_126#TRANSCRIPT:1#ABBREVIATION:ADH1C#ECNUMBERS:1.1.1.1# LOCUS:_125#TRANSCRIPT:1#ABBREVIATION:ADH1B#ECNUMBERS:1.1.1.1# LOCUS:_128#TRANSCRIPT:1#ABBREVIATION:ADH5#ECNUMBERS:1.1.1.1# LOCUS:_127#TRANSCRIPT:1#ABBREVIATION:ADH4#ECNUMBERS:1.1.1.1# LOCUS:_124#TRANSCRIPT:1#ABBREVIATION:ADH1A#ECNUMBERS:1.1.1.1# LOCUS:_131#TRANSCRIPT:1#ABBREVIATION:ADH7#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:1#ABBREVIATION:ZADH2# LOCUS:_137872#TRANSCRIPT:1#ABBREVIATION:ADHFE1#ECNUMBERS:1.1.1.1# LOCUS:_284273#TRANSCRIPT:2#ABBREVIATION:ZADH2# proteins: NAME:alcohol dehydrogenase IV #ABBREVIATION:Adh4# NAME:alcohol dehydrogenase V#ABBREVIATION:Adh5# NAME:alcohol dehydrogenase 1A (class I), alpha polypeptide#ABBREVIATION:Adh1a.1# NAME:alcohol dehydrogenase IB (class I), beta polypeptide#ABBREVIATION:Adh1b.1# NAME:alcohol dehydrogenase 1C (class I), gamma polypeptide#ABBREVIATION:Adh1c.1# NAME:alcohol dehydrogenase 6 (class V)#ABBREVIATION:Adh6.1# NAME:alcohol dehydrogenase 7 (class IV), mu or sigma polypeptide#ABBREVIATION:Adh7.1# NAME:alcohol dehydrogenase, iron containing, 1 (liver)#ABBREVIATION:Adhfe1.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ab.1# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1ac# NAME:alcohol dehydrogenase#ABBREVIATION:Adh1bc# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.1# NAME:zinc binding alcohol dehydrogenase#ABBREVIATION:Zadh2.2# citations: AUTHORS:Deng Y, Wang Z, Gu S, Ji C, Ying K, Xie Y, Mao Y#TITLE:Cloning and characterization of a novel human alcohol dehydrogenase gene (ADHFe1)#JOURNAL:DNA Seq#YEAR:2002#PUBMED:12592711# AUTHORS:Ramchandani VA, Bosron WF, Li TK#TITLE:Research advances in ethanol metabolism#JOURNAL:Pathol Biol (Paris)#YEAR:2001#PUBMED:11762128# AUTHORS:Salway, JG#TITLE:Metabolism at a Glance, 2nd ed#YEAR:1999# AUTHORS:Dawidek-Pietryka K, Szczepaniak S, Dudka J, Mazur M#TITLE:In vitro studies of human alcohol dehydrogenase inhibition in the process of methanol and ethylene glycol oxidation#JOURNAL:Arch Toxicol#YEAR:1998#PUBMED:9806434# AUTHORS:Abramson S, Singh AK.#TITLE:Treatment of the alcohol intoxications: ethylene glycol, methanol and isopropanol.#JOURNAL:Curr Opin Nephrol Hypertens#YEAR:2000#PUBMED:11128434# GENE ASSOCIATION: (131.1) or (130.1) or (126.1 and 124.1) or (128.1) or (125.1) or (137872.1) or (125.1 and 124.1) or (284273.1) or (124.1) or (125.1 and 126.1) or (284273.2) or (127.1) or (126.1)
Abbreviation: R_UAG4E Synonyms: _0 EC Number: 5.1.3.7 SUBSYSTEM: Aminosugar Metabolism Equation: [c] : uacgam <==> udpacgal Confidence Level: 2 NCD genes: LOCUS:_2582#TRANSCRIPT:1#ABBREVIATION:GALE#ECNUMBERS:5.1.3.2# LOCUS:_2582#TRANSCRIPT:2#ABBREVIATION:GALE#ECNUMBERS:5.1.3.2# proteins: NAME:UDP-galactose-4-epimerase #ABBREVIATION:Gale.1# NAME:UDP-galactose-4-epimerase #ABBREVIATION:Gale.2# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# GENE ASSOCIATION: (2582.1) or (2582.2)
Abbreviation: R_EX_4nphsf_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 4nphsf <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
nucleoside-diphosphatase (UDP), Golgi apparatus
Abbreviation: R_NDP7g Synonyms: _0 EC Number: 3.6.1.6 SUBSYSTEM: Nucleotides Equation: [g] : h2o + udp --> h + pi + ump Confidence Level: 4 NCD genes: LOCUS:_9583#TRANSCRIPT:1#ABBREVIATION:LYSAL1#ECNUMBERS:3.6.1.5# proteins: NAME:ectonucleoside triphosphate diphosphohydrolase 4, Golgi apparatus#ABBREVIATION:Entpd4.1-g# citations: AUTHORS:Wang TF, Guidotti G#TITLE:Golgi localization and functional expression of human uridine diphosphatase#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9556635# AUTHORS:Biederbick A, Kosan C, Kunz J, Elsasser HP#TITLE:First apyrase splice variants have different enzymatic properties.#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10858452# AUTHORS:Biederbick A, Rose S, Elsasser HP#TITLE:A human intracellular apyrase-like protein, LALP70, localizes to lysosomal/autophagic vacuoles#JOURNAL:J Cell Sci#YEAR:1999#PUBMED:10393803# GENE ASSOCIATION: (9583.1)
Abbreviation: R_HSD11B1r Synonyms: _4 SUBSYSTEM: Steroid Metabolism Equation: [r] : cortsn + h + nadph <==> crtsl + nadp Confidence Level: 4 NJ genes: LOCUS:_3290#TRANSCRIPT:1#ABBREVIATION:HSD11B1#ECNUMBERS:1.1.1.146# LOCUS:_3290#TRANSCRIPT:2#ABBREVIATION:HSD11B1#ECNUMBERS:1.1.1.146# proteins: NAME:hydroxysteroid (11-beta) dehydrogenase 1#ABBREVIATION:Hsd11b1.1# NAME:hydroxysteroid (11-beta) dehydrogeanse 1#ABBREVIATION:Hsd11b1.2# citations: AUTHORS:Yau JL, Seckl JR.#TITLE:_11beta-hydroxysteroid dehydrogenase type I in the brain; thickening the glucocorticoid soup.#JOURNAL:Mol Psychiatry#YEAR:2001#PUBMED:11673786# AUTHORS:Albiston AL, Obeyesekere VR, Smith RE, Krozowski ZS.#TITLE:Cloning and tissue distribution of the human 11 beta-hydroxysteroid dehydrogenase type 2 enzyme.#JOURNAL:Mol Cell Endocrinol#YEAR:1994#PUBMED:7859916# AUTHORS:Tomlinson JW, Walker EA, Bujalska IJ, Draper N, Lavery GG, Cooper MS, Hewison M, Stewart PM.#TITLE:_11beta-hydroxysteroid dehydrogenase type 1: a tissue-specific regulator of glucocorticoid response.#JOURNAL:Endocr Rev#YEAR:2004#PUBMED:15466942# GENE ASSOCIATION: (3290.1) or (3290.2)
_11-beta-hydroxysteroid dehydrogenase type 1
Abbreviation: R_THMt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: h[c] + thm[c] --> h[m] + thm[m] Confidence Level: 4 NCD genes: none proteins: none citations: AUTHORS:Song Q, Singleton CK. #TITLE:Mitochondria from cultured cells derived from normal and thiamine-responsive megaloblastic anemia individuals efficiently import thiamine diphosphate.#JOURNAL:BMC Biochem.#YEAR:2005#PUBMED:12014993# GENE ASSOCIATION:
Thiamine transport in via proton symport, mitochondrial
Abbreviation: R_EX_glu_DASH_L_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : glu-L <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NKCC2t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) cl[e] + na1[e] + nh4[e] <==> (2) cl[c] + na1[c] + nh4[c] Confidence Level: 4 NCD genes: LOCUS:_6558#TRANSCRIPT:1#ABBREVIATION:SLC12A2# LOCUS:_6557#TRANSCRIPT:1#ABBREVIATION:SLC12A1# proteins: NAME:solute carrier family 12 (sodium/potassium/chloride transporters), member 1#ABBREVIATION:Slc12a1.1-e# NAME:solute carrier family 12 (sodium/potassium/chloride transporters), member 2#ABBREVIATION:Slc12a2.1-e# citations: AUTHORS:Simon DB, Karet FE, Hamdan JM, DiPietro A, Sanjad SA, Lifton RP#TITLE:Bartter's syndrome, hypokalaemic alkalosis with hypercalciuria, is caused by mutations in the Na-K-2Cl cotransporter NKCC2#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8640224# AUTHORS:Hebert SC, Mount DB, Gamba G#TITLE:Molecular physiology of cation-coupled Cl- cotransport: the SLC12 family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12739168# AUTHORS:Payne JA, Xu JC, Haas M, Lytle CY, Ward D, Forbush B 3rd#TITLE:Primary structure, functional expression, and chromosomal localization of the bumetanide-sensitive Na-K-Cl cotransporter in human colon#JOURNAL:J Biol Chem#YEAR:1995#PUBMED:7629105# AUTHORS:Russell JM#TITLE:Sodium-potassium-chloride cotransport#JOURNAL:Physiol Rev#YEAR:2000#PUBMED:10617769# AUTHORS:Bergeron MJ, Gagnon E, Wallendorff B, Lapointe JY, Isenring P.#TITLE:Ammonium transport and pH regulation by K(+)-Cl(-) cotransporters#JOURNAL:Am J Physiol Renal Physiol#YEAR:2003#PUBMED:12657561# GENE ASSOCIATION: (6557.1) or (6558.1)
Abbreviation: R_EX_tchola_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : tchola <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_LCTStg Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lcts[g] --> lcts[e] Confidence Level: 2 this reaciton is only found in lactating mammary cells NCD genes: none proteins: none citations: AUTHORS:Alberts B, Johnson A, Lewis J, Raff M, Roberts K, Walter P#TITLE:Molecular Biology of the Cell, 4th ed.#YEAR:2002# GENE ASSOCIATION:
lactose transport from Golgi to extracellular (via vesicle)
Abbreviation: R_THMPPtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: (2) oh1[m] + thmpp[c] --> (2) oh1[c] + thmpp[m] Confidence Level: 4 IT genes: none proteins: none citations: AUTHORS:Song Q, Singleton CK. #TITLE:Mitochondria from cultured cells derived from normal and thiamine-responsive megaloblastic anemia individuals efficiently import thiamine diphosphate.#JOURNAL:BMC Biochem.#YEAR:2005#PUBMED:12014993# GENE ASSOCIATION:
Thiamine diphosphate transport in via anion antiport, mitochondria
Abbreviation: R_GLYt2rL Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: gly[l] + h[l] <==> gly[c] + h[c] Confidence Level: 4 NCD SAB lyso genes: LOCUS:_206358#TRANSCRIPT:1#ABBREVIATION:SLC36A1# proteins: NAME:solute carrier family 36 (proton/amino acid symporter), member 1#ABBREVIATION:Slc36a1.1# citations: AUTHORS:Sagne C, Agulhon C, Ravassard P, Darmon M, Hamon M, El Mestikawy S, Gasnier B, Giros B#TITLE:Identification and characterization of a lysosomal transporter for small neutral amino acids#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390972# AUTHORS:Boll M, Foltz M, Rubio-Aliaga I, Kottra G, Daniel H#TITLE:Functional characterization of two novel mammalian electrogenic proton-dependent amino acid cotransporters#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11959859# GENE ASSOCIATION: (206358.1)
glycine reversible transport via proton symport (lysosome)
Abbreviation: R_DESAT18_7 Synonyms: _1 EC Number: 1.14.19.1 SUBSYSTEM: Fatty acid elongation Equation: [c] : h + nadh + o2 + vacccoa --> (2) h2o + lneldccoa + nad Confidence Level: 4 for homo sapiens, noted to actually be NADH-cyt b5, using NADH at present NJ genes: LOCUS:_9415#TRANSCRIPT:1#ABBREVIATION:FADS2#ECNUMBERS:1.14.99.25# proteins: NAME:fatty acid desaturase 2#ABBREVIATION:Fads2.1# citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Horrobin DF#TITLE:Omega-6 Essential Fatty Acids#YEAR:1990# AUTHORS:Leonard AE, Bobik EG, Dorado J, Kroeger PE, Chuang LT, Thurmond JM, Parker-Barnes JM, Das T, Huang YS, Mukerji P#TITLE:Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids#JOURNAL:Biochem J#YEAR:2000# AUTHORS:Cho HP, Nakamura MT, Clarke SD#TITLE:Cloning, expression, and nutritional regulation of the mammalian delta-6 desaturase#JOURNAL:The Journal of Biological Chemistry#YEAR:1999# AUTHORS:Pereira SL, Leonard AE, Mukerji P#TITLE:Recent advances in the study of fatty acid desaturases from animals and lower eukaryotes#JOURNAL:Prostaglandins Leukotrienes and Essential Fatty Acids#YEAR:2003# GENE ASSOCIATION: (9415.1)
Abbreviation: R_EX_estradiolglc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : estradiolglc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EBASTINEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ebastine[e] <==> ebastine[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EBASTINEtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: ebastine[r] <==> ebastine[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DINt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: din[e] <==> din[c] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION:
Abbreviation: R_THMDt5 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: (2) na1[e] + thymd[e] --> (2) na1[c] + thymd[c] Confidence Level: 4 IT genes: LOCUS:_64078#TRANSCRIPT:1#ABBREVIATION:SLC28A3# proteins: NAME:solute carrier family 28 (sodium-coupled nucleoside transporter), member 3, extracellular #ABBREVIATION:Slc28a3.1-e# citations: AUTHORS:Gray JH, Owen RP, Giacomini KM.#TITLE:The concentrative nucleoside transporter family, SLC28. #YEAR:2004#PUBMED:12856181# GENE ASSOCIATION: (64078.1)
thymidine transport (1:2 Na/Thymd cotransport)
glycoprotein 6-alpha-L-fucosyltransferase
Abbreviation: R_F6Tg Synonyms: _5 EC Number: 2.4.1.68 SUBSYSTEM: N-Glycan Biosynthesis Equation: [g] : gdpfuc + n2m2masn --> fn2m2masn + gdp + h Confidence Level: 0 NCD genes: LOCUS:_2530#TRANSCRIPT:1#ABBREVIATION:FUT8# LOCUS:_2530#TRANSCRIPT:2#ABBREVIATION:FUT8# LOCUS:_2530#TRANSCRIPT:4#ABBREVIATION:FUT8# LOCUS:_2530#TRANSCRIPT:3#ABBREVIATION:FUT8# LOCUS:_2530#TRANSCRIPT:5#ABBREVIATION:FUT8# proteins: NAME:fucosyltransferase 8 (alpha (1,6) fucosyltransferase), Golgi apparatus (ubiquitous)#ABBREVIATION:Fut8.1-g# NAME:fucosyltransferase 8 (alpha (1,6) fucosyltransferase), Golgi apparatus (ubiquitous)#ABBREVIATION:Fut8.2-g# NAME:fucosyltransferase 8 (alpha (1,6) fucosyltransferase), Golgi apparatus (ubiquitous)#ABBREVIATION:Fut8.3-g# NAME:fucosyltransferase 8 (alpha (1,6) fucosyltransferase), Golgi apparatus (ubiquitous)#ABBREVIATION:Fut8.4-g# NAME:fucosyltransferase 8 (alpha (1,6) fucosyltransferase), Golgi apparatus (ubiquitous)#ABBREVIATION:Fut8.5-g# citations: AUTHORS:Activity and tissue distribution of splice variants of alpha6-fucosyltransferase in human embryogenesis.#TITLE:Martinez-Duncker I, Michalski JC, Bauvy C, Candelier JJ, Mennesson B, Codogno P, Oriol R, Mollicone R.#JOURNAL:Glycobiology#YEAR:2004#PUBMED:14514715# GENE ASSOCIATION: (2530.4) or (2530.1) or (2530.3) or (2530.2) or (2530.5)
Abbreviation: R_P4504F81r Synonyms: _0 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : arachd + h + nadph + o2 --> 18harachd + h2o + nadp Confidence Level: 4 w-2 hydroxylation of arachidonic acid -> 19R-Hydroxyprostaglandins NJ genes: LOCUS:_11283#TRANSCRIPT:1#ABBREVIATION:CYP4F8# proteins: NAME:cytochrome P450, family 4, subfamily F, polypeptide 8#ABBREVIATION:Cyp4f8.1# citations: AUTHORS:Bylund J, Finnstrom N, Oliw EH.#TITLE:Gene expression of a novel cytochrome P450 of the CYP4F subfamily in human seminal vesicles.#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10405341# AUTHORS:Lewis DF.#TITLE:_57 varieties: the human cytochromes P450.#JOURNAL:Pharmacogenomics#YEAR:2004#PUBMED:15579107# GENE ASSOCIATION: (11283.1)
Abbreviation: R_G5SADrm Synonyms: _0 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : glu5sa <==> 1pyr5c + h + h2o Confidence Level: 4 spontaneous reaction NCD genes: none proteins: none citations: AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# GENE ASSOCIATION:
L-glutamate 5-semialdehyde dehydratase, reversible, mitochondrial
Abbreviation: R_NADtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: nad[n] <==> nad[c] Confidence Level: 2 it genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FUT33g Synonyms: _5 EC Number: 2.4.1.65 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : acgalfucgalacglcgalgluside_hs + gdpfuc --> fucacgalfucgalacglcgalgluside_hs + gdp + h Confidence Level: 4 NJ genes: LOCUS:_2525#TRANSCRIPT:1#ABBREVIATION:FUT3#ECNUMBERS:2.4.1.65# proteins: NAME:fucosyltransferase 3 (galactoside 3(4)-L-fucosyltransferase, Lewis blood group)#ABBREVIATION:Fut3.1# citations: AUTHORS:Cakir B, Pankow JS, Salomaa V, Couper D, Morris TL, Brantley KR, Hiller KM, Heiss G, Weston BW.#TITLE:Distribution of Lewis (FUT3)genotype and allele: frequencies in a biethnic United States population.#JOURNAL:Ann Hematol#YEAR:2002#PUBMED:12424536# AUTHORS:Sousa VL, Brito C, Costa T, Lanoix J, Nilsson T, Costa J.#TITLE:Importance of Cys, Gln, and Tyr from the transmembrane domain of human alpha 3/4 fucosyltransferase III for its localization and sorting in the Golgi of baby hamster kidney cells.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12493760# AUTHORS:Kukowska-Latallo JF, Larsen RD, Nair RP, Lowe JB.#TITLE:A cloned human cDNA determines expression of a mouse stage-specific embryonic antigen and the Lewis blood group alpha(1,3/1,4)fucosyltransferase.#JOURNAL:Genes Dev#YEAR:1990#PUBMED:1977660# GENE ASSOCIATION: (2525.1)
Abbreviation: R_VITD3Hm Synonyms: _0 SUBSYSTEM: Vitamin D Equation: [m] : h + nadph + o2 + vitd3 --> 25hvitd3 + h2o + nadp Confidence Level: 4 based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.196 IT genes: LOCUS:_1593#TRANSCRIPT:1#ABBREVIATION:CYP27A1# proteins: NAME:cytochrome P450, family 27, subfamily A, polypeptide 1#ABBREVIATION:Cyp27a1.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Guo YD, Strugnell S, Back DW, Jones G.#TITLE:Transfected human liver cytochrome P-450 hydroxylates vitamin D analogs at different side-chain positions.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1993#PUBMED:7690968# GENE ASSOCIATION: (1593.1)
Abbreviation: R_MELATN23DOX Synonyms: _0 EC Number: 1.13.11.42 SUBSYSTEM: Tryptophan metabolism Equation: [c] : melatn + o2 --> fna5moxam Confidence Level: 2 SAB genes: LOCUS:_3620#TRANSCRIPT:1#ABBREVIATION:INDO#ECNUMBERS:1.13.11.42# proteins: NAME:indoleamine-pyrrole 2,3 dioxygenase#ABBREVIATION:Indo.1# citations: AUTHORS:Hirata F, Hayaishi O, Tokuyama T, Seno S#TITLE:In vitro and in vivo formation of two new metabolites of melatonin#JOURNAL:J Biol Chem#YEAR:1974#PUBMED:4814344# GENE ASSOCIATION: (3620.1)
Melatonin:oxygen 2,3-dioxygenase (indole-decyclizing)
Abbreviation: R_PEPLYStn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: peplys[e] <==> peplys[n] Confidence Level: 2 MM genes: none proteins: none citations: none GENE ASSOCIATION:
peptide (lysine) nuclear transport via diffusion
Abbreviation: R_NDPK9m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + idp <==> adp + itp Confidence Level: 2 NCD genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:IDP), mitochondrial
Abbreviation: R_EX_imp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : imp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DCYTt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: dcyt[e] <==> dcyt[c] Confidence Level: 2 genes: none proteins: none citations: AUTHORS:Rampazzo C, Gallinaro L, Milanesi E, Frigimelica E, Reichard P, Bianchi V#TITLE:A deoxyribonucleotidase in mitochondria: involvement in regulation of dNTP pools and possible link to genetic disease.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10899995# GENE ASSOCIATION:
Abbreviation: R_EX_atp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : atp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FPGS6m Synonyms: _0 EC Number: 6.3.2.17 SUBSYSTEM: Folate Metabolism Equation: [m] : 6dhf + atp + glu-L --> 7dhf + adp + h + pi Confidence Level: 4 IT genes: LOCUS:_2356#TRANSCRIPT:1#ABBREVIATION:FPGS#ECNUMBERS:6.3.2.17# proteins: NAME:folylpolyglutamate synthase #ABBREVIATION:Fpgs.1-m# citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:McGuire JJ, Russell CA, Balinska M.#TITLE:Human cytosolic and mitochondrial folylpolyglutamate synthetase are electrophoretically distinct. Expression in antifolate-sensitive and -resistant human cell lines.#JOURNAL: J Biol Chem.#YEAR:2000#PUBMED:10777604# GENE ASSOCIATION: (2356.1)
Abbreviation: R_DPPS Synonyms: _1 SUBSYSTEM: Miscellaneous Equation: [c] : frdp + (7) ipdp --> decdp + (7) ppi Confidence Level: 2 by analogy to other dimethyl transferase rxns. See PMID: 10586494, human interemidate (from C15 ->C50) not explicitly determined yet, however it is known to occur since this pathway is essential in Q10 synthesis. For human metabolism: may be associated w/ GeneID: 23590 TPRT - recheck lit in future IT and NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NAGAlby Synonyms: _3 SUBSYSTEM: Sphingolipid Metabolism Equation: [l] : acgbgbside_hs + h + udp --> gbside_hs + udpacgal Confidence Level: 1 Beta-galactosidase B - no evidence to support this actually happens yet; added by analogy with alpha-galactosidase B (NAGAIy) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_It Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: i[e] <==> i[c] Confidence Level: 4 NCD genes: LOCUS:_160728#TRANSCRIPT:1#ABBREVIATION:SLC5A8# proteins: NAME:solute carrier family 5 (iodide transporter), member 8, extracellular#ABBREVIATION:Slc5a8.1-e# citations: AUTHORS:Rodriguez AM, Perron B, Lacroix L, Caillou B, Leblanc G, Schlumberger M, Bidart JM, Pourcher T#TITLE:Identification and characterization of a putative human iodide transporter located at the apical membrane of thyrocytes#JOURNAL:J Clin Endocrinol Metab#YEAR:2002#PUBMED:1217270# GENE ASSOCIATION: (160728.1)
Abbreviation: R_DOLASNT_Ler Synonyms: _0 EC Number: 2.4.1.119 SUBSYSTEM: N-Glycan Biosynthesis Equation: [r] : Asn-X-Ser/Thr + (0.1) g3m8mpdol_L --> (0.1) doldp_L + g3m8masn + h Confidence Level: 0 NCD genes: LOCUS:_6185#TRANSCRIPT:1#ABBREVIATION:RPN2# LOCUS:_6184#TRANSCRIPT:1#ABBREVIATION:RPN1# LOCUS:_1650#TRANSCRIPT:1#ABBREVIATION:DDOST# LOCUS:_1603#TRANSCRIPT:1#ABBREVIATION:DAD1# proteins: NAME:oligosaccharyltransferase complex, endoplasmic reticulum (ubiquitous)#ABBREVIATION:Ost-er# citations: none GENE ASSOCIATION: (6185.1 and 1650.1 and 6184.1 and 1603.1)
Dolichyl-diphosphooligosaccharide:protein-L-asparagine oligopolysaccharidotransferase (liver)
Abbreviation: R_RETI2 Synonyms: _0 EC Number: 5.2.1.7 SUBSYSTEM: Vitamin A Metabolism Equation: [c] : retinol <==> retinol-9-cis Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Blomhoff R, Green MH, Norum KR.#TITLE:Vitamin A: physiological and biochemical processing. #JOURNAL:Annu Rev Nutr.#YEAR:1992#PUBMED:1503811# GENE ASSOCIATION:
Abbreviation: R_ST6GALNAC25 Synonyms: _5 SUBSYSTEM: Sphingolipid Metabolism Equation: [g] : cmpacna + gd1alpha_hs --> cmp + gt1alpha_hs + h Confidence Level: 2 NJ genes: LOCUS:_10610#TRANSCRIPT:1#ABBREVIATION:SIAT7B#ECNUMBERS:2.4.99.7# proteins: NAME:ST6 (alpha-N-acetyl-neuraminyl-2,3-beta-galactosyl-1,3)-N-acetylgalactosaminide alpha-2,6-sialyltransferase 2#ABBREVIATION:St6galnac2.1# citations: AUTHORS:Kolter T, Sandhoff K#TITLE:Recent advances in the biochemistry of sphingolipidoses#JOURNAL:Brain Pathology#YEAR:1998# GENE ASSOCIATION: (10610.1)
Abbreviation: R_GLCAASE8ly Synonyms: _3 EC Number: 3.2.1.31 SUBSYSTEM: Hyaluronan Metabolism Equation: [l] : h2o + ha --> glcur + ha_deg1 Confidence Level: 4 NCD genes: LOCUS:_2990#TRANSCRIPT:1#ABBREVIATION:GUSB#ECNUMBERS:3.2.1.31# proteins: NAME:glucuronidase, beta, lysosomal#ABBREVIATION:Gusb.1-l# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Guise KS, Korneluk RG, Waye J, Lamhonwah AM, Quan F, Palmer R, Ganschow RE, Sly WS, Gravel RA#TITLE:Isolation and expression in Escherichia coli of a cDNA clone encoding human beta-glucuronidase#JOURNAL:Gene#YEAR:1985#PUBMED:3924735# AUTHORS:Ho YC, Ho LH, Ho KJ#TITLE:Human hepatic beta-glucuronidase: an enzyme kinetic study#JOURNAL:Enzyme#YEAR:1985#PUBMED:3987656# AUTHORS:Oshima A, Kyle JW, Miller RD, Hoffmann JW, Powell PP, Grubb JH, Sly WS, Tropak M, Guise KS, Gravel RA#TITLE:Cloning, sequencing, and expression of cDNA for human beta-glucuronidase#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:1987#PUBMED:3468507# GENE ASSOCIATION: (2990.1)
Abbreviation: R_PUNP7 Synonyms: _0 EC Number: 2.4.2.1 SUBSYSTEM: Nucleotides Equation: [c] : pi + xtsn <==> r1p + xan Confidence Level: 4 Specificity not completely determined. Can also catalyse ribosyltransferase reactions of the type catalysed by EC 2.4.2.5, nucleoside ribosyltransferasextsn genes: LOCUS:_4860#TRANSCRIPT:1#ABBREVIATION:NP#ECNUMBERS:3.4.2.1# proteins: NAME:nucleoside phosphorylase #ABBREVIATION:Np.1# citations: AUTHORS:Erion MD, Stoeckler JD, Guida WC, Walter RL, Ealick SE.#TITLE:Purine nucleoside phosphorylase. 2. Catalytic mechanism. #JOURNAL:Biochemistry#YEAR:1997#PUBMED:9305963# AUTHORS:Williams SR, Goddard JM, Martin DW Jr. #TITLE:Human purine nucleoside phosphorylase cDNA sequence and genomic clone characterization.#JOURNAL:Nucleic Acids Res.#YEAR:1984#PUBMED:6087295# GENE ASSOCIATION: (4860.1)
purine-nucleoside phosphorylase (Xanthosine)
Abbreviation: R_SUCCtp Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: succ[c] <==> succ[x] Confidence Level: 0 unknown mechanism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_HCOUMARINte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: hcoumarin[e] <==> hcoumarin[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_UDPGLCter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: udpg[c] + ump[r] <==> udpg[r] + ump[c] Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Trombetta ES, Helenius A.#TITLE:Glycoprotein reglucosylation and nucleotide sugar utilization in the secretory pathway: identification of a nucleoside diphosphatase in the endoplasmic reticulum.#JOURNAL:EMBO J#YEAR:1999#PUBMED:10369669# GENE ASSOCIATION:
UDP-Glc endoplasmic reticulum transport via CMP antiport
Abbreviation: R_P5CR Synonyms: _0 EC Number: 1.5.1.2 SUBSYSTEM: Urea cycle/amino group metabolism Equation: [c] : 1pyr5c + (2) h + nadph --> nadp + pro-L Confidence Level: 4 genes: LOCUS:_65263#TRANSCRIPT:1#ABBREVIATION:PYCRL# LOCUS:_29920#TRANSCRIPT:1#ABBREVIATION:PYCR2# proteins: NAME:pyrroline-5-carboxylate reductase family, member 2#ABBREVIATION:Pycr2.1# NAME:pyrroline-5-carboxylate reductase-like#ABBREVIATION:Pycrl.1# citations: AUTHORS:Jeremy M. Berg, John L. Tymoczko, Lubert Stryer, Neil D. Clarke#TITLE:Biochemistry#YEAR:2002# AUTHORS:Merrill MJ, Yeh GC, Phang JM.#TITLE:Purified human erythrocyte pyrroline-5-carboxylate reductase. Preferential oxidation of NADPH.#JOURNAL: J Biol Chem#YEAR:1989#PUBMED:2722838# GENE ASSOCIATION: (29920.1) or (65263.1)
Abbreviation: R_EX_hspg_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : hspg <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_DOLGLCP_Uter Synonyms: _0 SUBSYSTEM: N-Glycan Biosynthesis Equation: (0.1) dolglcp_U[c] --> (0.1) dolglcp_U[r] Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Helenius J, Aebi M.#TITLE:Transmembrane movement of dolichol linked carbohydrates during N-glycoprotein biosynthesis in the endoplasmic reticulum#JOURNAL:Semin Cell Dev Biol#YEAR:2002#PUBMED:12137737# GENE ASSOCIATION:
Dolichyl beta-D-glucosyl phosphate flippase (uterus)
Abbreviation: R_AHANDROSTANGLCtr Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: ahandrostanglc[c] <==> ahandrostanglc[r] Confidence Level: 2 unknown mechanism, however glucuronidated metabolites need to get out of the cell NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_GLYCK2 Synonyms: _7 EC Number: 2.7.1.31 SUBSYSTEM: Glyoxylate and Dicarboxylate Metabolism Equation: [c] : atp + glyc-R --> 2pg + adp + h Confidence Level: 2 JLR genes: none proteins: none citations: AUTHORS:Poore RE, Hurst CH, Assimos DG, Holmes RP#TITLE:Pathways of hepatic oxalate synthesis and their regulation#JOURNAL:Am J Physiol.#YEAR:1997#PUBMED:9038835# GENE ASSOCIATION:
Abbreviation: R_S4T3g Synonyms: _1 EC Number: 2.8.2.5 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : cs_e_pre4 + paps --> cs_e_pre5a + h + pap Confidence Level: 4 NCD genes: LOCUS:_50515#TRANSCRIPT:1#ABBREVIATION:CHST11# LOCUS:_55501#TRANSCRIPT:1#ABBREVIATION:CHST12# LOCUS:_166012#TRANSCRIPT:1#ABBREVIATION:CHST13# LOCUS:_113189#TRANSCRIPT:1#ABBREVIATION:D4ST1# proteins: NAME:carbohydrate (chondroitin 4) sulfotransferase 11, Golgi#ABBREVIATION:Chst11.1-g# NAME:carbohydrate (chondroitin 4) sulfotransferase 12, Golgi apparatus#ABBREVIATION:Chst12.1-g# NAME:carbohydrate (chondroitin 4) sulfotransferase 13, Golgi apparatus#ABBREVIATION:Chst13.1-g# NAME:dermatan 4 sulfotransferase 1, Golgi apparatus#ABBREVIATION:D4st.1-g# citations: AUTHORS:Silbert JE, Sugumaran G#TITLE:Biosynthesis of chondroitin/dermatan sulfate#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512856# AUTHORS:Yamauchi S, Mita S, Matsubara T, Fukuta M, Habuchi H, Kimata K, Habuchi O#TITLE:Molecular cloning and expression of chondroitin 4-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10722746# AUTHORS:Okuda T, Mita S, Yamauchi S, Matsubara T, Yagi F, Yamamori D, Fukuta M, Kuroiwa A, Matsuda Y, Habuchi O#TITLE:Molecular cloning, expression, and chromosomal mapping of human chondroitin 4-sulfotransferase, whose expression pattern in human tissues is different from that of chondroitin 6-sulfotransferase#JOURNAL:J Biochem (Tokyo)#YEAR:2000#PUBMED:11056388# AUTHORS:Hiraoka N, Nakagawa H, Ong E, Akama TO, Fukuda MN, Fukuda M#TITLE:Molecular cloning and expression of two distinct human chondroitin 4-O-sulfotransferases that belong to the HNK-1 sulfotransferase gene family#JOURNAL:J Biol Chem#YEAR:2000#PUBMED:10781601# AUTHORS:Kang HG, Evers MR, Xia G, Baenziger JU, Schachner M#TITLE:Molecular cloning and characterization of chondroitin-4-O-sulfotransferase-3. A novel member of the HNK-1 family of sulfotransferases#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:12080076# AUTHORS:Evers MR, Xia G, Kang HG, Schachner M, Baenziger JU#TITLE:Molecular cloning and characterization of a dermatan-specific N-acetylgalactosamine 4-O-sulfotransferase#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:1147079# AUTHORS:Mikami T, Mizumoto S, Kago N, Kitagawa H, Sugahara K.#TITLE:Specificities of three distinct human chondroitin/dermatan N-acetylgalactosamine 4-O-sulfotransferases demonstrated using partially desulfated dermatan sulfate as an acceptor: implication of differential roles in dermatan sulfate biosynthesis.#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12847091# GENE ASSOCIATION: (55501.1) or (166012.1) or (50515.1) or (113189.1)
Abbreviation: R_S6TASE16ly Synonyms: _5 EC Number: 3.1.6.14 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg21 <==> h + ksi_deg22 + so4 Confidence Level: 4 NCD genes: LOCUS:_2799#TRANSCRIPT:1#ABBREVIATION:GNS#ECNUMBERS:3.1.6.14# proteins: NAME:glucosamine (N-acetyl)-6-sulfatase (Sanfilippo disease IIID), lysosomal#ABBREVIATION:Gns.1-l# citations: AUTHORS:Robertson DA, Freeman C, Nelson PV, Morris CP, Hopwood JJ#TITLE:Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3196333# AUTHORS:Freeman C, Clements PR, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689314# AUTHORS:Freeman C, Hopwood JJ#TITLE:Human liver N-acetylglucosamine-6-sulphate sulphatase. Catalytic properties#JOURNAL:Biochem J#YEAR:1987#PUBMED:3689315# GENE ASSOCIATION: (2799.1)
Abbreviation: R_CBL2tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: cbl2[c] --> cbl2[m] Confidence Level: 2 IT genes: LOCUS:_166785#TRANSCRIPT:1#ABBREVIATION:MMAA# proteins: NAME:methylmalonic aciduria (cobalamin deficiency) type A #ABBREVIATION:Mmaa1.1-m# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# AUTHORS:Seetharam B, Alpers DH.#TITLE:Absorption and transport of cobalamin (vitamin B12).#JOURNAL:Annu Rev Nutr.#YEAR:1982#PUBMED:6313022# AUTHORS:Carmel R, Green R, Rosenblatt DS, Watkins D.#TITLE:Update on cobalamin, folate, and homocysteine.#JOURNAL:Hematology (Am Soc Hematol Educ Program).#YEAR:2003#PUBMED:14633777# AUTHORS:Dobson CM, Wai T, Leclerc D, Wilson A, Wu X, Dore C, Hudson T, Rosenblatt DS, Gravel RA.#TITLE:Identification of the gene responsible for the cblA complementation group of vitamin B12-responsive methylmalonic acidemia based on analysis of prokaryotic gene arrangements. #JOURNAL:Proc Natl Acad Sci U S A. #YEAR:2002#PUBMED:12438653# GENE ASSOCIATION: (166785.1)
Abbreviation: R_TYRDOPO Synonyms: _1 EC Number: 1.14.18.1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : o2 + (2) tyr-L --> (2) 34dhphe Confidence Level: 4 SAB genes: LOCUS:_7299#TRANSCRIPT:1#ABBREVIATION:TYR#ECNUMBERS:1.14.18.1# proteins: NAME:tyrosinase#ABBREVIATION:Tyr.1# citations: AUTHORS:Aaron Bunsen Lerner, Thomas B. Fitzpatrick, Evan Calkins, and William H. Summerson#TITLE:MAMMALIAN TYROSINASE: PREPARATION AND PROPERTIES#JOURNAL:J. Biol. Chem#YEAR:1949# AUTHORS:Pomerantz SH#TITLE:The tyrosine hydroxylase activity of mammalian tyrosinase#JOURNAL:J Biol Chem#YEAR:1968#PUBMED:5294951# GENE ASSOCIATION: (7299.1)
Abbreviation: R_TRIODTHYSULT Synonyms: _0 EC Number: 2.8.2.1 SUBSYSTEM: Tyrosine metabolism Equation: [c] : paps + triodthy --> h + pap + triodthysuf Confidence Level: 4 RS/TV (6/14/05) genes: LOCUS:_6817#TRANSCRIPT:1#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:2#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:3#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:4#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# LOCUS:_6817#TRANSCRIPT:5#ABBREVIATION:SULT1A1#ECNUMBERS:2.8.2.1# proteins: NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.1# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.2# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.3# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.4# NAME:Sulfotransferase family, cytosolic, 1A, phenol-preferring, member 1#ABBREVIATION:Sult1a1.5# citations: AUTHORS:Chapman E, Best MD, Hanson SR, Wong CH.#TITLE:Sulfotransferases: structure, mechanism, biological activity, inhibition, and synthetic utility.#JOURNAL:Angew Chem Int Ed Engl#YEAR:2004#PUBMED:15293241# AUTHORS:Glatt H, Boeing H, Engelke CE, Ma L, Kuhlow A, Pabel U, Pomplun D, Teubner W, Meinl W.#TITLE:Human cytosolic sulphotransferases: genetics, characteristics, toxicological aspects.#JOURNAL:Mutat Res#YEAR:2001#PUBMED:11535246# GENE ASSOCIATION: (6817.4) or (6817.1) or (6817.3) or (6817.2) or (6817.5)
Abbreviation: R_LTC4Sr Synonyms: _2 SUBSYSTEM: Eicosanoid Metabolism Equation: [r] : gthrd + leuktrA4 --> leuktrC4 Confidence Level: 4 previous EC 2.5.1.37 EC: 4.4.1.20 NJ genes: LOCUS:_4259#TRANSCRIPT:1#ABBREVIATION:MGST3# LOCUS:_4258#TRANSCRIPT:1#ABBREVIATION:MGST2# LOCUS:_4056#TRANSCRIPT:1#ABBREVIATION:LTC4S#ECNUMBERS:2.5.1.37# LOCUS:_4056#TRANSCRIPT:2#ABBREVIATION:LTC4S#ECNUMBERS:2.5.1.37# proteins: NAME:leukotriene C4 synthase#ABBREVIATION:Ltc4s.1# NAME:microsomal glutathione S-transferase 2#ABBREVIATION:Mgst2.1-r# NAME:microsomal glutathione S-transferase 3#ABBREVIATION:Mgst3.1-r# NAME:leukotriene C4 synthase#ABBREVIATION:Ltc4s.2# citations: TITLE:Goodman & Gilman's the pharmacological basis of therapeutics#YEAR:2001# AUTHORS:Leff AR.#TITLE:Regulation of leukotrienes in the management of asthma: biology and clinical therapy.#JOURNAL:Annu Rev Med#YEAR:2001#PUBMED:11160764# GENE ASSOCIATION: (4056.1) or (4259.1) or (4056.2) or (4258.1)
gulonate dehydrogenase, endoplasmic reticulum
Abbreviation: R_GULNDer Synonyms: _1 EC Number: 1.1.1.19 SUBSYSTEM: Pentose and Glucuronate Interconversions Equation: [r] : glcur + h + nadph <==> guln + nadp Confidence Level: 2 NCD genes: none proteins: none citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Orten JM, Neuhaus OW.#TITLE:Human Biochemistry#YEAR:1975# AUTHORS:Banhegyi G, Braun L, Csala M, Puskas F, Mandl J#TITLE:Ascorbate metabolism and its regulation in animals#JOURNAL:Free Radic Biol Med#YEAR:1997#PUBMED:9296457# GENE ASSOCIATION:
Abbreviation: R_ESTRONESt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: estrones[e] + hco3[c] <==> estrones[c] + hco3[e] Confidence Level: 4 NJ genes: LOCUS:_11309#TRANSCRIPT:1#ABBREVIATION:SLCO2B1# LOCUS:_53919#TRANSCRIPT:1#ABBREVIATION:SLCO1C1# LOCUS:_28234#TRANSCRIPT:1#ABBREVIATION:SLCO1B3# LOCUS:_6579#TRANSCRIPT:1#ABBREVIATION:SLCO1A2# LOCUS:_28231#TRANSCRIPT:1#ABBREVIATION:SLCO4A1# LOCUS:_28232#TRANSCRIPT:1#ABBREVIATION:SLCO3A1# LOCUS:_10599#TRANSCRIPT:1#ABBREVIATION:SLCO1B1# proteins: NAME:solute carrier organic anion transporter family, member 1A2#ABBREVIATION:Slco1a2.1# NAME:solute carrier organic anion transporter family, member 1B1#ABBREVIATION:Slco1b1.1# NAME:solute carrier organic anion transporter family, member 1B3#ABBREVIATION:Slco1b3.1# NAME:solute carrier organic anion transporter family, member 2B1#ABBREVIATION:Slco2b1.1# NAME:solute carrier organic anion transporter family, member 3A1#ABBREVIATION:Slco3a1.1# NAME:solute carrier organic anion transporter family, member 4A1#ABBREVIATION:Slco4a1.1# NAME:solute carrier organic anion transporter family, member 1C1#ABBREVIATION:Slco1c1.1# citations: AUTHORS:Kullak-Ublick GA, Beuers U, Meier PJ, Domdey H, Paumgartner G.#TITLE:Assignment of the human organic anion transporting polypeptide (OATP) gene to chromosome 12p12 by fluorescence in situ hybridization.#JOURNAL:J Hepatol.#YEAR:1996#PUBMED:9007731# AUTHORS:Hagenbuch B, Meier PJ.#TITLE:The superfamily of organic anion transporting polypeptides.#JOURNAL:Biochim Biophys Acta.#YEAR:2003#PUBMED:12507753# AUTHORS:Satlin LM, Amin V, Wolkoff AW.#TITLE:Organic anion transporting polypeptide mediates organic anion/HCO3- exchange.#JOURNAL:J Biol Chem.#YEAR:1997#PUBMED:9334206# GENE ASSOCIATION: (28232.1) or (28234.1) or (28231.1) or (10599.1) or (53919.1) or (6579.1) or (11309.1)
estrone 3-sulfate transport via bicarbonate countertransport
Abbreviation: R_GLCNACT4g Synonyms: _3 SUBSYSTEM: Chondroitin / heparan sulfate biosynthesis Equation: [g] : hs_pre6 + uacgam --> h + hs_pre7 + udp Confidence Level: 4 EC 2.4.1.224 NCD genes: LOCUS:_2131#TRANSCRIPT:1#ABBREVIATION:EXT1# LOCUS:_2132#TRANSCRIPT:1#ABBREVIATION:EXT2#ECNUMBERS:2.4.1.-# LOCUS:_2137#TRANSCRIPT:1#ABBREVIATION:EXTL3#ECNUMBERS:2.4.1.-# LOCUS:_2134#TRANSCRIPT:1#ABBREVIATION:EXTL1# LOCUS:_2132#TRANSCRIPT:2#ABBREVIATION:EXT2# proteins: NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.1-g# NAME:exotoses (multiple), Golgi apparatus#ABBREVIATION:Ext12.2-g# NAME:exostoses (multiple)-like 1, Golgi apparatus#ABBREVIATION:Extl1.1-g# NAME:exostoses (multiple)-like 3, Golgi apparatus#ABBREVIATION:Extl3.1-g# citations: AUTHORS:Saito T, Seki N, Yamauchi M, Tsuji S, Hayashi A, Kozuma S, Hori T#TITLE:Structure, chromosomal location, and expression profile of EXTR1 and EXTR2, new members of the multiple exostoses gene family#JOURNAL:Biochem Biophys Res Commun#YEAR:1998#PUBMED:9473480# AUTHORS:Sugahara K, Kitagawa H#TITLE:Heparin and heparan sulfate biosynthesis#JOURNAL:IUBMB Life#YEAR:2002#PUBMED:12512855# AUTHORS:Kim BT, Kitagawa H, Tamura J, Saito T, Kusche-Gullberg M, Lindahl U, Sugahara K#TITLE:Human tumor suppressor EXT gene family members EXTL1 and EXTL3 encode alpha 1,4- N-acetylglucosaminyltransferases that likely are involved in heparan sulfate/ heparin biosynthesis#JOURNAL:Proc Natl Acad Sci U S A#YEAR:2001#PUBMED:11390981# AUTHORS:Van Hul W, Wuyts W, Hendrickx J, Speleman F, Wauters J, De Boulle K, Van Roy N, Bossuyt P, Willems PJ#TITLE:Identification of a third EXT-like gene (EXTL3) belonging to the EXT gene family#JOURNAL:Genomics#YEAR:1998#PUBMED:9479495# AUTHORS:McCormick C, Duncan G, Goutsos KT, Tufaro F#TITLE:The putative tumor suppressors EXT1 and EXT2 form a stable complex that accumulates in the Golgi apparatus and catalyzes the synthesis of heparan sulfate#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2000#PUBMED:10639137# AUTHORS:Kobayashi S, Morimoto K, Shimizu T, Takahashi M, Kurosawa H, Shirasawa T#TITLE:Association of EXT1 and EXT2, hereditary multiple exostoses gene products, in Golgi apparatus#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10679296# AUTHORS:Ahn J, Ludecke HJ, Lindow S, Horton WA, Lee B, Wagner MJ, Horsthemke B, Wells DE#TITLE:Cloning of the putative tumour suppressor gene for hereditary multiple exostoses (EXT1)#JOURNAL:Nat Genet#YEAR:1995#PUBMED:7550340# AUTHORS:McCormick C, Leduc Y, Martindale D, Mattison K, Esford LE, Dyer AP, Tufaro F#TITLE:The putative tumour suppressor EXT1 alters the expression of cell-surface heparan sulfate#JOURNAL:Nat Genet#YEAR:1998#PUBMED:9620772# AUTHORS:Lind T, Tufaro F, McCormick C, Lindahl U, Lidholt K#TITLE:The putative tumor suppressors EXT1 and EXT2 are glycosyltransferases required for the biosynthesis of heparan sulfate#JOURNAL:J Biol Chem#YEAR:1998#PUBMED:9756849# AUTHORS:Stickens D, Clines G, Burbee D, Ramos P, Thomas S, Hogue D, Hecht JT, Lovett M, Evans GA#TITLE:The EXT2 multiple exostoses gene defines a family of putative tumour suppressor genes#JOURNAL:Nat Genet#YEAR:1996#PUBMED:8782816# AUTHORS:Wise CA, Clines GA, Massa H, Trask BJ, Lovett M#TITLE:Identification and localization of the gene for EXTL, a third member of the multiple exostoses gene family#JOURNAL:Genome Res#YEAR:1997#PUBMED:9037597# GENE ASSOCIATION: (2131.1 and 2132.2) or (2137.1) or (2131.1 and 2132.1) or (2134.1)
Abbreviation: R_FUC13GALACGLCGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fuc13galacglcgal14acglcgalgluside_hs[e] <==> fuc13galacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_CYTK10 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : cmp + dgtp <==> cdp + dgdp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_LYSt4 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lys-L[e] + na1[e] --> lys-L[c] + na1[c] Confidence Level: 2 SAB genes: LOCUS:_11254#TRANSCRIPT:1#ABBREVIATION:SLC6A14# LOCUS:_6584#TRANSCRIPT:1#ABBREVIATION:SLC22A5# proteins: NAME:solute carrier family 6 (amino acid transporter), member 14#ABBREVIATION:Slc6a14.1# NAME:solute carrier family 22 (organic cation transporter), member 5#ABBREVIATION:Slc22a5.1# citations: AUTHORS:Chen NH, Reith ME, Quick MW#TITLE:Synaptic uptake and beyond: the sodium- and chloride-dependent neurotransmitter transporter family SLC6#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12719981# AUTHORS:Van Winkle LJ#TITLE:Amino acid transport regulation and early embryo development#JOURNAL:Biol Reprod#YEAR:2001#PUBMED:11133652# GENE ASSOCIATION: (11254.1) or (6584.1)
L-lysine transport in via sodium symport
Abbreviation: R_TOLBUTAMIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: tolbutamide[e] <==> tolbutamide[c] Confidence Level: 2 unknown mechanism, related cyp P450 metabolism reactions NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_FACOAL80i Synonyms: _0 EC Number: 6.2.1.2 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + octa --> amp + occoa + ppi Confidence Level: 4 JLR Also 6.2.1.3 genes: LOCUS:_2181#TRANSCRIPT:1#ABBREVIATION:ACSL3# LOCUS:_2181#TRANSCRIPT:2#ABBREVIATION:ACSL3# proteins: NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.1# NAME:acyl-CoA synthetase long-chain family member 3#ABBREVIATION:Acsl3.2# citations: none GENE ASSOCIATION: (2181.1) or (2181.2)
Abbreviation: R_ATPtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[c] + atp[m] --> adp[m] + atp[c] Confidence Level: 4 NCD genes: LOCUS:_291#TRANSCRIPT:1#ABBREVIATION:SLC25A4# LOCUS:_292#TRANSCRIPT:1#ABBREVIATION:SLC25A5# LOCUS:_293#TRANSCRIPT:1#ABBREVIATION:SLC25A6# proteins: NAME:Solute carrier family 25 (adenine nucleotide translocator) member 4, mitochondrial#ABBREVIATION:Slc25a4.1-m# NAME:Solute carrier family 25 (adenine nucleotide translocator) member 5, mitochondrial#ABBREVIATION:Slc25a5.1-m# NAME:Solute carrier family 25 (adenine nucleotide translocator) member 6, mitochondrial#ABBREVIATION:Slc25a6.1-m# citations: AUTHORS:Palmieri, F.#TITLE: The mitochondrial transporter family (SLC25): physiological and pathological implications.#JOURNAL: Pflugers Archive#YEAR:2004#PUBMED:14598172# GENE ASSOCIATION: (291.1) or (293.1) or (292.1)
Abbreviation: R_EX_strdnc_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : strdnc <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_NACHEXA2ly Synonyms: _0 SUBSYSTEM: Chondroitin sulfate degradation Equation: [l] : cs_b + (2) h2o --> acgal + cs_b_deg2 + h + so4 Confidence Level: 4 Note that this reaction is specific to beta-N-acetylhexosaminidase A and cannot be catalyzed by beta-N-acetylhexosaminidase B (or presumably beta-N-acetylhexosaminidase S). This is also a lumped reaction, representing the cleavage of GalNAc4S or GalNAc6S and N-acetylgalactosamine 4-sulfatase or 6-sulfatase. NCD genes: LOCUS:_3074#TRANSCRIPT:1#ABBREVIATION:HEXB#ECNUMBERS:3.2.1.52# LOCUS:_3073#TRANSCRIPT:1#ABBREVIATION:HEXA#ECNUMBERS:3.2.1.52# proteins: NAME:beta-N-acetylhexosaminidase A, lysosomal#ABBREVIATION:HexA-l# NAME:beta-N-acetylhexosaminidase S, lysosomal#ABBREVIATION:HexS-l# citations: AUTHORS:Maier T, Strater N, Schuette CG, Klingenstein R, Sandhoff K, Saenger W.#TITLE:The X-ray crystal structure of human beta-hexosaminidase B provides new insights into Sandhoff disease#JOURNAL:J Mol Biol#YEAR:2003#PUBMED:12706724# AUTHORS:Liu B, Ahmad W, Aronson NN Jr#TITLE:Structure of the human gene for lysosomal di-N-acetylchitobiase#JOURNAL:Glycobiology#YEAR:1999#PUBMED:10336991# AUTHORS:Winchester BG#TITLE:Lysosomal metabolism of glycoconjugates#JOURNAL:Subcell Biochem#YEAR:1996#PUBMED:8993162# AUTHORS:Hepbildikler ST, Sandhoff R, Kolzer M, Proia RL, Sandhoff K#TITLE:Physiological substrates for human lysosomal beta -hexosaminidase S.#JOURNAL:J Biol Chem#YEAR:2002#PUBMED:11707436# GENE ASSOCIATION: (3073.1) or (3073.1 and 3074.1)
Abbreviation: R_BILGLCURte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: atp[c] + bilglcur[c] + h2o[c] <==> adp[c] + bilglcur[e] + h[c] + pi[c] Confidence Level: 2 NJ genes: LOCUS:_4363#TRANSCRIPT:1#ABBREVIATION:ABCC1.1# proteins: NAME:ATP-binding cassette, subfamily C (CFTR/MRP), member 1#ABBREVIATION:ABCC1.1# citations: none GENE ASSOCIATION: (4363.1)
Abbreviation: R_S6T2g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core2_pre8 + paps --> h + ksii_core2_pre9 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_BPNT2 Synonyms: _0 EC Number: 3.1.3.7 SUBSYSTEM: Nucleotides Equation: [c] : h2o + paps --> aps + pi Confidence Level: 4 genes: LOCUS:_10380#TRANSCRIPT:1#ABBREVIATION:BPNT1#ECNUMBERS:3.1.3.7# proteins: NAME:_3'(2'), 5'-bisphosphate nucleotidase 1 #ABBREVIATION:Bpnt1# citations: AUTHORS:Spiegelberg BD, Xiong JP, Smith JJ, Gu RF, York JD.#TITLE:Cloning and characterization of a mammalian lithium-sensitive bisphosphate 3'-nucleotidase inhibited by inositol 1,4-bisphosphate#JOURNAL:J Biol Chem#YEAR:1999#PUBMED:10224133# GENE ASSOCIATION: (10380.1)
Abbreviation: R_DGTPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: dgtp[c] <==> dgtp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SEAHCYStn Synonyms: _0 SUBSYSTEM: Selenoamino acid metabolism Equation: seahcys[c] <==> seahcys[n] Confidence Level: 1 SAB based on yeast genes: none proteins: none citations: none GENE ASSOCIATION:
Se-adenosyl-seleno-L-homocysteine nuclear transport
Abbreviation: R_ARGDCm Synonyms: _0 EC Number: 4.1.1.19 SUBSYSTEM: Arginine and Proline Metabolism Equation: [m] : arg-L + h --> agm + co2 Confidence Level: 4 SAB m genes: LOCUS:_113451#TRANSCRIPT:1#ABBREVIATION:ODC-p# proteins: NAME:ornithine decarboxylase-like#ABBREVIATION:Odc-p.1-m# citations: AUTHORS:Zhu MY, Iyo A, Piletz JE, Regunathan S#TITLE:Expression of human arginine decarboxylase, the biosynthetic enzyme for agmatine#JOURNAL:Biochim Biophys Acta#YEAR:2004# AUTHORS:Grillo MA, Colombatto S#TITLE:Metabolism and function in animal tissues of agmatine, a biogenic amine formed from arginine#JOURNAL:Amino Acids#YEAR:2004#PUBMED:14752610# GENE ASSOCIATION: (113451.1)
Abbreviation: R_CCA_D3t Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: cca_d3[c] --> cca_d3[e] Confidence Level: 2 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
Abbreviation: R_G12MT2_L Synonyms: _0 EC Number: 2.4.1.131 SUBSYSTEM: N-Glycan Biosynthesis Equation: [c] : gdpmann + (0.1) m3mpdol_L --> gdp + h + (0.1) m4mpdol_L Confidence Level: 0 NCD genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# GENE ASSOCIATION:
Abbreviation: R_FACOAL260 Synonyms: _0 EC Number: 6.2.1.3 SUBSYSTEM: Fatty acid activation Equation: [c] : atp + coa + hexc <==> amp + hexccoa + ppi Confidence Level: 4 cytoplasmic version -NJ genes: LOCUS:_2180#TRANSCRIPT:1#ABBREVIATION:ACSL1#ECNUMBERS:6.2.1.3# proteins: NAME:acyl-CoA synthestase long-chain family member 1#ABBREVIATION:Acsl1.1# citations: AUTHORS:Mashek DG, Bornfeldt KE, Coleman RA, Berger J, Bernlohr DA, Black P, DiRusso CC, Farber SA, Guo W, Hashimoto N, Khodiyar V, Kuypers FA, Maltais LJ, Nebert DW, Renieri A, Schaffer JE, Stahl A, Watkins PA, Vasiliou V, Yamamoto TT.#TITLE:Revised nomenclature for the mammalian long-chain acyl-CoA synthetase gene family.#JOURNAL:J Lipid Res#YEAR:2004#PUBMED:15292367# AUTHORS:Reich, Sel'kov#TITLE:Energy metabolism of the cell : a theoretical treatise#YEAR:1981# GENE ASSOCIATION: (2180.1)
Abbreviation: R_CO2ter Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: co2[c] <==> co2[r] Confidence Level: 1 NCD genes: none proteins: none citations: none GENE ASSOCIATION:
CO2 endoplasmic reticular transport via diffusion
Abbreviation: R_DNDPt56m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: atp[m] + dctp[c] --> atp[c] + dctp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_GAMYe Synonyms: _0 EC Number: 3.2.1.3 SUBSYSTEM: Starch and Sucrose Metabolism Equation: [e] : glygn5 + h2o --> Tyr-ggn + malt Confidence Level: 4 NCD genes: LOCUS:_8972#TRANSCRIPT:1#ABBREVIATION:MGAM# proteins: NAME:maltase-glucoamylase (alpha-glucosidase), extracellular (intestine)#ABBREVIATION:Mgam.1-e# citations: AUTHORS:Naim HY, Sterchi EE, Lentze MJ#TITLE:Structure, biosynthesis, and glycosylation of human small intestinal maltase-glucoamylase#JOURNAL:J Biol Chem#YEAR:1988#PUBMED:3143729# GENE ASSOCIATION: (8972.1)
glucoamylase, extracellular (glygn5 -> malt)
Abbreviation: R_FAH1 Synonyms: _1 SUBSYSTEM: CYP Metabolism Equation: [c] : ddca + h + nadph + o2 --> h2o + nadp + whddca Confidence Level: 4 fatty acid hydroxylation NJ genes: LOCUS:_1579#TRANSCRIPT:1#ABBREVIATION:CYP4A11# proteins: NAME:cytochrome P450, family 4, subfamily A, polypeptide#ABBREVIATION:Cyp4a11.1# citations: AUTHORS:Rendic S.#TITLE:Summary of information on human CYP enzymes: human P450 metabolism data.#JOURNAL:Drug Metab Rev#YEAR:2002#PUBMED:11996015# AUTHORS:Palmer CN, Richardson TH, Griffin KJ, Hsu MH, Muerhoff AS, Clark JE, Johnson EF.#TITLE:Characterization of a cDNA encoding a human kidney, cytochrome P-450 4A fatty acid omega-hydroxylase and the cognate enzyme expressed in Escherichia coli.#JOURNAL:Biochim Biophys Acta#YEAR:1993#PUBMED:7679927# AUTHORS:Imaoka S, Ogawa H, Kimura S, Gonzalez FJ.#TITLE:Complete cDNA sequence and cDNA-directed expression of CYP4A11, a fatty acid omega-hydroxylase expressed in human kidney.#JOURNAL:DNA Cell Biol#YEAR:1993#PUBMED:8274222# GENE ASSOCIATION: (1579.1)
Abbreviation: R_EX_5htrp_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : 5htrp <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_PDE4n Synonyms: _0 EC Number: 3.1.4.17 SUBSYSTEM: Nucleotides Equation: [n] : 35cgmp + h2o --> gmp + h Confidence Level: 4 NCD. IT genes: LOCUS:_5152#TRANSCRIPT:1#ABBREVIATION:PDE9A#ECNUMBERS:3.1.4.17# proteins: NAME:phosphodiesterase 9A #ABBREVIATION:Pde9a.1-n# citations: AUTHORS:Wang P, Wu P, Egan RW, Billah MM.#TITLE:Identification and characterization of a new human type 9 cGMP-specific phosphodiesterase splice variant (PDE9A5). Differential tissue distribution and subcellular localization of PDE9A variants.#JOURNAL:Gene#YEAR:2003#PUBMED:14527714# AUTHORS:Identification and distribution of different mRNA variants produced by differential splicing in the human phosphodiesterase 9A gene. #TITLE:Identification and distribution of different mRNA variants produced by differential splicing in the human phosphodiesterase 9A gene. #JOURNAL:Biochem Biophys Res Commun.#YEAR:2003#PUBMED:12565835# AUTHORS:Fisher DA, Smith JF, Pillar JS, St Denis SH, Cheng JB.#TITLE:Isolation and characterization of PDE9A, a novel human cGMP-specific phosphodiesterase.#JOURNAL:J Biol Chem.#YEAR:1998#PUBMED:9624146# GENE ASSOCIATION: (5152.1)
_3',5'-cyclic-nucleotide phosphodiesterase, Nucleus
Abbreviation: R_EX_adrn_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : adrn <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_EX_carveol_LPAREN_e_RPAREN_ Synonyms: _0 Equation: [e] : carveol <==> Confidence Level: genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_ABO5g Synonyms: _10 SUBSYSTEM: Blood Group Biosynthesis Equation: [g] : fucgalacgalfuc12gal14acglcgalgluside_hs + udpacgal --> acgalfucgalacgalfuc12gal14acglcgalgluside_hs + h + udp Confidence Level: 4 NJ genes: LOCUS:_28#TRANSCRIPT:1#ABBREVIATION:ABO# proteins: NAME:ABO blood group#ABBREVIATION:Abo.1# citations: AUTHORS:Lloyd KO.#TITLE:The chemistry and immunochemistry of blood group A, B, H, and Lewis antigens: past, present and future.#JOURNAL:Glycoconj J#YEAR:2000#PUBMED:11421346# AUTHORS:Yamamoto F.-I., Hakomori S.-I.;#TITLE:Sugar-nucleotide donor specificity of histo-blood group A and B transferases is based on amino acid substitutions#JOURNAL:J. Biol. Chem#YEAR:1990#PUBMED:2121736# AUTHORS:Patenaude S.I., Seto N.O.L., Borisova S.N., Szpacenko A., Marcus S.L., Palcic M.M., Evans S.V.;#TITLE:The structural basis for specificity in human ABO(H) blood group biosynthesis#JOURNAL:Nat. Struct. Biol#YEAR:2002#PUBMED:12198488# GENE ASSOCIATION: (28.1)
Abbreviation: R_NADPHtru Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: nadph[c] --> nadph[r] Confidence Level: 1 diffusion - but unidirectional uptake into ER (to avoid loops w/ NAD/NADP) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
NADPH transporter, endoplasmic reticulum
Abbreviation: R_ITPtn Synonyms: _0 SUBSYSTEM: Transport, Nuclear Equation: itp[c] <==> itp[n] Confidence Level: 2 IT genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_5THFtl Synonyms: _0 SUBSYSTEM: Transport, Lysosomal Equation: _5thf[c] --> 5thf[l] Confidence Level: 2 carrier-mediated transport - but which and how is unknown IT genes: none proteins: none citations: AUTHORS:Suh JR, Herbig AK, Stover PJ. #TITLE:New perspectives on folate catabolism. #JOURNAL:Annu Rev Nutr.#YEAR:2001#PUBMED:11375437# GENE ASSOCIATION:
Abbreviation: R_DNDPt12m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: adp[m] + dadp[c] --> adp[c] + dadp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_BTNt2m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: btn[c] + h[c] <==> btn[m] + h[m] Confidence Level: 4 NCD genes: LOCUS:_6566#TRANSCRIPT:1#ABBREVIATION:SLC16A1# proteins: NAME:solute carrier family 16 (monocarboxylic acid transporters), member 1 #ABBREVIATION:Slc16a1.1-m# citations: AUTHORS:Bender DA#TITLE:Nutritional Biochemistry of the Vitamins#YEAR:2003# AUTHORS:Zempleni J.#TITLE:Uptake, localization, and noncarboxylase roles of biotin.#JOURNAL:Annu Rev Nutr#YEAR:2005#PUBMED:16011464# AUTHORS:Daberkow RL, White BR, Cederberg RA, Griffin JB, Zempleni J.#TITLE:Monocarboxylate transporter 1 mediates biotin uptake in human peripheral blood mononuclear cells.#JOURNAL:J Nutr. #YEAR:2003#PUBMED:12949353# AUTHORS:Butz CE, McClelland GB, Brooks GA.#TITLE:MCT1 confirmed in rat striated muscle mitochondria.#JOURNAL:J Appl Physiol.#YEAR:2004#PUBMED:15121743# GENE ASSOCIATION: (6566.1)
Biotin reversible transport via proton symport, mitochondria
Abbreviation: R_CERT1rt Synonyms: _0 SUBSYSTEM: Transport, Endoplasmic Reticular Equation: crm_hs[c] <==> crm_hs[r] Confidence Level: 2 non-vesicular intracellular transport of ceramide genes: LOCUS:_10087#TRANSCRIPT:1#ABBREVIATION:COL4A3BP# proteins: NAME:collagen, type IV, alpha 3 (Goodpasture antigen) binding protein#ABBREVIATION:Col4a3bp.1# citations: AUTHORS:Kumagai K, Yasuda S, Okemoto K, Nishijima M, Kobayashi S, Hanada K.#TITLE:CERT mediates intermembrane transfer of various molecular species of ceramides.#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15596449# AUTHORS:Hanada K, Kumagai K, Yasuda S, Miura Y, Kawano M, Fukasawa M, Nishijima M.#TITLE:Molecular machinery for non-vesicular trafficking of ceramide.#JOURNAL:Nature#YEAR:2003#PUBMED:14685229# AUTHORS:Perry RJ, Ridgway ND.#TITLE:Molecular mechanisms and regulation of ceramide transport.#JOURNAL:Biochim Biophys Acta#YEAR:2005#PUBMED:15907394# AUTHORS:van Meer G, Wolthoorn J, Degroote S.#TITLE:The fate and function of glycosphingolipid glucosylceramide.#JOURNAL:Philos Trans R Soc Lond B Biol Sci#YEAR:2003#PUBMED:12803919# GENE ASSOCIATION: (10087.1)
Abbreviation: R_FALDtm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: fald[m] <==> fald[c] Confidence Level: 1 MM genes: none proteins: none citations: none GENE ASSOCIATION:
formaldehyde transport via diffusion (mitochondrial)
Abbreviation: R_H6_APOS_ET2er Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : em3gacpail_hs + pe_hs --> dag_hs + emem2gacpail_hs Confidence Level: 4 NCD genes: LOCUS:_5281#TRANSCRIPT:1#ABBREVIATION:PIGF# LOCUS:_5281#TRANSCRIPT:2#ABBREVIATION:PIGF# LOCUS:_54872#TRANSCRIPT:1#ABBREVIATION:GPI7# proteins: NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.1-r# NAME:phosphatidylinositol glycan, class F, endoplasmic reticulum#ABBREVIATION:Pigf.2-r# NAME:GPI protein, endoplasmic reticulum#ABBREVIATION:Gpi7.1-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Shishioh N, Hong Y, Ohishi K, Ashida H, Maeda Y, Kinoshita T#TITLE:GPI7 is the second partner of PIG-F and involved in modification of glycosylphosphatidylinositol#JOURNAL:J Biol Chem#YEAR:2005#PUBMED:15632136# GENE ASSOCIATION: (5281.1) and (54872.1) or (5281.2) and (54872.1)
Abbreviation: R_AGPex Synonyms: _0 SUBSYSTEM: Transport, Peroxisomal Equation: akdhap_hs[c] <==> akdhap_hs[x] Confidence Level: 0 for alkly glycerol phosphate reductase (transmembrane rxn) - in homo sapiens glycerolipid metabolism NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_OAGT3tg Synonyms: _0 SUBSYSTEM: Transport, Golgi Apparatus Equation: oagt3_hs[c] <==> oagt3_hs[g] Confidence Level: 1 unknown mechanism for transport - intracellular transport occurs, most details are unknown - see for example Varki glycobiology text or numerous reviews (PMID: 8206147) NJ genes: none proteins: none citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Sandhoff K, Klein A.#TITLE:Intracellular trafficking of glycosphingolipids: role of sphingolipid activator proteins in the topology of endocytosis and lysosomal digestion.#JOURNAL:FEBS Lett.#YEAR:1994#PUBMED:8206147# GENE ASSOCIATION:
Abbreviation: R_S6T17g Synonyms: _0 SUBSYSTEM: Keratan sulfate biosynthesis Equation: [g] : ksii_core4_pre8 + paps --> h + ksii_core4_pre9 + pap Confidence Level: 4 NCD genes: LOCUS:_10164#TRANSCRIPT:1#ABBREVIATION:CHST4# LOCUS:_9435#TRANSCRIPT:1#ABBREVIATION:CHST2# LOCUS:_23563#TRANSCRIPT:1#ABBREVIATION:CHST5# LOCUS:_23563#TRANSCRIPT:2#ABBREVIATION:CHST5# LOCUS:_4166#TRANSCRIPT:1#ABBREVIATION:CHST6# proteins: NAME:carbohydrate (N-acetylglucosamine-6-O) sulfotransferase 2#ABBREVIATION:Chst2.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 4, Golgi#ABBREVIATION:Chst4.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.1-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 5, Golgi#ABBREVIATION:Chst5.2-g# NAME:carbohydrate (N-acetylglucosamine 6-O) sulfotransferase 6, Golgi apparatus#ABBREVIATION:Chst6.1-g# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Uchimura K, Fasakhany F, Kadomatsu K, Matsukawa T, Yamakawa T, Kurosawa N, Muramatsu T#TITLE:Diversity of N-acetylglucosamine-6-O-sulfotransferases: molecular cloning of a novel enzyme with different distribution and specificities#JOURNAL:Biochem Biophys Res Commun#YEAR:2000#PUBMED:10913333# AUTHORS:Akama TO, Nakayama J, Nishida K, Hiraoka N, Suzuki M, McAuliffe J, Hindsgaul O, Fukuda M, Fukuda MN#TITLE:Human corneal GlcNac 6-O-sulfotransferase and mouse intestinal GlcNac 6-O-sulfotransferase both produce keratan sulfate#JOURNAL:J Biol Chem#YEAR:2001#PUBMED:11278593# AUTHORS:de Graffenried CL, Bertozzi CR#TITLE:Golgi localization of carbohydrate sulfotransferases is a determinant of L-selectin ligand biosynthesis#JOURNAL:J Biol Chem#YEAR:2003#PUBMED:12855678# AUTHORS:Uchimura K, Muramatsu H, Kaname T, Ogawa H, Yamakawa T, Fan QW, Mitsuoka C, Kannagi R, Habuchi O, Yokoyama I, Yamamura K, Ozaki T, Nakagawara A, Kadomatsu K, Muramatsu T#TITLE:Human N-acetylglucosamine-6-O-sulfotransferase involved in the biosynthesis of 6-sulfo sialyl Lewis X: molecular cloning, chromosomal mapping, and expression in various organs and tumor cells#JOURNAL:J Biochem (Tokyo)#YEAR:1998#PUBMED:9722682# AUTHORS:Bistrup A, Bhakta S, Lee JK, Belov YY, Gunn MD, Zuo FR, Huang CC, Kannagi R, Rosen SD, Hemmerich S#TITLE:Sulfotransferases of two specificities function in the reconstitution of high endothelial cell ligands for L-selectin#JOURNAL:J Cell Biol#YEAR:1999#PUBMED:10330415# AUTHORS:Lee JK, Bhakta S, Rosen SD, Hemmerich S#TITLE:Cloning and characterization of a mammalian N-acetylglucosamine-6-sulfotransferase that is highly restricted to intestinal tissue#JOURNAL:Biochem Biophys Res Commun#YEAR:1999#PUBMED:10491328# AUTHORS:Grunwell JR, Bertozzi CR#TITLE:Carbohydrate sulfotransferases of the GalNAc/Gal/GlcNAc6ST family#JOURNAL:Biochemistry#YEAR:2002#PUBMED:12403612# GENE ASSOCIATION: (23563.2) or (9435.1) or (23563.1) or (10164.1) or (4166.1)
Abbreviation: R_H3MTer_U Synonyms: _0 SUBSYSTEM: Glycosylphosphatidylinositol (GPI)-anchor biosynthesis Equation: [r] : (0.1) dolmanp_U + m2gacpail_hs --> (0.1) dolp_U + h + m3gacpail_hs Confidence Level: 4 NCD genes: LOCUS:_9488#TRANSCRIPT:1#ABBREVIATION:PIGB# proteins: NAME:phosphatidylinositol glycan, class B, endoplasmic reticulum#ABBREVIATION:Pigb.1-r# citations: AUTHORS:Varki, Cummings, Esko, Freeze, Hart, Marth#TITLE:Essentials of Glycobiology#YEAR:1999# AUTHORS:Takahashi M, Inoue N, Ohishi K, Maeda Y, Nakamura N, Endo Y, Fujita T, Takeda J, Kinoshita T#TITLE:PIG-B, a membrane protein of the endoplasmic reticulum with a large lumenal domain, is involved in transferring the third mannose of the GPI anchor#JOURNAL:EMBO J#YEAR:1996#PUBMED:8861954# GENE ASSOCIATION: (9488.1)
Abbreviation: R_ARTCOAL2 Synonyms: _0 SUBSYSTEM: R Group Synthesis Equation: [c] : Rtotal2 + coa <==> Rtotal2coa Confidence Level: 0 HR fatty acid metabolism - R groups = acyl groups, Rtotal, Rtotal2, Rtotal3 correspond to three possible acyl groups added to glycerol Carbon stoichiometry normalized wrt C16 R1 - C16 groups R2 - essential FA + associated derivatives R3 - non-essential, dietary FA (C18) R4 - monounsaturated FA R5 -other (C26, C24) NJ genes: none proteins: none citations: none GENE ASSOCIATION:
Abbreviation: R_SERtN1 Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: h[c] + (2) na1[e] + ser-L[e] <==> h[e] + (2) na1[c] + ser-L[c] Confidence Level: 4 SAB based on PMID 12845534 genes: LOCUS:_92745#TRANSCRIPT:1#ABBREVIATION:SLC38A5# proteins: NAME:solute carrier family 38, member 5#ABBREVIATION:Slc38a5.1# citations: AUTHORS:Mackenzie B, Erickson JD#TITLE:Sodium-coupled neutral amino acid (System N/A) transporters of the SLC38 gene family#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:12845534# GENE ASSOCIATION: (92745.1)
Abbreviation: R_LYStiDF Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: lys-L[e] --> lys-L[c] Confidence Level: 4 SAB genes: LOCUS:_84889#TRANSCRIPT:1#ABBREVIATION:SLC7A3# LOCUS:_6542#TRANSCRIPT:1#ABBREVIATION:SLC7A2# LOCUS:_6541#TRANSCRIPT:1#ABBREVIATION:SLC7A1# LOCUS:_55089#TRANSCRIPT:1#ABBREVIATION:SLC38A4# proteins: NAME:solute carrier family 38, member 4#ABBREVIATION:Slc38a4.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 1#ABBREVIATION:Slc7a1.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 2#ABBREVIATION:Slc7a2.1# NAME:solute carrier family 7 (cationic amino acid transporter, y+ system), member 3#ABBREVIATION:Slc7a3.1# citations: AUTHORS:Gu S, Adan-Rice D, Leach RJ, Jiang JX#TITLE:A novel human amino acid transporter, hNAT3: cDNA cloning, chromosomal mapping, genomic structure, expression, and functional characterization#JOURNAL:Genomics#YEAR:2001#PUBMED:11414754# AUTHORS:Hatanaka T, Huang W, Ling R, Prasad PD, Sugawara M, Leibach FH, Ganapathy V#TITLE:Evidence for the transport of neutral as well as cationic amino acids by ATA3, a novel and liver-specific subtype of amino acid transport system A#JOURNAL:Biochim Biophys Acta#YEAR:2001#PUBMED:11342143# AUTHORS:Albritton LM, Bowcock AM, Eddy RL, Morton CC, Tseng L, Farrer LA, Cavalli-Sforza LL, Shows TB, Cunningham JM#TITLE:The human cationic amino acid transporter (ATRC1): physical and genetic mapping to 13q12-q14#JOURNAL:Genomics#YEAR:1992#PUBMED:1348489# AUTHORS:Cariappa R, Heath-Monnig E, Furesz TC, Kamath SG, Smith CH#TITLE:Stable polarized expression of hCAT-1 in an epithelial cell line#JOURNAL:J Membr Biol#YEAR:2002#PUBMED:11891586# AUTHORS:Hoshide R, Ikeda Y, Karashima S, Matsuura T, Komaki S, Kishino T, Niikawa N, Endo F, Matsuda I#TITLE:Molecular cloning, tissue distribution, and chromosomal localization of human cationic amino acid transporter 2 (HCAT2)#JOURNAL:Genomics#YEAR:1996#PUBMED:8954799# AUTHORS:Furesz TC, Moe AJ, Smith CH#TITLE:Lysine uptake by human placental microvillous membrane: comparison of system y+ with basal membrane#JOURNAL:Am J Physiol#YEAR:1995#PUBMED:7534987# AUTHORS:Wolf S, Janzen A, Vekony N, Martine U, Strand D, Closs EI#TITLE:Expression of solute carrier 7A4 (SLC7A4) in the plasma membrane is not sufficient to mediate amino acid transport activity#JOURNAL:Biochem J#YEAR:2002#PUBMED:12049641# AUTHORS:Vekony N, Wolf S, Boissel JP, Gnauert K, Closs EI#TITLE:Human cationic amino acid transporter hCAT-3 is preferentially expressed in peripheral tissues#JOURNAL:Biochemistry#YEAR:2001#PUBMED:11591158# AUTHORS:Verrey F, Closs EI, Wagner CA, Palacin M, Endou H, Kanai Y#TITLE:CATs and HATs: the SLC7 family of amino acid transporters#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14770310# GENE ASSOCIATION: (84889.1) or (55089.1) or (6542.1) or (6541.1)
L-lysine transport via diffusion (extracellular to cytosol)
Abbreviation: R_DNDPt38m Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: dudp[m] + dutp[c] --> dudp[c] + dutp[m] Confidence Level: 4 IT genes: LOCUS:_60386#TRANSCRIPT:1#ABBREVIATION:SLC25A19# proteins: NAME:solute carrier family 25 (mitochondrial deoxynucleotide carrier), member 19 #ABBREVIATION:Slc25A19.1-m# citations: AUTHORS:Dolce V, Fiermonte G, Runswick MJ, Palmieri F, Walker JE. #TITLE:The human mitochondrial deoxynucleotide carrier and its role in the toxicity of nucleoside antivirals.#JOURNAL:Proc Natl Acad Sci U S A.#YEAR:2001#PUBMED:11226231# GENE ASSOCIATION: (60386.1)
Abbreviation: R_CYSALANaEx Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: ala-L[c] + cys-L[e] + na1[e] --> ala-L[e] + cys-L[c] + na1[c] Confidence Level: 4 SAB see Kanai and Hediger 2004 (2003), PMID 14530974 genes: LOCUS:_6509#TRANSCRIPT:1#ABBREVIATION:SLC1A4# LOCUS:_6510#TRANSCRIPT:1#ABBREVIATION:SLC1A5# proteins: NAME:solute carrier family 1 (glutamate/neutral amino acid transporter), member 4#ABBREVIATION:Slc1a4.1# NAME:solute carrier family 1 (neutral amino acid transporter), member 5#ABBREVIATION:Slc1a5.1# citations: AUTHORS:Kanai Y, Hediger MA#TITLE:The glutamate/neutral amino acid transporter family SLC1: molecular, physiological and pharmacological aspects#JOURNAL:Pflugers Arch#YEAR:2004#PUBMED:14530974# GENE ASSOCIATION: (6509.1) or (6510.1)
L-alanine/L-cysteine Na-dependent exchange (Cys-L in)
Abbreviation: R_SALMCOM2 Synonyms: _0 EC Number: 2.1.1.6 SUBSYSTEM: Tyrosine metabolism Equation: [c] : adrnl + amet --> ahcys + h + mepi Confidence Level: 2 copied SAB's original NJ genes: LOCUS:_118881#TRANSCRIPT:1#ABBREVIATION:COMTD1# proteins: NAME:catechol-O-methyltransferase domain containing 1#ABBREVIATION:Comtd1.1# citations: AUTHORS:Schluckebier G, O'Gara M, Saenger W, Cheng X.#TITLE:Universal catalytic domain structure of AdoMet-dependent methyltransferases.#JOURNAL:J Mol Biol#YEAR:1995#PUBMED:7897657# GENE ASSOCIATION: (118881.1)
Abbreviation: R_CYTK8 Synonyms: _0 EC Number: 2.7.4.14 SUBSYSTEM: Nucleotides Equation: [c] : cmp + datp <==> cdp + dadp Confidence Level: 4 genes: LOCUS:_51727#TRANSCRIPT:1#ABBREVIATION:UMP-CMPK#ECNUMBERS:2.7.4.14# proteins: NAME:UMP-CMP kinase #ABBREVIATION:UmpK.1# citations: AUTHORS:Van Rompay AR, Johansson M, Karlsson A.#TITLE:Phosphorylation of deoxycytidine analog monophosphates by UMP-CMP kinase: molecular characterization of the human enzyme. #JOURNAL:Mol Pharmacol. #YEAR:1999#PUBMED:10462544# AUTHORS:Liou JY, Dutschman GE, Lam W, Jiang Z, Cheng YC.#TITLE:Characterization of human UMP/CMP kinase and its phosphorylation of D- and L-form deoxycytidine analogue monophosphates.#JOURNAL:Cancer Res. #YEAR:2002#PUBMED:11912132# GENE ASSOCIATION: (51727.1)
Abbreviation: R_MTHFC Synonyms: _0 EC Number: 3.5.4.9 SUBSYSTEM: Folate Metabolism Equation: [c] : h2o + methf <==> 10fthf + h Confidence Level: 4 genes: LOCUS:_4522#TRANSCRIPT:1#ABBREVIATION:MTHFD1#ECNUMBERS:1.5.1.5,3.5.4.9,6.3.4.3# LOCUS:_80068#TRANSCRIPT:1#ABBREVIATION:FLJ13105# LOCUS:_285216#TRANSCRIPT:1#ABBREVIATION:LOC285216# proteins: NAME:methylenetetrahydrofolate dehydrogenase (NADP+ dependent) 1, methenyltetrahydrofolate cyclohydrolase, formyltetrahydrofolate synthetase#ABBREVIATION:Mthfd1.1# NAME:hypothetical protein FLJ13105#ABBREVIATION:Flj13105.1# NAME:hypothetical protein LOC285216#ABBREVIATION:Loc285216.1# citations: AUTHORS:Devlin, TM#TITLE:Textbook of Biochemistry with Clinical Correlations#YEAR:2001# AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION: (4522.1) or (285216.1) or (80068.1)
Abbreviation: R_PCRNtc Synonyms: _0 SUBSYSTEM: Carnitine shuttle Equation: pcrn[x] --> pcrn[c] Confidence Level: 4 NJ genes: none proteins: none citations: AUTHORS:Jakobs BS, Wanders RJ.#TITLE:Fatty acid beta-oxidation in peroxisomes and mitochondria: the first, unequivocal evidence for the involvement of carnitine in shuttling propionyl-CoA from peroxisomes to mitochondria.#JOURNAL:Biochem Biophys Res Commun.#YEAR:1995#PUBMED:7654220# GENE ASSOCIATION:
transport into the cytosol from peroxisome (carnitine)
Abbreviation: R_GTHPe Synonyms: _0 EC Number: 1.11.1.9 SUBSYSTEM: Glutathione Metabolism Equation: [e] : (2) gthrd + h2o2 <==> gthox + (2) h2o Confidence Level: 4 SAB genes: LOCUS:_2878#TRANSCRIPT:1#ABBREVIATION:GPX3#ECNUMBERS:1.11.1.9# LOCUS:_257202#TRANSCRIPT:1#ABBREVIATION:GPX6#ECNUMBERS:1.11.1.9# proteins: NAME:glutathione peroxidase 3 (plasma)#ABBREVIATION:Gpx3.1-e# NAME:glutathione peroxidase 6 (olfactory)#ABBREVIATION:Gpx6.1-e# citations: AUTHORS:Metzler, David E #TITLE:Biochemistry : the chemical reactions of living cells 2 ed vol 1#YEAR:2001# AUTHORS:Takahashi K, Avissar N, Whitin J, Cohen H.#TITLE:Purification and characterization of human plasma glutathione peroxidase: a selenoglycoprotein distinct from the known cellular enzyme#JOURNAL:Arch Biochem Biophys#YEAR:1987#PUBMED:3619451# GENE ASSOCIATION: (2878.1) or (257202.1)
Abbreviation: R_NRVNCt Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: nrvnc[e] <==> nrvnc[c] Confidence Level: 2 NJ genes: none proteins: none citations: AUTHORS:Schaffer JE.#TITLE:Fatty acid transport: the roads taken.#JOURNAL:Am J Physiol Endocrinol Metab.#YEAR:2002#PUBMED:11788354# AUTHORS:Pownall HJ, Hamilton JA.#TITLE:Energy translocation across cell membranes and membrane models.#JOURNAL:Acta Physiol Scand#YEAR:2003#PUBMED:12864740# GENE ASSOCIATION:
Abbreviation: R_GALASE8ly Synonyms: _12 EC Number: 3.2.1.23 SUBSYSTEM: Keratan sulfate degradation Equation: [l] : h2o + ksi_deg20 --> gal + ksi_deg21 Confidence Level: 4 NCD genes: LOCUS:_4758#TRANSCRIPT:1#ABBREVIATION:NEU1#ECNUMBERS:3.2.1.18# LOCUS:_2720#TRANSCRIPT:1#ABBREVIATION:GLB1#ECNUMBERS:3.2.1.23# LOCUS:_2588#TRANSCRIPT:1#ABBREVIATION:GALNS#ECNUMBERS:3.1.6.4# LOCUS:_5476#TRANSCRIPT:1#ABBREVIATION:PPGB# proteins: NAME:sialidase I, lysosomal#ABBREVIATION:Neu1.1-l# NAME:beta-galactosidase, lysosomal#ABBREVIATION:Glb1.1-l# NAME:cathepsin A (protective protein), lysosomal #ABBREVIATION:Ppgb.1-l# NAME:galactosamine (N-acetyl)-6-sulfate sulfatase (Morquio syndrome, mucopolysaccharidosis type IVA), lysosomal#ABBREVIATION:Galns.1-l# citations: AUTHORS:Oshima A, Tsuji A, Nagao Y, Sakuraba H, Suzuki Y.#TITLE:Cloning, sequencing, and expression of cDNA for human beta-galactosidase.#JOURNAL:Biochem Biophys Res Commun#YEAR:1988#PUBMED:3143362# AUTHORS:Lim CT, Horwitz AL#TITLE:Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochim Biophys Acta#YEAR:1981#PUBMED:7213753# AUTHORS:Glossl J, Truppe W, Kresse H#TITLE:Purification and properties of N-acetylgalactosamine 6-sulphate sulphatase from human placenta#JOURNAL:Biochem J#YEAR:1979#PUBMED:39554# AUTHORS:Bielicki J, Hopwood JJ#TITLE:Human liver N-acetylgalactosamine 6-sulphatase. Purification and characterization#JOURNAL:Biochem J#YEAR:1991#PUBMED:1953646# AUTHORS:Masue M, Sukegawa K, Orii T, Hashimoto T#TITLE:N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics#JOURNAL:J Biochem (Tokyo)#YEAR:1991#PUBMED:1794986# AUTHORS:Tomatsu S, Fukuda S, Masue M, Sukegawa K, Fukao T, Yamagishi A, Hori T, Iwata H, Ogawa T, Nakashima Y, et al#TITLE:Morquio disease: isolation, characterization and expression of full-length cDNA for human N-acetylgalactosamine-6-sulfate sulfatase#JOURNAL:Biochem Biophys Res Commun#YEAR:1991#PUBMED:1755850# AUTHORS:Bielicki J, Fuller M, Guo XH, Morris CP, Hopewood JJ, Anson DS#TITLE:Expression, purification and characterization of recombinant human N-acetylgalactosamine-6-sulphatase#JOURNAL:Biochem J#YEAR:1995#PUBMED:7575473# AUTHORS:Yamamoto Y, Hake CA, Martin BM, Kretz KA, Ahern-Rindell AJ, Naylor SL, Mudd M, O'Brien JS#TITLE:Isolation, characterization, and mapping of a human acid beta-galactosidase cDNA#JOURNAL:DNA Cell Biol#YEAR:1990#PUBMED:2111707# AUTHORS:Pshezhetsky AV, Ashmarina M#TITLE:Lysosomal multienzyme complex: biochemistry, genetics, and molecular pathophysiology.#JOURNAL:Prog Nucleic Acid Res Mol Biol#YEAR:2001#PUBMED:11550799# AUTHORS:Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M#TITLE:Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis#JOURNAL:Nat Genet#YEAR:1997#PUBMED:9054950# AUTHORS:Bonten E, van der Spoel A, Fornerod M, Grosveld G, d'Azzo A#TITLE:Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis.#JOURNAL:Genes Dev#YEAR:1996#PUBMED:8985184# AUTHORS:Milner CM, Smith SV, Carrillo MB, Taylor GL, Hollinshead M, Campbell RD#TITLE:Identification of a sialidase encoded in the human major histocompatibility complex#JOURNAL:J Biol Chem#YEAR:1997#PUBMED:9020182# GENE ASSOCIATION: (5476.1) and (2720.1) and (2588.1) and (4758.1) or (5476.1) and (2720.1)
Abbreviation: R_NDPK1m Synonyms: _0 EC Number: 2.7.4.6 SUBSYSTEM: Nucleotides Equation: [m] : atp + gdp <==> adp + gtp Confidence Level: 2 Many nucleoside diphosphates (eight described) can act as acceptors. While many ribo- and deoxyribonucleoside triphosphates can act as donors, only ATP is chosen as a donor to minimize the number of total reactions. genes: LOCUS:_4833#TRANSCRIPT:1#ABBREVIATION:NME4# proteins: NAME:Nucleoside diphosphate kinase 4#ABBREVIATION:NME4.1# citations: AUTHORS:Milon L, Rousseau-Merck MF, Munier A, Erent M, Lascu I, Capeau J, Lacombe ML.#TITLE:nm23-H4, a new member of the family of human nm23/nucleoside diphosphate kinase genes localised on chromosome 16p13.#JOURNAL:Hum Genet. #YEAR:1997#PUBMED:9099850# GENE ASSOCIATION: (4833.1)
nucleoside-diphosphate kinase (ATP:GDP), mitochondrial
Perillyl aldehyde:NAD+ oxidoreductase (m)
Abbreviation: R_PYLALDOXm Synonyms: _0 EC Number: 1.2.1.3 SUBSYSTEM: Limonene and pinene degradation Equation: [m] : h2o + nad + pylald --> (2) h + nadh + peracd Confidence Level: 1 SAB genes: LOCUS:_8659#TRANSCRIPT:1#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# LOCUS:_217#TRANSCRIPT:1#ABBREVIATION:ALDH2#ECNUMBERS:1.2.1.3# LOCUS:_219#TRANSCRIPT:1#ABBREVIATION:ALDH1B1#ECNUMBERS:1.2.1.3# LOCUS:_8659#TRANSCRIPT:2#ABBREVIATION:ALDH4A1#ECNUMBERS:1.5.1.12# proteins: NAME:aldehyde dehydrogenase 2 family (mitochondrial)#ABBREVIATION:Aldh2.1-m# NAME:aldehyde dehydrogenase 1 family, member B1#ABBREVIATION:Aldh1b1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.1-m# NAME:aldehyde dehydrogenase 4 family, member A1#ABBREVIATION:Aldh4a1.2-m# citations: none GENE ASSOCIATION: (8659.2) or (217.1) or (8659.1) or (219.1)
Abbreviation: R_24_COMMA_25DHVITD3tm Synonyms: _0 SUBSYSTEM: Transport, Mitochondrial Equation: _2425dhvitd3[m] --> 2425dhvitd3[c] Confidence Level: 2 is found in blood --> subsequently has to be transported but how? based on Vitamins, G.F.M. Ball,2004, Blackwell publishing, 1st ed (book) pg.193 IT genes: none proteins: none citations: AUTHORS:Ball, G.F.M#TITLE:Vitamins: Their Role in the Human Body#YEAR:2004# GENE ASSOCIATION:
_24,25-Dihydroxyvitamin D3 transport from mitochondria
Abbreviation: R_FAOXC2452253x Synonyms: _0 EC Number: 1.3.99.3 SUBSYSTEM: Fatty acid oxidation, peroxisome Equation: [x] : coa + h2o + nad + o2 + tetpent3coa --> accoa + clpndcoa + h + h2o2 + nadh Confidence Level: 4 - in peroxisome, no fad, 1ste set of beta-ox is by acyl-coa oxidase (passes electrons directly to o2 -> h2o2) confusing name, sorry - accounting for omega3 vs omega6 FA peroxisomal (very long chain FA) c24:5,n-3 -> c22:5, n-3 4 step FAO lumped also includes EC 1.1.1.35, 1.1.1.211, 1.3.3.6, 4.2.1.17, 4.2.1.74 NJ genes: LOCUS:_3295#TRANSCRIPT:1#ABBREVIATION:HSD17B4# LOCUS:_30#TRANSCRIPT:1#ABBREVIATION:ACAA1#ECNUMBERS:2.3.1.16# LOCUS:_51#TRANSCRIPT:1#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# LOCUS:_1962#TRANSCRIPT:1#ABBREVIATION:EHHADH#ECNUMBERS:4.2.1.17.1.1.1.# LOCUS:_51#TRANSCRIPT:2#ABBREVIATION:ACOX1#ECNUMBERS:1.3.3.6# proteins: NAME:peroxisomal beta-oxidation (lumped)#ABBREVIATION:Perox2.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox2.2-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.1-x# NAME:peroxisomal beta oxidation (lumped)#ABBREVIATION:Perox3.2-x# citations: none GENE ASSOCIATION: (3295.1 and 30.1 and 51.2) or (30.1 and 51.2 and 1962.1) or (51.1 and 30.1 and 1962.1) or (51.1 and 3295.1 and 30.1)
alkyl acylglycerol phosphocholine transferase
Abbreviation: R_AGLPC Synonyms: _1 SUBSYSTEM: Glycerophospholipid Metabolism Equation: [c] : ak2g_hs + cdpchol --> ak2gchol_hs + cmp + h + h2o Confidence Level: 0 NJ genes: none proteins: none citations: AUTHORS:Voet D, Voet JG#TITLE:Biochemistry#YEAR:1995# AUTHORS:Murray RK, Granner DK, Mayes PA, Rodwell VW#TITLE:Harper's Biochemistry#YEAR:1999# AUTHORS:Brites P, Waterham HR, Wander RJA#TITLE:Functions and biosynthesis of plasmalogens in health and disease#JOURNAL:Biochimica et Biophysica Acta#YEAR:2004# AUTHORS:Nagan N, Zoeller RA#TITLE:Plasmalogens: biosynthesis and functions#JOURNAL:Progress in Lipid Research#YEAR:2001# GENE ASSOCIATION:
Abbreviation: R_FBA Synonyms: _0 EC Number: 4.1.2.13 SUBSYSTEM: Glycolysis/Gluconeogenesis Equation: [c] : fdp <==> dhap + g3p Confidence Level: 0 genes: LOCUS:_226#TRANSCRIPT:1#ABBREVIATION:ALDOA#ECNUMBERS:4.1.2.13# LOCUS:_229#TRANSCRIPT:1#ABBREVIATION:ALDOB#ECNUMBERS:4.1.2.13# LOCUS:_230#TRANSCRIPT:1#ABBREVIATION:ALDOC#ECNUMBERS:4.1.2.13# LOCUS:_226#TRANSCRIPT:2#ABBREVIATION:ALDOA#ECNUMBERS:4.1.2.13# LOCUS:_226#TRANSCRIPT:3#ABBREVIATION:ALDOA#ECNUMBERS:4.1.2.13# proteins: NAME:aldolase A, fructose-bisphosphate (muscle)#ABBREVIATION:Aldoa.1# NAME:aldolase A, fructose-bisphosphate (muscle)#ABBREVIATION:Aldoa.2# NAME:aldolase A, fructose-bisphosphate (muscle)#ABBREVIATION:Aldoa.3# NAME:aldolase B, fructose-bisphosphate (liver)#ABBREVIATION:Aldob.1# NAME:aldolase C, fructose-bisphosphate (brain)#ABBREVIATION:Aldoc.1# citations: none GENE ASSOCIATION: (226.2) or (226.1) or (226.3) or (230.1) or (229.1)
Abbreviation: R_LGNCCPT1 Synonyms: _0 EC Number: 2.3.1.21 SUBSYSTEM: Carnitine shuttle Equation: [c] : crn + lgnccoa --> coa + lgnccrn Confidence Level: 4 NJ genes: LOCUS:_1375#TRANSCRIPT:1#ABBREVIATION:CPT1B# LOCUS:_1374#TRANSCRIPT:1#ABBREVIATION:CPT1A#ECNUMBERS:2.3.1.21# LOCUS:_126129#TRANSCRIPT:1#ABBREVIATION:CPT1C# LOCUS:_1375#TRANSCRIPT:2#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:3#ABBREVIATION:CPT1B# LOCUS:_1375#TRANSCRIPT:4#ABBREVIATION:CPT1B# proteins: NAME:carnitine palmitoyltransferase 1A (liver)#ABBREVIATION:Cpt1a.1# NAME:carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.1# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.2# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.3# NAME:Carnitine palmitoyltransferase 1B (muscle)#ABBREVIATION:Cpt1b.4# NAME:Carnitine palmitoyltransferase 1C(brain)#ABBREVIATION:Cpt1c.1# citations: none GENE ASSOCIATION: (1375.1) or (1375.3) or (1375.4) or (126129.1) or (1374.1) or (1375.2)
Abbreviation: R_FUCFUCFUCGALACGLCGAL14ACGLCGALGLUSIDEte Synonyms: _0 SUBSYSTEM: Transport, Extracellular Equation: fucfucfucgalacglcgal14acglcgalgluside_hs[e] <==> fucfucfucgalacglcgal14acglcgalgluside_hs[c] Confidence Level: 2 unknown mechanism, however these blood groups need to make it to the extracellular surface of cells NJ genes: none proteins: none citations: none GENE ASSOCIATION: