R00703Cyto (S)-Lactate+NAD+-->Pyruvate+NADH+H+
C04405MM (2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA
R00658Cyto 2-Phospho-D-glycerate-->Phosphoenolpyruvate+H2O
Transporter26 Ornithine carrier - L-Ornithine for H+
R00355Cyto L-Aspartate+2-Oxoglutarate-->Oxaloacetate+L-Glutamate
Transporter27 Ornithine carrier - L-Arginine for H+
Transporter10 Dicarboxylate carrier - Succinate for phosphate
Transporter28 Ornithine carrier - L-Citrulline for H+
Transporter29 Graves disease carrier - CoA
Transporter30 Glutamate carrier - Glutamate and H+
Transporter9 Dicarboxylate carrier - Malate for phosphate
R00709MM Isocitrate+NAD+-->2-Oxoglutarate+CO2+NADH+H+
R00710MM Acetaldehyde+NAD++H2O-->Acetate+NADH+H+
R00711MM Acetaldehyde+NADP++H2O-->Acetate+NADPH+H+
R00713MM Succinatesemialdehyde+NAD++H2O-->Succinate+NADH+H+
C04246MM But-2-enoyl-[acyl-carrier protein]
C04076MM L-2-Aminoadipate 6-semialdehyde
R00708MM (S)-1-Pyrroline-5-carboxylate+NADP++2H2O-->L-Glutamate+NADPH+H+
R00707MM (S)-1-Pyrroline-5-carboxylate+NAD++2H2O-->L-Glutamate+NADH+H+
R00706MM 3-Oxopropanoate+CoA+NADP+-->Acetyl-CoA+CO2+NADPH+H+
R01702MM 4-Methyl-2-oxopentanoate+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]S-(3-methylbutanoyl)dihydrolipoyllysine+CO2
R00659MM UTP+Pyruvate-->UDP+Phosphoenolpyruvate
R01706MM Hexadecanoyl-[acp]+H2O-->Acyl-carrierprotein+Hexadecanoicacid
C04618MM (3R)-3-Hydroxybutanoyl-[acyl-carrier protein]
R00705MM 3-Oxopropanoate+CoA+NAD+-->Acetyl-CoA+CO2+NADH+H+
C04620MM (3R)-3-Hydroxyoctanoyl-[acyl-carrier protein]
R00667MM L-Ornithine+2-Oxoglutarate-->L-Glutamate5-semialdehyde+L-Glutamate
Transporter14 Dicarboxylate carrier - Succinate for sulphite
R01801MM CDP-diacylglycerol+sn-Glycerol3-phosphate-->CMP+Phosphatidylglycerophosphate
R00214Cyto (S)-Malate+NAD+-->Pyruvate+CO2+NADH+H+
Transporter32 deoxynucleotide carrier - dGTP for ATP
R00342Cyto (S)-Malate+NAD+-->Oxaloacetate+NADH+H+
Transporter13 Dicarboxylate carrier - Malate for thiosulfate
Transporter31 deoxynucleotide carrier - dATP for ATP
Transporter12 Dicarboxylate carrier - Malate for sulfate
R01778MM (3S)-3-Hydroxyacyl-CoA+NAD+-->3-Oxoacyl-CoA+NADH+H+
Transporter11 Dicarboxylate carrier - Malate for sulphite
Transporter33 deoxynucleotide carrier - dCTP for ATP
R01799MM CTP+Phosphatidate-->Pyrophosphate+CDP-diacylglycerol
R00200Cyto ATP+Pyruvate-->ADP+Phosphoenolpyruvate
R00084Cyto 4Porphobilinogen+H2O-->Hydroxymethylbilane+4NH3
R01859MM ATP+Propanoyl-CoA+HCO3--->ADP+Orthophosphate+(S)-2-Methyl-3-oxopropanoyl-CoA
Transporter16 Dicarboxylate carrier - Succinate for thiosulfate
R00004Cyto Pyrophosphate+H2O-->2Orthophosphate
R00036Cyto 25-Aminolevulinate-->Porphobilinogen+2H2O
R01858MM dGTP+Pyruvate-->dGDP+Phosphoenolpyruvate
Transporter15 Dicarboxylate carrier - Succinate for sulfate
Transporter36 deoxynucleotide carrier - dGDP for ATP
R01701MM 3-Methyl-2-oxobutanoicacid+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]S-(2-methylpropanoyl)dihydrolipoyllysine+CO2
Transporter37 deoxynucleotide carrier - dCDP for ATP
R08657MM sn-Glycerol 3-phosphate+Ubiquinone-->Glyceronephosphate+Ubiquinol
R04224MM 2-Methylprop-2-enoyl-CoA+H2O-->(S)-3-Hydroxyisobutyryl-CoA
Transporter35 deoxynucleotide carrier - dADP for ATP
Transporter17 Oxoglutarate carrier - Oxoglutarate for malate
Transporter18 Aspartate/glutamate carrier - Glutamate and H+ for aspartate
Transporter40 deoxynucleotide carrier - GDP for ATP
Transporter19 Ornithine carrier - Ornithine for citrulline and H+
Transporter20 Ornithine carrier - Lysine for citrulline and H+
Transporter38 deoxynucleotide carrier - dTDP for ATP
Transporter39 deoxynucleotide carrier - ADP for ATP
R00572MM CTP+Pyruvate-->CDP+Phosphoenolpyruvate
R00621MM 2-Oxoglutarate+Thiamindiphosphate-->3-Carboxy-1-hydroxypropyl-ThPP+CO2
C05125MM 2-(alpha-Hydroxyethyl)thiamine diphosphate
Transporter34 deoxynucleotide carrier - dTTP for ATP
R00551MM L-Arginine+H2O-->L-Ornithine+Urea
R00432MM GTP+Succinate+CoA-->GDP+Orthophosphate+Succinyl-CoA
R07618MM EnzymeN6-(dihydrolipoyl)lysine+NAD+-->EnzymeN6-(lipoyl)lysine+NADH+H+
R00431MM GTP+Oxaloacetate-->GDP+Phosphoenolpyruvate+CO2
R00014MM Pyruvate+Thiamindiphosphate-->2-(alpha-Hydroxyethyl)thiaminediphosphate+CO2
R08157MM Octanoyl-[acp]+H2O-->Acyl-carrierprotein+Octanoicacid
R00430MM GTP+Pyruvate-->GDP+Phosphoenolpyruvate
R00410MM Succinyl-CoA+Acetoacetate-->Succinate+Acetoacetyl-CoA
R00004MM Pyrophosphate+H2O-->2Orthophosphate
R01360MM (S)-3-Hydroxy-3-methylglutaryl-CoA-->Acetyl-CoA+Acetoacetate
R01361MM (R)-3-Hydroxybutanoate+NAD+-->Acetoacetate+NADH+H+
Transporter22 Ornithine carrier - Ornithine for lysine and H+
R01624MM Acetyl-CoA+Acyl-carrierprotein-->CoA+Acetyl-[acyl-carrierprotein]
R07602MM 3-Methyl-1-hydroxybutyl-ThPP+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]S-(3-methylbutanoyl)dihydrolipoyllysine+Thiamindiphosphate
Transporter21 Ornithine carrier - Arginine for citrulline and H+
R07603MM (S)-3-Methyl-2-oxopentanoicacid+Thiamindiphosphate-->2-Methyl-1-hydroxybutyl-ThPP+CO2
R01626MM Malonyl-CoA+Acyl-carrierprotein-->CoA+Malonyl-[acyl-carrierprotein]
Transporter24 Ornithine carrier - Lysine for arginine and H+
Transporter41 deoxynucleotide carrier - UDP for ATP
R07604MM 2-Methyl-1-hydroxybutyl-ThPP+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]S-(2-methylbutanoyl)dihydrolipoyllysine+Thiamindiphosphate
Transporter23 Ornithine carrier - Ornithine for arginine and H+
R01648MM 4-Aminobutanoate+2-Oxoglutarate-->Succinatesemialdehyde+L-Glutamate
R07390MM 2Phosphatidylglycerol-->Cardiolipin+Glycerol
R01655MM 5,10-Methenyltetrahydrofolate+H2O-->10-Formyltetrahydrofolate+H+
Transporter25 Ornithine carrier - L-Lysine for H+
R07599MM 3-Methyl-2-oxobutanoicacid+Thiamindiphosphate-->2-Methyl-1-hydroxypropyl-ThPP+CO2
R01699MM Pyruvate+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residueacetyltransferase]S-acetyldihydrolipoyllysine+CO2
R07600MM 2-Methyl-1-hydroxypropyl-ThPP+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]S-(2-methylpropanoyl)dihydrolipoyllysine+Thiamindiphosphate
R01700MM 2-Oxoglutarate+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residuesuccinyltransferase]S-succinyldihydrolipoyllysine+CO2
R07601MM 4-Methyl-2-oxopentanoate+Thiamindiphosphate-->3-Methyl-1-hydroxybutyl-ThPP+CO2
Transporter48 oxodicarboxylate carrier - citrate for oxoglutarate
Transporter49 oxodicarboxylate carrier - oxaloacetate for oxoglutarate
Transporter50 Unknown pyruvate carrier - Pyruvate
Transporter45 carnitine carrier - Carnitine out
Transporter46 oxodicarboxylate carrier - Oxoadipate for oxoglutarate
Transporter47 oxodicarboxylate carrier - 2-aminoadipate for oxoglutarate
Transporter42 deoxynucleotide carrier - CDP for ATP
C05378Cyto beta-D-Fructose 1,6-bisphosphate
Transporter44 carnitine carrier - Carnitine out, palmitoyl/acyl carnitine in
R00391MM Acyl-CoA+Acetyl-CoA-->CoA+3-Oxoacyl-CoA
R00405MM ATP+Succinate+CoA-->ADP+Orthophosphate+Succinyl-CoA
R02163MM COMPLEX_I_Ubiquinone+NADH-->Ubiquinol+NAD+4H+0.002O2.-
R00316MM ATP+Acetate-->Pyrophosphate+Acetyladenylate
R02164MM COMPLEX_II_Ubiquinone+Succinate-->Ubiquinol+Fumarate
R07162MM Acyl-CoA+NADP+-->trans-2,3-Dehydroacyl-CoA+NADPH+H+
R00342MM (S)-Malate+NAD+-->Oxaloacetate+NADH+H+
R05066MM (S)-3-Hydroxyisobutyrate+NAD+-->(S)-Methylmalonatesemialdehyde+NADH+H+
R02161MM COMPLEX_III_Ubiquinol+2Ferricytochromec-->Ubiquinone+2Ferrocytochromec
R06985MM trans-Hex-2-enoyl-CoA+NADPH+H+-->Hexanoyl-CoA+NADP+
R00351MM Citrate+CoA-->Acetyl-CoA+H2O+Oxaloacetate
R02085MM (S)-3-Hydroxy-3-methylglutaryl-CoA-->3-Methylglutaconyl-CoA+H2O
R00344MM ATP+Pyruvate+HCO3--->ADP+Orthophosphate+Oxaloacetate
R00355MM L-Aspartate+2-Oxoglutarate-->Oxaloacetate+L-Glutamate
R02030MM Phosphatidylglycerol+CDP-diacylglycerol-->Cardiolipin+CMP
R04965MM (R)-3-Hydroxydodecanoyl-[acp]-->trans-Dodec-2-enoyl-[acp]+H2O
R00388MM Acyl-CoA+Oxygen-->trans-2,3-Dehydroacyl-CoA+H2O2
R04967MM Tetradecanoyl-[acp]+NADP+-->trans-Tetradec-2-enoyl-[acp]+NADPH+H+
R00371MM Acetyl-CoA+Glycine-->CoA+L-2-Amino-3-oxobutanoicacid
R02313MM N6-(L-1,3-Dicarboxypropyl)-L-lysine+NAD++H2O-->L-Glutamate+L-2-Aminoadipate6-semialdehyde+NADH+H+
R04970MM Hexadecanoyl-[acp]+NADP+-->trans-Hexadec-2-enoyl-[acp]+NADPH+H+
R05064MM (S)-3-Hydroxyisobutyryl-CoA+H2O-->CoA+(S)-3-Hydroxyisobutyrate
R02199MM L-Isoleucine+2-Oxoglutarate-->(S)-3-Methyl-2-oxopentanoicacid+L-Glutamate
R02241MM Phosphatidate+CoA-->1-Acyl-sn-glycerol3-phosphate+Palmitoyl-CoA
R04968MM Tetradecanoyl-[acp]+Malonyl-[acyl-carrierprotein]-->3-Oxohexadecanoyl-[acp]+CO2+Acyl-carrierprotein
Transporter58 Phosphatidylserine carrier - Phosphatidylserine
C00671MM (S)-3-Methyl-2-oxopentanoic acid
R00248MM L-Glutamate+NADP++H2O-->2-Oxoglutarate+NH3+NADPH+H+
R04959MM Octanoyl-[acp]+NADP+-->trans-Oct-2-enoyl-[acp]+NADPH+H+
R04960MM Octanoyl-[acp]+Malonyl-[acyl-carrierprotein]-->3-Oxodecanoyl-[acp]+CO2+Acyl-carrierprotein
R00245MM L-Glutamate5-semialdehyde+NAD++H2O-->L-Glutamate+NADH+H+
R01923MM Palmitoyl-CoA+L-Carnitine-->CoA+L-Palmitoylcarnitine
R04962MM Decanoyl-[acp]+NADP+-->trans-Dec-2-enoyl-[acp]+NADPH+H+
R01939MM L-2-Aminoadipate+2-Oxoglutarate-->2-Oxoadipate+L-Glutamate
R00261MM L-Glutamate-->4-Aminobutanoate+CO2
R04963MM Decanoyl-[acp]+Malonyl-[acyl-carrierprotein]-->3-Oxododecanoyl-[acp]+CO2+Acyl-carrierprotein
R04964MM (R)-3-Hydroxydodecanoyl-[acp]+NADP+-->3-Oxododecanoyl-[acp]+NADPH+H+
R00258MM L-Alanine+2-Oxoglutarate-->Pyruvate+L-Glutamate
R00256MM L-Glutamine+H2O-->L-Glutamate+NH3
R01899MM Isocitrate+NADP+-->Oxalosuccinate+NADPH+H+
R02029MM Phosphatidylglycerophosphate+H2O-->Phosphatidylglycerol+Orthophosphate
Transporter5 Citrate carrier - Citrate for Isocitrate
R00274MM H2O2+2Glutathione-->Glutathionedisulfide+2H2O
Transporter7 ADP/ATP carrier - ADP for ATP
Transporter6 Phosphate carrier - Phosphate and H+ together
R00268MM Oxalosuccinate-->2-Oxoglutarate+CO2
R01940MM 2-Oxoadipate+Lipoamide-->S-Glutaryldihydrolipoamide+CO2
Transporter2 Citrate carrier - Malate for isocitrate
R04954MM (R)-3-Hydroxyhexanoyl-[acp]-->trans-Hex-2-enoyl-[acp]+H2O
Transporter1 Citrate carrier - Malate for citrate
R01975MM (S)-3-Hydroxybutanoyl-CoA+NAD+-->Acetoacetyl-CoA+NADH+H+
R04956MM Hexanoyl-[acp]+NADP+-->trans-Hex-2-enoyl-[acp]+NADPH+H+
Transporter4 Citrate carrier - PEP for isocitrate
R01978MM (S)-3-Hydroxy-3-methylglutaryl-CoA+CoA-->Acetyl-CoA+H2O+Acetoacetyl-CoA
R04957MM Hexanoyl-[acp]+Malonyl-[acyl-carrierprotein]-->3-Oxooctanoyl-[acp]+CO2+Acyl-carrierprotein
Transporter3 Citrate carrier - PEP for citrate
Transporter59 Phosphatidylcholine carrier - Phosphatidylcholine
Transporter60 Phosphatidylethanolamine carrier - Phosphatidylethanolamine
C15973MM Enzyme N6-(dihydrolipoyl)lysine
R00243MM L-Glutamate+NAD++H2O-->2-Oxoglutarate+NH3+NADH+H+
This model is from the article: A metabolic model of the mitochondrion and its use in modelling diseases of the tricarboxylic acid cycle. Smith AC, Robinson AJ. BMC Syst Biol. 2011 Jun 29;5(1):102. 21714867 , Abstract: BACKGROUND:Mitochondria are a vital component of eukaryotic cells and their dysfunction is implicated in a large number of metabolic, degenerative and age-related human diseases. The mechanism or these disorders can be difficult to elucidate due to the inherent complexity of mitochondrial metabolism. To understand how mitochondrial metabolic dysfunction contributes to these diseases, a metabolic model of a human heart mitochondrion was created. RESULTS: A new model of mitochondrial metabolism was built on the principle of metabolite availability using MitoMiner, a mitochondrial proteomics database, to evaluate the subcellular localisation of reactions that have evidence for mitochondrial localisation. Extensive curation and manual refinement was used to create a model called iAS253, containing 253 reactions, 245 metabolites and 89 transport steps across the inner mitochondrial membrane. To demonstrate the predictive abilities of the model, flux balance analysis was used to calculate metabolite fluxes under normal conditions and to simulate three metabolic disorders that affect the TCA cycle: fumarase deficiency, succinate dehydrogenase deficiency and alpha-ketoglutarate dehydrogenase deficiency. CONCLUSION: The results of simulations using the new model corresponded closely with phenotypic data under normal conditions and provided insight into the complicated and unintuitive phenotypes of the three disorders, including the effect of interventions that may be of therapeutic benefit, such as low glucose diets or amino acid supplements. The model offers the ability to investigate other mitochondrial disorders and can provide the framework for the integration of experimental data in future studies. Created by Anthony C. Smith (Mon Mar 14 11:03:11 2011) MRC - Mitochondrial Biology Unit, Cambridge, UK. acs@mrc-mbu.cam.ac.uk This metabolic network is a stocihiometric model of a human heart mitochondrion. Identifiers are KEGG Ids. This model originates from BioModels Database: A Database of Annotated Published Models (http://www.ebi.ac.uk/biomodels/). It is copyright (c) 2005-2011 The BioModels.net Team. To the extent possible under law, all copyright and related or neighbouring rights to this encoded model have been dedicated to the public domain worldwide. Please refer to CC0 Public Domain Dedication for more information. In summary, you are entitled to use this encoded model in absolutely any manner you deem suitable, verbatim, or with modification, alone or embedded it in a larger context, redistribute it, commercially or not, in a restricted way or not.. To cite BioModels Database, please use: Li C, Donizelli M, Rodriguez N, Dharuri H, Endler L, Chelliah V, Li L, He E, Henry A, Stefan MI, Snoep JL, Hucka M, Le Novère N, Laibe C (2010) BioModels Database: An enhanced, curated and annotated resource for published quantitative kinetic models. BMC Syst Biol., 4:92.
R00238MM 2Acetyl-CoA-->CoA+Acetoacetyl-CoA
Transporter62 Diffusion? - Butanoic acid
R00239MM ATP+L-Glutamate-->ADP+L-Glutamyl5-phosphate
C00449MM N6-(L-1,3-Dicarboxypropyl)-L-lysine
Transporter64 Unknown amino acid carrier - L-Asparagine
R00236MM Acetyladenylate+CoA-->AMP+Acetyl-CoA
C15979MM [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]
R03026MM (S)-3-Hydroxybutanoyl-CoA-->Crotonoyl-CoA+H2O
Transporter65 Unknown amino acid carrier - L-Cysteine
R02765MM (R)-Methylmalonyl-CoA --> (S)-Methylmalonyl-CoA
R00216MM (S)-Malate+NADP+-->Pyruvate+CO2+NADPH+H+
R00205MM Methylglyoxal+NADP++H2O-->Pyruvate+NADPH+H+
R03103MM L-2-Aminoadipate6-semialdehyde+NADP++H2O-->L-2-Aminoadipate+NADPH+H+
R03102MM L-2-Aminoadipate6-semialdehyde+NAD++H2O-->L-2-Aminoadipate+NADH+H+
R00214MM (S)-Malate+NAD+-->Pyruvate+CO2+NADH+H+
Transporter66 Unknown amino acid carrier - L-Glutamine
C15975MM [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]
R03105MM Mercaptopyruvate+Sulfite-->Thiosulfate+Pyruvate
C15977MM [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]
C04633MM (3R)-3-Hydroxypalmitoyl-[acyl-carrier protein]
C04619MM (3R)-3-Hydroxydecanoyl-[acyl-carrier protein]
R03170MM 2(S)-2-Methylbutanoyl-CoA+Oxygen-->22-Methylbut-2-enoyl-CoA+2H2O
R04747MM Hexanoyl-CoA+Acetyl-CoA-->CoA+3-Oxooctanoyl-CoA
R04746MM (S)-Hydroxyoctanoyl-CoA-->trans-Oct-2-enoyl-CoA+H2O
R03172MM (S)-2-Methylbutanoyl-CoA+FAD-->2-Methylbut-2-enoyl-CoA+FADH2
R03174MM (S)-2-Methylbutanoyl-CoA+EnzymeN6-(dihydrolipoyl)lysine-->CoA+[Dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]S-(2-methylbutanoyl)dihydrolipoyllysine
R03222MM 2ProtoporphyrinogenIX+3Oxygen-->2Protoporphyrin+6H2O
R03270MM 2-(alpha-Hydroxyethyl)thiaminediphosphate+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residueacetyltransferase]S-acetyldihydrolipoyllysine+Thiamindiphosphate
R04953MM (R)-3-Hydroxyhexanoyl-[acp]+NADP+-->3-Oxohexanoyl-[acp]+NADPH+H+
R04952MM Butyryl-[acp]+Malonyl-[acyl-carrierprotein]-->3-Oxohexanoyl-[acp]+CO2+Acyl-carrierprotein
R04754MM Decanoyl-CoA+FAD-->trans-Dec-2-enoyl-CoA+FADH2
R04753MM Decanoyl-CoA+NADP+-->trans-Dec-2-enoyl-CoA+NADPH+H+
R04751MM Hexanoyl-CoA+FAD-->trans-Hex-2-enoyl-CoA+FADH2
R04749MM (S)-Hydroxyhexanoyl-CoA-->trans-Hex-2-enoyl-CoA+H2O
Transporter67 Unknown amino acid carrier - L-Proline
R04748MM (S)-Hydroxyhexanoyl-CoA+NAD+-->3-Oxohexanoyl-CoA+NADH+H+
Transporter70 Unknown amino acid carrier - L-Methionine
R01220MM 5,10-Methylenetetrahydrofolate+NADP+-->5,10-Methenyltetrahydrofolate+NADPH+H+
Transporter69 Unknown amino acid carrier - L-Histidine
R01218MM 5,10-Methylenetetrahydrofolate+NAD+-->5,10-Methenyltetrahydrofolate+NADH+H+
Transporter68 Unknown amino acid carrier - L-Tyrosine
R02661MM 2-Methylpropanoyl-CoA+FAD-->2-Methylprop-2-enoyl-CoA+FADH2
Transporter74 Unknown amino acid carrier - L-Isoleucine
R01214MM L-Valine+2-Oxoglutarate-->3-Methyl-2-oxobutanoicacid+L-Glutamate
R00086MM ATP_SYNTHASE_ADP+Orthophosphate+4H+-->ATP+H2O
R04745MM (S)-Hydroxyoctanoyl-CoA+NAD+-->3-Oxooctanoyl-CoA+NADH+H+
Transporter73 Unknown amino acid carrier - Glycine
R00094MM 2Glutathione+NAD+-->Glutathionedisulfide+NADH+H+
Transporter72 Unknown amino acid carrier - L-Tryptophan
R01177MM Acetyl-CoA+Butanoyl-CoA-->CoA+3-Oxohexanoyl-CoA
Transporter71 Unknown amino acid carrier - L-Phenylalanine
R00115MM 2Glutathione+NADP+-->Glutathionedisulfide+NADPH+H+
R01176MM ATP+Butanoicacid+CoA-->AMP+Pyrophosphate+Butanoyl-CoA
C00356MM (S)-3-Hydroxy-3-methylglutaryl-CoA
R01175MM Butanoyl-CoA+FAD-->FADH2+Crotonoyl-CoA
R02569MM Acetyl-CoA+EnzymeN6-(dihydrolipoyl)lysine-->CoA+[Dihydrolipoyllysine-residueacetyltransferase]S-acetyldihydrolipoyllysine
R01171MM Butanoyl-CoA+NAD+-->Crotonoyl-CoA+NADH+H+
R02529MM Aminoacetone+H2O+Oxygen-->Methylglyoxal+NH3+H2O2
R02487MM Glutaryl-CoA+FAD-->FADH2+Crotonoyl-CoA+CO2
C06157MM [Dihydrolipoyllysine-residue succinyltransferase]
R00081MM COMPLEX_IV_Oxygen+4Ferrocytochromec-->4Ferricytochromec+2H2O
Transporter75 Unknown amino acid carrier - L-Leucine
C06002MM (S)-Methylmalonate semialdehyde
R02660MM 22-Methylpropanoyl-CoA+Oxygen-->22-Methylprop-2-enoyl-CoA+2H2O
R02571MM Glutaryl-CoA+Dihydrolipoamide-->CoA+S-Glutaryldihydrolipoamide
R02570MM Succinyl-CoA+EnzymeN6-(dihydrolipoyl)lysine-->CoA+[Dihydrolipoyllysine-residuesuccinyltransferase]S-succinyldihydrolipoyllysine
R02685MM (3S)-3-Hydroxyacyl-CoA-->trans-2,3-Dehydroacyl-CoA+H2O
R02662MM 2-Methylpropanoyl-CoA+EnzymeN6-(dihydrolipoyl)lysine-->CoA+[Dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]S-(2-methylpropanoyl)dihydrolipoyllysine
R04742MM Decanoyl-CoA+Acetyl-CoA-->CoA+3-Oxododecanoyl-CoA
R04741MM (S)-3-Hydroxydodecanoyl-CoA+NAD+-->3-Oxododecanoyl-CoA+NADH+H+
R04744MM (S)-Hydroxydecanoyl-CoA-->trans-Dec-2-enoyl-CoA+H2O
R04743MM (S)-Hydroxydecanoyl-CoA+NAD+-->3-Oxodecanoyl-CoA+NADH+H+
Transporter76 Unknown amino acid carrier - L-Valine
R04739MM (S)-3-Hydroxytetradecanoyl-CoA+NAD+-->3-Oxotetradecanoyl-CoA+NADH
Transporter77 Unknown carrier - Glutathione
R04738MM (S)-3-Hydroxyhexadecanoyl-CoA-->trans-Hexadec-2-enoyl-CoA+H2O
R04740MM (S)-3-Hydroxytetradecanoyl-CoA-->trans-Tetradec-2-enoyl-CoA+H2O
R00941MM 10-Formyltetrahydrofolate+NADP++H2O-->Tetrahydrofolate+CO2+NADPH+H+
R03197Cyto UroporphyrinogenIII-->CoproporphyrinogenIII+4CO2
Transporter82 Unknown carrier - Acetoacetate
R04726MM Dodecanoyl-[acyl-carrierprotein]+Malonyl-[acyl-carrierprotein]-->3-Oxotetradecanoyl-[acp]+CO2+Acyl-carrierprotein
R00935MM (S)-Methylmalonatesemialdehyde+CoA+NAD+-->Propanoyl-CoA+CO2+NADH+H+
R03165Cyto Hydroxymethylbilane-->UroporphyrinogenIII+H2O
Transporter81 Unknown carrier - Fumarate
R04737MM (S)-3-Hydroxyhexadecanoyl-CoA+NAD+-->3-Oxopalmitoyl-CoA+NADH+H+
R04779Cyto ATP+beta-D-Fructose6-phosphate-->ADP+beta-D-Fructose1,6-bisphosphate
R00945MM 5,10-Methylenetetrahydrofolate+Glycine+H2O-->Tetrahydrofolate+L-Serine
R03220Cyto CoproporphyrinogenIII+Oxygen-->ProtoporphyrinogenIX+2CO2+2H2O
R04725MM Dodecanoyl-[acyl-carrierprotein]+NADP+-->trans-Dodec-2-enoyl-[acp]+NADPH+H+
Transporter79 Unknown carrier - (R)-3-Hydroxybutanoate
R01090MM L-Leucine+2-Oxoglutarate-->4-Methyl-2-oxopentanoate+L-Glutamate
Transporter78 Unknown carrier - 5-Aminolevulinate
R01138MM dATP+Pyruvate-->dADP+Phosphoenolpyruvate
R01354MM ATP+Propanoate-->Pyrophosphate+Propinoladenylate
R03989MM Tetradecanoyl-CoA+NADP+-->trans-Tetradec-2-enoyl-CoA+NADPH+H+
R03858MM Lauroyl-CoA+Acetyl-CoA-->CoA+3-Oxotetradecanoyl-CoA
R00927MM Propanoyl-CoA+Acetyl-CoA-->CoA+2-Methylacetoacetyl-CoA
R03990MM Tetradecanoyl-CoA+FAD-->trans-Tetradec-2-enoyl-CoA+FADH2
R00926MM Propinoladenylate+CoA-->AMP+Propanoyl-CoA
R04170MM (S)-3-Hydroxydodecanoyl-CoA-->2-trans-Dodecenoyl-CoA+H2O
R01280MM ATP+Hexadecanoicacid+CoA-->AMP+Palmitoyl-CoA+Pyrophosphate
R04203MM (2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA+NAD+-->2-Methylacetoacetyl-CoA+NADH+H+
R01279MM Palmitoyl-CoA+FAD-->trans-Hexadec-2-enoyl-CoA+FADH2
R04204MM (2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA-->2-Methylbut-2-enoyl-CoA+H2O
R01278MM Palmitoyl-CoA+NADP+-->trans-Hexadec-2-enoyl-CoA+NADPH+H+
R01253MM L-Proline+FAD-->(S)-1-Pyrroline-5-carboxylate+FADH2
R04095MM 3-Methylbutanoyl-CoA+FAD-->3-Methylcrotonyl-CoA+FADH2
R01251MM L-Proline+NADP+-->(S)-1-Pyrroline-5-carboxylate+NADPH+H+
R04097MM 3-Methylbutanoyl-CoA+EnzymeN6-(dihydrolipoyl)lysine-->CoA+[Dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]S-(3-methylbutanoyl)dihydrolipoyllysine
R01248MM L-Proline+NAD+-->(S)-1-Pyrroline-5-carboxylate+NADH+H+
R04138MM ATP+3-Methylcrotonyl-CoA+HCO3--->ADP+Orthophosphate+3-Methylglutaconyl-CoA
R04537MM (3R)-3-Hydroxyoctanoyl-[acyl-carrierprotein]-->trans-Oct-2-enoyl-[acp]+H2O
R00895MM L-Cysteine+2-Oxoglutarate-->Mercaptopyruvate+L-Glutamate
C00236Cyto 3-Phospho-D-glyceroyl phosphate
R03991MM Tetradecanoyl-CoA+Acetyl-CoA-->CoA+3-Oxopalmitoyl-CoA
R04568MM (3R)-3-Hydroxytetradecanoyl-[acyl-carrierprotein]-->trans-Tetradec-2-enoyl-[acp]+H2O
R04566MM (3R)-3-Hydroxytetradecanoyl-[acyl-carrierprotein]+NADP+-->3-Oxotetradecanoyl-[acp]+NADPH+H+
R04544MM (3R)-3-Hydroxypalmitoyl-[acyl-carrierprotein]-->trans-Hexadec-2-enoyl-[acp]+H2O
R04543MM (3R)-3-Hydroxypalmitoyl-[acyl-carrierprotein]+NADP+-->3-Oxohexadecanoyl-[acp]+NADPH+H+
C16254MM [Dihydrolipoyllysine-residue succinyltransferase]
R00740MM 3-Oxopropanoate+CoA+NADP+-->Malonyl-CoA+NADPH+H+
R01070Cyto beta-D-Fructose1,6-bisphosphate-->Glyceronephosphate+D-Glyceraldehyde3-phosphate
R04433MM Ubiquinone+FADH2-->Ubiquinol+FAD+
R01061Cyto D-Glyceraldehyde3-phosphate+Orthophosphate+NAD+-->3-Phospho-D-glyceroylphosphate+NADH+H+
R00727MM ITP+Succinate+CoA-->IDP+Orthophosphate+Succinyl-CoA
R04533MM (3R)-3-Hydroxybutanoyl-[acyl-carrierprotein]+NADP+-->Acetoacetyl-[acp]+NADPH+H+
R01015Cyto D-Glyceraldehyde3-phosphate-->Glyceronephosphate
R00726MM ITP+Oxaloacetate-->IDP+Phosphoenolpyruvate+CO2
R04534MM (3R)-3-Hydroxydecanoyl-[acyl-carrierprotein]+NADP+-->3-Oxodecanoyl-[acp]+NADPH
R00842Cyto sn-Glycerol3-phosphate+NAD+-->Glyceronephosphate+NADH+H+
R00851MM sn-Glycerol3-phosphate+Palmitoyl-CoA-->1-Acyl-sn-glycerol3-phosphate+CoA
R04535MM (3R)-3-Hydroxydecanoyl-[acyl-carrierprotein]-->trans-Dec-2-enoyl-[acp]+H2O
R00833MM (R)-2-Methyl-3-oxopropanoyl-CoA-->Succinyl-CoA
R04536MM (3R)-3-Hydroxyoctanoyl-[acyl-carrierprotein]+NADP+-->3-Oxooctanoyl-[acp]+NADPH+H+
R01280Cyto ATP+Hexadecanoicacid+CoA-->AMP+Palmitoyl-CoA+Pyrophosphate
R00830MM Succinyl-CoA+Glycine-->5-Aminolevulinate+CoA+CO2
R00742MM ATP+Acetyl-CoA+HCO3--->ADP+Orthophosphate+Malonyl-CoA
C16255MM [Dihydrolipoyllysine-residue acetyltransferase]
R03758MM Aminoacetone+CO2-->L-2-Amino-3-oxobutanoicacid
R03381MM (S)-Methylmalonatesemialdehyde+CoA+NAD+-->(R)-2-Methyl-3-oxopropanoyl-CoA+NADH+H+
R03316MM 3-Carboxy-1-hydroxypropyl-ThPP+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residuesuccinyltransferase]S-succinyldihydrolipoyllysine+Thiamindiphosphate
R03314MM L-Glutamate5-semialdehyde-->(S)-1-Pyrroline-5-carboxylate+H2O
R03313MM L-Glutamate5-semialdehyde+Orthophosphate+NADP+-->L-Glutamyl5-phosphate+NADPH+H+
R03857MM Lauroyl-CoA+FAD-->2-trans-Dodecenoyl-CoA+FADH2
R03778MM Octanoyl-CoA+Acetyl-CoA-->CoA+3-Oxodecanoyl-CoA
R03856MM Lauroyl-CoA+NADP+-->2-trans-Dodecenoyl-CoA+NADPH+H+
R03776MM Octanoyl-CoA+NADP+-->trans-Oct-2-enoyl-CoA+NADPH+H+
C05223MM Dodecanoyl-[acyl-carrier protein]
R03777MM Octanoyl-CoA+FAD-->trans-Oct-2-enoyl-CoA+FADH2
C04688MM (3R)-3-Hydroxytetradecanoyl-[acyl-carrier protein]
R01512Cyto ATP+3-Phospho-D-glycerate-->ADP+3-Phospho-D-glyceroylphosphate
R00724MM ITP+Pyruvate-->IDP+Phosphoenolpyruvate
R01518Cyto 2-Phospho-D-glycerate-->3-Phospho-D-glycerate
R00716MM N6-(L-1,3-Dicarboxypropyl)-L-lysine+NADP++H2O-->L-Lysine+2-Oxoglutarate+NADPH+H+
R04355MM Acetyl-[acyl-carrierprotein]+Malonyl-[acyl-carrierprotein]-->Acetoacetyl-[acp]+CO2+Acyl-carrierprotein
R02740Cyto alpha-D-Glucose6-phosphate-->beta-D-Fructose6-phosphate
R04225MM (S)-3-Methyl-2-oxopentanoicacid+EnzymeN6-(lipoyl)lysine-->[Dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]S-(2-methylbutanoyl)dihydrolipoyllysine+CO2
R04430MM Butyryl-[acp]+NADP+-->But-2-enoyl-[acyl-carrierprotein]+NADPH+H+
R01786Cyto ATP+alpha-D-Glucose-->ADP+alpha-D-Glucose6-phosphate
R01923Cyto Palmitoyl-CoA+L-Carnitine-->CoA+L-Palmitoylcarnitine
R04428MM (3R)-3-Hydroxybutanoyl-[acyl-carrierprotein]-->But-2-enoyl-[acyl-carrierprotein]+H2O