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Pyruvate dehydrogenase E1 component subunit beta, mitochondrial Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial Glutathione reductase, mitochondrial Hydroxymethylglutaryl- CoA lyase, mitochondrial Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial Pyruvate dehydrogenase E1 component subunit beta, mitochondrial 2- oxoisovalerate dehydrogenase subunit alpha, mitochondrial 2- oxoisovalerate dehydrogenase subunit beta, mitochondrial Branched- chain-amino- acid aminotransferase, cytosolic Branched- chain-amino- acid aminotransferase, cytosolic Branched- chain-amino- acid aminotransferase, cytosolic 2- oxoisovalerate dehydrogenase Dihydrolipoyllysine- residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial Isovaleryl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial Pyruvate carboxylase, mitochondrial Propionyl-CoA carboxylase beta chain, mitochondrial Glutathione reductase, mitochondrial Dihydrolipoyllysine- residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 2- oxoisovalerate dehydrogenase 2- oxoisovalerate dehydrogenase Dihydrolipoyllysine- residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial Short-chain specific acyl-CoA dehydrogenase, mitochondrial Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial Isobutyryl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial 3- hydroxyisobutyryl- CoA hydrolase, mitochondrial 3- hydroxyisobutyrate dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial 4-aminobutyrate aminotransferase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Short-chain specific acyl-CoA dehydrogenase, mitochondrial Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial 15- hydroxyprostaglandin dehydrogenase [NAD(+)] Acetyl-CoA acetyltransferase, mitochondrial Succinate- semialdehyde dehydrogenase, mitochondrial Pyruvate carboxylase, mitochondrial Propionyl-CoA carboxylase beta chain, mitochondrial Methylmalonyl- CoA epimerase, mitochondrial Methylmalonyl- CoA mutase, mitochondrial Aldehyde oxidase 1 Succinate- semialdehyde dehydrogenase, mitochondrial Methylglutaconyl- CoA hydratase, mitochondrial Hydroxymethylglutaryl- CoA synthase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Succinyl- CoA:3- ketoacid coenzyme A transferase 1, mitochondrial (S)-beta- Aminoisobutyric acid Acetyl-CoA Propionyl-CoA Acetyl-CoA Succinyl-CoA 3-Methyl-1-hydroxybutyl-ThPP Lipoamide S-(3-Methylbutanoyl)- dihydrolipoamide-E Dihydrolipoamide NAD NADH H+ 3-Hydroxy-3-methylglutaryl-CoA Acetoacetic acid 2-Methyl-1-hydroxypropyl-ThPP Thiamine pyrophosphate S-(2-Methylpropionyl)- dihydrolipoamide-E Lipoamide 2-Methyl-1-hydroxypropyl-ThPP S-(2-Methylbutanoyl)- dihydrolipoamide Thiamine pyrophosphate L-Valine Oxoglutaric acid α- Ketoisovaleric acid L-Glutamic acid L-Leucine Oxoglutaric acid Ketoleucine L-Glutamic acid L-Isoleucine Oxoglutaric acid 3-Methyl-2- oxovaleric acid L-Glutamic acid Thiamine pyrophosphate CO2 CoA Isovaleryl-CoA 3-Methylcrotonyl-CoA 3-Hydroxyisovaleryl-CoA H2O ATP Hydrogen carbonate 3-Methylglutaconyl-CoA ADP Dihydrolipoamide NAD NADH H+ CoA Isobutyryl-CoA CO2 CO2 CoA 2-Methylbutyryl-CoA Methacrylyl-CoA H2O (S)-3-Hydroxyisobutyryl-CoA H2O Accumulation (S)-3- Hydroxyisobutyric acid CoA NAD (S)- Methylmalonic acid semialdehyde NADH H+ Oxoglutaric acid L-Glutamic acid Tiglyl-CoA H2O 2-Methyl-3-hydroxybutyryl-CoA NAD 2-Methylacetoacetyl-CoA NADH CoA H2O NAD CoA NADH Hydrogen carbonate ATP Hydrogen carbonate S-Methylmalonyl-CoA ADP Pi R-Methylmalonyl-CoA H2O O2 Methylmalonic acid Hydrogen peroxide NAD H2O NADH H2O Acetoacetyl-CoA H2O CoA CoA Succinyl-CoA Succinic acid Thiamine pyrophosphate FAD Thiamine pyrophosphate Pyridoxal Pyridoxal Pyridoxal Pyridoxal 5'-phosphate Lipoyl-GMP FAD FAD Biotin FAD Lipoyl-GMP Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Lipoyl-GMP FAD FAD FAD FAD Pyridoxal 5'-phosphate FAD FAD FAD Biotin Adenosylcobalamin FAD Molybdopterin 2Fe-2S electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Pyrimidine Metabolism Citric Acid Cycle Propanoate Metabolism Citric Acid Cycle Citric Acid Cycle
Mitochondria Pdhb Pdha2 Gsr Hmgcl Pdha2 Pdhb Pdha2 Pdhb Bcat1 Bcat1 Bcat1 Pdha2 Pdhb Dlst Ivd Acadm Echs1 Pc Pccb Gsr Dlst Pdha2 Pdhb Pdha2 Pdhb Dlst Acads Acadsb Acad8 Acadm Echs1 Hibch Hibadh Echs1 Abat Acadm Acads Acadsb Echs1 Hpgd Acat1 Aldh5a1 Pc Pccb Mcee Mut Aox1 Aldh5a1 Auh Hmgcs2 Acat1 Oxct1 (S)-beta- Aminoisobutyric acid Acetyl-CoA Propionyl-CoA Acetyl-CoA Succinyl-CoA 3-Methyl-1- hydroxybutyl- ThPP Lipoamide S-(3- Methylbutanoyl)- dihydrolipoamide- E Dihydrolipoamide NAD NADH Hydrogen Ion 3-Hydroxy-3- methylglutaryl- CoA Acetoacetic acid 2-Methyl-1- hydroxypropyl- ThPP S-(2- Methylpropionyl)- dihydrolipoamide- E Lipoamide 2-Methyl-1- hydroxypropyl- ThPP S-(2- Methylbutanoyl)- dihydrolipoamide Thiamine pyrophosphate L-Valine Oxoglutaric acid α- Ketoisovaleric acid L-Glutamic acid L-Leucine Oxoglutaric acid Ketoleucine L-Glutamic acid L-Isoleucine Oxoglutaric acid 3-Methyl-2- oxovaleric acid L-Glutamic acid Thiamine pyrophosphate Carbon dioxide Coenzyme A Isovaleryl-CoA 3- Methylcrotonyl- CoA 3- Hydroxyisovaleryl- CoA Water Adenosine triphosphate Hydrogen carbonate 3- Methylglutaconyl- CoA Adenosine diphosphate Dihydrolipoamide NAD NADH Hydrogen Ion Coenzyme A Isobutyryl-CoA Carbon dioxide Carbon dioxide Coenzyme A 2- Methylbutyryl- CoA Methacrylyl-CoA Water (S)-3- Hydroxyisobutyryl- CoA Water Accumulation (S)-3- Hydroxyisobutyric acid Coenzyme A NAD (S)- Methylmalonic acid semialdehyde NADH Hydrogen Ion Oxoglutaric acid L-Glutamic acid Tiglyl-CoA Water 2-Methyl-3- hydroxybutyryl- CoA NAD 2- Methylacetoacetyl- CoA NADH Coenzyme A Water NAD Coenzyme A NADH Hydrogen carbonate Adenosine triphosphate Hydrogen carbonate S- Methylmalonyl- CoA Adenosine diphosphate Phosphate R- Methylmalonyl- CoA Water Oxygen Methylmalonic acid Hydrogen peroxide NAD Water NADH Water Acetoacetyl-CoA Water Coenzyme A Coenzyme A Succinyl-CoA Succinic acid electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Pyrimidine Metabolism Citric Acid Cycle Propanoate Metabolism Citric Acid Cycle Citric Acid Cycle
Pdhb Pdha2 Gsr Hmgcl Pdha2 Pdhb Pdha2 Pdhb Bcat1 Bcat1 Bcat1 Pdha2 Pdhb Dlst Ivd Acadm Echs1 Pc Pccb Gsr Dlst Pdha2 Pdhb Pdha2 Pdhb Dlst Acads Acadsb Acad8 Acadm Echs1 Hibch Hibadh Echs1 Abat Acadm Acads Acadsb Echs1 Hpgd Acat1 Aldh5a1 Pc Pccb Mcee Mut Aox1 Aldh5a1 Auh Hmgcs2 Acat1 Oxct1 L2MBA Ac-CoA PropCoA Ac-CoA Suc-CoA 3MhThPP Lipoamd S3MdipE DHLipoa NAD NADH H+ HMG-CoA LIN 2M1ThPP ThiamPP S2MDE Lipoamd 2M1ThPP S2MD ThiamPP Val AKG Ketoval Glu Leu AKG Ketoleu Glu Ile AKG 3M2Oxva Glu ThiamPP CO2 CoA IsovCoA 3McroCA HIV-CoA H2O ATP HCO3 3MCoA ADP DHLipoa NAD NADH H+ CoA IsoBCoA CO2 CO2 CoA 2IbuCoA McryCoA H2O 3HIsoCA H2O Accumulation 3-HIBA CoA NAD 22M3O NADH H+ AKG Glu Tig-CoA H2O 2M3HC NAD 2Ma-CoA NADH CoA H2O NAD CoA NADH HCO3 ATP HCO3 S-MmCoA ADP Pi R-MmCoA H2O O2 Isosca H2O2 NAD H2O NADH H2O ActaCoA H2O CoA CoA Suc-CoA Succini ThiamPP FAD ThiamPP Pyridox Pyridox Pyridox Pyr-5'P L-GMP FAD FAD Biotin FAD L-GMP Pyr-5'P Pyr-5'P L-GMP FAD FAD FAD FAD Pyr-5'P FAD FAD FAD Biotin Adnscbn FAD mlyBdpn 2Fe2S ETF RETF ETF RETF ETF RETF PW000160 PW000005 PW000149 PW000005 PW000005
Mitochondria Pdhb Pdha2 Gsr Hmgcl Pdha2 Pdhb Pdha2 Pdhb Bcat1 Bcat1 Bcat1 Pdha2 Pdhb Dlst Ivd Acadm Echs1 Pc Pccb Gsr Dlst Pdha2 Pdhb Pdha2 Pdhb Dlst Acads Acadsb Acad8 Acadm Echs1 Hibch Hibadh Echs1 Abat Acadm Acads Acadsb Echs1 Hpgd Acat1 Aldh5a1 Pc Pccb Mcee Mut Aox1 Aldh5a1 Auh Hmgcs2 Acat1 Oxct1 L2MBA Ac-CoA PropCoA Ac-CoA Suc-CoA 3MhThPP Lipoamd S3MdipE DHLipoa NAD NADH H+ HMG-CoA LIN 2M1ThPP S2MDE Lipoamd 2M1ThPP S2MD ThiamPP Val AKG Ketoval Glu Leu AKG Ketoleu Glu Ile AKG 3M2Oxva Glu ThiamPP CO2 CoA IsovCoA 3McroCA HIV-CoA H2O ATP HCO3 3MCoA ADP DHLipoa NAD NADH H+ CoA IsoBCoA CO2 CO2 CoA 2IbuCoA McryCoA H2O 3HIsoCA H2O Accumulation 3-HIBA CoA NAD 22M3O NADH H+ AKG Glu Tig-CoA H2O 2M3HC NAD 2Ma-CoA NADH CoA H2O NAD CoA NADH HCO3 ATP HCO3 S-MmCoA ADP Pi R-MmCoA H2O O2 Isosca H2O2 NAD H2O NADH H2O ActaCoA H2O CoA CoA Suc-CoA Succini ETF RETF ETF RETF ETF RETF PW000160 PW000005 PW000149 PW000005 PW000005