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2-oxoisovalerate dehydrogenase subunit beta, mitochondrial 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial Hydroxymethylglutaryl- CoA lyase, mitochondrial 2-oxoisovalerate dehydrogenase subunit beta, 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 Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial Isovaleryl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial Methylcrotonoyl- CoA carboxylase subunit alpha, mitochondrial Methylcrotonoyl- CoA carboxylase beta chain, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial 2-oxoisovalerate dehydrogenase 2-oxoisovalerate dehydrogenase Lipoamide acyltransferase component of branched-chain alpha-keto acid 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 3-hydroxyacyl-CoA dehydrogenase type-2 3-ketoacyl-CoA thiolase, mitochondrial Methylmalonate- semialdehyde dehydrogenase [acylating], mitochondrial Propionyl-CoA carboxylase alpha chain, mitochondrial Propionyl-CoA carboxylase beta chain, mitochondrial Methylmalonyl-CoA epimerase, mitochondrial Methylmalonyl-CoA mutase, mitochondrial Aldehyde oxidase Aldehyde dehydrogenase, mitochondrial Methylglutaconyl- CoA hydratase, mitochondrial Hydroxymethylglutaryl- CoA synthase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Succinyl-CoA:3- ketoacid coenzyme A transferase 1, mitochondrial 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial 2-oxoisovalerate dehydrogenase subunit alpha, 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 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 HCO3- 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 (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