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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 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