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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 alpha, mitochondrial 2- oxoisovalerate dehydrogenase 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 acetyltransferase component of pyruvate 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 Dihydrolipoyllysine- residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial 2- oxoisovalerate dehydrogenase 2- oxoisovalerate dehydrogenase Dihydrolipoyllysine- residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial Short-chain specific acyl-CoA dehydrogenase, mitochondrial Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial Short-chain specific acyl-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 Methylmalonyl CoA mutase Aldehyde oxidase 1 Aldehyde 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 Accumulation 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 (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