Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
Probable enoyl-CoA hydratase 2, mitochondrial
Hydroxymethylglutaryl-CoA lyase, mitochondrial
Dihydrolipoyl dehydrogenase 2, mitochondrial
2-oxoisovalerate dehydrogenase subunit alpha 2, mitochondrial
2-oxoisovalerate dehydrogenase subunit beta 2, mitochondrial
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
Branched-chain-amino-acid aminotransferase 2, chloroplastic
Branched-chain-amino-acid aminotransferase 3, chloroplastic
Branched-chain-amino-acid aminotransferase 5, chloroplastic
3-Hydroxy-3-methylglutaryl-CoA → Acetoacetic acid + Acetyl-CoA
Isovaleryl-CoA + Oxidized flavoprotein → 3-Methylcrotonyl-CoA + Reduced flavoprotein
3-Methylglutaconyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA
3-Methylcrotonyl-CoA + Adenosine triphosphate + Hydrogen carbonate → 3-Methylglutaconyl-CoA + Adenosine diphosphate + Hydrogen Ion + Phosphate
Branched-chain-amino-acid aminotransferase 1, mitochondrial
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic acid
Coenzyme A + Ketoleucine + NAD → Carbon dioxide + Isovaleryl-CoA + NADH
branched-chain keto-acid dehydrogenase complex