MOLECULE

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

Involved in oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).