FEATURES

FAMILY

DESCRIPTION

Also known as ODBA_HUMAN, BCKDHA. 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). Heterotetramer of alpha and beta chains.

... More

EXTERNAL LINKS

UNIPROT
ENZYME CLASSIFICATION
PhosphoSitePlus

BCKDHA

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

ligandability Mutations

BCKDHA

Molecular Synopsis