FEATURES

FAMILY

DESCRIPTION

Also known as ODBA_HUMAN, BCKDHA. E1 subunit of the branched-chain alpha-keto dehydrogenase complex that catalyzes both the decarboxylation of the alpha-ketoacid and the subsequent reductive acylation of the lipoyl moiety (another catalytic cofactor) that is covalently bound to E2 (PubMed:7883996). 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.

EXTERNAL LINKS

uniprot
enzyme classification
PhosphoSitePlus

BCKDHA

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

BCKDHA

Molecular Synopsis