Literature associating BCKDHA and branched-chain keto acid dehydrogenase kinase deficiency
BCKDHA [ENSP00000269980]
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; 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).
Synonyms: BCKDHA, A0A024R0K3, F5GXU9, H0YH20, H0YH31 ...