branched-chain keto acid dehydrogenase E1 or 2-oxoisovalerate dehydrogenase

Function

• branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA & CO2

Structure

• heterotetramer of alpha & beta chains
• contains multiple copies of 3 enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) lipoamide dehydrogenase (E3

More general terms

• dehydrogenase
• multisubunit protein

Database

• OMIM: https://mirror.omim.org/entry/248600
• OMIM: https://mirror.omim.org/entry/248611