FEATURES

FAMILY

DESCRIPTION

Also known as ODB2_BOVIN, DBT. 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). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component. Forms a 24-polypeptide structural core with octahedral symmetry.

EXTERNAL LINKS

DBT

Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial

Mutations

Mutation(s) distribution

Mutations table

Search Mutation