branched-chain ketoacid dehydrogenase

Function

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

2-methylpropanoyl-CoA + enzyme N(6)-(dihydrolipoyl)lysine
                    <-->
CoA + enzyme N(6)-(S-(2-methylpropanoyl)dihydrolipoyl)lysine

Structure

• it contains multiple copies of 3 enzymatic components:
• branched-chain alpha-keto acid decarboxylase (E1),
• lipoamide acyltransferase (E2)
• lipoamide dehydrogenase (E3)
• forms a 24-polypeptide structural core with octahedral symmetry

Pathology

defect gives rise to maple syrup urine disease

More general terms

• ketone oxidoreductase
• dehydrogenase
• mitochondrial membrane protein
• multienzyme complex

Database

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