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: 1) branched-chain alpha-keto acid decarboxylase (E1), 2) lipoamide acyltransferase (E2) 3) lipoamide dehydrogenase (E3) - forms a 24-polypeptide structural core with octahedral symmetry Pathology: defect gives rise to maple syrup urine disease

General

dehydrogenase ketone oxidoreductase mitochondrial membrane protein multienzyme complex

Properties

COMPARTMENT: inner mitochondrial membrane MOTIF: membrane region active site

References

OMIM :accession 248600

Components

branched-chain keto acid dehydrogenase E1 or 2-oxoisovalerate dehydrogenase dihydrolipamide acyltransferase E2 (keto acid dehydrogenase E2, BCATE2 E2 subunit, DBT, BCKAD) lipoamide dehydrogenase (glycine cleavage system L protein, DLD, GCSL, LAD, LADH, PHE3)