dihydrolipamide acyltransferase E2 (keto acid dehydrogenase E2, BCATE2 E2 subunit, DBT, BCKAD)

Function

• branched-chain alpha-keto aciddehydrogenase complex component
• catalytic function of BCATE2 is to accept & transfer to coenzyme A, acyl groups generated by branched-chain alpha-keto acid decarboxylase component of branched-chain alpha-keto acid dehydrogenase

Cofactor: binds 1 lipoyl cofactor covalently

Structure

• belongs to the 2-oxoacid dehydrogenase family
• contains 1 lipoyl-binding domain

Compartment

mitochondrial matrix

Pathology

• defects in DBT are the cause of maple syrup urine disease type 2

More general terms

• acyltransferase

Database

• OMIM: https://mirror.omim.org/entry/248600
• OMIM: https://mirror.omim.org/entry/248610
• UniProt: http://www.uniprot.org/uniprot/P11182.html