dihydrolipamide acetyltransferase E2 (pyruvate dehydrogenase E2, DLAT)

Function

• pyruvate dehydrogenase subunit

Cofactor: binds 2 lipoyl cofactors covalently

Structure

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

Compartment

mitochondrial matrix

Pathology

• defects in DLAT are the cause of pyruvate dehydrogenase deficiency
• autoantibodies to DLAT may be associated with primary biliary cirrhosis

More general terms

• acyltransferase

Component of

• pyruvate dehydrogenase

Database

• UniProt: http://www.uniprot.org/uniprot/P10515.html
• OMIM: https://mirror.omim.org/entry/245348
• OMIM: https://mirror.omim.org/entry/608770
• OMIM: https://mirror.omim.org/entry/109720