Dihydrolipoamide S-acetyltransferase (DLAT)

Dihydrolipoamide S-acetyltransferase (DLAT) is a mitochondrial enzyme constituting the core E2 component of the pyruvate dehydrogenase multi-enzyme complex. It catalyzes the transfer of the acetyl group from dihydrolipoamide to coenzyme A, producing acetyl-CoA, a crucial intermediate connecting glycolysis to the citric acid cycle and ultimately cellular energy production. In humans, DLAT is encoded by the *DLAT* gene and is a major autoantigen in primary biliary cirrhosis. Deficiency or dysfunction of DLAT disrupts energy metabolism, leading to clinical manifestations such as lactic acidosis and autoimmune cholestatic liver disease[1][2][5]. Structural details include a multi-domain organization: three tandem lipoyl domains, a peripheral subunit-binding domain, and a catalytic domain, forming part of a dodecahedral core within the pyruvate dehydrogenase complex[1][3].

Other names

Dihydrolipoyl transacetylaseDihydrolipoamide acetyltransferasePyruvate dehydrogenase complex component E2PDCE2DLAT (gene/protein symbol)

Enzymatic acetyl group transfer from dihydrolipoamide to coenzyme A, producing acetyl-CoA for metabolic use. Autoantibody binding in primary biliary cirrhosis leads to tissue destruction.