Dihydrolipoyl-Transacetylase

• OMIM: 608770
• UniProt: P10515

The dihydrolipoyl transacetylase (E2 ) ( correct: Dihydrolipoyllysinrest acetyltransferase also: dihydrolipoamide S- acetyltransferase PDHE2 ) is the enzyme that functions in all living creatures as a sub-unit E2 of the pyruvate dehydrogenase complex (PDH) and as essential for the maintenance of glycolysis and the citric acid cycle is. Within the PDH E2 has the task of transferring the acetyl residue from acetyl- CoA to the lipoic acid cofactor bound to PDH and back. PDHE2 is the people's car allergen in primary biliary cirrhosis, an autoimmune disease. Mutations in the gene DLAT are responsible for certain forms of pyruvate dehydrogenase deficiency.

In primary biliary cirrhosis probably E2 is first modified by the environment occurring Methyloctinoat, whereupon it becomes such a strong antigen that in apoptosis and antibodies against normal E2 are formed.