Dihydrolipoamide S-acetyltransferase (DLAT) is a key component of the pyruvate dehydrogenase complex (PDC), which is located in the mitochondria. This enzyme plays a crucial role in cellular energy production by facilitating the conversion of pyruvate into acetyl-CoA, a process that links glycolysis to the citric acid cycle. DLAT accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A (CoA). It is encoded by the DLAT gene, which is conserved across various species. Mutations in this gene can lead to pyruvate dehydrogenase E2 deficiency, causing primary lactic acidosis in infancy and early childhood. Additionally, DLAT is the antigen for antimitochondrial antibodies, which are present in nearly 95% of patients with primary biliary cirrhosis (PBC), an autoimmune liver disease.