PDC-E2, also called dihydrolipoamide acetyltransferase, is the acceptor molecule for acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Of note, in addition to its essential role in cellular metabolism, PDC-E2 is the key autoantigen in primary biliary cirrhosis (PBC) [53]. The gene discussed is DLAT; the disease is primary biliary cholangitis.