However, other mitochondrial proteins have been documented to play a role in autoimmune pathology including the E2 subunit of pyruvate dehydrogenase, which has been shown to act as a target antigen in the mediation of primary biliary cirrhosis 32, 33 and ANT1 21–40 that we have previously shown to induce myocarditis in a manner similar to BCKDk peptides 2. This evidence concerns the gene BCKDK and primary biliary cholangitis.