The mitochondrial adenine nucleotide translocator is an antigen in primary biliary cirrhosis.

Circulating antibodies reacting specifically with the adenine nucleotide translocator from liver mitochondria were detected in sera from 12 patients with proven primary biliary cirrhosis (PBC) by a solid phase double antibody immunoradiometric assay (IRMA). Furthermore these antibodies were absorbed with the isolated adenine nucleotide translocator from liver mitochondria. None of the sera from 20 normal individuals, four patients with anti-mitochondrial positive pseudolupus syndrome (PLE) sera (M-3) and three patients with syphilis (anti-M-l) had antibodies directed against this protein from inner mitochondrial membrane. The adenine nucleotide translocator as antigen in PBC could clearly be distinguished from the ATPase associated PBC specific M-2 antigen. With the present study, for the first time, a well characterized protein from inner mitochondrial membrane has been clearly defined as an autoantigen in primary biliary cirrhosis.