Mitochondrial(AMA)-M2-Ab ELISA

Mitochondrial (AMA)-M2-Ab ELISA is a solid phase enzyme immunoassay employing native mitochondial M2 antigen for the quantitative detection of antibodies against M2 in human serum. The assay is a tool in the diagnosis of the primary biliary cirrhosis (PBC). Primary biliary cirrhosis (PBC) is a chronic inflammatory disorder of the small and medium bile ducts and serologically characterized by the presence of circulating M2 autoantibodies. Anti-M2 autoantibodies belong to the group of anti-mitochondrial antibodies (AMA). The heterogeneously reacting specific AMA of the M2 subtype are directed against three related proteins of the a-keto acid dehydrogenase complex, which is located at the inner mitochondrial membrane. The major epitope recognized is located on the E2 subunit and the protein X of the pyruvate dehydrogenase complex (PDC). Additionally, AMA-M2 autoantibodies recognize the (E1a and E1ß) subunits of the same complex and the E2 subunit of several other multienzyme complexes, such as the 2-oxo-glutarate dehydrogenase complex (OGDC) and the branched chain 2-oxo acid dehydrogenase complex (BCOADC). The determination of AMA-M2 is a powerful tool in diagnosing PBC.