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The Mitochondrial IgG ELISA test system is an enzyme linked immunosorbent assay (ELISA) for the detection of IgG class antibody to Mitochondrial in human serum or plasma. Mitochondrial Antibodies (MA) are directed against the E2 subunit of the pyruvate dehydrogenase enzyme complex located at the inner mitochondrial membrane (PDC-E2), the E2 subunit of the branched chain 2-oxo acid dehydrogenase complex (BCOADC-E2), the E2 subunit of the 2-oxo-glutarate dehydrogenase complex (OGDC-E2), protein X, and PDC-Ela and PDC-E1. MA are found in ~95% of patients with primary biliary cirrhosis (PBC). MA in low titers are common in chronic active hepatitis and their presence does not preclude response to corticosteroids. MA disappear in about one month after orthotopic liver transplantation (OLT) and decrease with cyclosporine treatment which might be useful in PBC. MA are found in <1% of apparently healthy Caucasoid adults. Approximately 3% of patients with PBC have scleroderma, usually of the CREST syndrome variety. In addition, MA reactive with the PDC-E2 complex are found in some patients with CREST or diffuse scleroderma, sometimes in the absence of overt liver disease. Scleroderma typically precedes PBC in those patients with both diseases.