The mitochondrial antigen M2 is a cluster of four major mitochondrial innermembrane proteins of approximately 74, 52, 45, and 39kD, which are found widely in nature. (2) These antigens have been identified as the E2 subunit of branched chain 2-oxo acid dehydrogenase, the E2 subunit of the 2-oxo dehydrogenase complex, protein X, and the E1a and E1b subunits of the pyruvate dehydrogenase complex (3). Autoantibodies to mitochondrial antigens were originally described in patients with Primary Biliary Cirrhosis. It was later realized that Primary Biliary Cirrhosis is an autoimmune liver disease characterized by autoantibodies to the mitochondrial antigen M2 in serum (1). The presence of autoantibodies against the mitochondrial antigens has been reported to have prognostic value in the diagnosis Primary Biliary Cirrhosis, as the disease rarely occurs without anti-M2. (2) Autoantibodies to M2 in Primary Biliary Cirrhosis usually occur in a very high titer and have an IgG3 subclass restriction (2). Recent developments in ELISA methology have increased the sensitivity and the capacity of the diagnostician to quantitate patient antibody titers. Unit Definition: One unit of activity is defined as the amount of antigen that will generate an absorbance of 1.2 when assayed using standard procedure for enzyme immunoassay. Activity will vary with assay conditions. Application: Recommended for use in standard solid phase immunoassays. Storage Conditions: Product unstable above 0C, aliquot and store frozen at -70C. Avoid repeated freeze-thaw.
Reinheit:
Purified from bovine spleen and/or thymus through the use of immobilized human anti-Mitochondrial immunoglobulins. Other antigens (Sm, RNP, Scl-70, SS-A(Ro), SS-B(La), Jo-1) are not detected with ELISA assays.
Formulierung:
Supplied as a frozen liquid in 0.01M Tris, 0.5M sodium chloride, 0.02% sodium azide.
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