Mitochondrial Antigen

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).\n\nThe 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). \n\nRecent developments in ELISA methology have increased the sensitivity and the capacity of the diagnostician to quantitate patient antibody titers.\n\nUnit Definition:\nOne 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.\n\nApplication:\nRecommended for use in standard solid phase immunoassays.\n\nStorage Conditions:\nProduct unstable above 0 degrees C, aliquot and store frozen at -70 degrees C. Avoid repeated freeze-thaw.\n

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SPECIFICATIONS

Catalog Number

M3990-01

Size

1000U

Applications

ELISA

Form

Supplied as a frozen liquid in 0.01M Tris, 0.5M sodium chloride, 0.02% sodium azide.\n

Purity

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.

References

1. Baum H, and Palmer C. Mol. Aspects Med. 8, 1985, pg 201-234. \n2. \"Molecular basis of mitochondrial autoreactivity in primary biliary cirrhosis.\" Immunology Today, Vol. 10, 1989. \n3. American College of Rheumatology, 55th Annual Scientific Meeting, Boston Mass, Poster Presentation, 1991.