| 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. The heterogeneously reacting
specific anti-mitochondrial antibodies of the M2 subtype
are directed against three related proteins of the
a-keto acid dehydrogenase complex which is located
at the inside of the mitochondrial membrane. The
recognized major epitope is located on the E2 subunit
and the protein X of the pyruvate dehydrogenase
complex (PDC). Additionally AMA-M2 autoantibodies
recognize the (E1a und E1b) subunits of the same
complex and the E2 subunit of several other multi
enzyme 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. |
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 |
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| Tests |
12 x 8 Tests |
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| Calculation |
quantitative |
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| Range |
0 – 200 IU/ml |
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| Cut-off |
10 IU/ml |
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| Sensitivity |
1 IU/ml |
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| Calibration |
WHO 67/183 |
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| Substrate |
TMB / 450 nm |
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Instruction for Use: ORG 516-0508-01-gb.pdf
Material-Safety-Datasheet: ORG 516.pdf