Anti-mitochondrial antibody

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Anti-mitochondrial antibodies (AMA) are antibodies (immunoglobulins) formed against mitochondria, primarily mitochondria in cells of the liver. The presence of AMAs in the blood or serum of a person is indicative of a disease called primary biliary cirrhosis (PBC) (a scarring of liver tissue, confined primarily to the bile duct drainage system of the liver). It is present in about 95% of cases.Primary biliary cirrhosis is seen primarily in middle-aged women, and in those afflicted with other autoimmune diseases. PBC is an autoimmune disorder, a condition in which the human body's immune defense system mistakenly attacks the body's own cells, or in this case parts of the cells.Cause of AMAs is postulated that xenobiotic-induced and/or oxidative modification of mitochondrial autoantigens is a critical step leading to loss of tolerance. In acute liver failure AMA are found against all major liver antigens.
  • pyruvate dehydrogenase, E2 subunits
  • 2-oxo-glutarate dehydrogenase
  • branched chain 2-oxo-acid dehydrogenase
    • Anti-cardiolipin antibodies are another type of AMA, cardiolipin is found on the inner mitochondrial membrane.=Correlation with non-mitochodrial antigens= Fifty seven percent of acute liver failure patients had elevated anti-transglutaminase antibodies (anti-tTG), which correlate with gluten-sensitive enteropathy (see coeliac disease, Gluten-sensitive enteropathy associated conditions). The inflamation produced by gluten-sensitive cellular immunity may cause the oxidative stress resulting in the modification of mitochondrial antigens and acute liver failure. Anti-gp210 antibodies are also found in 47% of PBC patients.

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