Autoantibodies, consisting of immunoglobulins formed against mitochondria, primarily mitochondria in cells of the liver.
Its presence is indicative of several autoimmune diseases such as 2242t are contained in multienzyme complexes within the inner lining of the mitochondria.
Antimitochondrial antibodies react against a component of this multienzyme complex called E2, the pyruvate dehydrogenase complex PDC-E2).
Accumulation of PDC-E2 within the biliary epithelial cells is observed exclusively in the livers of patients with primary biliary cirrhosis.
In acute liver failure such autoantobodies are found against all major liver antigens.
Anti-cardiolipin antibodies are another type of antimitochondrial antibodies.
Cardiolipin is found on the inner mitochondrial membrane.
Patients with acute liver failure patients have elevated anti-transglutaminase antibodies in more than 50% of cases.
With gluten-sensitive enteropathy anti-transglutiaminase antibodies are present.