Anti-mitochondrial antibodies and other immunological tests in primary biliary cirrhosis.
Primary biliary cirrhosis (PBC) has been classified as an autoimmune disease. It is characterized by a multitude of immune-mediated symptoms and phenomena. Humoral autoimmunity directed against mitochondrial auto-antigens or structures cross-reactive with these proteins represents the basis of one of the most specific tests in any autoimmune disease: the detection of auto-antibodies against pyruvate dehydrogenase. The molecular cloning and expression of antigens of the ketoacid dehydrogenase complex has led to the establishment of highly specific and reliable testing systems for anti-mitochondrial antibodies (AMA). The characterization of specific antinuclear auto-antibodies has contributed to the diagnosis of AMA-negative PBC and is an important marker in overlap syndromes of PBC and other autoimmune liver diseases.
Department of Gastroenterology and Hepatology, Medical School of Hannover, Germany.
C P
CP
Strassburg
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Jaeckel
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MP
Manns
D000888:Antibodies, Anti-Idiotypic; D000974:Antibodies, Antinuclear / Q000276:immunology; D000917:Antibody Formation; D001327:Autoimmune Diseases / Q000276:immunology*; D015551:Autoimmunity; D006801:Humans; D007655:Ketoglutarate Dehydrogenase Complex / Q000276:immunology; D008105:Liver Cirrhosis, Biliary / Q000276:immunology*; D008930:Mitochondria, Liver / Q000276:immunology*
D000888:Antibodies, Anti-Idiotypic; D000974:Antibodies, Antinuclear; D007655:Ketoglutarate Dehydrogenase Complex