Anti-mitochondrial antibody
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Anti-mitochondrial antibodies (AMA) are antibodies (immunoglobulins) formed against mitochondria,[1] 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)[2] (a scarring of liver tissue, confined primarily to the bile duct drainage system of the liver). It is present in about 95% of cases.[3]
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.[4]
- 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).[4] The inflammation 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.[5][6]
See also
References
- ↑ MedlinePlus Encyclopedia 003529
- ↑ GI111 at FPnotebook
- ↑ Oertelt S, Rieger R, Selmi C, Invernizzi P, Ansari A, Coppel R, Podda M, Leung P, Gershwin M (2007). "A sensitive bead assay for antimitochondrial antibodies: Chipping away at AMA-negative primary biliary cirrhosis". Hepatology 45 (3): 659-65. PMID 17326160.
- ↑ 4.0 4.1 Leung PS, Rossaro L, Davis PA, et al (2007). "Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis". doi:10.1002/hep.21828. PMID 17657817.
- ↑ Nickowitz RE, Worman HJ (1993). "Autoantibodies from patients with primary biliary cirrhosis recognize a restricted region within the cytoplasmic tail of nuclear pore membrane glycoprotein Gp210". J. Exp. Med. 178 (6): 2237-42. PMID 7504063.
- ↑ Bauer A, Habior A (2007). "Measurement of gp210 autoantibodies in sera of patients with primary biliary cirrhosis". J. Clin. Lab. Anal. 21 (4): 227-31. doi:10.1002/jcla.20170. PMID 17621358.
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
