Anti-mitochondrial antibody

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


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]

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]

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References

  1. MedlinePlus Encyclopedia 003529
  2. GI111 at FPnotebook
  3. 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. 4.0 4.1 Leung PS, Rossaro L, Davis PA; et al. (2007). "Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis". PMID 17657817. doi:10.1002/hep.21828. 
  5. 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. 
  6. 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. PMID 17621358. doi:10.1002/jcla.20170. 

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