Anti-mitochondrial antibodies (AMA) are antibodies (immunoglobulins) formed against mitochondria,[1] primarily mitochondria in cells of the liver. Antibodies (also known as immunoglobulins, abbreviated Ig) are Gamma globulin Proteins that are found in Blood or other Bodily Antibodies (also known as immunoglobulins, abbreviated Ig) are Gamma globulin Proteins that are found in Blood or other Bodily In Cell biology, a mitochondrion (plural mitochondria) is a membrane-enclosed Organelle found in most eukaryotic cells. The cell is the structural and functional unit of all known living Organisms It is the smallest unit of an organism that is classified as living and is often called The liver is a vital organ in the human body and is present in Vertebrates and some other animals The presence of AMAs in the blood or serum of a person is indicative of several autoimmune diseases such as primary biliary cirrhosis (PBC)[2] (a scarring of liver tissue, confined primarily to the bile duct drainage system of the liver). Blood is a specialized Bodily fluid that delivers necessary substances to the body's cells €”such as nutrients and oxygen—and transports Waste products Blood plasma is the Liquid component of Blood, in which the Blood cells are suspended Autoimmune diseases arise from an overactive Immune response of the body against substances and tissues normally present in the body Primary biliary cirrhosis is an Autoimmune disease of the Liver marked by the slow progressive destruction of the small bile ducts ( Bile canaliculi) within 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. Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues
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. Pyruvate dehydrogenase (E1 is the first component Enzyme of Pyruvate dehydrogenase complex (PDC Oxoglutarate dehydrogenase (aka α-ketoglutarate dehydrogenase) is an enzyme complex most commonly known for its role in the Citric acid cycle. The branched-chain α-keto acid dehydrogenase complex is a combination of Enzymes responsible for the degradation of the Branched chain amino acids. Anti-cardiolipin antibodies (ACA are antibodies often directed against Cardiolipin and found in several diseases including Syphilis, Antiphospholipid syndrome Cardiolipin (bis phosphatidyl Glycerol) is an important component of the inner Mitochondrial membrane, where it constitutes about 20% of the total lipid The Mitochondrial inner membrane forms internal compartments known as Cristae which allow greater space for the proteins such as Cytochromes to function properly
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). Anti-transglutaminase antibodies (ATA are Antibodies found more frequently in certain Autoimmune diseases High levels ( Titers of ATA are found in Coeliac Gluten-sensitive enteropathy has a wide variety of associated conditions however the key symptoms are typically restricted to the bowel and associated tissues [4] The inflamation produced by gluten-sensitive cellular immunity may cause the oxidative stress resulting in the modification of mitochondria l antigens and acute liver failure. Anti-gp210 antibodies are also found in 47% of PBC patients. Anti-glycoprotein-210 antibodies ( AGPA, anti-gp210 anti-nup210 anti-np210 are directed at gp210 and are found within Primary biliary cirrhosis (PBC [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). In Cell biology, a mitochondrion (plural mitochondria) is a membrane-enclosed Organelle found in most eukaryotic cells. Antibodies (also known as immunoglobulins, abbreviated Ig) are Gamma globulin Proteins that are found in Blood or other Bodily MedlinePlus, with the MedlinePlus Medical Encyclopedia, is a website network containing Health information from the world's largest medical Library The Family Practice Notebook is a medical database focused on Family practice. "A sensitive bead assay for antimitochondrial antibodies: Chipping away at AMA-negative primary biliary cirrhosis". Hepatology 45 (3): 659–65. doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 17326160.
- ^ a b Leung PS, Rossaro L, Davis PA, et al (2007). "Antimitochondrial antibodies in acute liver failure: Implications for primary biliary cirrhosis". doi:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. 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:. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 17621358.