Anti-mitochondrial antibodies
Encyclopedia
Anti-mitochondrial antibodies (AMA) are autoantibodies, consisting of immunoglobulins formed against mitochondria, primarily mitochondria in cells
of the liver
. The presence of AMAs in the blood
or serum
of a person is indicative of several autoimmune disease
s such as primary biliary cirrhosis
(PBC) (a scarring of liver tissue, confined primarily to the bile duct drainage system of the liver). It is present in about 95% of cases.
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.
Anti-cardiolipin antibodies
are another type of AMA, cardiolipin
is found on the inner mitochondrial membrane
.
(anti-tTG), which correlate with gluten-sensitive
enteropathy (see coeliac disease
, Gluten-sensitive enteropathy associated conditions).
The inflammation 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.
Cell (biology)
The cell is the basic structural and functional unit of all known living organisms. It is the smallest unit of life that is classified as a living thing, and is often called the building block of life. The Alberts text discusses how the "cellular building blocks" move to shape developing embryos....
of the liver
Liver
The liver is a vital organ present in vertebrates and some other animals. It has a wide range of functions, including detoxification, protein synthesis, and production of biochemicals necessary for digestion...
. The presence of AMAs in the blood
Blood
Blood is a specialized bodily fluid in animals that delivers necessary substances such as nutrients and oxygen to the cells and transports metabolic waste products away from those same cells....
or serum
Blood plasma
Blood plasma is the straw-colored liquid component of blood in which the blood cells in whole blood are normally suspended. It makes up about 55% of the total blood volume. It is the intravascular fluid part of extracellular fluid...
of a person is indicative of several autoimmune disease
Autoimmune disease
Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. In other words, the body actually attacks its own cells. The immune system mistakes some part of the body as a pathogen and attacks it. This may be restricted to...
s such as primary biliary cirrhosis
Primary biliary cirrhosis
Primary biliary cirrhosis, often abbreviated PBC, is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts within the liver. When these ducts are damaged, bile builds up in the liver and over time damages the tissue. This can lead to scarring,...
(PBC) (a scarring of liver tissue, confined primarily to the bile duct drainage system of the liver). It is present in about 95% of cases.
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.
- pyruvate dehydrogenasePyruvate dehydrogenasePyruvate dehydrogenase complex is a complex of three enzymes that transform pyruvate into acetyl-CoA by a process called pyruvate decarboxylation. Acetyl-CoA may then be used in the citric acid cycle to carry out cellular respiration, and this complex links the glycolysis metabolic pathway to the...
, E2 subunits - 2-oxo-glutarate dehydrogenaseOxoglutarate dehydrogenaseThe oxoglutarate dehydrogenase complex or α-ketoglutarate dehydrogenase complex is an enzyme complex, most commonly known for its role in the citric acid cycle.-Units:...
- branched chain 2-oxo-acid dehydrogenaseBranched-chain alpha-keto acid dehydrogenase complexThe 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
Anti-cardiolipin antibodies
Anti-cardiolipin antibodies are antibodies often directed against cardiolipin and found in several diseases including syphilis, antiphospholipid syndrome, livedoid vasculitis, vertebrobasilar insufficiency, Behçet's syndrome, idiopathic spontaneous abortion, and systemic lupus erythematosus. They...
are another type of AMA, cardiolipin
Cardiolipin
Cardiolipin is an important component of the inner mitochondrial membrane, where it constitutes about 20% of the total lipid composition. The only other place that cardiolipin can be found is in the membranes of most bacteria. The name ‘cardiolipin’ is derived from the fact that it was first...
is found on the inner mitochondrial membrane
Inner mitochondrial membrane
The mitochondrial inner membrane forms internal compartments known as cristae, which allow greater space for the proteins such as cytochromes to function properly and efficiently. The electron transport chain is located on the inner membrane of the mitochondria...
.
Correlation with non-mitochondrial antigens
Fifty seven percent of acute liver failure patients had elevated anti-transglutaminase antibodiesAnti-transglutaminase antibodies
Anti-transglutaminase antibodies are autoantibodies against the transglutaminase protein. Antibodies serve an important role in the immune system by detecting cells and substances that the rest of the immune system then eliminates. These cells and substance can be foreign and also can be producd...
(anti-tTG), which correlate with gluten-sensitive
enteropathy (see coeliac disease
Coeliac disease
Coeliac disease , is an autoimmune disorder of the small intestine that occurs in genetically predisposed people of all ages from middle infancy onward...
, Gluten-sensitive enteropathy associated conditions).
The inflammation 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
Anti-glycoprotein-210 antibodies
Anti-glycoprotein-210 antibodies are directed at gp210 and are found within primary biliary cirrhosis patients in high frequency. AGPA recognize the cytoplasmic oriented carboxyl terminus of the protein...
are also found in 47% of PBC patients.