Cardiolipin
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Cardiolipin (bisphosphatidyl glycerol) is an important component of the inner mitochondrial membrane, where it constitutes about 20% of the total lipid.
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[edit] Function and structure
It is typically present in metabolically active cells of the heart and skeletal muscle. It has also been observed in certain bacterial membranes.
It serves as an insulator and stabilizes the activity of protein complexes important to the electron transport chain.
Cardiolipin is a "double" phospholipid because it has four fatty acid tails, instead of the usual two.
[edit] Clinical significance
[edit] Barth syndrome
Barth syndrome is a rare genetic disorder that was recognised in the 1970's to cause infantile death. It has a mutation in the gene coding for tafazzin, an enzyme involved in the biosynthesis of cardiolipin. Girls heterozygous for the trait are unaffected. Sufferers of this condition have mitochondria that are abnormal, and they cannot sustain adequate production of ATP. Cardiomyopathy and general weakness is common to these patients. Cardiolipin treats the symptoms of BTHS and prevents infections.
[edit] Antiphospholipid syndrome
Patients with anticardiolipin antibodies (Antiphospholipid syndrome) can have recurrent thrombotic events even early in their mid-late teen years. These events can occur in vessels where thrombosis may be relatively uncommon, such as the hepatic or renal veins. These antibodies are usually picked up in young women with recurrent spontaneous abortions.
[edit] Syphilis
Cardiolipin from a cow heart is used as an antigen in the Wassermann test for syphilis. Anti-cardiolipin antibodies can also be increased in numerous other conditions, including malaria and tuberculosis, so this test is not specific
[edit] External links
Glycerophospholipids/Phosphoglycerides: Phosphatidylethanolamine - Phosphatidylcholine - Dipalmitoylphosphatidylcholine - Cardiolipin - Phosphatidylserine - Ether lipids (Plasmalogen, Platelet-activating factor)
Phosphatidylinositol - Glycophosphatidylinositol - Phosphatidylinositol 3-phosphate - Phosphatidylinositol (3,4)-bisphosphate - Phosphatidylinositol (3,5)-bisphosphate - Phosphatidylinositol (4,5)-bisphosphate - Phosphatidylinositol (3,4,5)-trisphosphate