Cardiolipin 1799250 221163335 2008-06-23T09:09:20Z DOI bot 6652755 Citation maintenance. You can [[WP:DOI|use this bot]] yourself! Please [[User:DOI_bot/bugs|report any bugs]]. [[Image:cardiolipin.png|Cardiolipin|right|thumb|300px]] [[Image:Diphosphatidyl-Glycerol.png|Cardiolipin (alternate image)|right|thumb|300px]] '''Cardiolipin''' (bis[[phosphatidate|phosphatidyl]] [[glycerol]]) is an important component of the inner [[mitochondrial membrane]], where it constitutes about 20% of the total lipid. ==Function and structure== It is typically present in metabolically active cells of the heart and skeletal muscle, in the [[membrane]]s of their [[mitochondria]], mostly in the inner membrane, and consists roughly 20% of its lipids <ref>Krebs, Hauser and Carafoli, [http://www.jbc.org/cgi/reprint/254/12/5308 Asymmetric Distribution of Phospholipids in the Inner Membrane of Beef Heart Mitochondria], Journal of Biological Chemistry, Vol. 254, No. 12, June 25, pp. 5308-5316, 1979.</ref>. It has also been observed in certain bacterial membranes. It serves as an insulator and stabilizes the activity of [[protein complex]]es important to the [[electron transport chain]] {{Fact|date=September 2007}}. It also "glues" them together<ref>Zhang, Mileykovskaya and Dowhan, [http://www.jbc.org/cgi/content/full/277/46/43553 Gluing the Respiratory Chain Together: cardiolipin is required for supercomplex formation in the inner mitochondrial membrane], J. Biol. Chem., Vol. 277, Issue 46, 43553-43556, November 15, 2002.</ref>. Cardiolipin is a "double" phospholipid because it has four fatty acid tails, instead of the usual two. While most lipids are made in the endoplasmic reticulum, cardiolipin is synthesized in the mitochondria themselves, on the matrix side of the inner mitochondrial membrane. ==Clinical significance== ===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. ===Diabetes=== Heart disease hits people with diabetes twice as often as people without diabetes. In those with diabetes, cardiovascular complications occur at an earlier age and often result in premature death, making heart disease the major killer of diabetic people. Cardiolipin has recently been found to be deficient in the heart at the earliest stages of diabetes, possibly due to a lipid-digesting enzyme that becomes more active in diabetic heart muscle.[http://www.eurekalert.org/pub_releases/2007-08/wuso-afm081007.php] ===Antiphospholipid syndrome=== Patients with [[anti-cardiolipin 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. In anti-cardiolipin mediated autoimmune disease there is a dependency on the [[apolipoprotein H]] for recognition.<ref name="pmid2349221">{{cite journal | author = McNeil HP, Simpson RJ, Chesterman CN, Krilis SA | title = Anti-phospholipid antibodies are directed against a complex antigen that includes a lipid-binding inhibitor of coagulation: beta 2-glycoprotein I (apolipoprotein H) | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 87 | issue = 11 | pages = 4120–4 | year = 1990 | pmid = 2349221 | doi =10.1073/pnas.87.11.4120 }}</ref> ===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. ==See also== * [[Phosphatidylglycerol]] ==References== {{Reflist}} ==External links== * {{MeshName|Cardiolipin}} {{Phospholipids}} {{Autoantigens}} [[Category:Phospholipids]] [[Category:Membrane biology]] {{biochem-stub}} [[cs:Kardiolipin]] [[de:Cardiolipin]] [[fr:Cardiolipine]] [[nl:Cardiolipine]] [[pl:Kardiolipina]] [[ru:Кардиолипин]]