Cardiolipin (bisphosphatidyl glycerol) is an important component of the inner mitochondrial membrane, where it constitutes about 20% of the total lipid. Phosphatidic acid (PA is the acid form of phosphatidate a common Phospholipid that is a major constituent of Cell membranes Phosphatidate acid is the smallest of In Cell biology, a mitochondrion (plural mitochondria) is a membrane-enclosed Organelle found in most eukaryotic cells.

Contents 1 Function and structure 2 Clinical significance 2.1 Barth syndrome 2.2 Diabetes 2.3 Antiphospholipid syndrome 2.4 Syphilis 3 See also 4 References 5 External links

Function and structure

It is typically present in metabolically active cells of the heart and skeletal muscle, in the membranes of their mitochondria, mostly in the inner membrane, and consists roughly 20% of its lipids ^[1]. In Cell biology, a mitochondrion (plural mitochondria) is a membrane-enclosed Organelle found in most eukaryotic cells. 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. A protein complex is a group of two or more Proteins Protein complexes are a form of Quaternary structure. An electron transport chain couples a chemical reaction between an electron donor (such as NADH) and an electron acceptor (such as O2) to the transfer It also "glues" them together^[2].

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. Barth syndrome ( BTHS) also known as 3-Methylglutaconic aciduria type II and Cardiomyopathy-neutropenia syndrome is a rare Genetic disorder classified It has a mutation in the gene coding for tafazzin, an enzyme involved in the biosynthesis of cardiolipin. Tafazzin is a Protein highly expressed in cardiac and Skeletal muscle. Biosynthesis is a phenomenon wherein Chemical compounds are produced from simpler Reagents Biosynthesis unlike Chemosynthesis, takes place within living 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. Cardiomyopathy, which literally means "heart muscle disease" is the deterioration of the function of the Myocardium (i 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. [1]

Antiphospholipid syndrome

Patients with anti-cardiolipin antibodies (Antiphospholipid syndrome) can have recurrent thrombotic events even early in their mid-late teen years. Anti-cardiolipin antibodies (ACA are antibodies often directed against Cardiolipin and found in several diseases including Syphilis, Antiphospholipid syndrome Antiphospholipid syndrome (APS or APLS or antiphospholipid antibody syndrome is a disorder of Coagulation, which causes blood clots ( Thrombosis) in both 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. ^[3]

Syphilis

Cardiolipin from a cow heart is used as an antigen in the Wassermann test for syphilis. The Wassermann test is a Complement-fixation ( Complement system) Antibody test for Syphilis, named after the bacteriologist August von Wassermann Syphilis is a Sexually transmitted disease caused by the spirochetal Bacterium Treponema pallidum pallidum. 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

External links

• MeSH Cardiolipin

Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books

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