• It is remodeled by the enzymes monolysocardiolipin acyltransferase, lysocardiolipin acyltransferase, and tafazzin, which transfer a fourth fatty acid chain onto the phospholipid. (wikipedia.org)
  • The tafazzin protein is involved in altering a fat (lipid) called cardiolipin, which plays critical roles in the mitochondrial inner membrane. (medlineplus.gov)
  • Once altered by tafazzin, cardiolipin is key in maintaining mitochondrial shape, energy production, and protein transport within cells. (medlineplus.gov)
  • As a result, tafazzin cannot alter cardiolipin, and levels of functional cardiolipin are reduced. (medlineplus.gov)
  • Barth Syndrome, a rare X-linked disorder affecting 1:300,000 live births, results from defects in Tafazzin, an acyltransferase that remodels cardiolipin and is essential for mitochondrial respiration. (bvsalud.org)
  • MLCL is normally present as part of the metabolic cycle of mitochondrial lipids, such as cardiolipin. (wikipedia.org)
  • A lack of functional cardiolipin and an excess of MLCL are thought to impair normal mitochondrial shape and functions. (medlineplus.gov)
  • This strain produced triglycerides (TG) as well as four common phospholipids: phosphatidylethanolamine (PE), phosphatidylglycerol (PG), cardiolipin (CL) and lysocardiolipin (LCL). (nature.com)
  • Monolysocardiolipin (MLCL) is a phospholipid with three fatty acid chains located in the inner membrane of mitochondria. (wikipedia.org)
  • In addition, for unknown reasons, a variant of cardiolipin called monolysocardiolipin (MLCL) is formed. (medlineplus.gov)
  • Biochemical abnormalities include decreased levels of the mitochondrial phospholipid cardiolipin, increased levels of monolysocardiolipin, and a lower degree of unsaturation of the (monolyso)cardiolipin acyl chains. (nih.gov)
  • Monolysocardiolipin (MLCL) is a phospholipid with three fatty acid chains located in the inner membrane of mitochondria. (wikipedia.org)
  • Monolyso-cardiolipin-biomarker of mitochondrial dysfunction induced by total body irradiation. (cdc.gov)
  • Mitochondria targeting of nonperoxidizable triphenylphosphonium-conjugated oleic acid protects mouse embryonic cells against apoptosis: role of cardiolipin remodeling. (cdc.gov)
  • Because a validated confirmatory method is not yet available, we set up and validated a high-performance liquid chromatography-mass spectrometry (HPLC-MS) method for BTHS in cultured fibroblasts, lymphocytes, and skeletal muscle based on cardiolipin, monolysocardiolipin, and the monolysocardiolipin/cardiolipin ratio. (nih.gov)
  • In addition, we performed retrospective analysis of 121 muscle samples of patients with myopathy of which mitochondrial origin was presumed, and we identified one patient with cardiolipin abnormalities similar to BTHS patients. (nih.gov)