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Cardiolipin molecule

This complex lipid can be viewed as a polar headgroup consisting of a glycerol backbone. C1 and C3 are esterified to the phosphate groups of glycerol phosphatide (diacylglycerol phosphate) on each side, each carrying two hydrophophobic acyl-chains. The composition of the 4 side chains is variable. The drawn structure carries 4 linoleic chains, the predominant composition in various tissues, including skeletal and heart muscle. The side chain composition is regulated by tafazzin, a transacylase, deficient in Barth syndrome. The polar headgroup is coloured in cyan, and the apolar tail region in green. (Contributed by Dr. Peter Barth.)

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  • Congestive heart failure
  • Endocardial fibroelastosis
  • Exuberant leukocytosis
  • Growth retardation
  • Hypoglycemia
  • Learning disability
  • Monocytosis
  • Neutropenia
  • Ventricular dysrhythmia