Article
Defective remodeling of cardiolipin and phosphatidylglycerol in Barth syndrome.
Biochemical and biophysical research communications - 20 Dec 2000
Vreken P, Valianpour F, Nijtmans L G, Grivell L A, Plecko B, Wanders R J, Barth P G
Abstract excerpt
Cardiolipin (CL) and phosphatidylglycerol (PG) are the major polyglycerophospholipids observed in mammalian tissues. CL is exclusively found in the inner mitochondrial membrane and is required for optimal function of many of the respiratory and ATP-synthesizing enzymes. The role of CL in oxidative phosphorylation is, however, not fully understood and although reduced CL content leads to aberrant cell function, no...
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