CEREBRAL LIPIDS IN PHENYLKETONURIA

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Abstract
The lipid fraction of cerebral grey and white matter from three phenylketonuric patients was examined chemically. Total lipid and phospholipid content fell within the range of normal. Cholesterol was slightly decreased in two of the three cases. Lipid hexose was low in all three specimens of white matter, and the concentration of pure cerebroside was at or beyond the lower limit of normal in the three patients. The molecular composition of cerebrosides and sulfatides was examined in all three cases. The structure of cerebrosides was normal in two cases. In one brain, the quantity of kerasin was decreased and its major component was an iso-C24 branchedchain fatty acid of unknown structure. An analogous reduction of kerasin sulfate was observed in all three brains. It has been postulated that a relative deficiency of non-hydroxy fatty acids in the glycolipid fraction, particularly in sulfatides, may represent one of the consistent defects in the chemical structure of phenylketonuric white matter.

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