Abstract
ABSTRACT: Vanier, M.‐Th. and Svennerholm, L. (Department of Neurochemistry, Psychiatric Research Centre, University of Göeborg, Göteborg, Sweden). Chemical Pathology of Krabbe's Disease. II. Fatty acid composition of cerebrosides, sulfatides and sphingomyelins in brain. Acta Paediatr Scand, 63: 501, 1974.—The fatty acid composition of sphingomyelins, cerebrosides and sulfatides was determined in cerebral tissue from 18 Swedish children aged 7–32 months, with Krabbe's disease. Sphingomyelins of the white matter had a fatty acid pattern which indicated a serious deficiency of myelin. The fatty acid pattern also suggested that a substantial amount of the sphingomyelins was derived from cells of mesenchymal origin. The cerebrosides, and to a certain extent the sulfatides of cerebral cortex and white matter, had a higher concentration of saturated very‐long‐chain C23‐C26 acids than normal brains. The difference from the normal fatty acid pattern of cerebrosides was smaller in myelin. This suggests that the cerebrosides stored in the globoid cells have an abnormal fatty acid composition.
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