BRAIN SPHINGOGLYCOLIPIDS IN KRABBE'S GLOBOID CELL LEUCODYSTROPHY1

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Abstract
Abstract— Seven sphingoglycolipids were isolated from the white matter of a patient with globoid cell leucodystrophy (Krabbe's disease). After purification by saponification and column and preparative thin‐layer chromatography, these compounds were analysed for the carbohydrate composition and sequence and for fatty acid composition by paper and gas‐liquid chromatography. The compounds were identified as gluco‐ and galactocerebrosides, lactosyl‐ceramide, digalactosy I‐glucosyl‐ceramide, two types of tetrahexosyl‐ceramides (asialo‐ganglioside and globoside), and sulphatide. Glucocerebrosideconstituted 13 percent of total cerebroside in white matter, but sulphatide contained only galactose. Galactocere‐broside and sulphatide exhibited compositions of fatty acids similar to those in normal white matter, with only minor abnormalities. Other sphingoglycolipids showed fatty acid patterns with relatively high proportions of longer‐chain fatty acids, rather than the predominant C18:0 acid usually found in ceramide hexosides of the brain. Hematoside, also found in the white matter in a significant amount, similarly contained a large proportion of longer‐chain fatty acids, whereas other gangliosides contained predominantly C18:0 acid. The abnormal ceramide hexoside pattern was restricted mostly to white matter except for glucocerebroside, which constituted 32 per cent of grey matter cerebroside. We postulate that the visceral type of sphingoglycolipids may be constituents of globoid cells, abundantly present in white matter and considered to be cells of mesenchymal origin.

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