Abstract
Two glycosphingolipids that accumulate in patients with Fabry's disease were isolated from abnormal kidney by gradient elution chromatography on silicic acid. A combination of periodate oxidation, mild acid hydrolysis, permethylation, and gas chromatographic studies of the sugar moieties established the arrangement of sugars and positions of their anomeric linkages in the oligosaccharide portions of the lipids. Comparisons of the NMR spectra of trimethylsilyl derivatives of the lipids and several degradation products with those of reference compounds allowed the assignment of configurations of glycosidic linkages in the trihexosylceramide. These studies provide conclusive evidence for the chemical structure of the major accumulated glycosphingolipid in Fabry's disease, which is galactosyl-(β1→4)-galactosyl-(β1→4)-glucosyl-(β1→1)-ceramide. The other glycosphingolipid isolated from the kidney was not completely characterized; a partial chemical structure is galactosyl-(1→4)-galactosyl-(1→1)-ceramide.
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