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Journal of Lipid Research, Vol. 11, 31-37, January 1970
Copyright © 1970 by Lipid Research, Inc.

A method for the quantitative determination of neutral glycosphingolipids in urine sediment

R. J. Desnick ¹, C. C. Sweeley ², and William Krivit ²

¹ Dight Institute for Human Genetics, University of Minnesota, Minneapolis, Minnesota 55455, Department of Biochemistry, Michigan State University, East Lansing, Michigan 48823, and Department of Pediatrics, University of Minnesota Medical School, Minneeapolis, Minnesota 55455
² Dight Institute for Human Genetics, University of Minnesota, Minneapolis, Minnesota 55455, Department of Biochemistry, Michigan State University, East Lansing, Michigan 48823, and Department of Pediatrics, University of Minnesota Medical School, Minnesota 55455

A method is described for the isolation and quantitation of six neutral glycosyl ceramides from human urinary sediment. Total lipids were extracted from sediments of 24-hr urine collections, and the glycosyl ceramides were isolated by silicic acid column chromatography followed by thin-layer chromatography. Methanolysis of the individual glycosyl ceramides yielded methyl glycosides which were quantitated as the trimethylsilyl ethers by gas-liquid chromatography.

By this technique, the submicromolar concentrations of six glycosyl ceramides in normal subjects and in individuals with Fabry's disease, an hereditary glycosphingolipid storage disease, were determined. Trihexosyl ceramide (galactosyl-galactosylglucosyl ceramide) and a digalactosyl ceramide accumulated in the urinary sediment of patients with Fabry's disease.

Supplementary key words neutral glycosyl ceramides • thin-layer chromatography • gas-liquid chromatography • trimethylsilyl methyl glycosides • Fabry's disease • trihexosyl ceramide • digalactosyl ceramide

Submitted on June 16, 1969
Accepted on September 11, 1969

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