Animal and cellular models of sphingolipid storage disorders of humans.

ABSTRACT

The synthesis of L-galactosylceramide is described. Data are presented indicating that this enantiomorph of D-galactocerebroside is not cleaved by galactocerebroside-beta-galactosidase obtained from mammalian tissues. The synthesis of L-glucosylceramide and beta-D-glucothiocerebroside are outlined. These compounds are also refractory to catabolism by glycosidases in mammalian tissues that catalyze the hydrolysis of naturally occurring cerebrosides. L-Hexosyl- and thioanalogs of cerebrosides and perhaps psychosines as well may be helpful for investigating the pathogenesis of Krabbe's disease and Gaucher's disease.