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  • Title: Animal and cellular models of sphingolipid storage disorders of humans.
    Author: Gal AE, Weis AL, Quirk JM, Tokoro T, Brady RO.
    Journal: Chem Phys Lipids; 1986 Dec 15; 42(1-3):199-207. PubMed ID: 3829206.
    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.
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