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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.
AuthorsA E Gal, A L Weis, J M Quirk, T Tokoro, R O Brady
JournalChemistry and physics of lipids (Chem Phys Lipids) Vol. 42 Issue 1-3 Pg. 199-207 (Dec 15 1986) ISSN: 0009-3084 [Print] Ireland
PMID3829206 (Publication Type: Journal Article)
Chemical References
  • Cerebrosides
  • Galactosylceramides
  • Glucosylceramides
  • Galactosylceramidase
  • Galactose
Topics
  • Animals
  • Brain (enzymology)
  • Cerebrosides (metabolism)
  • Disease Models, Animal
  • Galactose (metabolism)
  • Galactosylceramidase (metabolism)
  • Galactosylceramides (metabolism)
  • Gaucher Disease
  • Glucosylceramides (metabolism)
  • Humans
  • Intestine, Small (enzymology)
  • Leukodystrophy, Globoid Cell
  • Rats
  • Rats, Inbred Strains

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