Animal and cellular models of sphingolipid storage disorders of humans.
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.
D000818:Animals; D001921:Brain / Q000201:enzymology; D002554:Cerebrosides / Q000378:metabolism*; D004195:Disease Models, Animal; D005690:Galactose / Q000378:metabolism; D005698:Galactosylceramidase / Q000378:metabolism; D005699:Galactosylceramides / Q000378:metabolism*; D005776:Gaucher Disease; D005963:Glucosylceramides / Q000378:metabolism; D006801:Humans; D007421:Intestine, Small / Q000201:enzymology; D007965:Leukodystrophy, Globoid Cell; D051381:Rats; D011919:Rats, Inbred Strains
D002554:Cerebrosides; D005699:Galactosylceramides; D005963:Glucosylceramides; D005698:Galactosylceramidase; D005690:Galactose