L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro.
Procedures for the synthesis and radioactive labeling of L-glucosylceramide are described. This compound is a stereoisomeric analogue of D-glucosylceramide which occurs in nature and accumulates in pathological quantity in the organs and tissues of patients with Gaucher disease. The properties of L-glucosylceramide that have been examined so far have been found to be indistinguishable from those of the naturally occurring glycolipid. However, L-glucosylceramide is completely refractory to enzymatic hydrolysis by purified placental glucocerebrosidase and enzyme(s) present in whole tissue extracts. It is anticipated that L-glucosylceramide will be a uniquely useful substance for exploring pathogenetic processes in animal analogues of Gaucher disease.
D000818:Animals; D002554:Cerebrosides / Q000138:chemical synthesis*; D005776:Gaucher Disease / Q000209:etiology / Q000378:metabolism; D005959:Glucosidases / Q000494:pharmacology*; D005962:Glucosylceramidase / Q000494:pharmacology*; D005963:Glucosylceramides / Q000138:chemical synthesis* / Q000378:metabolism; D006801:Humans; D066298:In Vitro Techniques; D051379:Mice; D012107:Research Design; D013237:Stereoisomerism
D002554:Cerebrosides; D005963:Glucosylceramides; D005959:Glucosidases; D005962:Glucosylceramidase