L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Gal, AE; Pentchev, PG; Massey, JM; Brady, RO

Published Date

  • July 1, 1979

Published In

Volume / Issue

  • 76 / 7

Start / End Page

  • 3083 - 3086

PubMed ID

  • 290986

Pubmed Central ID

  • PMC383767

International Standard Serial Number (ISSN)

  • 0027-8424

Digital Object Identifier (DOI)

  • 10.1073/pnas.76.7.3083


  • eng

Conference Location

  • United States