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.
- Gal, AE; Pentchev, PG; Massey, JM; Brady, RO
- July 1, 1979
Volume / Issue
- 76 / 7
Start / End Page
- 3083 - 3086
Pubmed Central ID
International Standard Serial Number (ISSN)
Digital Object Identifier (DOI)
- United States