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

Abstract

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 examined so far are indistinguishable from those of the naturally occurring glycolipid. L-Glucosylceramide is completely refractory to enzymatic hydrolysis by purified placental glucocerebrosidase and enzyme(s) present in whole [mouse] tissue extracts. L-Glucosylceramide will probably be a uniquely useful substance for exploring pathogenetic processes in animal analogues of Gaucher disease.