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  • Title: L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro.
    Author: Gal AE, Pentchev PG, Massey JM, Brady RO.
    Journal: Proc Natl Acad Sci U S A; 1979 Jul; 76(7):3083-6. PubMed ID: 290986.
    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 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.
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