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

Publication ,  Journal Article
Gal, AE; Pentchev, PG; Massey, JM; Brady, RO
Published in: Proc Natl Acad Sci U S A
July 1979
Published version (DOI) Link to item

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.

Duke Scholars

Janice Munn Massey
Author Janice Munn Massey Neurology, Neuromuscular Disease

Published In

Proc Natl Acad Sci U S A

DOI

10.1073/pnas.76.7.3083

ISSN

0027-8424

Publication Date

July 1979

Volume

76

Issue

7

Start / End Page

3083 / 3086

Location

United States

Related Subject Headings

  • Stereoisomerism
  • Research Design
  • Mice
  • In Vitro Techniques
  • Humans
  • Glucosylceramides
  • Glucosylceramidase
  • Glucosidases
  • Gaucher Disease
  • Cerebrosides
 

Citation

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Gal, A. E., Pentchev, P. G., Massey, J. M., & Brady, R. O. (1979). L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro. Proc Natl Acad Sci U S A, 76(7), 3083–3086. https://doi.org/10.1073/pnas.76.7.3083
Gal, A. E., P. G. Pentchev, J. M. Massey, and R. O. Brady. “L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro.” Proc Natl Acad Sci U S A 76, no. 7 (July 1979): 3083–86. https://doi.org/10.1073/pnas.76.7.3083.
Gal AE, Pentchev PG, Massey JM, Brady RO. L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro. Proc Natl Acad Sci U S A. 1979 Jul;76(7):3083–6.
Gal, A. E., et al. “L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro.” Proc Natl Acad Sci U S A, vol. 76, no. 7, July 1979, pp. 3083–86. Pubmed, doi:10.1073/pnas.76.7.3083.
Gal AE, Pentchev PG, Massey JM, Brady RO. L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro. Proc Natl Acad Sci U S A. 1979 Jul;76(7):3083–3086.
Journal cover image

Published In

Proc Natl Acad Sci U S A

DOI

10.1073/pnas.76.7.3083

ISSN

0027-8424

Publication Date

July 1979

Volume

76

Issue

7

Start / End Page

3083 / 3086

Location

United States

Related Subject Headings

  • Stereoisomerism
  • Research Design
  • Mice
  • In Vitro Techniques
  • Humans
  • Glucosylceramides
  • Glucosylceramidase
  • Glucosidases
  • Gaucher Disease
  • Cerebrosides