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.
Full text
PDF
Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Adachi M., Volk B. W. Gaucher disease in mice induced by conduritol-B-epoxide: morphologic features. Arch Pathol Lab Med. 1977 May;101(5):255–259. [PubMed] [Google Scholar]
- BRADY R. O., KANFER J. N., SHAPIRO D. METABOLISM OF GLUCOCEREBROSIDES. II. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHER'S DISEASE. Biochem Biophys Res Commun. 1965 Jan 18;18:221–225. doi: 10.1016/0006-291x(65)90743-6. [DOI] [PubMed] [Google Scholar]
- Brady R. O. Inherited metabolic diseases of the nervous system. Science. 1976 Aug 27;193(4255):733–739. doi: 10.1126/science.822514. [DOI] [PubMed] [Google Scholar]
- Burton R. M., Sodd M. A., Lewis W., 3rd The distribution and excretion of tritium labeled cerebroside (galactosyl ceramide) radioactivity by the monkey. Lipids. 1969 Nov;4(6):496–500. doi: 10.1007/BF02531031. [DOI] [PubMed] [Google Scholar]
- FOLCH J., LEES M., SLOANE STANLEY G. H. A simple method for the isolation and purification of total lipides from animal tissues. J Biol Chem. 1957 May;226(1):497–509. [PubMed] [Google Scholar]
- Furbish F. S., Blair H. E., Shiloach J., Pentchev P. G., Brady R. O. Enzyme replacement therapy in Gaucher's disease: large-scale purification of glucocerebrosidase suitable for human administration. Proc Natl Acad Sci U S A. 1977 Aug;74(8):3560–3563. doi: 10.1073/pnas.74.8.3560. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Gal A. E., Pentchev P. G., Fash F. J. A novel chromogenic substrate for assaying glucocerebrosidase activity. Proc Soc Exp Biol Med. 1976 Nov;153(2):363–366. doi: 10.3181/00379727-153-39546. [DOI] [PubMed] [Google Scholar]
- Gal A. E. Separation and identification of monosaccharides from biological materials by thin-layer chromatography. Anal Biochem. 1968 Sep;24(3):452–461. doi: 10.1016/0003-2697(68)90152-8. [DOI] [PubMed] [Google Scholar]
- Neskovic N. M., Nussbaum J. L., Mandel P. [Thin-layer chromatographic separation of glycolipids in animal lipid mixtures]. J Chromatogr. 1970 Jun 3;49(2):255–261. doi: 10.1016/s0021-9673(00)93630-6. [DOI] [PubMed] [Google Scholar]
- Pentchev P. G., Brady R. O., Blair H. E., Britton D. E., Sorrell S. H. Gaucher disease: isolation and comparison of normal and mutant glucocerebrosidase from human spleen tissue. Proc Natl Acad Sci U S A. 1978 Aug;75(8):3970–3973. doi: 10.1073/pnas.75.8.3970. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Stephens M. C., Bernatsky A., Burachinsky V., Legler G., Kanfer J. N. The Gaucher mouse: differential action of conduritol B epoxide and reversibility of its effects. J Neurochem. 1978 May;30(5):1023–1027. doi: 10.1111/j.1471-4159.1978.tb12395.x. [DOI] [PubMed] [Google Scholar]