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

作者信息

Gal A E, Pentchev P G, Massey J M, Brady R O

出版信息

Proc Natl Acad Sci U S A. 1979 Jul;76(7):3083-6. doi: 10.1073/pnas.76.7.3083.

DOI:10.1073/pnas.76.7.3083
PMID:290986
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC383767/
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.

摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/383767/648669b8fac2/pnas00007-0052-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/383767/ba7dfc757f4b/pnas00007-0050-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/383767/a80b33e0d789/pnas00007-0050-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/383767/e697b192d495/pnas00007-0051-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/383767/648669b8fac2/pnas00007-0052-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/383767/ba7dfc757f4b/pnas00007-0050-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/383767/a80b33e0d789/pnas00007-0050-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/383767/e697b192d495/pnas00007-0051-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ce1d/383767/648669b8fac2/pnas00007-0052-a.jpg

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Proc Natl Acad Sci U S A. 1979 Jul;76(7):3083-6. doi: 10.1073/pnas.76.7.3083.
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本文引用的文献

1
METABOLISM OF GLUCOCEREBROSIDES. II. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHER'S DISEASE.葡萄糖脑苷脂的代谢。II. 戈谢病中酶缺乏的证据。
Biochem Biophys Res Commun. 1965 Jan 18;18:221-5. doi: 10.1016/0006-291x(65)90743-6.
2
A simple method for the isolation and purification of total lipides from animal tissues.一种从动物组织中分离和纯化总脂质的简单方法。
J Biol Chem. 1957 May;226(1):497-509.
3
Separation and identification of monosaccharides from biological materials by thin-layer chromatography.通过薄层色谱法从生物材料中分离和鉴定单糖
Anal Biochem. 1968 Sep;24(3):452-61. doi: 10.1016/0003-2697(68)90152-8.
4
Thin-layer chromatographic separation of glycolipids in animal lipid mixtures.
J Chromatogr. 1970 Jun 3;49(2):255-61. doi: 10.1016/s0021-9673(00)93630-6.
5
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.
6
A novel chromogenic substrate for assaying glucocerebrosidase activity.一种用于检测葡糖脑苷脂酶活性的新型显色底物。
Proc Soc Exp Biol Med. 1976 Nov;153(2):363-6. doi: 10.3181/00379727-153-39546.
7
Inherited metabolic diseases of the nervous system.
Science. 1976 Aug 27;193(4255):733-9. doi: 10.1126/science.822514.
8
Gaucher disease in mice induced by conduritol-B-epoxide: morphologic features.
Arch Pathol Lab Med. 1977 May;101(5):255-9.
9
Enzyme replacement therapy in Gaucher's disease: large-scale purification of glucocerebrosidase suitable for human administration.戈谢病的酶替代疗法：适用于人体给药的葡萄糖脑苷脂酶的大规模纯化。
Proc Natl Acad Sci U S A. 1977 Aug;74(8):3560-3. doi: 10.1073/pnas.74.8.3560.
10
The Gaucher mouse: differential action of conduritol B epoxide and reversibility of its effects.
J Neurochem. 1978 May;30(5):1023-7. doi: 10.1111/j.1471-4159.1978.tb12395.x.