文章基本信息

标题：L-glucosylceramide: synthesis, properties, and resistance to catabolism by glucocerebrosidase in vitro
本地全文：下载
作者：A E Gal ; P G Pentchev ; J M Massey 等
期刊名称：Proceedings of the National Academy of Sciences
印刷版ISSN：0027-8424
电子版ISSN：1091-6490
出版年度：1979
卷号：76
期号：7
页码：3083-3086
DOI：10.1073/pnas.76.7.3083
语种：English
出版社：The National Academy of Sciences of the United States of America
摘要：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.