SAPOSIN PROTEINS - STRUCTURE, FUNCTION, AND ROLE IN HUMAN LYSOSOMAL STORAGE DISORDERS

被引：329

作者：

OBRIEN, JS

KISHIMOTO, Y

机构：

[1] Dept. of Neurosciences, Center for Molecular Genetics, University of California, San Diego, La Jolla, CA

来源：

FASEB JOURNAL | 1991年 / 5卷 / 03期

关键词：

SPHINGOLIPID ACTIVATOR PROTEINS; GAUCHER DISEASE; FUCOSIDOSIS; LYSOSOMAL STORAGE DISEASE; TAY-SACHS DISEASE;

D O I：

10.1096/fasebj.5.3.2001789

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Saposins are sphingolipid activator proteins, four of which are derived from a single precursor, prosaposin, by proteolytic processing. These small heat-stable glycoproteins (12-14 kDa) are required for the lysosomal hydrolysis of a variety of sphingolipids. Characterization of these four activator proteins, two of which were recently discovered, and their importance in human health and disease are reviewed in this article.

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页码：301 / 308

页数：8

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