Niemann-Pick type C mutations cause lipid traffic jam

被引：124

作者：

Liscum, L ^{[1
]}

机构：

[1] Tufts Univ, Dept Physiol, Sch Med, Boston, MA 02111 USA

来源：

TRAFFIC | 2000年 / 1卷 / 03期

关键词：

cholesterol; late endosome; lysobisphosphatidic acid; lysosome; lipid transport; multivesicular body; Niemann-Pick C; sphingolipid;

D O I：

10.1034/j.1600-0854.2000.010304.x

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

The Niemann-Pick C protein (NPC1) is required for cholesterol transport from late endosomes and lysosomes to other cellular membranes. Mutations in NPC1 cause lysosomal lipid storage and progressive neurological degeneration. Cloning of the NPC1 gene has given us tools with which to investigate the function of this putative cholesterol transporter. Here, we discuss recent studies indicating that NPC1 is not a cholesterol-specific transport molecule. Instead, NPC1 appears to be required for the vesicular shuttling of both lipids and fluid-phase constituents from multivesicular late endosomes to destinations such as the trans-Golgi network.

引用

页码：218 / 225

页数：8