Endoplasmic reticulum stress in disease pathogenesis

被引：622

作者：

Lin, Jonathan H. ^{[1
,2
]}

Walter, Peter ^{[1
,3
]}

Yen, T. S. Benedict ^{[2
,4
]}

机构：

[1] Univ Calif San Francisco, Dept Biochem & Biophys, San Francisco, CA 94143 USA

[2] Univ Calif San Francisco, Dept Pathol, San Francisco, CA 94143 USA

[3] Howard Hughes Med Inst, Chevy Chase, MD USA

[4] San Francisco VA Med Ctr, Pathol Serv, San Francisco, CA 94121 USA

来源：

ANNUAL REVIEW OF PATHOLOGY-MECHANISMS OF DISEASE | 2008年 / 3卷

关键词：

protein misfolding; unfolded protein response; IRE1; PERK; ATF-6;

D O I：

10.1146/annurev.pathmechdis.3.121806.151434

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

The endoplasmic reticulum (ER) is the site of synthesis and folding of membrane and secretory proteins, which, collectively, represent a large fraction of the total protein output of a mammalian cell. Therefore; the protein flux through the ER must be carefully monitored for abnormalities, including the buildup of misfolded proteins. Mammalian cells have. evolved an intricate set of signaling pathways from the ER to the cytosol and nucleus, to allow the cell to respond to the of fold presence misfold proteins within the ER. These pathways, known collectively as-the unfolded protein response, are important for normal cellular homeostasis and organismal development and may also play key roles in the pathogenesis of many diseases. This review provides background information on the unfolded protein response and discusses a selection of diseases whose pathogenesis involves ER stress.

引用

页码：399 / 425

页数：27

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