Stressing out the EIR: A role of the unfolded protein response in prion-related disorders

被引：87
作者：
Hetz, CA
Soto, C
机构：
[1] Harvard Univ, Sch Publ Hlth, Dept Immunol & Infect Dis, Boston, MA 02115 USA
[2] Univ Chile, Inst Biomed Sci, Santiago, Chile
[3] Univ Texas, Med Branch, Dept Neurol Neurosci & Cell Biol, George & Cynthia Mitchell Ctr Alzheimers Dis Res, Galveston, TX 77555 USA
来源：
CURRENT MOLECULAR MEDICINE | 2006年 / 6卷 / 01期
关键词：
prion related disorders; apoptosis; PrPsc; proteasome; ER stress; glucose-regulated proteins; caspase-12; PrPSC-like; aggresomes;
D O I：
10.2174/156652406775574578
中图分类号：
R-3 [医学研究方法]; R3 [基础医学];
学科分类号：
1001 ;
摘要：
Transmissible Spongiform Encephalopathies are fatal and infectious neurodegenerative diseases characterized by extensive neuronal apoptosis and the accumulation of an abnormally folded form of the cellular prion protein (PrP), denoted PrPSC. Compelling evidence suggests the involvement of several signaling pathways in prion pathogenesis, including proteasome dysfunction, alterations in the protein maturation pathways and the unfolded protein response. Recent reports indicate that endoplasmic reticulum stress due to the PrP misfolding may be a critical factor mediating neuronal dysfunction in prion diseases. These findings have applications for developing novel strategies for treatment and early diagnosis of transmissible spongiform encephalopathies and other neurodegenerative diseases.
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收藏
页码：37 / 43
页数：7
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