Role of ubiquitin protein ligases in the pathogenesis of polyglutamine diseases

被引：6

作者：

Dikshit, Priyanka ^{[1
]}

Jana, Nihar Ranjan ^{[1
]}

机构：

[1] Natl Brain Res Ctr, Cellular & Mol Neurosci Lab, Manesar 122050, Gurgaon, India

来源：

NEUROCHEMICAL RESEARCH | 2008年 / 33卷 / 05期

关键词：

E3 ubiquitin ligases; CHIP; polyglutamine; E6-AP; Parkin;

D O I：

10.1007/s11064-007-9459-x

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：

The accumulation of intracellular protein deposits as inclusion bodies is the common pathological hallmark of most age related neurodegenerative disorders including polyglutamine diseases. Appearances of aggregates of the misfolded mutant disease proteins suggest that the cells are unable to efficiently degrade them, and failure of clearance leads to the severe disturbances of the cellular quality control system. The quality control ubiquitin ligases are now increasingly implicated in the biology of polyglutamine diseases, Parkinson's diseases, Amyotrophic lateral sclerosis and Alzheimer's disease. Here we review the recent studies that have revealed a critical role of E3 ubiquitin ligases in understanding the pathogenesis of polyglutamine diseases.

引用

页码：945 / 951

页数：7

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