Polyglutamine diseases: a transcription disorder?

被引：67

作者：

Okazawa, H ^{[1
]}

机构：

[1] Japan Sci Technol Corp, PRESTO, Tokyo Metropolitan Inst Neurosci, Dept Mol Therapeut, Fuchu, Tokyo 1838526, Japan

来源：

CELLULAR AND MOLECULAR LIFE SCIENCES | 2003年 / 60卷 / 07期

关键词：

polyglutamine disease; transcription; cofactor; splicing; RNA; nuclear body; aggregation; ubiquitin; SUMO; proteasome; neuronal death;

D O I：

10.1007/s00018-003-3013-z

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：

Various molecular processes including unfolded protein response, protein transport, synaptic transmission and transcription are implicated in the pathology of polyglutamine diseases caused by the expanded polyglutamine-containing proteins. More than 20 transcription-related factors have been reported to interact with disease proteins, and the pathological interaction is known to repress gene expression. The whole shape of nuclear events evoked by disease proteins is now emerging with information on these transcription-related factors and with findings on the similarity between nuclear bodies and pathological inclusion bodies. This article reviews 'transcription theory', a rapidly growing hypothesis in polyglutamine diseases.

引用

页码：1427 / 1439

页数：13

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