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Published in: Molecular Brain 1/2009

Open Access 01-12-2009 | Review

Nuclear accumulation of polyglutamine disease proteins and neuropathology

Authors: Lauren S Havel, Shihua Li, Xiao-Jiang Li

Published in: Molecular Brain | Issue 1/2009

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Abstract

There are nine inherited neurodegenerative disorders caused by polyglutamine (polyQ) expansion in various disease proteins. Although these polyglutamine proteins have different functions and are localized in different subcellular regions, all the polyQ diseases share a common pathological feature: the nuclear accumulation of polyQ disease proteins and the formation of inclusions. The nuclear accumulation of polyQ proteins in turn leads to gene transcriptional dysregulation and neuropathology. Here we will discuss potential mechanisms behind the nuclear accumulation of mutant polyQ proteins, since an understanding of how polyQ proteins accumulate in the nucleus could help elucidate the pathogenesis of these diseases and develop their treatment.
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Metadata
Title
Nuclear accumulation of polyglutamine disease proteins and neuropathology
Authors
Lauren S Havel
Shihua Li
Xiao-Jiang Li
Publication date
01-12-2009
Publisher
BioMed Central
Published in
Molecular Brain / Issue 1/2009
Electronic ISSN: 1756-6606
DOI
https://doi.org/10.1186/1756-6606-2-21

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