Volume 20, No 4, Apr 2010

ISSN: 1001-0602 
EISSN: 1748-7838 2018 
impact factor 17.848* 
(Clarivate Analytics, 2019)

Volume 20 Issue 4, April 2010: 400-407

REVIEWS

Polyglutamine toxicity in non-neuronal cells

Polyglutamine toxicity in non-neuronal cells

Polyglutamine toxicity in non-neuronal cells Correspondence: Xiao-Jiang Li,(xli2@emory.edu)

The neurodegenerative polyglutamine diseases are caused by an expansion of unstable polyglutamine repeats in various disease proteins. Although these mutant proteins are expressed ubiquitously in neuronal and non-neuronal cells, they cause selective degeneration of specific neuronal populations. Recently, increasing evidence shows that polyglutamine disease proteins also affect non-neuronal cells. However, it remains unclear how the expression of polyglutamine proteins in non-neuronal cells contributes to the course of the polyglutamine diseases. Here, we discuss recent findings about the expression of mutant polyglutamine proteins in non-neuronal cells and their influence on neurological symptoms. Understanding the contribution of non-neuronal polyglutamine proteins to disease progression will help elucidate disease mechanisms and also help in the development of new treatment options.

Cell Research (2010) 20:400-407. doi: 10.1038/cr.2010.32; published online 16 March 2010

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