Abstract
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.
Original language | English (US) |
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Pages (from-to) | 400-407 |
Number of pages | 8 |
Journal | Cell Research |
Volume | 20 |
Issue number | 4 |
DOIs | |
State | Published - Apr 2010 |
Externally published | Yes |
Keywords
- Aggregation
- Glia
- Huntington's disease
- Misfolding
- Neurodegeneration
- Polyglutamine
ASJC Scopus subject areas
- Molecular Biology
- Cell Biology