Polyglutamine toxicity in non-neuronal cells.
Cell Res
; 20(4): 400-7, 2010 Apr.
Article
in En
| MEDLINE
| ID: mdl-20231860
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Peptides
/
Neuroglia
/
Heredodegenerative Disorders, Nervous System
Limits:
Humans
Language:
En
Journal:
Cell Res
Year:
2010
Document type:
Article
Affiliation country:
United States