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Polyglutamine toxicity in non-neuronal cells Bradford JW; Li S; Li XJCell Res 2010[Apr]; 20 (4): 400-7The 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.|Heredodegenerative Disorders, Nervous System/*genetics/metabolism[MESH]|Humans[MESH]|Huntington Disease/metabolism[MESH]|Muscular Disorders, Atrophic/metabolism[MESH]|Myoclonic Epilepsies, Progressive/metabolism[MESH]|Neuroglia/cytology/*metabolism[MESH]|Peptides/*toxicity[MESH]|Spinocerebellar Ataxias/metabolism[MESH] |
