Polyglutamine toxicity in non-neuronal cells  

作　　者：Jennifer W Bradford Shihua Li Xiao-Jiang Li 

机构地区：[1]Department of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA

出　　处：《Cell Research》2010年第4期400-407,共8页细胞研究（英文版）

摘　　要：The neurodegenerative polyglutamine diseases are caused various disease proteins. Although these mutant proteins are by an expansion of unstable polyglutamine repeats in 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 progres- sion will help elucidate disease mechanisms and also help in the development of new treatment options.

关 键 词：POLYGLUTAMINE Huntington＇s disease NEURODEGENERATION GLIA MISFOLDING AGGREGATION 

分 类 号：Q550.3[生物学—生物化学] S858.315.3[农业科学—临床兽医学]