Polyglutamine toxicity in non-neuronal cells.
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.
Bradford
Jennifer W
JW
Department of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA.
Li
Shihua
S
Li
Xiao-Jiang
XJ
D020271:Heredodegenerative Disorders, Nervous System / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D006816:Huntington Disease / Q000378:metabolism; D020966:Muscular Disorders, Atrophic / Q000378:metabolism; D020191:Myoclonic Epilepsies, Progressive / Q000378:metabolism; D009457:Neuroglia / Q000166:cytology / Q000378:metabolism*; D010455:Peptides / Q000633:toxicity*; D020754:Spinocerebellar Ataxias / Q000378:metabolism
D010455:Peptides; C097188:polyglutamine