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.
Department of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA.
Jennifer W
JW
Bradford
Shihua
S
Li
Xiao-Jiang
XJ
Li
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