Polyglutamine toxicity in non-neuronal cells

Jennifer Webster Bradford, Shihua Li, Xiao Jiang Li

Research output: Contribution to journalReview article

15 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)400-407
Number of pages8
JournalCell Research
Volume20
Issue number4
DOIs
StatePublished - Apr 1 2010

Fingerprint

Mutant Proteins
Proteins
polyglutamine
Neurodegenerative Diseases
Disease Progression
Population

Keywords

  • Aggregation
  • Glia
  • Huntington's disease
  • Misfolding
  • Neurodegeneration
  • Polyglutamine

ASJC Scopus subject areas

  • Molecular Biology
  • Cell Biology

Cite this

Polyglutamine toxicity in non-neuronal cells. / Bradford, Jennifer Webster; Li, Shihua; Li, Xiao Jiang.

In: Cell Research, Vol. 20, No. 4, 01.04.2010, p. 400-407.

Research output: Contribution to journalReview article

Bradford, Jennifer Webster ; Li, Shihua ; Li, Xiao Jiang. / Polyglutamine toxicity in non-neuronal cells. In: Cell Research. 2010 ; Vol. 20, No. 4. pp. 400-407.
@article{2437c9a3b96b4af58a0ada171d46dbca,
title = "Polyglutamine toxicity in non-neuronal cells",
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.",
keywords = "Aggregation, Glia, Huntington's disease, Misfolding, Neurodegeneration, Polyglutamine",
author = "Bradford, {Jennifer Webster} and Shihua Li and Li, {Xiao Jiang}",
year = "2010",
month = "4",
day = "1",
doi = "10.1038/cr.2010.32",
language = "English (US)",
volume = "20",
pages = "400--407",
journal = "Cell Research",
issn = "1001-0602",
publisher = "Nature Publishing Group",
number = "4",

}

TY - JOUR

T1 - Polyglutamine toxicity in non-neuronal cells

AU - Bradford, Jennifer Webster

AU - Li, Shihua

AU - Li, Xiao Jiang

PY - 2010/4/1

Y1 - 2010/4/1

N2 - 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.

AB - 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.

KW - Aggregation

KW - Glia

KW - Huntington's disease

KW - Misfolding

KW - Neurodegeneration

KW - Polyglutamine

UR - http://www.scopus.com/inward/record.url?scp=77950547307&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77950547307&partnerID=8YFLogxK

U2 - 10.1038/cr.2010.32

DO - 10.1038/cr.2010.32

M3 - Review article

C2 - 20231860

AN - SCOPUS:77950547307

VL - 20

SP - 400

EP - 407

JO - Cell Research

JF - Cell Research

SN - 1001-0602

IS - 4

ER -