| Polyglutamine neurodegeneration: protein misfolding revisited. | |
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MedLine Citation:
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PMID: 18778858 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Polyglutamine diseases are a major cause of neurodegeneration worldwide. Recent studies highlight the importance of protein quality control mechanisms in regulating polyglutamine-induced toxicity. Here we discuss a model of disease pathogenesis that integrates current understanding of the role of protein folding in polyglutamine disease with emerging evidence that alterations in native protein interactions contribute to toxicity. We also incorporate new findings on other age-related neurodegenerative diseases in an effort to explain how protein aggregation and normal aging processes might be involved in polyglutamine disease pathogenesis. |
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Authors:
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Aislinn J Williams; Henry L Paulson |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Review Date: 2008-09-06 |
Journal Detail:
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Title: Trends in neurosciences Volume: 31 ISSN: 0166-2236 ISO Abbreviation: Trends Neurosci. Publication Date: 2008 Oct |
Date Detail:
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Created Date: 2008-09-23 Completed Date: 2009-04-09 Revised Date: 2011-06-01 |
Medline Journal Info:
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Nlm Unique ID: 7808616 Medline TA: Trends Neurosci Country: England |
Other Details:
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Languages: eng Pagination: 521-8 Citation Subset: IM |
Affiliation:
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Program in Neuroscience and Medical Scientist Training Program, University of Iowa, 2206 MERF, Iowa City, IA 52242, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Aging
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physiology Animals Humans Models, Biological Nerve Degeneration / genetics, metabolism* Neurotoxicity Syndromes / etiology, pathology* Peptides / metabolism* Protein Folding* |
| Grant Support | |
ID/Acronym/Agency:
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F31 NS056609-02/NS/NINDS NIH HHS; NS056609/NS/NINDS NIH HHS; NS38712/NS/NINDS NIH HHS; R01 NS038712-01A1/NS/NINDS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Peptides; 26700-71-0/polyglutamine |
| Comments/Corrections | |
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