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Polyglutamine neurodegeneration: protein misfolding revisited
Journal article   Peer reviewed

Polyglutamine neurodegeneration: protein misfolding revisited

Aislinn J Williams and Henry L Paulson
Trends in neurosciences (Regular ed.), Vol.31(10), pp.521-528
10/2008
DOI: 10.1016/j.tins.2008.07.004
PMCID: PMC2580745
PMID: 18778858

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Abstract

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.
Neurotoxicity Syndromes - etiology Animals Peptides - metabolism Aging - physiology Neurotoxicity Syndromes - pathology Models, Biological Humans Nerve Degeneration - genetics Nerve Degeneration - metabolism Protein Folding

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