Polyglutamine neurodegeneration: protein misfolding revisited.

Polyglutamine neurodegeneration: protein misfolding revisited.
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DOI:
10.1016/j.tins.2008.07.004
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发表时间:
2008-10
影响因子:
15.9
通讯作者:
Paulson, Henry L.
Paulson, Henry L.
中科院分区:
医学1区
文献类型:
--
作者:
Williams, Aislinn J.;Paulson, Henry L.

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Polyglutamine diseases are a major cause of neurodegenerative disease worldwide. Recent studies highlight the importance of protein quality control mechanisms in regulating polyglutamine-induced toxicity. Drawing on these studies, we propose a model of disease pathogenesis that integrates current understanding of the role of protein folding in polyglutamine disease. 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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