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Inhibiting the ubiquitin–proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments

An expanded polyglutamine (polyQ) domain in the N-terminal region of huntingtin (htt) causes misfolding and accumulation of htt in neuronal cells and the subsequent neurodegeneration of Huntington's disease (HD). Clearing the misfolded htt is critical for preventing neuropathology, and this pro...

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Detalhes bibliográficos
Main Authors: Li, Xiang, Wang, Chuan-En, Huang, Shanshan, Xu, Xingshun, Li, Xiao-Jiang, Li, He, Li, Shihua
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2010
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2876889/
https://ncbi.nlm.nih.gov/pubmed/20354076
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddq127
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