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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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Dettagli Bibliografici
Autori principali: Li, Xiang, Wang, Chuan-En, Huang, Shanshan, Xu, Xingshun, Li, Xiao-Jiang, Li, He, Li, Shihua
Natura: Artigo
Lingua:Inglês
Pubblicazione: Oxford University Press 2010
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Accesso online: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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