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Bifunctional Anti-Huntingtin Proteasome-Directed Intrabodies Mediate Efficient Degradation of Mutant Huntingtin Exon 1 Protein Fragments

Huntington's disease (HD) is a fatal autosomal dominant neurodegenerative disorder caused by a trinucleotide (CAG)(n) repeat expansion in the coding sequence of the huntingtin gene, and an expanded polyglutamine (>37Q) tract in the protein. This results in misfolding and accumulation of hunt...

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Autori principali: Butler, David C., Messer, Anne
Natura: Artigo
Lingua:Inglês
Pubblicazione: Public Library of Science 2011
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3245261/
https://ncbi.nlm.nih.gov/pubmed/22216210
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0029199
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