Proteolysis of Mutant Huntingtin Produces an Exon 1 Fragment That Accumulates as an Aggregated Protein in Neuronal Nuclei in Huntington Disease

Huntingtin proteolysis has been implicated in the molecular pathogenesis of Huntington disease (HD). Despite an intense effort, the identity of the pathogenic smallest N-terminal fragment has not been determined. Using a panel of anti-huntingtin antibodies, we employed an unbiased approach to genera...

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Detalhes bibliográficos
Main Authors: Landles, Christian, Sathasivam, Kirupa, Weiss, Andreas, Woodman, Ben, Moffitt, Hilary, Finkbeiner, Steve, Sun, Banghua, Gafni, Juliette, Ellerby, Lisa M., Trottier, Yvon, Richards, William G., Osmand, Alex, Paganetti, Paolo, Bates, Gillian P.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2010
Assuntos:
Molecular Bases of Disease
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2838303/
https://ncbi.nlm.nih.gov/pubmed/20086007
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M109.075028
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