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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Bibliographic Details
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.
Format: Artigo
Language:Inglês
Published: American Society for Biochemistry and Molecular Biology 2010
Subjects:
Molecular Bases of Disease
Online Access: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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