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Perturbation with Intrabodies Reveals That Calpain Cleavage Is Required for Degradation of Huntingtin Exon 1

BACKGROUND: Proteolytic processing of mutant huntingtin (mHtt), the protein that causes Huntington's disease (HD), is critical for mHtt toxicity and disease progression. mHtt contains several caspase and calpain cleavage sites that generate N-terminal fragments that are more toxic than full-len...

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Detalles Bibliográficos
Autores principales: Southwell, Amber L., Bugg, Charles W., Kaltenbach, Linda S., Dunn, Denise, Butland, Stefanie, Weiss, Andreas, Paganetti, Paolo, Lo, Donald C., Patterson, Paul H.
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3031625/
https://www.ncbi.nlm.nih.gov/pubmed/21304966
http://dx.doi.org/10.1371/journal.pone.0016676