Perturbation with intrabodies reveals that calpain cleavage is required for degradation of huntingtin exon 1.

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-length mHtt. Fu...

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Bibliographic Details
Main Authors: Amber L Southwell, Charles W Bugg, Linda S Kaltenbach, Denise Dunn, Stefanie Butland, Andreas Weiss, Paolo Paganetti, Donald C Lo, Paul H Patterson
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3031625?pdf=render