Article
Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions.
Molecular cell - 1 Aug 2002
Lunkes Astrid, Lindenberg Katrin S, Ben-Haïem Léa, Weber Chantal, Devys Didier, Landwehrmeyer G Bernhard, Mandel Jean-Louis, Trottier Yvon
Abstract excerpt
Proteolytic processing of mutant huntingtin (mhtt) is regarded as a key event in the pathogenesis of Huntington's disease (HD). Mhtt fragments containing a polyglutamine expansion form intracellular inclusions and are more cytotoxic than full-length mhtt. Here, we report that two distinct mhtt fragments, termed cp-A and cp-B, differentially build up nuclear and cytoplasmic inclusions in HD brain and in a cellular...
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