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Accumulation of Mutant Huntingtin Fragments in Aggresome-like Inclusion Bodies as a Result of Insufficient Protein Degradation

The huntingtin exon 1 proteins with a polyglutamine repeat in the pathological range (51 or 83 glutamines), but not with a polyglutamine tract in the normal range (20 glutamines), form aggresome-like perinuclear inclusions in human 293 Tet-Off cells. These structures contain aggregated, ubiquitinate...

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Main Authors: Waelter, Stephanie, Boeddrich, Annett, Lurz, Rudi, Scherzinger, Eberhard, Lueder, Gerhild, Lehrach, Hans, Wanker, Erich E.
Format: Artigo
Jezik:Inglês
Izdano: The American Society for Cell Biology 2001
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Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC34592/
https://ncbi.nlm.nih.gov/pubmed/11359930
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