Heat shock response activation exacerbates inclusion body formation in a cellular model of huntington disease

Kirill Bersuker, Mark S. Hipp, Barbara Calamini, Richard I. Morimoto, Ron R. Kopito*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

41 Scopus citations

Abstract

Background: Aggregation of mutant huntingtin into inclusion bodies is a pathogenic feature of Huntington disease. Results: Genetic and pharmacological activation of the heat shock stress response (HSR) promotes inclusion body formation. Conclusion: Activation of the HSR contributes to mutant huntingtin aggregation. Significance: HSR activation as a therapeutic strategy may promote rather than suppress aggregation of proteins implicated in neurodegenerative disease.

Original languageEnglish (US)
Pages (from-to)23633-23638
Number of pages6
JournalJournal of Biological Chemistry
Volume288
Issue number33
DOIs
StatePublished - Aug 16 2013

ASJC Scopus subject areas

  • Molecular Biology
  • Biochemistry
  • Cell Biology

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