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

doi:10.1074/jbc.C113.481945

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

Molecular Biosciences

Research output: Contribution to journal › Article › peer-review

43 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 language	English (US)
Pages (from-to)	23633-23638
Number of pages	6
Journal	Journal of Biological Chemistry
Volume	288
Issue number	33
DOIs	https://doi.org/10.1074/jbc.C113.481945
State	Published - Aug 16 2013

Funding

ASJC Scopus subject areas

Molecular Biology
Biochemistry
Cell Biology

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title = "Heat shock response activation exacerbates inclusion body formation in a cellular model of huntington disease",

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.",

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AU - Bersuker, Kirill

AU - Hipp, Mark S.

AU - Calamini, Barbara

AU - Morimoto, Richard I.

AU - Kopito, Ron R.

PY - 2013/8/16

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N2 - 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.

AB - 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.

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

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