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Under conditions of cellular stress, activation of the heat shock response (HSR) results in an increase in the levels of molecular chaperones and is a first line of cellular defence against inclusion formation. It remains to be established whether neurodegenerative disease-associated proteins and inclusions are themselves capable of inducing an HSR in neuronal cells. To address this, we generated a neuroblastoma cell line that expresses a fluorescent reporter protein under conditions of heat shock transcription factor 1 (HSF1)-mediated HSR induction. We show that the HSR is not induced by exogenous treatment with aggregated forms of recombinant \u03b1-synuclein or the G93A mutant of superoxide dismutase-1 (SOD1G93A) nor intracellular expression of SOD1G93A or a pathogenic form of polyglutamine-expanded huntingtin (Htt72Q). These results suggest that pathogenic proteins evade detection or impair induction of the HSR in neuronal cells. A failure of protein aggregation to induce an HSR might contribute to the development of inclusion pathology in neurodegenerative diseases.<\/jats:p>\n                  <jats:p>This article has an associated First Person interview with the first author of the paper.<\/jats:p>","DOI":"10.1242\/jcs.243709","type":"journal-article","created":{"date-parts":[[2020,7,13]],"date-time":"2020-07-13T11:17:02Z","timestamp":1594639022000},"source":"Crossref","is-referenced-by-count":11,"title":["Neurodegenerative disease-associated protein aggregates are poor inducers of the heat shock response in neuronal cells"],"prefix":"10.1242","volume":"133","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5725-5512","authenticated-orcid":false,"given":"Rebecca","family":"San Gil","sequence":"first","affiliation":[{"name":"Illawarra Health and Medical Research Institute 1 , Wollongong, NSW 2522 , Australia"},{"name":"Molecular Horizons and School of Chemistry and Molecular Bioscience, University of Wollongong 2 , Wollongong, NSW , Australia"},{"name":"Queensland Brain Institute, University of Queensland 3 Neurodegeneration Pathobiology Laboratory , , St Lucia, QLD 4072 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5345-8360","authenticated-orcid":false,"given":"Dezerae","family":"Cox","sequence":"additional","affiliation":[{"name":"Bio21 Molecular Science and Biotechnology Institute, University of Melbourne 4 Department of Biochemistry and Molecular Biology , , Parkville, VIC 3052 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1764-3809","authenticated-orcid":false,"given":"Luke","family":"McAlary","sequence":"additional","affiliation":[{"name":"Illawarra Health and Medical Research Institute 1 , Wollongong, NSW 2522 , Australia"},{"name":"Molecular Horizons and School of Chemistry and Molecular Bioscience, University of Wollongong 2 , Wollongong, NSW , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tracey","family":"Berg","sequence":"additional","affiliation":[{"name":"Illawarra Health and Medical Research Institute 1 , Wollongong, NSW 2522 , Australia"},{"name":"Molecular Horizons and School of Chemistry and Molecular Bioscience, University of Wollongong 2 , Wollongong, NSW , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7954-5801","authenticated-orcid":false,"given":"Adam K.","family":"Walker","sequence":"additional","affiliation":[{"name":"Queensland Brain Institute, University of Queensland 3 Neurodegeneration Pathobiology Laboratory , , St Lucia, QLD 4072 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2528-7039","authenticated-orcid":false,"given":"Justin J.","family":"Yerbury","sequence":"additional","affiliation":[{"name":"Illawarra Health and Medical Research Institute 1 , Wollongong, NSW 2522 , Australia"},{"name":"Molecular Horizons and School of Chemistry and Molecular Bioscience, University of Wollongong 2 , Wollongong, NSW , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9241-8268","authenticated-orcid":false,"given":"Lezanne","family":"Ooi","sequence":"additional","affiliation":[{"name":"Illawarra Health and Medical Research Institute 1 , Wollongong, NSW 2522 , Australia"},{"name":"Molecular Horizons and School of Chemistry and Molecular Bioscience, University of Wollongong 2 , Wollongong, NSW , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7574-0044","authenticated-orcid":false,"given":"Heath","family":"Ecroyd","sequence":"additional","affiliation":[{"name":"Illawarra Health and Medical Research Institute 1 , Wollongong, NSW 2522 , Australia"},{"name":"Molecular Horizons and School of Chemistry and Molecular Bioscience, University of Wollongong 2 , Wollongong, NSW , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2020,8,5]]},"reference":[{"key":"2024061318561365400_JCS243709C1","doi-asserted-by":"publisher","first-page":"3761","DOI":"10.1128\/MCB.8.9.3761","article-title":"Key features of heat shock regulatory elements","volume":"8","author":"Amin","year":"1988","journal-title":"Mol. 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