{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T22:30:10Z","timestamp":1775082610096,"version":"3.50.1"},"reference-count":64,"publisher":"The Company of Biologists","license":[{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"am","delay-in-days":1598,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0"}],"funder":[{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Background: Polyglutamine expansion diseases are a group of hereditary neurodegenerative disorders that develop when a CAG repeat in the causative genes are unstably expanded above a certain threshold. The expansion of trinucleotide CAG repeats cause hereditary adult-onset neurodegenerative disorders such as Huntington's disease, dentatorubral-pallidoluysian atrophy, spinobulbar muscular atrophy and multiple forms of spinocerebellar ataxia (SCA). The most common dominantly inherited spinocerebellar ataxia is the type 3 (SCA3) also known as Machado-Joseph disease (MJD), is an autosomal dominant, progressive neurological disorder. The gene causatively associated with MJD is ATXN3. Recent studies have shown that this gene modulates endoplasmic reticulum (ER) stress.<\/jats:p>\n               <jats:p>Results: We generated transgenic C. elegans strains expressing human ATXN3 genes in motor neurons, and animals expressing mutant ATXN3-CAG89 alleles showed decreased lifespan, impaired movement, and rates of neurodegeneration greater than wild type ATXN3-CAG10 controls. We tested three neuroprotective compounds (methylene blue, guanabenz and salubrinal) believed to modulate ER stress and observed that these molecules rescued ATXN3-CAG89 phenotypes. Furthermore, these compounds required specific branches of ER unfolded protein response (UPRER), reduced global ER and oxidative stress, as well as polyglutamine aggregation.<\/jats:p>\n               <jats:p>Conclusions: We introduce novel C. elegans models for MJD based on the expression of full-length ATXN3 in a limited number of neurons. Using these models, we discovered that chemical modulation of the UPRER reduced neurodegeneration and warrants investigation in mammalian models of MJD.<\/jats:p>","DOI":"10.1242\/dmm.029736","type":"journal-article","created":{"date-parts":[[2017,10,24]],"date-time":"2017-10-24T00:11:04Z","timestamp":1508803864000},"source":"Crossref","is-referenced-by-count":14,"title":["Rescue of ATXN3 neuronal toxicity in <i>C. elegans<\/i> by chemical modification of ER stress"],"prefix":"10.1242","author":[{"given":"Yasmin","family":"Fardghassemi","sequence":"first","affiliation":[{"name":"CRCHUM and Department of Biochemistry, University of Montreal, Montreal, Quebec, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arnaud","family":"Tauffenberger","sequence":"additional","affiliation":[{"name":"CRCHUM and Department of Pathology and Cell Biology, University of Montreal, Montreal, Quebec, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah","family":"Gosselin","sequence":"additional","affiliation":[{"name":"CRCHUM and Department of Neuroscience, University of Montreal, Montreal, Quebec, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. 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