{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T21:30:55Z","timestamp":1785879055010,"version":"3.56.0"},"reference-count":62,"publisher":"The Company of Biologists","issue":"10","license":[{"start":{"date-parts":[[2021,10,11]],"date-time":"2021-10-11T00:00:00Z","timestamp":1633910400000},"content-version":"vor","delay-in-days":10,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"MJD Foundation"},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"crossref","award":["1069235"],"award-info":[{"award-number":["1069235"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"crossref","award":["1146750"],"award-info":[{"award-number":["1146750"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"crossref"}]},{"name":"The Snow Foundation"},{"name":"Motor Neuron Disease Research Australia"},{"DOI":"10.13039\/501100001230","name":"Macquarie University","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100001230","id-type":"DOI","asserted-by":"crossref"}]},{"name":"SCA Network of Sweden"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,10,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Spinocerebellar ataxia 3 (SCA3, also known as Machado\u2013Joseph disease) is a neurodegenerative disease caused by inheritance of a CAG repeat expansion within the ATXN3 gene, resulting in polyglutamine (polyQ) repeat expansion within the ataxin-3 protein. In this study, we have identified protein aggregates in both neuronal-like (SHSY5Y) cells and transgenic zebrafish expressing human ataxin-3 with expanded polyQ. We have adapted a previously reported flow cytometry methodology named flow cytometric analysis of inclusions and trafficking, allowing rapid quantification of detergent insoluble forms of ataxin-3 fused to a GFP in SHSY5Y cells and cells dissociated from the zebrafish larvae. Flow cytometric analysis revealed an increased number of detergent-insoluble ataxin-3 particles per nuclei in cells and in zebrafish expressing polyQ-expanded ataxin-3 compared to those expressing wild-type human ataxin-3. Treatment with compounds known to modulate autophagic activity altered the number of detergent-insoluble ataxin-3 particles in cells and zebrafish expressing mutant human ataxin-3. We conclude that flow cytometry can be harnessed to rapidly count ataxin-3 aggregates, both in vitro and in vivo, and can be used to compare potential therapies targeting protein aggregates.<\/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\/dmm.049023","type":"journal-article","created":{"date-parts":[[2021,9,2]],"date-time":"2021-09-02T10:25:54Z","timestamp":1630578354000},"source":"Crossref","is-referenced-by-count":10,"title":["Flow cytometry allows rapid detection of protein aggregates in cellular and zebrafish models of spinocerebellar ataxia 3"],"prefix":"10.1242","volume":"14","author":[{"given":"Katherine J.","family":"Robinson","sequence":"first","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Department of Biomedical Sciences, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madelaine C.","family":"Tym","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Department of Biomedical Sciences, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alison","family":"Hogan","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Department of Biomedical Sciences, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxinne","family":"Watchon","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Department of Biomedical Sciences, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristy C.","family":"Yuan","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Department of Biomedical Sciences, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stuart K.","family":"Plenderleith","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Department of Biomedical Sciences, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily K.","family":"Don","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Department of Biomedical Sciences, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6860-9192","authenticated-orcid":false,"given":"Angela S.","family":"Laird","sequence":"additional","affiliation":[{"name":"Centre for Motor Neuron Disease Research, Department of Biomedical Sciences, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2021,10,11]]},"reference":[{"key":"2021101811191722400_DMM049023C1","doi-asserted-by":"publisher","first-page":"882","DOI":"10.1212\/WNL.51.3.882","article-title":"CAG repeat number correlates with the rate of brainstem and cerebellar atrophy in Machado-Joseph disease","volume":"51","author":"Abe","year":"1998","journal-title":"Neurology"},{"key":"2021101811191722400_DMM049023C2","doi-asserted-by":"publisher","first-page":"bio036475","DOI":"10.1242\/bio.036475","article-title":"Neuronal cell culture from transgenic zebrafish models of neurodegenerative disease","volume":"7","author":"Acosta","year":"2018","journal-title":"Biol. 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