{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T02:23:55Z","timestamp":1791685435072,"version":"4.3.4"},"reference-count":207,"publisher":"Portland Press Ltd.","issue":"6","license":[{"start":{"date-parts":[[2024,3,18]],"date-time":"2024-03-18T00:00:00Z","timestamp":1710720000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,3,20]]},"abstract":"<jats:p>Machado-Joseph disease (MJD) is a devastating and incurable neurodegenerative disease characterised by progressive ataxia, difficulty speaking and swallowing. Consequently, affected individuals ultimately become wheelchair dependent, require constant care, and face a shortened life expectancy. The monogenic cause of MJD is expansion of a trinucleotide (CAG) repeat region within the ATXN3 gene, which results in polyglutamine (polyQ) expansion within the resultant ataxin-3 protein. While it is well established that the ataxin-3 protein functions as a deubiquitinating (DUB) enzyme and is therefore critically involved in proteostasis, several unanswered questions remain regarding the impact of polyQ expansion in ataxin-3 on its DUB function. Here we review the current literature surrounding ataxin-3's DUB function, its DUB targets, and what is known regarding the impact of polyQ expansion on ataxin-3's DUB function. We also consider the potential neuroprotective effects of ataxin-3's DUB function, and the intersection of ataxin-3's role as a DUB enzyme and regulator of gene transcription. Ataxin-3 is the principal pathogenic protein in MJD and also appears to be involved in cancer. As aberrant deubiquitination has been linked to both neurodegeneration and cancer, a comprehensive understanding of ataxin-3's DUB function is important for elucidating potential therapeutic targets in these complex conditions. In this review, we aim to consolidate knowledge of ataxin-3 as a DUB and unveil areas for future research to aid therapeutic targeting of ataxin-3's DUB function for the treatment of MJD and other diseases.<\/jats:p>","DOI":"10.1042\/bcj20240017","type":"journal-article","created":{"date-parts":[[2024,3,18]],"date-time":"2024-03-18T11:35:55Z","timestamp":1710761755000},"page":"461-480","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["The deubiquitinase function of ataxin-3 and its role in the pathogenesis of Machado-Joseph disease and other diseases"],"prefix":"10.1042","volume":"481","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-2650-4441","authenticated-orcid":false,"given":"Anastasiya","family":"Potapenko","sequence":"first","affiliation":[{"name":"Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7996-1386","authenticated-orcid":false,"given":"Jennilee M.","family":"Davidson","sequence":"additional","affiliation":[{"name":"Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5156-0567","authenticated-orcid":false,"given":"Albert","family":"Lee","sequence":"additional","affiliation":[{"name":"Motor Neuron Disease Research Centre, Macquarie Medical School, 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":"Motor Neuron Disease Research Centre, Macquarie Medical School, Faculty of Medicine, Health and Human Sciences, Macquarie University, Sydney, NSW 2109, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2024,3,18]]},"reference":[{"key":"2024031811354205700_BCJ-2024-0017C1","doi-asserted-by":"publisher","first-page":"300","DOI":"10.1038\/ng0793-300","article-title":"The gene for Machado-Joseph disease maps to human chromosome 14q","volume":"4","year":"1993","journal-title":"Nat. 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