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This mutant ataxin-3 protein affects several cellular pathways, leading to neuroinflammation and neuronal death in specific brain regions resulting in severe clinical manifestations. Presently, there is no therapy able to modify the disease progression. Nevertheless, anti-inflammatory pharmacological intervention has been associated with positive outcomes in other neurodegenerative diseases. Thus, the present work aimed at investigating whether ibuprofen treatment would alleviate Machado\u2013Joseph disease.<\/jats:p><jats:p>We found that ibuprofen-treated mouse models presented a significant reduction in the neuroinflammation markers, namely Il1b and TNFa mRNA and IKB-\u03b1 protein phosphorylation levels. Moreover, these mice exhibited neuronal preservation, cerebellar atrophy reduction, smaller mutant ataxin-3 inclusions and motor performance improvement. Additionally, neural cultures of Machado\u2013Joseph disease patients\u2019 induced pluripotent stem cells-derived neural stem cells incubated with ibuprofen showed increased levels of neural progenitors proliferation and synaptic markers such as MSI1, NOTCH1 and SYP. These findings were further confirmed in ibuprofen-treated mice that display increased neural progenitor numbers (Ki67 positive) in the subventricular zone. Furthermore, interestingly, ibuprofen treatment enhanced neurite total length and synaptic function of human neurons. Therefore, our results indicate that ibuprofen reduces neuroinflammation and induces neuroprotection, alleviating Machado\u2013Joseph disease-associated neuropathology and motor impairments. Thus, our findings demonstrate that ibuprofen treatment has the potential to be used as a neuroprotective therapeutic approach in Machado\u2013Joseph disease.<\/jats:p>","DOI":"10.1093\/hmg\/ddz097","type":"journal-article","created":{"date-parts":[[2019,5,23]],"date-time":"2019-05-23T19:16:23Z","timestamp":1558638983000},"page":"3691-3703","source":"Crossref","is-referenced-by-count":32,"title":["Ibuprofen enhances synaptic function and neural progenitors proliferation markers and improves neuropathology and motor coordination in Machado\u2013Joseph disease models"],"prefix":"10.1093","volume":"28","author":[{"given":"Liliana S","family":"Mendon\u00e7a","sequence":"first","affiliation":[{"name":"Vectors and Gene Therapy Group, Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cl\u00e9vio","family":"N\u00f3brega","sequence":"additional","affiliation":[{"name":"Vectors and Gene Therapy Group, Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Tavino","sequence":"additional","affiliation":[{"name":"Vectors and Gene Therapy Group, Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maximilian","family":"Brinkhaus","sequence":"additional","affiliation":[{"name":"Vectors and Gene Therapy Group, Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Matos","sequence":"additional","affiliation":[{"name":"Vectors and Gene Therapy Group, Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Tom\u00e9","sequence":"additional","affiliation":[{"name":"Vectors and Gene Therapy Group, Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ricardo","family":"Moreira","sequence":"additional","affiliation":[{"name":"Vectors and Gene Therapy Group, Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Henriques","sequence":"additional","affiliation":[{"name":"Vectors and Gene Therapy Group, Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian K","family":"Kaspar","sequence":"additional","affiliation":[{"name":"The Research Institute at Nationwide Children\u2019s Hospital, Ohio State University School of Medicine, Columbus, Ohio 43205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5831-3307","authenticated-orcid":false,"given":"Lu\u00eds","family":"Pereira de Almeida","sequence":"additional","affiliation":[{"name":"Vectors and Gene Therapy Group, Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"},{"name":"Faculty of Pharmacy, University of Coimbra, 3000-548 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,5,25]]},"reference":[{"key":"2019122822374108400_ref1","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1038\/ng1194-221","article-title":"CAG expansions in a novel gene for Machado\u2013Joseph disease at chromosome 14q32.1","volume":"8","author":"Kawaguchi","year":"1994","journal-title":"Nat. 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