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Here, we used a mouse model to test a possible connection between SCA and Ronin (Thap11), a polyglutamine-containing transcriptional regulator encoded in a region of human chromosome 16q22.1 that has been genetically linked to SCA type 4. We report that transgenic expression of Ronin in mouse cerebellar Purkinje cells leads to detrimental loss of these cells and the development of severe ataxia as early as 10 weeks after birth. Mechanistically, we find that several SCA-causing genes harbor Ronin DNA-binding motifs and are transcriptionally deregulated in transgenic animals. In addition, ectopic expression of Ronin in embryonic stem cells significantly increases the protein level of Ataxin-1, the protein encoded by Atxn1, alterations of which cause SCA type 1. This increase is also seen in the cerebellum of transgenic animals, although the latter was not statistically significant. Hence, our data provide evidence for a link between Ronin and SCAs, and suggest that Ronin may be involved in the development of other neurodegenerative diseases.<\/jats:p>","DOI":"10.1242\/dmm.044834","type":"journal-article","created":{"date-parts":[[2021,6,24]],"date-time":"2021-06-24T14:55:36Z","timestamp":1624546536000},"source":"Crossref","is-referenced-by-count":5,"title":["Ronin overexpression induces cerebellar degeneration in a mouse model of ataxia"],"prefix":"10.1242","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9271-2456","authenticated-orcid":false,"given":"Thomas P.","family":"Zwaka","sequence":"first","affiliation":[{"name":"Department for Cell, Regenerative and Developmental Biology, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Black Family Stem Cell Institute, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Huffington Center for Cell-Based Research in Parkinson's Disease, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marta","family":"Skowronska","sequence":"additional","affiliation":[{"name":"Department for Cell, Regenerative and Developmental Biology, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Black Family Stem Cell Institute, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Huffington Center for Cell-Based Research in Parkinson's Disease, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0441-8354","authenticated-orcid":false,"given":"Ronald","family":"Richman","sequence":"additional","affiliation":[{"name":"Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA"},{"name":"Jan and Dan Duncan Neurological Research Institute at Texas Children's Hospital, Houston, TX 77030, USA"},{"name":"Howard Hughes Medical Institute, Baylor College of Medicine, Houston, TX 77030, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1360-3260","authenticated-orcid":false,"given":"Marion","family":"Dejosez","sequence":"additional","affiliation":[{"name":"Department for Cell, Regenerative and Developmental Biology, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Black Family Stem Cell Institute, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"},{"name":"Huffington Center for Cell-Based Research in Parkinson's Disease, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","published-online":{"date-parts":[[2021,6,24]]},"reference":[{"key":"2021062414553042500_DMM044834C1","doi-asserted-by":"publisher","first-page":"1440","DOI":"10.1523\/JNEUROSCI.16-04-01440.1996","article-title":"Glutamate receptors mediate dynamic regulation of nitric oxide synthase expression in cerebellar granule cells","volume":"16","author":"Baader","year":"1996","journal-title":"J. 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