{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,11]],"date-time":"2026-01-11T18:03:59Z","timestamp":1768154639679,"version":"3.49.0"},"reference-count":39,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2025,3,14]],"date-time":"2025-03-14T00:00:00Z","timestamp":1741910400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"NIH","award":["R01NS109077"],"award-info":[{"award-number":["R01NS109077"]}]},{"name":"NIH","award":["R01NS134916"],"award-info":[{"award-number":["R01NS134916"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Spinocerebellar ataxia type 1 (SCA1) is a rare autosomal dominant inherited neurodegenerative disease caused by the expansion of glutamine (Q)-encoding CAG repeats in the gene ATAXIN1 (ATXN1). Patients with SCA1 suffer from movement and cognitive deficits and severe cerebellar pathology. Previous studies identified sex differences in disease progression in SCA1 patients, but whether these differences are present in mouse models is unclear. Using a battery of behavioral tests, immunohistochemistry of brain slices, and RNA sequencing, we examined sex differences in motor and cognitive performance, cerebellar pathology, and cerebellar gene expression changes in a recently created conditional knock-in mouse model f-ATXN1146Q expressing human coding regions of ATXN1 with 146 CAG repeats. We found worse motor performance and weight loss accompanied by increased microglial activation and an increase in immune viral response pathways in male f-ATXN1146Q mice.<\/jats:p>","DOI":"10.3390\/ijms26062623","type":"journal-article","created":{"date-parts":[[2025,3,14]],"date-time":"2025-03-14T07:02:16Z","timestamp":1741935736000},"page":"2623","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Sex Differences in a Novel Mouse Model of Spinocerebellar Ataxia Type 1 (SCA1)"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0334-2033","authenticated-orcid":false,"given":"Adem","family":"Selimovic","sequence":"first","affiliation":[{"name":"Department of Neuroscience, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kaelin","family":"Sbrocco","sequence":"additional","affiliation":[{"name":"Graduate Medical Sciences-Anatomy & Neurobiology, Boston University, Boston, MA 02118, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8988-6682","authenticated-orcid":false,"given":"Gourango","family":"Talukdar","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adri","family":"McCall","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8952-6417","authenticated-orcid":false,"given":"Stephen","family":"Gilliat","sequence":"additional","affiliation":[{"name":"Department of Pharmacology, Yale School of Medicine, Yale University, New Haven, CT 06511, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3764-7329","authenticated-orcid":false,"given":"Ying","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"},{"name":"Institute for Translational Neuroscience, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marija","family":"Cvetanovic","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"},{"name":"Institute for Translational Neuroscience, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,3,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.biopsych.2021.01.002","article-title":"Sex Differences in Neurodegeneration: The Role of the Immune System in Humans","volume":"91","author":"Kodama","year":"2022","journal-title":"Biol. 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