{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T02:41:24Z","timestamp":1772160084055,"version":"3.50.1"},"reference-count":27,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T00:00:00Z","timestamp":1761436800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,10,26]],"date-time":"2025-10-26T00:00:00Z","timestamp":1761436800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100004829","name":"Science and Technology Department of Sichuan Province","doi-asserted-by":"publisher","award":["2019YFS0214"],"award-info":[{"award-number":["2019YFS0214"]}],"id":[{"id":"10.13039\/501100004829","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Genetics"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by CAG repeat expansion in the HTT gene. Existing toxin\u2010induced and genetic models provide important insights, but none fully replicate the progressive pathology of HD. An AAV9\u2010mediated striatal mouse model expressing mutant HTT with 82 CAG repeats was established to reproduce hallmark neuropathological changes and behavioral deficits. Male C57BL\/6 mice received bilateral intrastriatal injections of AAV9\u2010HTT\u201082Q or control AAV9\u2010GFP. Behavioral performance was assessed by rotarod, balance beam, open field, and Y\u2010maze tests. Neuropathology was examined with HE\/Nissl staining, TUNEL assay, and immunofluorescence for mHTT, DARPP\u201032, GFAP, and Iba1. AAV9\u201082Q mice exhibited progressive motor coordination deficits on the rotarod from Week 4 and impaired beam traversal from Week 18. Open field testing revealed persistent hyperactivity from Week 8, while anxiety\u2010like and cognitive measures showed only mild, non\u2010significant trends. Histological analysis demonstrated extensive mHTT aggregation in the striatum, accompanied by neuronal pyknosis, vacuolization, and significant loss of Nissl\u2010positive neurons. TUNEL staining confirmed increased apoptosis. Immunofluorescence further revealed selective reduction of DARPP\u201032+ medium spiny neurons, along with marked astrogliosis and microgliosis, indicating robust neurodegeneration and inflammatory responses. The AAV9\u201082Q model induces adult\u2010onset, progressive HD\u2010like pathology with early motor impairments, neuronal loss, and glial activation. It complements existing models and provides a reproducible platform for mechanistic studies and preclinical therapeutic evaluation.<\/jats:p>","DOI":"10.1111\/cge.70076","type":"journal-article","created":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T05:45:06Z","timestamp":1761543906000},"page":"521-528","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["<scp>AAV9<\/scp>\n                    \u2010Mediated Intrastriatal Delivery of Mutant\n                    <scp>HTT<\/scp>\n                    With 82\n                    <scp>CAG<\/scp>\n                    Repeats Induces Huntington's Disease\u2010Like Pathology and Behavioral Deficits in Mice"],"prefix":"10.1111","volume":"109","author":[{"given":"Lin","family":"Huang","sequence":"first","affiliation":[{"name":"Department of Neurology Zigong First People's Hospital  Zigong Sichuan 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