{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T11:42:19Z","timestamp":1790854939656,"version":"4.1.0"},"reference-count":55,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2008,2,21]],"date-time":"2008-02-21T00:00:00Z","timestamp":1203552000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2008,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Huntington's disease (HD) is a neurodegenerative disorder caused by an elongation of CAG repeats in the <jats:italic>HD<\/jats:italic> gene, which encodes a mutant copy of huntingtin with an expanded polyglutatmine repeat. Individuals who are affected by the disease suffer from motor, cognitive, and emotional impairments. Levels of certain striatal\u2010enriched mRNAs decrease in both HD patients and transgenic HD mice prior to the development of motor symptoms and neuronal cell death. Ciliary neurotrophic factor (CNTF) has been shown to protect neurons against chemically induced toxic insults in vitro and in vivo. To test the hypothesis that CNTF might protect neurons from the negative effects of the mutant huntingtin protein in vivo, CNTF was continuously expressed following transduction of the striatum by recombinant adeno\u2010associated viral vectors (rAAV2). Wild\u2010type and R6\/1 HD transgenic (R6\/1) mice that received bilateral or unilateral intrastriatal injections of rAAV2\u2010CNTF experienced weight loss. The CNTF\u2010treated R6\/1 HD transgenic mice experienced motor impairments at an earlier age than expected compared with age\u2010matched control R6\/1 HD transgenic animals. CNTF also caused abnormal behavior in WT mice. In addition to behavioral impairments, in situ hybridization showed that, in both WT and R6\/1 mice, CNTF expression caused a significant decrease in the levels of striatal\u2010enriched transcripts. Overall, continuous expression of striatal CNTF at the dose mediated by the expression cassette used in this study was detrimental to HD and wild\u2010type mice. \u00a9 2008 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jnr.21636","type":"journal-article","created":{"date-parts":[[2008,2,22]],"date-time":"2008-02-22T00:46:32Z","timestamp":1203641192000},"page":"1748-1757","source":"Crossref","is-referenced-by-count":16,"title":["Sustained striatal ciliary neurotrophic factor expression negatively affects behavior and gene expression in normal and R6\/1 mice"],"prefix":"10.1002","volume":"86","author":[{"given":"Eileen 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