{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T02:54:06Z","timestamp":1790045646267,"version":"4.0.1"},"reference-count":43,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2007,12,10]],"date-time":"2007-12-10T00:00:00Z","timestamp":1197244800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Gene Medicine"],"published-print":{"date-parts":[[2008,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Huntington's disease (HD) is an inherited autosomal dominant neurodegenerative disease caused by the expansion of a CAG trinucleotide repeat in exon 1 of the huntingtin (htt) gene. Vector\u2010mediated delivery of N\u2010terminal fragments of mutant htt has been used to study htt function <jats:italic>in vitro<\/jats:italic> and to establish HD models in rats. Due to the large size of the htt cDNA vector\u2010mediated delivery of full\u2010length htt has not been achieved so far.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>High\u2010capacity adenoviral (HC\u2010Ad) vectors were generated expressing mutant and wild\u2010type versions of N\u2010terminal truncated and full\u2010length htt either <jats:italic>in vitro<\/jats:italic> in primary neuronal cells or in the striatum of mice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p><jats:italic>In vitro<\/jats:italic> these vectors were used for transduction of primary neuronal cells isolated from E17 mouse embryos. Expression of mutant htt resulted in the formation of htt inclusions, a surrogate marker of the HD pathology. Kinetics of generation and localization of htt inclusions differed between truncated and full\u2010length htt carrying identical mutations. Following injection into the striatum vector\u2010mediated expression of mutant truncated htt led to prominent accumulation of htt inclusions in cell nuclei, while inclusions formed upon expression of mutant full\u2010length htt localized to the cytoplasm.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These results indicate that HC\u2010Ad vector\u2010mediated <jats:italic>in vitro<\/jats:italic> and <jats:italic>in vivo<\/jats:italic> delivery of truncated and full\u2010length mutant htt results in prominent inclusion formation in neuronal cells but in different cell compartments. These vectors will be useful tools for studying HD and may be used to generate large animal HD models. Copyright \u00a9 2007 John Wiley &amp; Sons, Ltd.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jgm.1150","type":"journal-article","created":{"date-parts":[[2007,12,10]],"date-time":"2007-12-10T10:17:11Z","timestamp":1197281831000},"page":"269-279","source":"Crossref","is-referenced-by-count":10,"title":["Inducing huntingtin inclusion formation in primary neuronal cell culture and <i>in vivo<\/i> by high\u2010capacity adenoviral vectors expressing truncated and full\u2010length huntingtin with polyglutamine expansion"],"prefix":"10.1002","volume":"10","author":[{"given":"Bin","family":"Huang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes","family":"Schiefer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Sass","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph 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