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Genetically modified animals should provide a more accurate representation of human HD, as they emulate the genetic basis of its etiology.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In this study, we aimed to assess the therapeutic potential of a human striatal neural stem cell line (STROC05) implanted in the R6\/2 transgenic mouse model of HD. As DARPP-32 GABAergic output neurons are predominately lost in HD, STROC05 cells were also pre-differentiated using purmorphamine, a hedgehog agonist, to yield a greater number of DARPP-32 cells. A bilateral injection of 4.5x10<jats:sup>5<\/jats:sup>cells of either undifferentiated or pre-differentiated DARPP-32 cells, however, did not affect outcome compared to a vehicle control injection. Both survival and neuronal differentiation remained poor with a mean of only 161 and 81 cells surviving in the undifferentiated and differentiated conditions respectively. Only a few cells expressed the neuronal marker Fox3.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Although the rapid brain atrophy and short life-span of the R6\/2 model constitute adverse conditions to detect potentially delayed treatment effects, significant technical hurdles, such as poor cell survival and differentiation, were also sub-optimal. Further consideration of these aspects is therefore needed in more enduring transgenic HD models to provide a definite assessment of this cell line\u2019s therapeutic relevance. However, a combination of treatments is likely needed to affect outcome in transgenic models of HD.<\/jats:p><\/jats:sec>","DOI":"10.1186\/1471-2202-13-97","type":"journal-article","created":{"date-parts":[[2012,8,9]],"date-time":"2012-08-09T14:22:23Z","timestamp":1344522143000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["Implantation of undifferentiated and pre-differentiated human neural stem cells in the R6\/2 transgenic mouse model of Huntington\u2019s disease"],"prefix":"10.1186","volume":"13","author":[{"given":"Gehan","family":"El-Akabawy","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivan","family":"Rattray","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saga M","family":"Johansson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Gale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gillian","family":"Bates","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Modo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2012,8,9]]},"reference":[{"issue":"3","key":"2790_CR1","first-page":"311","volume":"84","author":"GO Abdulrahman Jr","year":"2011","unstructured":"Abdulrahman GO: Therapeutic Advances in the Management of Huntington's Disease. 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