Department of Neuroscience, Maastricht University Medical Center, 6229 ER Maastricht, The Netherlands.
Neural Plast. 2012;2012:682712. doi: 10.1155/2012/682712. Epub 2012 Jul 18.
Huntington's disease (HD) is a fatal inherited disorder leading to selective neurodegeneration and neuropsychiatric symptoms. Currently, there is no treatment to slow down or to stop the disease. There is also no therapy to effectively reduce the symptoms. In the investigation of novel therapies, different animal models of Huntington's disease, varying from insects to nonhuman primates, have been created and used. Few years ago, the first transgenic rat model of HD, carrying a truncated huntingtin cDNA fragment with 51 CAG repeats under control of the native rat huntingtin promoter, was introduced. We have been using this animal model in our research and review here our experience with the behavioural, neurophysiological, and histopathological phenotype of the transgenic Huntington's disease rats with relevant literature.
亨廷顿病(HD)是一种致命的遗传性疾病,导致选择性神经退行性变和神经精神症状。目前,尚无治疗方法可以减缓或阻止疾病的发展。也没有有效的治疗方法可以减轻症状。在新型疗法的研究中,已经创建并使用了不同的亨廷顿病动物模型,从昆虫到非人类灵长类动物不等。几年前,携带控制天然大鼠亨廷顿启动子的截断亨廷顿 cDNA 片段的第一个转 Huntington's 病大鼠模型被引入。我们一直在使用这种动物模型进行研究,并在此回顾了我们使用该模型的经验,以及相关文献中关于转 Huntington's 病大鼠的行为、神经生理学和组织病理学表型。