Department of Physiology, Development and Neuroscience, University of Cambridge, Cambridge, UK.
J Huntingtons Dis. 2023;12(2):133-148. doi: 10.3233/JHD-230574.
Sleep and circadian disruption affects most individuals with Huntington's disease (HD) at some stage in their lives. Sleep and circadian dysregulation are also present in many mouse and the sheep models of HD. Here I review evidence for sleep and/or circadian dysfunction in HD transgenic animal models and discuss two key questions: 1) How relevant are such findings to people with HD, and 2) Whether or not therapeutic interventions that ameliorate deficits in animal models of HD might translate to meaningful therapies for people with HD.
睡眠和昼夜节律紊乱在亨廷顿病(HD)患者的某些阶段影响大多数人。许多 HD 的小鼠和绵羊模型也存在睡眠和昼夜节律失调。在这里,我回顾了 HD 转基因动物模型中睡眠和/或昼夜节律功能障碍的证据,并讨论了两个关键问题:1)这些发现与人 HD 有何关联,2)是否可以将改善 HD 动物模型中缺陷的治疗干预转化为对人 HD 有意义的治疗方法。