Abnormal motor behavior and vestibular dysfunction in the stargazer mouse mutant
Abnormal motor behavior and vestibular dysfunction in the stargazer mouse mutant
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DOI：
10.1016/j.neuroscience.2004.05.052
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发表时间：
2004-01-01
期刊：
影响因子：
3.3
通讯作者：
Jinnah, HA
中科院分区：
文献类型：
--
作者：
Khan, Z;Carey, J;Jinnah, HA
在stargazer突变小鼠中，编码stargazin基因的突变导致缺失性癫痫、小脑性共济失调和特征性异常运动综合征。当前研究的主要目的是表征异常运动行为的性质和来源。由于占星者运动综合症与其他前庭功能障碍的啮齿动物相似，因此将运动异常与使用已知毒素治疗的正常小鼠进行了比较，这些毒素会损害前庭系统。定量开放场评估显示，观星小鼠表现出与毒素诱导的前庭神经病变小鼠非常相似的运动综合征。然而，观星者小鼠也表现出一些额外的行为，比如共济失调的步态和持续的颈部伸肌运动。此外，在大多数运动功能的标准测试中，观星小鼠比毒素诱导的前庭病变小鼠表现更差。在前庭功能敏感的四项行为测试中，运动功能也受到损害。由于前庭和听觉系统之间的密切联系，听觉功能的测试也被采用。观察星突变体表现出相对正常的听觉脑干诱发反应，但没有明显的声惊吓反射。在光镜和电镜下对前庭感觉上皮的组织学检查证实了占星突变体的异常存在。这些结果暗示了一种以前未被认识到的观星蛋白在前庭系统正常功能中的作用，并表明一些（但不是全部）观星小鼠的异常运动综合征可归因于前庭功能障碍。(c) 2004 ibro。Elsevier Ltd.出版。版权所有。
In stargazer mutant mice, a mutation in the gene encoding stargazin results in absence epilepsy, cerebellar ataxia, and a characteristic abnormal motor syndrome. The main goal of the current studies was to characterize the nature and source of the abnormal motor behavior. Because the stargazer motor syndrome resembles that of other rodents with vestibular dysfunction, the motor abnormalities were compared with those of normal mice treated with toxins known to damage the vestibular system. Quantitative open field assessments revealed that the stargazer mice display a motor syndrome very similar to that exhibited by mice with toxin-induced vestibulopathy. However, stargazer mice also displayed several additional behaviors, such as ataxic gait and sustained extensor movements of the neck. In addition, stargazer mice performed worse than mice with toxin-induced vestibulopathy in most standard tests of motor function. Motor function was also impaired on each of four behavioral tests sensitive to vestibular function. Because of the close associations between the vestibular and auditory systems, tests of auditory function were also employed. The stargazer mutants exhibited relatively normal auditory brainstem evoked responses but no apparent acoustic startle reflex. Histological examination of vestibular sensory epithelium at the light and electron microscopic levels confirmed the existence of abnormalities in the stargazer mutants. These results imply a previously unrecognized role for stargazin in the normal functions of the vestibular system and indicate that some, but not all, of the abnormal motor syndrome of stargazer mice can be attributed to vestibular dysfunction. (C) 2004 IBRO. Published by Elsevier Ltd. All rights reserved.