p150glued功能缺失导致小鼠脊髓运动神经元高尔基体病变  

Loss-of-function of p150glued causes Golgi pathology in spinal motor neurons of mice

作　　者：郑佳音 甄然 杨璇 宋彬彬 彭宇 丁思妍 于佳 Zheng Jiayin;Zhen Ran;Yang Xuan;Song Binbin;Peng Yu;Ding Siyan;Yu Jia(Institute of Geriatrics and Rehabilitation,the Beijing Geriatric Hospital,Beijing 100095,China)

机构地区：[1]北京中医药大学附属北京老年医院老年医学基础研究中心,北京100095

出　　处：《脑与神经疾病杂志》2022年第6期365-369,共5页Journal of Brain and Nervous Diseases

基　　金：国家自然科学基金资助项目(82071438);北京市自然科学基金资助项目(7202077);北京市百千万人才工程资助项目(2017002);北京老年医院老年医学科研专项(2020bjlnyy-自-3,2019bjlnyy-青-4)。

摘　　要：目的 探讨神经元中特异性敲除运动神经元病致病基因DCTN1编码的p150glued蛋白对于小鼠脊髓运动神经元高尔基体结构的影响。方法 通过免疫荧光染色技术标记高尔基体蛋白RCAS1观察对照组(Dctn1^(LoxP/LoxP))小鼠及p150glued条件性敲除(Dctn1^(LoxP/LoxP);Thy1-Cre)小鼠脊髓前角运动神经元中高尔基体分布和结构的变化情况。结果 与Dctn1^(LoxP/LoxP)小鼠相比,6月龄Dctn1^(LoxP/LoxP);Thy1-Cre小鼠脊髓运动神经元中RCAS1阳性的高尔基体分布明显离散。18个月龄时,Dctn1^(LoxP/LoxP);Thy1-Cre小鼠脊髓运动神经元中RCAS1阳性的高尔基体分布更加离散,且连贯膜结构明显碎裂。结论 神经元中p150glued功能缺失可以引发小鼠脊髓运动神经元中早期高尔基体分散及晚期高尔基体碎裂。Objective To investigate the effects of neuron-specific loss-of-function of p150 glued encoded by motor neuron disease related Dctn 1 gene on the Golgi apparatus in spinal cord motor neurons of mice.Methods The Golgi structure was observed by using Golgi protein RCAS1 immunofluorescence staining in Dctn1^(LoxP/LoxP)and Dctn1^(LoxP/LoxP);Thyl-Cre mice.Results Compared to Dctn1^(LoxP/LoxP) mice,the Golgi dispersion at 6 months and fragmentation at 18 months were observed in the spinal cord motor neurons of Dctn1^(LoxP/LoxP);Thyl-Cre mice.Conclusion Loss-of-function of p150 glued in mice spinal motor neurons causes progressive pathology of Golgi apparatus.

关 键 词：p150glued 运动神经元病 高尔基体 脊髓运动神经元 

分 类 号：R744[医药卫生—神经病学与精神病学]