FMRP影响小鼠小脑皮质神经元发育与迁移的实验研究  

Effects of Fragile Mental Retardation Protein on Cerebellar Neuron Development and Migration in Mouse Model

作　　者：孙晓红[1] 董玉霞[1] 冯昱[2] 宋卫科[1] 何悦[1] 何志义[2] 

机构地区：[1]中国医科大学附属第四医院神经内科,沈阳110032 [2]中国医科大学附属第一医院神经内科,沈阳110001

出　　处：《中国医科大学学报》2010年第6期420-421,共2页Journal of China Medical University

基　　金：国家自然科学基金资助项目(30700220);中国博士后科研基金项目(200801398;20080431165)

摘　　要：目的观察脆性X智力障碍蛋白(FMRP)对小鼠小脑皮质神经元发育与迁移的影响。方法构建FMRPmutant-pEGFP与pEGFP质粒;将质粒转染到胚胎小鼠脑内并鉴定转基因脆性X综合征(FXS)小鼠类型;Nissl染色及免疫荧光组织化学染色观察小脑皮质神经核团发育及神经元迁移的形态学变化。结果 Nissl染色显示实验组小脑深部核团明显缩小且被白质分隔;Purkinje细胞呈现非极性紊乱排列,部分细胞滞留于内颗粒细胞层;免疫荧光染色显示实验组小脑单极刷状缘细胞迁移路径改变,细胞在侧脑室区聚集。结论 FMRP缺乏导致小鼠小脑皮质神经元发育与迁移异常。Objective To investigate the effects of fragile X mental retardation protein（FMRP）on the development and migration of cerebellar neurons in mouse model.Methods Plasmids containing FMRPmutant-EGFP or EGFP were established and transfected into the lateral ventricle of the embryo mouse.Fragile X syndrome（FXS）genotype of the mouse model was identified.Nissl staining and immunofluorescence staining were conducted to assess the changes in neuron development and migration.Results In the experimental group,Nissl staining showed that the deep cerebellar neuclei contracted and divided by white matter,and the non-polarized Purkinje cells retained in internal granular layer;while immunofluorescence staining showed that Tbr2-positive unipolar brush cells changed the migration pathway and accumulated in the ventricular zone.Conclusion Cerebellar neurons showed abnormal formation and migration with the absence of FMRP.

关 键 词：脆性X智力障碍蛋白 小脑深部核团 单极刷状缘细胞 浦肯耶细胞 

分 类 号：R341.32[医药卫生—基础医学]