FMRP影响小鼠小脑皮质神经元发育与迁移的实验研究

 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。

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

Objective To investigate the effects of fragile X mental retardation protein（FMRP）on the development and migration of cere-
bellar neurons in mouse model. Methods Plasmids containing FMRPmutant-EGFP or EGFP were established and transfected into the later-
al ventricle of the embryo mouse. Fragile X syndrome（FXS）genotype of the mouse model was identified. Nissl staining and immunofluores-
cence staining were conducted to assess the changes in neuron development and migration. Results In the experimental group，Nissl stain-
ing 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 accumu-
lated in the ventricular zone. Conclusion Cerebellar neurons showed abnormal formation and migration with the absence of FMRP.