C型Niemann-Pick病小鼠小脑Purkinje细胞的电生理特性研究

摘要

Objective: lo investigate the electrophysiological properties and calcium currents of Purkinje cells in mouse cerebellum of NPC and wild type mice. Methods: Fresh cerebel-lar slices were prepared and whole-cell patch clamp was applied to record the electrophysiological properties of Purkinje cells. The I-V curve of calcium channels and calcium current densities at different time points were establised. Results: No significant differences were found in the electrophysiological characteristics and calcium currents of Purkinje cells between NPC and wild type mice. Conclusion: In NPC mouse model, pathological cholesterol metabolism fails to modify the electrophysiological characteristics of Purkinje cells. It is reasonable to speculate that electrophysiological properties and calcium currents of Purkinje cells don't play an important role in NPC pathology.%目的:比较C型Niemann-Pick病(NPC)小鼠与野生型小鼠小脑Purkinje细胞膜电生理特性和钙电流的差异.方法:制备新鲜小鼠小脑脑片,利用组织膜片钳全细胞记录法记录出生后20～24 d(P20～24)、出生后5周(P5w)小鼠Purkinje 细胞膜的电生理特性、钙通道的I-V曲线和钙电流密度.结果:不同年龄段的NPC小鼠与野生型小鼠相比,Purkinje 细胞膜的电生理特性及钙电流变化无统计学差异.结论:NPC小鼠脂质代谢异常并未影响小脑Purkinje 细胞的电生理特性,推测Purkinje细胞电生理特性改变在NPC小鼠Purkinje细胞变性机制中起次要作用.