携带cdc2-siRNA重组腺相关病毒对C型尼曼-皮克病小鼠神经元细胞骨架损伤的保护作用  

Protective effect of recombinant adeno-associated virus encoding cdc2-siRNA on the injured neuronal cytoskeleton of Niemann-Pick disease type C mice

作　　者：魏佳军[1] 张旻[2] 张苏明[2] 降风[2] 

机构地区：[1]武汉大学人民医院神经内科,430060 [2]华中科技大学同济医学院附属同济医院神经内科

出　　处：《卒中与神经疾病》2009年第4期225-228,共4页Stroke and Nervous Diseases

基　　金：国家自然科学基金资助项目(No.30400141)

摘　　要：目的评估经小脑注射携带cdc2-siRNA的重组腺相关病毒(rAAV)对C型尼曼-皮克病(NPC)小鼠神经元细胞骨架损伤是否具有保护作用。方法将携带cdc2-siRNA重组腺相关病毒(rAAV)注射入2周龄npc-/-小鼠的小脑,采用免疫组织化学及免疫印迹方法观察该病毒对npc-/-小鼠神经元细胞骨架损伤的保护作用。结果(1)携带cdc2-siRNA的rAAV明显减少npc-/-小鼠轴突球状体的数量;(2)携带cdc2-siRNA的rAAV有效抑制磷酸化的神经丝(由SMI31识别)及磷酸化的Tau蛋白(由PHF-1识别)的表达。结论小脑注射携带cdc2-siRNA的rAAV对npc-/-小鼠神经元细胞骨架损害具有保护作用。Objective To assess the protective effect of cerebellar injection of recombinant adeno-associated virus （rAAV） encoding cde2 siRNA on the injured neuronal cytoskeleton of Niemann-Pick disease type C （NPC） mice. Methods RAAV encoding cdc2-siRNA was injected into the cerebellum of 2 weeks old npc-f- mice. The protective effect of the rAAV on the injured neuronal cytoskeleton in the treated mice was observed by immunohistology and immunoblotting technologies. Results （1）RAAV encoding cdc2-siRNA remarkably reduced the number of axonal spheroids in the treated mice. （2）rAAV encoding cdc2-siRNA markedly inhibited the expressions of phospho-neurofilament （recognized by SMI31）and phospho-Tau （recognized by PHF- 1 ）. Conclusions The rAAV encoding cdc2-siRNA injected into the cerebellum of npc-f- mice can protect the injured neuronal cytoskeleton of npc-f- mice.

关 键 词：轴突球状体 细胞分裂周期2 C型尼曼-皮克病 

分 类 号：R741[医药卫生—神经病学与精神病学]