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目的观察细胞周期依赖性蛋白激酶特异性抑制剂Roscovitine对C型尼曼-皮克病(NPC)小鼠(npc-/-)的治疗作用。方法采用72、144、300和600nmol/d的Roscovitine对5周龄的npc-/-小鼠(n=6~10)进行2周侧脑室灌注,以DMSO灌注的同龄npc-/-小鼠(n=6~10)为对照;采用免疫组织化学染色、H&E染色和小鼠衣架悬挂试验来进行检测。结果Roscovitine能显著减少球状轴突的数量,延缓浦肯野细胞的死亡并改善npc-/-小鼠的运动功能。结论Roscovitine对NPC的神经系统变性有一定的保护作用。
Objective To investigate the therapeutic effect of roscovitine, a specific inhibitor of cell cycle-dependent protein kinase, on N-type Niemann-Pick disease (NPC) mice (npc - / -). Methods Five-week-old npc - / - mice (n = 6 ~ 10) were infused with Roscovitine at 72, 144, 300 and 600 nmol / d for 2 weeks. n = 6 ~ 10) were used as control; immunohistochemical staining, H & E staining and hanger suspension test in mice were performed. Results Roscovitine significantly reduced the number of globular axons, slowed the death of Purkinje cells and improved the motor function of npc - / - mice. Conclusion Roscovitine has some protective effects on the NPC degeneration.