Roscovitine对C型尼曼-皮克病小鼠的神经保护作用  

The neuroprotective effects of roscovitine in Niemann-Pick Disease Type C mice

作　　者：张旻 卜碧涛[1] 王伟[1] Inez Vincent 

机构地区：[1]华中科技大学同济医学院附属同济医院神经内科,武汉430030 [2]美国华盛顿大学病理系

出　　处：《卒中与神经疾病》2005年第6期326-328,333,共4页Stroke and Nervous Diseases

基　　金：国家自然科学基金面上项目资助(No.30400141);国家自然科学基金重点项目资助(No.30230140)

摘　　要：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600 nmol/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 neuroprotective effects of cyclin dependent kinase activity inhibitor, roscovitine in Niemann-Pick Type C （NPC） mouse model （npc^-/-）. Methods Different concentrated roscovitine （72, 144, 300 and 600 nmol/day） were infused into 5 week old npc^-/- mice （n = 6-10） intracerebroventricularly for a 2-week period, and DMSO infused, same aged npc^-/- mice （n = 6- 10） were used as control. The immunohistochemistry, H＆E staining and mouse coat hanger test were adopted in this research. Result Roscovitine significantly reduced the number of spheroids, modulated Purkinje neuron death, and ameliorated motor defects in npc^-/- mice. Conclusion The study suggested the neuroprotective effects of roscovitine to the neurodegeneration in NPC.

关 键 词：C型尼曼-皮克病 细胞周期依赖性蛋白激酶 ROSCOVITINE 细胞骨架蛋白过度磷酸化 球状轴突 

分 类 号：R741[医药卫生—神经病学与精神病学]