RNA结合蛋白对神经发育和神经性疾病的影响  

The effect of RNA binding proteins on neurodevelopment and neurological disease

作　　者：韩星[1] 丁欣[1] 冯星[1] 

机构地区：[1]苏州医学院附属儿童医院新生儿科,215003

出　　处：《齐齐哈尔医学院学报》2015年第3期406-408,共3页Journal of Qiqihar Medical University

基　　金：国家自然基金(81471488);江苏省医学重点学科(XK201120);苏州市科教兴卫青年科技项目(KJXW2013018);苏州市重点实验室(SZS201108)

摘　　要：RNA结合蛋白(RNA binding proteins,RBPs)指导着细胞体内RNA的剪接、运输、翻译和降解,从而直接调控着蛋白质的时空表达。RBPs对神经元的发育和其功能的维持是不可或缺的。本文总结了RNA结合蛋白结构和功能上的共性,以FMRP,hnRNP K,TDP-43为例,具体阐述了其对于神经元轴突、树突发育的影响以及当蛋白功能突变或缺失引起的相关神经性疾病的机理,以期为神经发育和退行性疾病的研究提供新的思路和目标。RNA binding proteins （ RBPs） play a crucial role in neuronal development and pathogenesis of multiple neural diseases , containing functional domains for RNA -protein interaction , nuclear -cytoplasmic transportation .RBPs dynamically control the temporal and spatial expression of multiple proteins . Taking examples of FMRP, hnRNP-K and TDP-43, this article reviews how defective RBPs affect axon and dendrite development , and how to cause certain neural diseases , and in order to provide illuminating thoughts and targets for basic and clinical research in both neural development and degenerative neural diseases .

关 键 词：RNA结合蛋白 TDP-43 轴突发育 脆性X染色体综合征 TDP -43 

分 类 号：R512.62[医药卫生—内科学]