纤毛及纤毛相关疾病研究进展  

Cilia and Ciliopathies

作　　者：曹木青 潘俊敏[1] 

机构地区：[1]清华大学生命科学学院,北京100084

出　　处：《中国细胞生物学学报》2012年第9期849-856,共8页Chinese Journal of Cell Biology

基　　金：国家自然科学基金(No.30830057;No.30988004);国家重点基础研究发展计划(973)项目(No.2012CB945003)资助项目~~

摘　　要：纤毛是一种以细胞微管为主形成的突出于细胞表面的结构,分布于哺乳动物体内的大多数细胞。近年来研究发现,很多人类疾病都与纤毛结构、长度的失调相关,所以有关纤毛的研究是目前研究的热点领域。越来越多的证据证明,纤毛除了提供流体推动力参与细胞的运动功能之外,还具有信号传导的功能,在细胞生命活动的各个方面发挥着多种关键作用。它参与调控细胞生理活动、增殖与分化以及动物个体发育。因此,深入地探索纤毛调控机理对基础生物学理论的发展和人类纤毛相关疾病的攻克有重要意义。该文简要介绍了纤毛的结构、组装与解聚的机制、参与信号传导的功能以及纤毛缺陷同人类疾病的关系。Cilia are microtubule-based structures that protrude outside the cell surface of almost all types of mammalian cells.Recently,cilia have been under intensive studies because their defects are linked to various human diseases.In addition to roles in cell motility,cilia serve as an antenna to perform diverse cellular signaling.Cilia are involved in sensory transduction,developmental signaling and cell cycle progression.The study of cilia assembly,disassembly,length control as well as ciliary signaling has important implications for human disease.In this short review,we present recent advances on various aspects of ciliary biology and human disease resulted from ciliary defects.

关 键 词：纤毛 纤毛功能 纤毛内运输 纤毛相关疾病 

分 类 号：R363[医药卫生—病理学]