纤毛中Hedgehog信号调控机制研究进展  

Research progress of regulatory mechanism of Hedgehog signal in cilia

作　　者：王亚楠[1] 邹沙沙[1] 李铮[1] 胡洪亮[1] 

机构地区：[1]上海交通大学医学院附属仁济医院泌尿外科上海市男科学研究所精子发育与遗传学实验室上海市人类精子库,上海200001

出　　处：《上海交通大学学报（医学版）》2013年第4期502-505,共4页Journal of Shanghai Jiao tong University:Medical Science

基　　金：国家自然科学基金(30672146;30973069);浦江计划(08PJ1400);国家重点基础研究发展计划(2010CB945200;2011CB944504)~~

摘　　要：纤毛是多种信号通路的重要调节者,对Hedgehog(Hh)信号通路也起着至关重要的调控作用。研究表明Hh信号通路与鞭毛内转运密切相关,遗传学与细胞生物学的相关研究也表明了Hh信号对初级纤毛有依赖性。Hh信号通路的核心元件都定位于纤毛上,并通过鞭毛内转运实现其对信号刺激的应答,最终影响Hh基因的转录,而精子鞭毛的结构与纤毛类似,纤毛疾病也会造成精子鞭毛运动功能障碍。纤毛不动综合征等纤毛结构与功能的异常都会影响Hh通路,其诸多调节因子如Foxj1等对纤毛形成机制有重要意义,也为治疗不育症及避孕等相关措施研究提供了新思路。文章对纤毛中Hh信号调控机制研究进展进行综述。Cilia serve as an important regulator of various signaling pathways, as well as the Hedgehog （Hh） signaling pathway. Recent studies have revealed that there is a close relationship between Hh signaling pathway and intraflagellar transport of cilia, and further evidences from genetics and eytobiology have also proved the relationship between primary cilia and Hh signaling pathway. All of the core components of Hh signaling pathway are found on the primary cilia, and intraflagellar transport proteins are required for the transmission of information between the signaling molecules that mediate the activity of Hh signaling pathway. Flagella of sperm share the same structure with cilia, and eiliopathies can also lead to the defects of spermatie flagella. Ciliopathies and other diseases caused by the defects of structure and function of cilia could affect Hh signaling. Regulatory molecules such as Foxjl contribute to the construction of cilia, which brings new ideas in the field of infertility and contraception. The research progress of regulatory mechanism of Hedgehog signal in cilia is introduced in this paper.

关 键 词：纤毛 鞭毛 HEDGEHOG信号通路 调控分子 

分 类 号：R394[医药卫生—医学遗传学]