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 cytobiology 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 ciliopathies can also lead to the defects of spermatic flagella. Ciliopathies and other diseases caused by the defects of structure and function of cilia could affect Hh signaling. Regulatory molecules such as Foxj1 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.