Zhang Man-Li, Lu Yan-Ping, Li Ya-Li
Department of Gynaecology and Obstetrics, The PLA General Hospital,Beijing 100853, China.
Yi Chuan. 2015 Mar;37(3):233-239. doi: 10.16288/j.yczz.14-252.
Primary cilium is a microtubule-based organelle,which develops from the mother centriole of the centrosome. It is an antenna-like structure that anchors at the cell membrance, protruding from the cell surface. Primary cilium acts as a sensory organelle that receives different kinds of signals from the environment and transmits signals to cells to elicit cellular responses. Recent studies have revealed that primary cilium play an important role in transmitting Wnt signaling, which is critical for embryonic development. Dysfunction of primary cilium deregulates Wnt signaling, causing a series of pathological changes in different organs of the embryo, resulting in ciliopathies. In this review, we summarize correlation among primary cilium,Wnt/β-catenin signaling,Wnt/PCP signaling and ciliopathies. Current therapies in ciliopathies are also discussed. Highlights on these researches will encourage the development of Wnt-associated diagnostic tools and therapy for ciliopathies.
初级纤毛是一种基于微管的细胞器，它由中心体的母中心粒发育而来。它是一种类似天线的结构，锚定在细胞膜上，从细胞表面突出。初级纤毛作为一种感觉细胞器，接收来自环境的各种信号，并将信号传递给细胞以引发细胞反应。最近的研究表明，初级纤毛在传递Wnt信号中起重要作用，这对胚胎发育至关重要。初级纤毛功能障碍会使Wnt信号失调，导致胚胎不同器官发生一系列病理变化，从而引发纤毛病。在本综述中，我们总结了初级纤毛、Wnt/β-连环蛋白信号通路、Wnt/平面细胞极性（PCP）信号通路与纤毛病之间的相关性。还讨论了目前针对纤毛病的治疗方法。这些研究的亮点将促进与Wnt相关的纤毛病诊断工具和治疗方法的开发。
