初级纤毛是一类以微管为基础结构的细胞器,其来源于细胞的母中心粒,锚定在细胞膜并如“天线”般突出细胞表面。作为细胞感受器,初级纤毛从环境中接受各种信号,传导至细胞内引起细胞反应。近期的研究表明,初级纤毛对与胚胎发育密切相关的Wnt信号通路的传导起重要作用。纤毛的损害可造成Wnt信号通路的异常,并引起胚胎中多类脏器一系列的病理改变,导致初级纤毛相关疾病的发生。文章主要阐述了初级纤毛与Wnt/β-catenin、Wnt/PCP通路及初级纤毛相关疾病之间的关系,并对初级纤毛相关疾病的治疗进行了初步探讨。对初级纤毛与Wnt信号通路关系的深入研究将有助于人们对该类疾病的进一步诊断和治疗。
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.
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