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初级纤毛是近年来生物医学领域的一个研究热点。它存在于各种细胞表面，体形微小，结构复杂，具有重要的感官作用，可感知细胞外机械和化学信号变化，并协助其转导至细胞内部引发相应细胞应答。它自身组装、维持和分解所需的蛋白质，需要一组纤毛内转运蛋白来完成，即纤毛内转运系统（intraflagellar transport,IFT）。近年研究表明，初级纤毛与多种先天性疾病密切相关。随着免疫荧光染色、共聚焦显微镜观察等新实验方法的应用，人们对初级纤毛及IFT系统有了更深入的了解，为进一步指导纤毛相关疾病的干预诊疗奠定了基础。“,”The primary cilium attracts increasing attention in the biomedical field in recent years.It exists in the surface of various cells with a slim pattern,but complex structures and mighty functions as well.The primary cilium has an important role in sensory perception,through which it could receive the mechanical and chemical signals extracellularly,and then assist in their transduction to cells,causing cellular responses.However,assembling,maintaining and disintegrating the proteins of primary cilium depend on intraflagellar transport proteins to finish these processes. And recent research shows that it’s closely related with many congenital diseases.Accompanied with the appearance of various new experimental methods such as immunofluorescence staining and confocal microscopy,the knowledge of the primary cilium and IFT will be mastered better,which will further instruct us the prevention,diagnosis and treatment of the cilia-related diseases.