纤毛
睫状体病
生物
细胞生物学
运动纤毛
纤毛病
微管
调节器
多囊肾病
动力蛋白
激酶
中心体
纤毛形成
主轴装置
极光激酶B
磷酸化
驱动蛋白
鞭毛内运输
鞭毛
遗传学
表型
细胞
细胞周期
基因
肾
作者
Yu-Cheng Zhang,Yun-Feng Bai,Jin-Feng Yuan,Xiao-Lin Shen,Yu-Ling Xu,Xiao-Xiao Jian,Sen Li,Zeng-Qing Song,Huai-Bin Hu,Pei-Yao Li,Hai-Qing Tu,Qiu-Ying Han,Na Wang,Ai-Ling Li,Xue-Min Zhang,Min Wu,Tao Zhou,Hui-Yan Li
标识
DOI:10.1083/jcb.202003149
摘要
Primary cilia protrude from the cell surface and have diverse roles during development and disease, which depends on the precise timing and control of cilia assembly and disassembly. Inactivation of assembly often causes cilia defects and underlies ciliopathy, while diseases caused by dysfunction in disassembly remain largely unknown. Here, we demonstrate that CEP55 functions as a cilia disassembly regulator to participate in ciliopathy. Cep55−/− mice display clinical manifestations of Meckel–Gruber syndrome, including perinatal death, polycystic kidneys, and abnormalities in the CNS. Interestingly, Cep55−/− mice exhibit an abnormal elongation of cilia on these tissues. Mechanistically, CEP55 promotes cilia disassembly by interacting with and stabilizing Aurora A kinase, which is achieved through facilitating the chaperonin CCT complex to Aurora A. In addition, CEP55 mutation in Meckel–Gruber syndrome causes the failure of cilia disassembly. Thus, our study establishes a cilia disassembly role for CEP55 in vivo, coupling defects in cilia disassembly to ciliopathy and further suggesting that proper cilia dynamics are critical for mammalian development.
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