A polarized multicomponent foundation upholds ciliary central microtubules  

作　　者：Qingxia Chen Huijie Zhao Xinwen Pan Chuyu Fang Benhua Qiu Jingting Guo Xiumin Yan Xueliang Zhu 

机构地区：[1]Ministry of Education-Shanghai Key Laboratory of Children’s Environmental Health,Institute of Early Life Health,Xinhua Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China [2]State Key Laboratory of Cell Biology,Shanghai Institute of Biochemistry and Cell Biology,Center for Excellence in Molecular Cell Science,Chinese Academy of Sciences,Shanghai 200031,China [3]Institute of Biomedical Sciences,College of Life Sciences,Key Laboratory of Animal Resistance Biology of Shandong Province,Collaborative Innovation Center of Cell Biology in Universities of Shandong,Shandong Normal University,Jinan 250014,China [4]School of Life Science and Technology,ShanghaiTech University,Shanghai 201210,China [5]Center for Excellence in Molecular Cell Science,University of Chinese Academy of Sciences,Beijing 100049,China [6]School of Life Science,Hangzhou Institute for Advanced Study,University of Chinese Academy of Sciences,Hangzhou 310024,China

出　　处：《Journal of Molecular Cell Biology》2024年第8期19-32,共14页分子细胞生物学报(英文版)

基　　金：supported by the National Natural Science Foundation of China(31991192,32230027,32270725,32100551,and 32270807);Shandong Natural Science Foundation(2022HWYQ-075).

摘　　要：Cilia’s back-and-forth beat pattern requires a central pair(CP)of microtubules.However,the mechanism by which the CP is upheld above the transition zone(TZ)remains unclear.Here,we showed that a rod-like substructure marked by Cep131 and ciliary Centrin serves as a polarized CP-supporting foundation.This CP-foundation(CPF)was assembled independently of the CP during ciliogenesis in mouse ependymal cells.It protruded from the distal end of the basal body out of the TZ to enwrap the proximal end of the CP.Through proximity labeling,we identified 26 potential CPF components,among which Ccdc148 specifically localized at the proximal region of Centrin-decorated CPF and was complementary to the Cep131-enriched distal region.Cep131 deficiency abolished the CPF,resulting in CP penetration into the TZ.Consequently,cilia became prone to ultrastructural abnormality and paralysis,and Cep131-deficient mice were susceptible to late-onset hydrocephalus.In addition to Centrin,phylogenetic analysis also indicated conservations of Ccdc131 and Ccdc148 from protists to mammals,suggesting that the CPF is an evolutionarily conserved multicomponent CP-supporting platform in cilia.

关 键 词：basal plate central pair ciliary motility ependyma HYDROCEPHALUS motile cilia 

分 类 号：R741[医药卫生—神经病学与精神病学] Q24[医药卫生—临床医学]