RGS22 maintains the physiological function of ependymal cells to prevent hydrocephalus  

作　　者：Xue Pang Lin Gu Qiu-Ying Han Jia-Qing Xing Ming Zhao Shao-Yi Huang Jun-Xi Yi Jie Pan Hao Hong Wen Xue Xue-Qing Zhou Zhi-Hui Su Xin-Ran Zhang Li-Ming Sun Shao-Zhen Jiang Dan Luo Ling Chen Zheng-Jie Wang Yu Yu Tian Xia Xue-Min Zhang Ai-Ling Li Tao Zhou Hong Cai Tao Li 

机构地区：[1]Nanhu Laboratory,National Center of Biomedical Analysis,Beijing 100039,China [2]School of Basic Medical Sciences,Fudan University,Shanghai 200032,China [3]Department of Basic Medical Sciences,School of Medicine,Tsinghua University,Beijing 100084,China

出　　处：《Science China(Life Sciences)》2025年第2期441-453,共13页中国科学(生命科学英文版)

基　　金：supported by grants from the China National Natural Science Foundation(82130052 and 81925017 to Tao Li,82001690 to Hong Cai)。

摘　　要：Ependymal cells line the wall of cerebral ventricles and ensure the unidirectional cerebrospinal fluid(CSF)flow by beating their motile cilia coordinately.The ependymal denudation or ciliary dysfunction causes hydrocephalus.Here,we report that the deficiency of regulator of Gprotein signaling 22(RGS22)results in severe congenital hydrocephalus in both mice and rats.Interestingly,RGS22 is specifically expressed in ependymal cells within the brain.Using conditional knock-out mice,we further demonstrate that the deletion of Rgs22 exclusively in nervous system is sufficient to induce hydrocephalus.Mechanistically,we show that Rgs22 deficiency leads to the ependymal denudation and impaired ciliogenesis.This phenomenon can be attributed to the excessive activation of lysophosphatidic acid receptor(LPAR)signaling under Rgs22^(-/-)condition,as the LPAR blockade effectively alleviates hydrocephalus in Rgs22^(-/-)rats.Therefore,our findings unveil a previously unrecognized role of RGS22 in the central nervous system,and present RGS22 as a potential diagnostic and therapeutic target for hydrocephalus.

关 键 词：RGS22 HYDROCEPHALUS ependymal cells 

分 类 号：R73[医药卫生—肿瘤]