β-Catenin Deletion in Regional Neural Progenitors Leads to Congenital Hydrocephalus in Mice

作     者：Lin Ma Yanhua Du Xiangjie Xu Hexi Feng Yi Hui Nan Li Guanyu Jiang Xiaoqing Zhang Xiaocui Li Ling Liu Lin Ma;Yanhua Du;Xiangjie Xu;Hexi Feng;Yi Hui;Nan Li;Guanyu Jiang;Xiaoqing Zhang;Xiaocui Li;Ling Liu

作者机构：Translational Medical Center for Stem Cell TherapyShanghai East HospitalSchool of MedicineTongji UniversityShanghai200120China Key Laboratory of Spine and Spinal Cord Injury Repair and Regeneration of the Ministry of EducationOrthopaedic Department of Tongji HospitalShanghai200065China Brain and Spinal Cord Innovative Research CenterSchool of MedicineTongji UniversityShanghai200092China Key Laboratory of Neuroregeneration of Shanghai UniversitiesSchool of MedicineTongji UniversityShanghai200092China Tsingtao Advanced Research InstituteTongji UniversityQingdao266071China Shanghai Institute of Stem Cell Research and Clinical TranslationShanghai200120China Department of Pathology and PathophysiologySchool of MedicineTongji UniversityShanghai200092China Department of Immunology and MicrobiologyShanghai Institute of ImmunologyShanghai Jiao Tong University School of MedicineShanghai200025China Shanghai First Maternity and Infant HospitalSchool of MedicineTongji UniversityShanghai200092China 

出 版 物：《Neuroscience Bulletin》 (神经科学通报（英文版）)

年 卷 期：2022年第38卷第1期

页      面：81-94页

核心收录：

学科分类：0710[理学-生物学] 1002[医学-临床医学] 1001[医学-基础医学(可授医学、理学学位)] 100204[医学-神经病学] 10[医学] 

基　　金：This work was supported by grants from the National Key Research and Development Program of China(2018YFA0108000 and 2019YFA0110300) the National Natural Science Foundation of China(8205020,32000689,31400934,31771132,31872760,31801204,and 31800858) the Science and Technology Commission of Shanghai Municipality(19JC1415100 and 21140902300) the Shanghai Municipal Education Commission(C120114) China Postdoctoral Science Foundation(2017M621526) the Fundamental Research Funds for the Central Universities,and the Major Program of Development Fund for Shanghai Zhangjiang National Innovation Demonstration Zone(Stem Cell Strategic Biobank and Clinical Translation Platform of Stem Cell Technology,ZJ2018-ZD-004) 

主　　题：Congenital hydrocephalus β-Catenin Ependymal cells Nkx2.1 Neural development 

摘      要：Congenital hydrocephalus is a major neurological disorder with high rates of morbidity and mortality;however,the underlying cellular and molecular mechanisms remain largely *** animal models mirroring both embryonic and postnatal hydrocephalus are also ***,we describe a new mouse model of congenital hydrocephalus through knockout ofβ-catenin in Nkx2.1-expressing regional neural *** ventriculomegaly and an enlarged brain were consistently observed in knockout mice from embryonic day 12.5 through to *** profiling revealed severe dysfunctions in progenitor maintenance in the ventricular zone and therefore in cilium biogenesis afterβ-catenin *** analyses also revealed an aberrant neuronal layout in both the ventral and dorsal telencephalon in hydrocephalic mice at both embryonic and postnatal ***,knockout ofβ-catenin in regional neural progenitors leads to congenital hydrocephalus and provides a reproducible animal model for studying pathological changes and developing therapeutic interventions for this devastating disease.