在Sox2阳性细胞敲除β-catenin对小鼠内耳发育和极性的影响  

The role of β-catenin deletion in Sox2-positive cells in inner-ear development and polarity regulation in mice

作　　者：钱晓青 任冬冬 丛宁[1,3,4,5] 赵萌 迟放鲁[1,2,3,4] QIAN Xiao-qing;REN Dong-dong;CONG Ning;ZHAO Meng;CHI Fang-lu(Institute of ENT Eye and ENTH ospital,Fudan University,Shanghai 200031,China;Department of Otorhinolaryngology,Eye and ENT Hospital,Fudan University,Shanghai 200031,China;Shanghai Clinical Medical Center of Hearing Medicine,Shanghai 200031,China;Key Laboratory of Hearing Medicine,Ministry of Health,Shanghai 200031,China;Department of Research Center,Eye and ENT Hospital,Fudan University,Shanghai 200031,China)

机构地区：[1]复旦大学附属眼耳鼻喉科医院耳鼻喉科研究院,上海200031 [2]复旦大学附属眼耳鼻喉科医院耳鼻喉科,上海200031 [3]上海市听觉医学临床中心,上海200031 [4]卫生部听觉医学重点实验室,上海200031 [5]复旦大学附属眼耳鼻喉科医院实验中心,上海200031

出　　处：《复旦学报（医学版）》2018年第2期143-150,共8页Fudan University Journal of Medical Sciences

基　　金：国家自然科学基金(81420108010;81271084;81370022;81570920;81000413);上海市卫生系统优秀青年人才培养计划(XYQ2013084)~~

摘　　要：目的探究经典Wnt/β-catenin信号通路的核心蛋白β-catenin敲除对小鼠内耳发育和极性的影响。方法在胚胎小鼠内耳感觉上皮的前体细胞(Sox2阳性细胞)条件敲除β-catenin,观察内耳大体结构改变情况。对基底膜和椭圆囊斑、球囊斑行免疫荧光染色,观察毛细胞数量、排列和极性的变化情况。结果与对照组比较,β-catenin条件敲除后,听泡体积变小,耳蜗和前庭器变小,前庭感觉上皮毛细胞减少(P<0.01)。耳蜗静纤毛方向紊乱,动纤毛在位置和数量上发生变化,前庭毛细胞方向也产生散在性紊乱。结论β-catenin调控小鼠内耳发育和毛细胞极性,经典Wnt/β-catenin信号通路可能与非经典Wnt/PCP通路共同调控耳蜗伸展和内耳平面细胞极性。Objective To investigate the role ofβ-catenin,the core protein of canonical Wnt/β-catenin signaling,in the inner-ear development and polarity regulation.Methods p-catenin was conditionally deleted from precursor cells of inner-ear sensory epithelium(Sox2-positive cells)in embryonic mice.Morphological changes of the inner ear were observed.Immunofluorescence staining was performed to basilar membranes,utricles and saccules to observe the hair-cell changes in arrangement and polarity.Results Compared with the control group,knock-out of(3-catenin in Sox2-positive cells resulted in smaller otic vesicles,cochleas and vestibules,and fewer hair cells(H Cs)in vestibular sensory epithelium(P<0.01).Stereocilium showed misorientation and kinocillia had a change in location and quantity in the cochlea.Scattered misorented HCs were also observed in the vestibule.Conclusions(3-catenin is crucial for the development of inner ear and polarity manipulation of HCs in mice.The canonical Wnt/p-catenin signaling may be involved in the regulation of cochlea extension and planar cell polarity(PCP)of inner ear,which is known to be controlled by non-canonical Wnt/PCP signaling.

关 键 词：Β-CATENIN 耳蜗 前庭 毛细胞 发育 平面细胞极性 

分 类 号：R764[医药卫生—耳鼻咽喉科]