Critical role of spectrin in hearing development and deafness

doi:10.1126/sciadv.aav7803

KMS > 免疫化学研究所

	Critical role of spectrin in hearing development and deafness
	Liu, Yan1 ; Qi, Jieyu 1,2,3; Chen, Xin4 ; Tang, Mingliang 2,3; Chu, Cenfeng1,4 ; Zhu, Weijie 1,2,3; Li, Hui1 ; Tian, Cuiping1 ; Yang, Guang5 ; Zhong, Chao6 ; Zhang, Ying 7; Ni, Guangjian 8,9; He, Shuijin4 ; Chai, Renjie 2,3,10,11,12; Zhong, Guisheng1,3,4,13
	2019-04
发表期刊	SCIENCE ADVANCES (IF:11.7[JCR-2023],13.7[5-Year])
ISSN	2375-2548
卷号	5 期号:4
发表状态	已发表
DOI	10.1126/sciadv.aav7803
摘要	Inner ear hair cells (HCs) detect sound through the deflection of mechanosensory stereocilia. Stereocilia are inserted into the cuticular plate of HCs by parallel actin rootlets, where they convert sound-induced mechanical vibrations into electrical signals. The molecules that support these rootlets and enable them to withstand constant mechanical stresses underpin our ability to hear. However, the structures of these molecules have remained unknown. We hypothesized that alpha II- and beta II-spectrin subunits fulfill this role, and investigated their structural organization in rodent HCs. Using super-resolution fluorescence imaging, we found that spectrin formed ring-like structures around the base of stereocilia rootlets. These spectrin rings were associated with the hearing ability of mice. Further, HC-specific, beta II-spectrin knockout mice displayed profound deafness. Overall, our work has identified and characterized structures of spectrin that play a crucial role in mammalian hearing development.
收录类别	SCI ; SCIE ; EI
语种	英语
资助项目	National Natural Science Foundation of China[31771130] ; National Natural Science Foundation of China[31200743] ; National Natural Science Foundation of China[31671063] ; National Natural Science Foundation of China[81622013] ; National Natural Science Foundation of China[81470692] ; National Natural Science Foundation of China[31500852]
WOS研究方向	Science & Technology - Other Topics
WOS类目	Multidisciplinary Sciences
WOS记录号	WOS:000466398400081
出版者	AMER ASSOC ADVANCEMENT SCIENCE
EI入藏号	20191706840748
EI主题词	Fluorescence imaging ; Mammals ; Molecules ; Proteins ; Vibrations (mechanical)
EI分类号	Ergonomics and Human Factors Engineering:461.4 ; Organic Compounds:804.1 ; Mechanics:931.1 ; Atomic and Molecular Physics:931.3
WOS关键词	HAIR-CELL ; ACTIN-FILAMENTS ; CYTOSKELETAL STRUCTURE ; MEMBRANE SKELETON ; CUTICULAR PLATE ; BIRD COCHLEA ; STEREOCILIA ; ORGANIZATION ; MUTATIONS ; ROOTLETS
原始文献类型	Article
引用统计	被引频次：133[WOS] [WOS记录] [WOS相关记录]
文献类型	期刊论文
条目标识符	https://kms.shanghaitech.edu.cn/handle/2MSLDSTB/45559
专题	免疫化学研究所物质科学与技术学院_PI研究组_钟超组生命科学与技术学院_PI研究组_何水金组免疫化学研究所_特聘教授组_功能筛选实验室 iHuman研究所_特聘教授组_Raymond Stevens组 iHuman研究所_公共科研平台_超分辨生物成像平台 iHuman研究所_PI研究组_钟桂生组 iHuman研究所_PI研究组_Garth John Thompson组生命科学与技术学院_硕士生生命科学与技术学院_博士生
共同第一作者	Qi, Jieyu; Chen, Xin; Tang, Mingliang
通讯作者	He, Shuijin; Chai, Renjie; Zhong, Guisheng
作者单位	1.ShanghaiTech Univ, IHuman Inst, Shanghai, Peoples R China 2.Southeast Univ, Key Lab Dev Genes & Human Dis, Minist Educ, Inst Life Sci,Jiangsu Prov High Tech Key Lab Biom, Nanjing, Jiangsu, Peoples R China 3.Nantong Univ, Coinnovat Ctr Neuroregenerat, Jiangsu Key Lab Neuroregenerat, Nantong, Peoples R China 4.ShanghaiTech Univ, Sch Life Sci & Technol, Shanghai, Peoples R China 5.ShanghaiTech Univ, Shanghai Inst Adv Immunochem Studies, Shanghai, Peoples R China 6.ShanghaiTech Univ sity, Sch Phys Sci & Technol, Shanghai, Peoples R China 7.Dalhousie Univ, Dept Med Neurosci, Fac Med, 5850 Coll St, Halifax, NS, Canada 8.Tianjin Univ, Acad Med Engn & Translat Med, Tianjin, Peoples R China 9.Tianjin Univ, Dept Biomed Engn, Coll Precis Instruments & Optoelect Engn, Lab Neural Engn & Rehabil, Tianjin, Peoples R China 10.Chinese Acad Sci, Inst Stem Cell & Regenerat, Beijing, Peoples R China 11.Res Inst Otolaryngol, 321 Zhongshan Rd, Nanjing, Jiangsu, Peoples R China 12.Capital Med Univ, Beijing Key Lab Neural Regenerat & Repair, Beijing 100069, Peoples R China 13.Chinese Acad Sci, Guangzhou Inst Biomed & Hlth, Guangzhou, Guangdong, Peoples R China
第一作者单位	iHuman研究所
通讯作者单位	生命科学与技术学院; iHuman研究所
第一作者的第一单位	iHuman研究所
推荐引用方式 GB/T 7714	Liu, Yan,Qi, Jieyu,Chen, Xin,et al. Critical role of spectrin in hearing development and deafness[J]. SCIENCE ADVANCES,2019,5(4).
APA	Liu, Yan.,Qi, Jieyu.,Chen, Xin.,Tang, Mingliang.,Chu, Cenfeng.,...&Zhong, Guisheng.(2019).Critical role of spectrin in hearing development and deafness.SCIENCE ADVANCES,5(4).
MLA	Liu, Yan,et al."Critical role of spectrin in hearing development and deafness".SCIENCE ADVANCES 5.4(2019).