Absence of Serpinb6a causes progressive hair cell apoptosis and hearing loss in mice

查看全文 作　　者：Cheng [1,2]Cheng;Jieyu [3]Qi;Liyan [3]Zhang;He [4]Li;Jie [5]Lu;Siyu [1]Li;Zhong [3]Zhang;Yue [1]Qiu;Chen [6]Zhang;Lulu [7]Jiang;Chaorong [1]Yu;Xia [1,2]Gao;Phillip [8]I.Bird;Renjie [6,9,10,11,12]Chai 机构地区：[1]Department of Otolaryngology Head and Neck Surgery,Affiliated Drum Tower Hospital of Nanjing University Medical School,Jiangsu Provincial Key Medical Discipline(Laboratory),No.321 Zhongshan Road,Nanjing,Jiangsu 210008,China;[2]Research Institute of Otolaryngology,No.321 Zhongshan Road,Nanjing,Jiangsu 210008,China;[3]State Key Laboratory of Bioelectronics,School of Life Sciences and Technology,Jiangsu Province High-Tech Key Laboratory for Bio-Medical Research,Southeast University,Nanjing,Jiangsu 210096,China;[4]Department of Otolaryngology-Head and Neck Surgery,First Affiliated Hospital of Wenzhou Medical University,Wenzhou,Zhejiang 325015,China;[5]Northern Jiangsu People’s Hospital Affiliated to Yangzhou University,Yangzhou,Jiangsu 225009,China;[6]Beijing Key Laboratory of Neural Regeneration and Repair,Capital Medical University,Beijing 100069,China;[7]Suzhou Otovia Therapeutics Inc.,Suzhou,Jiangsu 215021,China;[8]Department of Biochemistry and Molecular Biology,Monash Biomedicine Discovery Institute,Monash University,Clayton,Victoria 3800,Australia;[9]State Key Laboratory of Bioelectronics,Department of Otolaryngology Head and Neck Surgery,Zhongda Hospital,School of Life Sciences and Technology,Advanced Institute for Life and Health,Jiangsu Province High-Tech Key Laboratory for Bio-Medical Research,Southeast University,Nanjing,Jiangsu 210009,China;[10]Co-Innovation Center of Neuroregeneration,Nantong University,Nantong,Jiangsu 226001,China;[11]Department of Otolaryngology Head and Neck Surgery,Sichuan Provincial People’s Hospital,University of Electronic Science and Technology of China,Chengdu,Sichuan 610072,China;[12]Institute for Stem Cell and Regeneration,Chinese Academy of Science,Beijing 100086,China 出　　处：《Journal of Genetics and Genomics》索引2023年第50卷第2期,共4页 基　　金：supported by grants from the National Key R&D Program of China(2017YFA0105201,2021YFA1101300,2020YFA0112503);the Strategic Priority Research Programof the Chinese Academy of Science(XDA16010303);the National Natural Science Foundation of China(82030029,81970882,81900941,82171144,82192862,81970884,81771019);the Natural Science Foundation from Jiangsu Province(BE2019711,BK20190121);the China Postdoctoral Science Foundation(2020M681555);theOutstanding Youth ProgramofNanjing Municipal Health Commission(JQX20003);the Science and Technology Department of Sichuan Province(2021YFS0371);the Shenzhen Fundamental Research Program(JCYJ20190814093401920,JCYJ20210324125608022);the Open Research Fund of State Key Laboratory of Genetic Engineering,Fudan University(SKLGE-2109). 摘　　要：Hearing impairment constitutes a global public health problem with severe social,political,and economic consequences,and the World Health Organization estimates that about 466 million individuals globally have some degree of hearing loss.Genetic factors account for about half of all deafness cases.Currently,more than 100 genes are known to be involved in hearing loss,but the underlying molecular mechanisms behind these deafness genes have not been fully characterized.The main clinical treatment for deafness is hearing aids and cochlear implants,but their efficacy depends heavily on the quantity and quality of residual hair cells(HCs)and spiral ganglion neurons and has only limited auditory effects. 关 键 词：DEAFNESS AUDITORY HEARING