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	The spatial distribution pattern of connexin26 expression in supporting cells and its role in outer hair cell survival
	Chen, Sen1,2; Xu, Kai1; Xie, Le1; Cao, Hai-Yan3; Wu, Xia1; Du, An-Na4; He, Zu-Hong2; Lin, Xi5; Sun, Yu1,2; Kong, Wei-Jia1,2
刊名	Cell death & disease
	2018-12-05
卷号	9页码:14
ISSN号	2041-4889
DOI	10.1038/s41419-018-1238-x
通讯作者	Sun, yu(sunyu@hust.edu.cn) ; Kong, wei-jia(entwjkong@hust.edu.cn)
英文摘要	Mutations in the gjb2 gene (which encodes connexin26 (cx26)) account for about a quarter of all cases of non-syndromic deafness. previous studies have indicated that knockout (ko) of gjb2 gene during early postnatal days can cause outer hair cell (ohc) loss in mouse models. however, the postnatal spatial distribution pattern of cx26 in different types of supporting cells (scs) and the role of such distributions for the survival of ohcs is still obscure. in this study, the spatial distribution patterns of cx26 in scs were observed, and based on these observations different spatial cx26-null mouse models were established in order to determine the effect of changes in the spatial distribution of cx26 in scs on the survival of ohcs. at postnatal day (p)3, unlike the synchronous expression of cx26 along both longitudinal and radial boundaries of most types of scs, cx26 expression was primarily observed along the longitudinal boundaries of rows of deiter's cells (dcs). from p5 to p7, radial expression of cx26 was gradually observed between adjacent rows of dcs. when gjb2 gene was knocked out at random in different types of scs, about 40% of the total dcs lost cx26 expression and these cx26-null dcs were distributed randomly in all three rows of dcs. the mice in this randomly cx26-null group showed normal hearing and no significant ohc loss. when using a longitudinal ko pattern to induce knockout of gjb2 gene specifically in the third row of dcs, about 33% of the total dcs lost cx26 expression in this specific longitudinally cx26-null group. the mice in this group showed late-onset hearing loss and significant ohc loss, however, the morphology of corresponding dcs was slightly altered. in both experimental groups, no substantial dc loss was observed. these results indicate that longitudinal cx26-based channels are predominant in dcs during p3-p5. the cx26 expression along rows of dcs might play a key role in the survival of ohcs, but this longitudinal ko pattern in dcs has a limited effect on dc survival or on its postnatal development.
WOS关键词	GAP-JUNCTIONS ; DEGENERATION ; ORGAN ; ABLATION ; NULL ; FORM
WOS研究方向	Cell Biology
WOS类目	Cell Biology
语种	英语
出版者	NATURE PUBLISHING GROUP
WOS记录号	WOS:000452323500016
内容类型	期刊论文
URI标识	http://www.corc.org.cn/handle/1471x/2373211
专题	武汉病毒研究所
通讯作者	Sun, Yu; Kong, Wei-Jia
作者单位	1.Huazhong Univ Sci & Technol, Dept Otorhinolaryngol, Union Hosp, Tongji Med Coll, Wuhan 430022, Hubei, Peoples R China 2.Huazhong Univ Sci & Technol, Tongji Med Coll, Inst Otorhinolaryngol, Wuhan 430022, Hubei, Peoples R China 3.Huazhong Univ Sci & Technol, Tongji Med Coll, Dept Ultrasound, Union Hosp, Wuhan 430022, Hubei, Peoples R China 4.Chinese Acad Sci, Wuhan Inst Virol, Ctr Instrumental Anal & Metrol, Wuhan 430071, Peoples R China 5.Emory Univ, Sch Med, Dept Otolaryngol Head & Neck Surg, Atlanta, GA 30322 USA
推荐引用方式 GB/T 7714	Chen, Sen,Xu, Kai,Xie, Le,et al. The spatial distribution pattern of connexin26 expression in supporting cells and its role in outer hair cell survival[J]. Cell death & disease,2018,9:14.
APA	Chen, Sen.,Xu, Kai.,Xie, Le.,Cao, Hai-Yan.,Wu, Xia.,...&Kong, Wei-Jia.(2018).The spatial distribution pattern of connexin26 expression in supporting cells and its role in outer hair cell survival.Cell death & disease,9,14.
MLA	Chen, Sen,et al."The spatial distribution pattern of connexin26 expression in supporting cells and its role in outer hair cell survival".Cell death & disease 9(2018):14.

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