Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2

Jervell and Lange-Nielsen syndrome is characterised by congenital deafness and vestibular dysfunction, and is caused by mutations in KCNE1 or KCNQ1. Here, the authors show that gene therapy via canalostomy at early postnatal stage can preserve the morphology of inner ear and auditory and vestibular...

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Autores principales:	Xuewen Wu, Li Zhang, Yihui Li, Wenjuan Zhang, Jianjun Wang, Cuiyun Cai, Xi Lin
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/2f9e5f7939d647e59aea11fefbf982d1
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spelling	oai:doaj.org-article:2f9e5f7939d647e59aea11fefbf982d12021-12-02T14:16:13ZGene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 210.1038/s41467-020-20808-72041-1723https://doaj.org/article/2f9e5f7939d647e59aea11fefbf982d12021-01-01T00:00:00Zhttps://doi.org/10.1038/s41467-020-20808-7https://doaj.org/toc/2041-1723Jervell and Lange-Nielsen syndrome is characterised by congenital deafness and vestibular dysfunction, and is caused by mutations in KCNE1 or KCNQ1. Here, the authors show that gene therapy via canalostomy at early postnatal stage can preserve the morphology of inner ear and auditory and vestibular functions in a mouse model of human JLNS2.Xuewen WuLi ZhangYihui LiWenjuan ZhangJianjun WangCuiyun CaiXi LinNature PortfolioarticleScienceQENNature Communications, Vol 12, Iss 1, Pp 1-12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Xuewen Wu Li Zhang Yihui Li Wenjuan Zhang Jianjun Wang Cuiyun Cai Xi Lin Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2
description	Jervell and Lange-Nielsen syndrome is characterised by congenital deafness and vestibular dysfunction, and is caused by mutations in KCNE1 or KCNQ1. Here, the authors show that gene therapy via canalostomy at early postnatal stage can preserve the morphology of inner ear and auditory and vestibular functions in a mouse model of human JLNS2.
format	article
author	Xuewen Wu Li Zhang Yihui Li Wenjuan Zhang Jianjun Wang Cuiyun Cai Xi Lin
author_facet	Xuewen Wu Li Zhang Yihui Li Wenjuan Zhang Jianjun Wang Cuiyun Cai Xi Lin
author_sort	Xuewen Wu
title	Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2
title_short	Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2
title_full	Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2
title_fullStr	Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2
title_full_unstemmed	Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2
title_sort	gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of jervell and lange-nielsen syndrome type 2
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/2f9e5f7939d647e59aea11fefbf982d1
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Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2

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