In Vitro Models of GJB2-Related Hearing Loss Recapitulate Ca²⁺ Transients via a Gap Junction Characteristic of Developing Cochlea

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• 作者：Ichiro Fukunaga¹ ; ² ; Ayumi Fujimoto¹ ; Kaori Hatakeyama¹ ; Toru Aoki¹ ; Atena Nishikawa¹ ; Tetsuo Noda³ ; ⁴ ; Osamu Minowa³ ; ⁴ ; Nagomi Kurebayashi⁵ ; Katsuhisa Ikeda¹ ; Kazusaku Kamiya¹ ; ^{kkamiya@juntendo.ac.jp}
• 关键词：hereditary deafness ; inner ear ; cochlea ; gap junction ; connexin ; GJB2 ; iPSC ; connexin 26 ; supporting cells ; Ca2+ transients
• 刊名：Stem Cell Reports
• 出版年：2016
• 出版时间：13 December 2016
• 年：2016
• 卷：7
• 期：6
• 页码：1023-1036
• 全文大小：5394 K
• 卷排序：7

文摘

Mutation in GJB2 (CX26) is the most frequent cause of hereditary deafness worldwide Functional CX26-gap junction plaque (GJP)-forming cells were generated from iPSCs These cells exhibited spontaneous Ca2+ transients typical of the developing cochlea The drastic disruption of GJP was observed in in vitro disease model of GJB2 mutation