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

Highlights 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 Summary Mutation of the Gap...