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There are &gt;120 forms of non-syndromic deafness associated with identified genetic loci. In particular, mutation of the gap junction beta 2 gene ( GJB2 ), which encodes connexin (CX)26 protein, is the most frequent cause of hereditary deafness worldwide. We previously described an induction method to develop functional CX26 gap junction-forming cells from mouse-induced pluripotent stem cells (iPSCs) and generated  in vitro  models for  GJB2 -related deafness. However, functional CX26 gap junction-forming cells derived from human iPSCs or embryonic stem cells (ESCs) have not yet been reported. In this study, we generated human iPSC-derived functional CX26 gap junction-forming cells (iCX26GJCs), which have the characteristics of cochlear supporting cells. These iCX26GJCs had gap junction plaque-like formations at cell–cell borders and co-expressed several markers that are expressed in cochlear supporting cells. Furthermore, we generated iCX26GJCs derived from iPSCs from two patients with the most common  GJB2  mutation in Asia, and these cells reproduced the pathology of  GJB2 -related deafness. These  in vitro  models may be useful for establishing optimal therapies and drug screening for various mutations in  GJB2 -related deafness.

Modeling gap junction beta 2 gene-related deafness with human iPSC