Kazusaku Kamiya, MD, PhD, and his colleagues replicated cochlear cells that could potentially be used to replace faulty ones in patients with hearing loss caused by mutation of the Gap Junction Beta 2 (GJB2) gene (Stem Cell Reports. 2016). The researchers from Jutendo University in Tokyo, Japan, produced induced pluripotent stem cells aimed at correcting this inherited condition, which causes disruption of gap junction plaques in the cochlea and leads to profound sensorineural hearing loss. They demonstrated in a mouse model that the stem-cell-derived gap junction cells were functional for forming gap junction intercellular communication networks and transient ion species typical of the developing cochlea. The authors of the study hoped the in vitrol models could be used to develop inner-ear therapies and drug screening that target GJB2-related hearing loss.
Figure. Schematic of In Vitro iPSC Differentiation into Functional iCx26GJCs and Disease Model Cells for GJB2-Related Hearing Loss
Approximately one in 1,000 children has severe hearing loss at birth or during early childhood, with about half of the cases attributable to genetic causes. Mutation of the GJB2 gene is the most common cause of hereditary hearing loss worldwide and accounts for up to 50 percent of non-syndromic sensorineural hearing loss cases in some populations.
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