Postnatal Developmental Expression in Gjb2 Transgenic Mice of the Organ of Corti

日本人中先天性双侧非综合征性感音神经性耳聋病例的比例非常高，这与编码连接蛋白的GJB2突变有关26。我们的目的是评估出生后的Corti器官在GJB2突变模型小鼠的发育。结果表明，小鼠耳蜗柱状细胞发育不全，细胞凋亡延迟，这为Gjb2基因突变所致的先天性耳聋提供了一种基本的治疗方法。

A strikingly high proportion of congenital bilateral nonsyndromic sensorineural deafness cases among Japanese have been linked to mutations in the GJB2 coding for the connexin26. We aimed to evaluate the postnatal development of the organ of Corti in GJB2 mutations model mice. The mice showed incomplete development of the cochlear pillar cells and delayed apoptosis in GER. The present findings contribute strongly to one of basic remedies for the congenital deafness caused by Gjb2 mutation.