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

日本先天性双侧非综合征性感音神经性耳聋病例中，有相当高的比例与连接蛋白 26 的 GJB2 编码突变有关。我们的目的是评估 GJB2 突变模型小鼠柯蒂氏器的出生后发育情况。小鼠的耳蜗柱细胞发育不完全，GER 细胞凋亡延迟。目前的研究结果为 Gjb2 突变引起的先天性耳聋的基本治疗方法之一做出了巨大贡献。

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.