Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
Targeted connexin26 ablation arrests postnatal development of the organ of Corti.
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DOI：
10.1016/j.bbrc.2009.05.023
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发表时间：
2009-07-17
影响因子：
3.1
通讯作者：
Lin, Xi
中科院分区：
文献类型：
--
作者：
Wang, Yunfeng;Chang, Qing;Tang, Wenxue;Sun, Yu;Zhou, Binfei;Li, Huawei;Lin, Xi
关键词：
编码连接蛋白26（Cx26）的基因突变是人类非综合征型遗传性耳聋的最常见原因。为了研究Cx26无效突变背后的耳聋机制，我们产生了三个独立的条件Cx26无效小鼠系。出生时细胞分化和总体耳蜗形态似乎正常。然而，出生后的Corti器官的发育被停滞，因为Corti隧道和Nuel空间从未打开。在P8左右的Claudius细胞中首次观察到细胞变性。外毛细胞的损失最初观察到周围P13在中间圈时，内毛细胞仍然完整。此后中回发生大量细胞死亡，并逐渐扩散至基底回，导致相应耳蜗位置的螺旋神经节神经元继发性变性。这些结果表明，Cx26在出生后的成熟和稳态的Corti器官的听力发病前起着至关重要的作用。
Mutations in the gene coding for connexin26 (Cx26) is the most common cause of human nonsyndromic hereditary deafness. To investigate deafness mechanisms underlying Cx26 null mutations, we generated three independent lines of conditional Cx26 null mice. Cell differentiation and gross cochlear morphology at birth seemed normal. However, postnatal development of the organ of Corti was stalled as the tunnel of Corti and the Nuel’s space were never opened. Cell degeneration was first observed in the Claudius cells around P8. Outer hair cell loss was initially observed around P13 at middle turn when inner hair cells were still intact. Massive cell death occurred in the middle turn thereafter and gradually spread to the basal turn, resulting in secondary degeneration of spiral ganglion neurons in the corresponding cochlear locations. These results demonstrated that Cx26 plays essential roles in postnatal maturation and homoeostasis of the organ of Corti before the onset of hearing.
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