Down regulated connexin26 at different postnatal stage displayed different types of cellular degeneration and formation of organ of Corti
Down regulated connexin26 at different postnatal stage displayed different types of cellular degeneration and formation of organ of Corti
复制标题
出生后不同阶段下调的connexin26表现出不同类型的细胞变性和柯蒂氏器的形成
DOI：
10.1016/j.bbrc.2014.01.154
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发表时间：
2014-02-28
影响因子：
3.1
通讯作者：
Kong, Weijia
中科院分区：
文献类型：
--
作者：
Chen, Sen;Sun, Yu;Kong, Weijia
连接蛋白26（Cx26）突变是非综合征型遗传性耳聋最常见的病因。不同的先天性Cx26基因缺失小鼠模型揭示了严重的听力损失模式和耳蜗发育缺陷。本研究旨在建立Cx26基因敲除小鼠模型，观察不同时间点Cx26基因敲除小鼠听力损失和细胞变性的时间进程和模式。观察5个月的形态学变化，以检测这些模型之间的长期功能障碍。根据Cx26表达降低的时间点，在不同的组中发现了轻度至重度听力损失模式。早期Cx26减少组的Corti器发育异常，中匝有明显的细胞脱落，而晚期Cx26减少组的Corti器发育正常，仅基底匝有少量毛发脱落。提示Cx26可能在耳蜗的生后成熟过程中起重要作用，在更成熟阶段的正常听力中起着不可替代的作用。(C)2014 Elsevier Inc. All rights reserved.
Connexin26 (Cx26) mutation is the most common cause for non-syndromic hereditary deafness. Different congenital Cx26 null mouse models revealed a profound hearing loss pattern and developmental defect in the cochlea. Our study aimed at establishing a Cx26 knocking down mouse model at different postnatal time points and to investigate the time course and pattern of the hearing loss and cell degeneration in these models. Morphologic changes were observed for 5 months to detect long-term diversities among these models. Depending on the time point when Cx26 expression was reduced, mild to profound hearing loss patterns were found in different groups. Malformed organ of Corti with distinct cell loss in middle turn was observed only in early Cx26 reduction group while mice in late Cx26 reduction group developed normal organ of Corti and only suffered a few hair loss in the basal turn. These results indicated that Cx26 may play essential roles in the postnatal maturation of the cochlea, and its role in normal hearing at more mature stage may be replaceable. (C) 2014 Elsevier Inc. All rights reserved.