Deafness and cochlear fibrocyte alterations in mice deficient for the inner ear protein otospiralin
Deafness and cochlear fibrocyte alterations in mice deficient for the inner ear protein otospiralin
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DOI：
10.1128/mcb.25.2.847-853.2005
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发表时间：
2005-01-01
影响因子：
5.3
通讯作者：
Hamel, CP
中科院分区：
文献类型：
--
作者：
Delprat, B;Ruel, J;Hamel, CP
在哺乳动物听觉器官耳蜗中，间充质非感觉区纤维细胞在耳蜗生理中发挥重要作用，包括维持内淋巴中的离子和水成分。人类耳聋发生在纤维细胞的改变强调了它们在听觉功能中的关键作用。我们最近描述了一个新的基因，Otos，它编码耳螺旋灵，一种由耳蜗和前庭的纤维细胞产生的功能未知的小蛋白质。我们现在已经产生了Otos缺失的小鼠，发现它们表现出中度耳聋，没有频率优势。组织病理学显示II型和IV型纤维细胞变性，而毛细胞和血管纹则正常。综上所述，这些发现表明由耳螺旋灵缺失引起的纤维细胞损伤导致耳蜗生理和听觉功能异常。这种中度功能障碍可能导致与年龄相关的听力损失。
In the cochlea, the mammalian auditory organ, fibrocytes of the mesenchymal nonsensory regions play important roles in cochlear physiology, including the maintenance of ionic and hydric components in the endolymph. Occurrence of human deafness in fibrocyte alterations underlines their critical roles in auditory function. We recently described a novel gene, Otos, which encodes otospiralin, a small protein of unknown function that is produced by the fibrocytes of the cochlea and vestibule. We now have generated mice with deletion of Otos and found that they show moderate deafness, with no frequency predominance. Histopathology revealed a degeneration of type II and IV fibrocytes, while hair cells and stria vascularis appeared normal. Together, these findings suggest that impairment of fibrocytes caused by the loss in otospiralin leads to abnormal cochlear physiology and auditory function. This moderate dysfunction may predispose to age-related hearing loss.