The tectorial membrane: one slice of a complex cochlear sandwich.

The tectorial membrane: one slice of a complex cochlear sandwich.
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DOI:
10.1097/moo.0b013e32830e20c4
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发表时间:
2008-10
影响因子:
1.6
通讯作者:
Russell IJ
Russell IJ
中科院分区:
医学3区
文献类型:
--
作者:
Richardson GP;Lukashkin AN;Russell IJ

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最近的研究结果表明,顶盖膜在听力中起着比迄今为止怀疑的更动态的作用,并且许多形式的耳聋可以由顶盖膜蛋白的突变引起,因此该综述既及时又相关。涵盖的主要主题是(i)分子组成,结构组织和特性的覆膜,(ii)的作用覆膜作为第二个谐振器和结构内有显着的纵向耦合,以及(iii)如何在覆膜蛋白突变导致耳聋的小鼠和男子。实验模型的结果表明,顶盖膜在听力中起着多重的关键作用。这些包括沿耳蜗长度沿着的耦合元件,支持行波并确保耳蜗反馈的增益和定时是最佳的。临床研究结果表明,稳定的,中度至重度形式的遗传性听力损失可能是TECTA突变的诊断,TECTA是编码顶盖膜的主要非胶原蛋白之一的基因。
The review is both timely and relevant as recent findings have shown the tectorial membrane plays a more dynamic role in hearing than hitherto suspected, and that many forms of deafness can result from mutations in tectorial membrane proteins. Main themes covered are (i) the molecular composition, structural organisation and properties of the tectorial membrane, (ii) the role of the tectorial membrane as a second resonator and a structure within which there is significant longitudinal coupling, and (iii) how mutations in tectorial membrane proteins cause deafness in mice and men. Findings from experimental models imply that the tectorial membrane plays multiple, critical roles in hearing. These include coupling elements along the length of the cochlea, supporting a travelling wave and ensuring the gain and timing of cochlear feedback are optimal. The clinical findings suggest stable, moderate-to-severe forms hereditary hearing loss may be diagnostic of a mutation in TECTA, the gene encoding one of the major, non-collagenous proteins of the tectorial membrane.