Genetics of auditory mechano-electrical transduction.
Genetics of auditory mechano-electrical transduction.
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DOI：
10.1007/s00424-014-1552-9
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发表时间：
2015-01
影响因子：
4.5
通讯作者：
Petit, Christine
中科院分区：
文献类型：
--
作者：
Michalski, Nicolas;Petit, Christine
耳蜗毛细胞的毛束在听觉机电转导（MET）过程中起着核心作用。耳蜗内的毛细胞数量非常少，因此用生化方法鉴定MET成分和相关分子复合物受到阻碍。相比之下，人类和老鼠的基因被证明是特别强大的。对遗传性耳聋的研究导致了MET机制的几个基本蛋白质的发现，这些蛋白质目前被用作破译相关分子网络的切入点。值得注意的是，MET不仅依赖于MET机制，而且还依赖于几个元素，以确保毛束的适当声诱导振荡或驱动MET电流所需的离子环境。在这里，我们回顾了从听力遗传学中出现的MET过程分子基础的最重要进展。
The hair bundles of cochlear hair cells play a central role in the auditory mechano-electrical transduction (MET) process. The identification of MET components and of associated molecular complexes by biochemical approaches is impeded by the very small number of hair cells within the cochlea. In contrast, human and mouse genetics have proven to be particularly powerful. The study of inherited forms of deafness led to the discovery of several essential proteins of the MET machinery, which are currently used as entry points to decipher the associated molecular networks. Notably, MET relies not only on the MET machinery but also on several elements ensuring the proper sound-induced oscillation of the hair bundle or the ionic environment necessary to drive the MET current. Here, we review the most significant advances in the molecular bases of the MET process that emerged from the genetics of hearing.
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