Reduced climbing and increased slipping adaptation in cochlear hair cells of mice with Myo7a mutations
Reduced climbing and increased slipping adaptation in cochlear hair cells of mice with Myo7a mutations
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DOI：
10.1038/nn784
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发表时间：
2002-01-01
影响因子：
25
通讯作者：
Steel, KP
中科院分区：
文献类型：
--
作者：
Kros, CJ;Marcotti, W;Steel, KP
MYO7A基因突变会导致小鼠和人类遗传性耳聋。我们描述了两个突变，MYO7A(6J)和MYO7A(4626SB)的影响。关于耳蜗毛细胞的力电转导。这两种突变都会导致两种主要的功能异常，从而干扰声音传导。为了打开机械换能器通道，需要将发束移位到其生理工作范围之外。换能器电流对兴奋性刺激的适应性也比正常更强。我们得出结论，肌球蛋白VIIA参与将膜结合的元件锚定和保持到立体纤毛的肌动蛋白核心。因此，肌球蛋白VIIA是传感器通道正常选通所必需的。
Mutations in Myo7a cause hereditary deafness in mice and humans. We describe the effects of two mutations, Myo7a(6J) and Myo7a(4626SB). on mechano-electrical transduction in cochlear hair cells. Both mutations result in two major functional abnormalities that would interfere with sound transduction. The hair bundles need to be displaced beyond their physiological operating range for mechanotransducer channels to open. Transducer currents also adapt more strongly than normal to excitatory stimuli. We conclude that myosin VIIA participates in anchoring and holding membrane-bound elements to the actin core of the stereocilium. Myosin VIIA is therefore required for the normal gating of transducer channels.