Inner ear defects induced by null mutation of the isk gene
Inner ear defects induced by null mutation of the isk gene
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DOI：
10.1016/s0896-6273(00)80255-x
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发表时间：
1996-12-01
期刊：
影响因子：
16.2
通讯作者：
Barhanin, J
中科院分区：
文献类型：
--
作者：
Vetter, DE;Mann, JR;Barhanin, J
Isk基因在许多组织中都有表达。来自内耳的药理学证据表明，ISK介导钾分泌到内淋巴。为了研究ISK零突变对内耳功能的影响，并建立一个可用于检测ISK在其他地方所起作用的系统(S)，我们培育了一种携带中断的ISK基因座的小鼠品系。基因敲除小鼠表现出典型的摇床/华尔兹舞曲行为。毛细胞退化，但不同内耳器官的毛细胞退化的时间不同。在功能上，我们发现在缺乏ISK的小鼠中，内耳的皱纹边缘细胞和前庭暗细胞在体外不能产生等量的短路电流，这表明缺乏跨上皮钾分泌。
The isk gene is expressed in many tissues. Pharmacological evidence from the inner ear suggests that Isk mediates potassium secretion into the endolymph. To examine the consequences of IsK null mutation on inner ear function, and to produce a system useful for examining the role(s) IsK plays elsewhere, we have produced a mouse strain that carries a disrupted isk locus. Knockout mice exhibit classic shaker/waltzer behavior. Hair cells degenerate, but those of different inner ear organs degenerate at different times. Functionally, we show that in mice lacking isk, the strial marginal cells and the vestibular dark cells of the inner ear are unable to generate an equivalent short circuit current in vitro, indicating a lack of transepithelial potassium secretion.