KCNQ1在小鼠耳蜗血管纹的表达及在听觉中的意义  

Expression of KCNQ1 in Stria Vascularis of Mouse Cochlea and Effects of KCNQ1 on Auditory Function

作　　者：褚汉启[1] 熊浩[1] 韩芳[1] 吴振恭[1] 周良强[1] 黄孝文[1] 崔永华[1] 

机构地区：[1]华中科技大学同济医学院附属同济医院耳鼻咽喉科华中科技大学同济医学院耳鼻咽喉科学研究所,武汉430030

出　　处：《听力学及言语疾病杂志》2007年第4期303-306,I0003,共5页Journal of Audiology and Speech Pathology

基　　金：国家自然科学基金资助项目(编号30672397)

摘　　要：目的探讨KCNQ1在耳蜗侧壁血管纹的表达及其在听觉中的作用。方法以不同基因型小鼠KC-NQ1-/-(突变纯合子)、KCNQ1+/-(杂合子)和KCNQ1+/+(野生型)以及C57BL/6J小鼠为实验对象,采用免疫组织化学和ABR检测技术,检测KCNQ1在小鼠耳蜗血管纹的表达及其听力。结果KCNQ1蛋白阳性颗粒集中在小鼠耳蜗血管纹边缘细胞顶膜。KCNQ1+/+小鼠的听力正常,短声ABR的阈值为36.67±7.13dBSPL;KCNQ1+/-小鼠听力低于同窝KCNQ1+/+野生型鼠,短声ABR的阈值为38.25±9.35dB SPL;KCNQ1-/-小鼠呈现全聋,ABR在100dB SPL时仍无反应。结论KCNQ1是位于耳蜗侧壁血管纹边缘细胞的重要通道蛋白,在维系耳蜗听觉功能中有重要作用。KCNQ1通道蛋白的缺失或功能受限可以不同程度地影响耳蜗的听觉功能。Objective To investigate the expression of KCNQ1 in stria vascularis of mouse cochlea and the role of KCNQ1 in auditory function. Methods The immunohistochemistry was performed in C57BL/6J mice and hearing thresholds was measured in the KCNQ1 knockout mice, heterozygous mice and wild- type mice using auditory brainstem response （ABR） respectively. Results KCNQ1 protein staining was localized in the lateral wall of marginal cells in stria vascularis. The auditory function of KCNQ1^＋/＋ mice were normal.The mean ABR thresholds in response to click sound stimulus was 36.67 ± 7.13 dB SPL while the mean value in KCNQ1^＋/- mice was 38.25 ± 9.35 dB SPL. In ralation to that observed in KCNQ1^＋/＋ mice, KCNQ1^-/- mice were completely deaf. No ABR responses were observed at even 100 dB SPL. Conclusion KCNQ1 is one of important channel proteins in stria vascularis of cochlea and plays a critical role in cochlear auditory. The differential expression of KCNQ1 channels in the cochlear stria vascularis may be related to auditory function of mouse.

关 键 词：KCNQ1 离子通道 耳蜗 听力 

分 类 号：R339.16[医药卫生—人体生理学]