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纤毛束功能的分子机制：遗传性耳聋小鼠模型研究

梁玲芝, 郑斌娇, 郑静, 方芳, 伍越, 管敏鑫

温州医学院Attardi线粒体生物医学研究院，温州 325035；浙江大学生命科学学院，杭州 310058

摘要

虽然人们在多年前已了解听觉系统末梢潜在功能的基本原理，但是对耳聋的分子机制却不甚明了。近年来，学者们在遗传性耳聋小鼠模型上进行了相关研究，探讨了纤毛束、毛细胞机械传感细胞器形成和功能相关的分子基础。本综述主要描述了1型Usher综合征基因产物形成的蛋白网状结构在纤毛束发育和机械电转换门控通道组装的作用，并介绍了静纤毛的小根对纤毛束机械性能的作用，纤毛束抑制隐蔽的特性，以及耳蜗中细胞外基质和盖膜在纤毛束刺激中的多种作用。

关键词： 纤毛束; 小鼠模型; 耳蜗; 机械电转换; 静纤毛; Usher综合征; 盖膜

分类号：R339.16

[Molecular mechanisms underlying function of hair bundle: study on genetic deafness in mouse models.] [Article in Chinese]

LIANG Ling-Zhi, ZHENG Bin-Jiao, ZHENG Jing, FANG Fang, WU Yue, GUAN Min-Xin

Attardi Institute of Mitochondrial Biomedicine, Wenzhou Medical College, Wenzhou 325035, China； College of Life Sciences, Zhejiang University, Hangzhou 310058, China

Abstract

Although the basic principles for the function of peripheral auditory system have been known for many years, the molecular mechanisms which affect deafness are not clear. In recent years, the study of hereditary deafness associated mouse models has revealed the molecular basis which is related with the formation and function of the hair bundle and the mechanosensory organelle of hair cell. This review focused on the role of protein network, which is formed by the proteins encoded by the Usher syndrome type 1 genes, in hair-bundle development and mechanotransducer channel gating. And the review also showed how the stereocilia rootlets contribute to the hair bundle’s mechanical properties and how the hair bundle produces suppressive masking. Finally, the review revealed multiple roles of the tectorial membrane and extracellular matrix in the hair bundles stimulating in the cochlea.

Key words: hair bundle; mouse model; cochlea; mechanoelectrical transduction; stereocilia; Usher syndrome; tectorial membrane

收稿日期：2011-12-15　　录用日期：2012-04-26

通讯作者：管敏鑫　　E-mail: gminxin88@gmail.com

引用本文：

梁玲芝, 郑斌娇, 郑静, 方芳, 伍越, 管敏鑫. 纤毛束功能的分子机制：遗传性耳聋小鼠模型研究[J]. 生理学报 2012; 64 (4): 481-488.

LIANG Ling-Zhi, ZHENG Bin-Jiao, ZHENG Jing, FANG Fang, WU Yue, GUAN Min-Xin. [Molecular mechanisms underlying function of hair bundle: study on genetic deafness in mouse models.] [Article in Chinese] . Acta Physiol Sin 2012; 64 (4): 481-488 (in Chinese with English abstract).