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[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

Received: 2011-12-15　　Accepted: 2012-04-26

Corresponding author: 管敏鑫　　E-mail: gminxin88@gmail.com

Citing This Article：

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).