[Molecular mechanisms underlying function of hair bundle: study on genetic deafness in mouse models].
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.
Attardi Institute of Mitochondrial Biomedicine, Wenzhou Medical College, Wenzhou 325035, China.
Ling-Zhi
LZ
Liang
Bin-Jiao
BJ
Zheng
Jing
J
Zheng
Fang
F
Fang
Yue
Y
Wu
Min-Xin
MX
Guan
D000818:Animals; D003051:Cochlea / Q000503:physiopathology*; D004195:Disease Models, Animal; D005109:Extracellular Matrix / Q000502:physiology; D006198:Hair Cells, Auditory / Q000473:pathology*; D006319:Hearing Loss, Sensorineural / Q000235:genetics*; D006801:Humans; D040542:Mechanotransduction, Cellular; D051379:Mice; D052245:Usher Syndromes / Q000235:genetics*