Molecular mechanisms underlying function of hair bundle: study on genetic deafness in mouse models.
- Author:
Ling-Zhi LIANG
1
;
Bin-Jiao ZHENG
;
Jing ZHENG
;
Fang FANG
;
Yue WU
;
Min-Xin GUAN
Author Information
1. Attardi Institute of Mitochondrial Biomedicine, Wenzhou Medical College, Wenzhou 325035, China.
- Publication Type:Journal Article
- MeSH:
Animals;
Cochlea;
physiopathology;
Disease Models, Animal;
Extracellular Matrix;
physiology;
Hair Cells, Auditory;
pathology;
Hearing Loss, Sensorineural;
genetics;
Humans;
Mechanotransduction, Cellular;
Mice;
Usher Syndromes;
genetics
- From:
Acta Physiologica Sinica
2012;64(4):481-488
- CountryChina
- Language:Chinese
-
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.
