Molecular mechanisms underlying function of hair bundle: study on genetic deafness in mouse models / 生理学报
Acta Physiologica Sinica
;
(6): 481-488, 2012.
Article
Dans Chinois
| WPRIM
| ID: wpr-333175
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.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Anatomopathologie
/
Physiologie
/
Cochlée
/
Mécanotransduction cellulaire
/
Modèles animaux de maladie humaine
/
Syndromes d'Usher
/
Matrice extracellulaire
/
Génétique
/
Cellules ciliées auditives
/
Surdité neurosensorielle
Limites du sujet:
Animaux
/
Humains
langue:
Chinois
Texte intégral:
Acta Physiologica Sinica
Année:
2012
Type:
Article
Documents relatifs à ce sujet
MEDLINE
...
LILACS
LIS