| First Author | Kitajiri S | Year | 2004 |
| Journal | J Cell Biol | Volume | 166 |
| Issue | 4 | Pages | 559-70 |
| PubMed ID | 15314067 | Mgi Jnum | J:124151 |
| Mgi Id | MGI:3720865 | Doi | 10.1083/jcb.200402007 |
| Citation | Kitajiri S, et al. (2004) Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia. J Cell Biol 166(4):559-70 |
| abstractText | Ezrin/radixin/moesin (ERM) proteins cross-link actin filaments to plasma membranes to integrate the function of cortical layers, especially microvilli. We found that in cochlear and vestibular sensory hair cells of adult wild-type mice, radixin was specifically enriched in stereocilia, specially developed giant microvilli, and that radixin-deficient (Rdx(-)(/)(-)) adult mice exhibited deafness but no obvious vestibular dysfunction. Before the age of hearing onset ( approximately 2 wk), in the cochlea and vestibule of Rdx(-)(/)(-) mice, stereocilia developed normally in which ezrin was concentrated. As these Rdx(-)(/)(-) mice grew, ezrin-based cochlear stereocilia progressively degenerated, causing deafness, whereas ezrin-based vestibular stereocilia were maintained normally in adult Rdx(-)(/)(-) mice. Thus, we concluded that radixin is indispensable for the hearing ability in mice through the maintenance of cochlear stereocilia, once developed. In Rdx(-)(/)(-) mice, ezrin appeared to compensate for radixin deficiency in terms of the development of cochlear stereocilia and the development/maintenance of vestibular stereocilia. These findings indicated the existence of complicate functional redundancy in situ among ERM proteins. |
