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Published 16 August 2004. doi:10.1083/jcb.200402007
The Rockefeller University Press, 0021-9525 $8.00
JCB, Volume 166, Number 4, 559-570
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Article

 Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia

Shin-ichiro Kitajiri1,2,3, Kanehisa Fukumoto1,4, Masaki Hata5, Hiroyuki Sasaki5,6, Tatsuya Katsuno1,7, Takayuki Nakagawa3, Juichi Ito3, Shoichiro Tsukita1,2, and Sachiko Tsukita1,2,8

1 Department of Cell Biology, Faculty of Medicine, Kyoto University, Sakyo-ku, Kyoto 606-8501, Japan
2 Solution Oriented Research for Science and Technology, Japan Science and Technology Corporation, Sakyo-ku, Kyoto 606-8501, Japan
3 Department of Otolaryngology Head and Neck Surgery, Faculty of Medicine, Kyoto University, Sakyo-ku, Kyoto 606-8507, Japan
4 Department of Surgery, Kyoto Prefectural University of Medicine, Kamigyo-ku, Kyoto 602-8566, Japan
5 KAN Research Institute, Kyoto Research Park, Shimogyo-ku, Kyoto 606-8317, Japan
6 Department of Molecular Cell Biology, Institute of DNA Medicine, The Jikei University School of Medicine, Nishi-Shinbashi, Minato-ku, Tokyo 105-8461, Japan
7 Department of Biological Sciences and Institute for Advanced Research, Nagoya University, Nagoya 464-8602, Japan
8 School of Health Sciences, Faculty of Medicine, Kyoto University, Sakyo-ku, Kyoto 606-8507, Japan

Address correspondence to Sachiko Tsukita, Dept. of Cell Biology, Kyoto University Faculty of Medicine, Yoshida-Konoe, Sakyo-ku, Kyoto 606-8501, Japan. Tel.: (81) 75-753-4373. Fax: (81) 75-753-4660. email: atsukita{at}mfour.med.kyoto-u.ac.jp

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 (~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.

Key Words: ERM; radixin; stereocilia; cochlea; deafness

Abbreviations used in this paper: ABR, auditory-evoked brainstem response; dB, decibel; ERM, ezrin/radixin/moesin; SPL, sound pressure level; VOR, vestibulo-ocular reflex.


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