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Animal Bioresource in Japan

.: Home > Animal Bioresource in Japan > 2013 > Volume 62 Number 4 > Yuki Miyasaka, Sari Suzuki, Yasuhiro Ohshiba, Kei Watanabe, Yoshihiko Sagara, Shumpei P. Yasuda, Kunie Matsuoka, Hiroshi Shitara, Hiromichi Yonekawa, Ryo Kominami, Yoshiaki Kikkawa

Compound Heterozygosity of the Functionally Null Cdh23v-ngt and Hypomorphic Cdh23ahl Alleles Leads to Early-onset Progressive Hearing Loss in Mice

Yuki Miyasaka, Sari Suzuki, Yasuhiro Ohshiba, Kei Watanabe, Yoshihiko Sagara, Shumpei P. Yasuda, Kunie Matsuoka, Hiroshi Shitara, Hiromichi Yonekawa, Ryo Kominami, Yoshiaki Kikkawa
Yuki Miyasaka, Sari Suzuki, Yasuhiro Ohshiba, Kei Watanabe, Yoshihiko Sagara, Shumpei P. Yasuda, Kunie Matsuoka, Hiroshi Shitara, Hiromichi Yonekawa, Ryo Kominami, Yoshiaki Kikkawa
Abstract :

The waltzer (v) mouse mutant harbors a mutation in Cadherin 23 (Cdh23) and is a model for Usher syndrome type 1D, which is characterized by congenital deafness, vestibular dysfunction, and prepubertal onset of progressive retinitis pigmentosa. In mice, functionally null Cdh23 mutations affect stereociliary morphogenesis and the polarity of both cochlear and vestibular hair cells. In contrast, the murine Cdh23ahl allele, which harbors a hypomorphic mutation, causes an increase in susceptibility to age-related hearing loss in many inbred strains. We produced congenic mice by crossing mice carrying the v niigata (Cdh23v-ngt) null allele with mice carrying the hypomorphic Cdh23ahl allele on the C57BL/6J background, and we then analyzed the animals’ balance and hearing phenotypes. Although the Cdh23v-ngt/ahl compound heterozygous mice exhibited normal vestibular function, their hearing ability was abnormal: the mice exhibited higher thresholds of auditory brainstem response (ABR) and rapid age-dependent elevation of ABR thresholds compared with Cdh23ahl/ahl homozygous mice. We found that the stereocilia developed normally but were progressively disrupted in Cdh23v-ngt/ahl mice. In hair cells, CDH23 localizes to the tip links of stereocilia, which are thought to gate the mechanoelectrical transduction channels in hair cells. We hypothesize that the reduction of Cdh23 gene dosage in Cdh23v-ngt/ahl mice leads to the degeneration of stereocilia, which consequently reduces tip link tension. These findings indicate that CDH23 plays an important role in the maintenance of tip links during the aging process.

Keywords :
Yuki Miyasaka, Sari Suzuki, Yasuhiro Ohshiba, Kei Watanabe, Yoshihiko Sagara, Shumpei P. Yasuda, Kunie Matsuoka, Hiroshi Shitara, Hiromichi Yonekawa, Ryo Kominami, Yoshiaki Kikkawa

Date Deposited : 06 Apr 2015 13:00

Last Modified : 06 Apr 2015 13:00

Official URL: http://https://www.jstage.jst.go.jp/browse/expanim/62/1/_contents

Volume 62, Number 4, - 2013

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