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論文
- タイトル
- タイトル（英）
- Myosin VI Haploinsufficiency Reduced Hearing Ability in Mice.
- 参照URL
- https://researchmap.jp/Yoshiaki_Kikkawa/published_papers/35697666
- 著者
- 著者（英）
- Yuta Seki,Hiroshi Shitara,Rie Ishii,Takafumi Ouchi,Shumpei P Yasuda,Yoshiaki Kikkawa
- 担当区分
- 概要
- 概要（英）
- In human, myosin VI (MYO6) haploinsufficiency causes postlingual progressive hearing loss. Because the usefulness of mouse models remains unclear, we produced novel Myo6 null (-/-) mutant mice and analyzed the hearing phenotypes of Myo6+/- (+/-) heterozygous mutants. We first recorded and compared the auditory brainstem responses and distortion product otoacoustic emissions in control Myo6+/+ (+/+) wild-type and +/- mice. These hearing phenotypes of +/- mice were mild; however, we confirmed that +/- mice developed progressive hearing loss. In particular, the hearing loss of female +/- mice progressed faster than that of male +/- mice. The stereocilia bundles of +/- mice exhibited progressive taper loss in cochlear inner hair cells (IHCs) and outer hair cells (OHCs). The loss of OHCs in +/- heterozygotes occurred at an earlier age than in +/+ mice. In particular, the OHCs at the basal area of the cochlea were decreased in +/- mice. IHC ribbon synapses from the area at the base of the cochlea were significantly reduced in +/- mice. Thus, our study indicated that MYO6 haploinsufficiency affected the detection of sounds in mice, and we suggest that +/- mice with Myo6 null alleles are useful animal models for gene therapy and drug treatment in patients with progressive hearing loss due to MYO6 haploinsufficiency.
- 出版者・発行元
- 出版者・発行元（英）
- 誌名
- 誌名（英）
- Neuroscience
- 巻
- 号
- 開始ページ
- 終了ページ
- 出版年月
- 2021年10月4日
- 査読の有無
- 査読有り
- 招待の有無
- 掲載種別
- 研究論文（学術雑誌）
- ISSN
- DOI URL
- https://doi.org/10.1016/j.neuroscience.2021.09.023
- 共同研究・競争的資金等の研究課題