※ 本業績情報は国立研究開発法人科学技術振興機構が運営するサービス「Researchmap」に登録されている研究者データ(プロフィール、業績等)を元に表示しています。

研究業績に対する検索条件
※ スペース区切りで絞り込み検索が可能です。
研究業績タイプによる絞り込み条件です。絞り込みは行っていません。
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
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
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
研究者
吉川 欣亮 (キッカワ ヨシアキ)