2021 Fiscal Year Final Research Report
Hair bundle formation and tonotopy in genetic hearing loss models and the iPS cells
| Project/Area Number |
18H02953
|
|---|
| Research Category |
Grant-in-Aid for Scientific Research (B)
|
| Allocation Type | Single-year Grants |
|---|
| Section | 一般 |
|---|
| Review Section |
Basic Section 56050:Otorhinolaryngology-related
|
|---|
| Research Institution | Juntendo University |
|---|
Principal Investigator |
|
|---|
| Co-Investigator(Kenkyū-buntansha) |
小池 卓二 電気通信大学, 大学院情報理工学研究科, 教授 (10282097)
安齋 崇 順天堂大学, 医学部, 助教 (20624852)
福永 一朗 順天堂大学, 大学院医学研究科, 非常勤助教 (20746581)
池田 勝久 順天堂大学, 医学部, 特任教授 (70159614)
|
|---|
| Project Period (FY) |
2018-04-01 – 2022-03-31
|
| Keywords | 蝸牛器官培養 / 遺伝性難聴 / 人工多能性幹細胞 / 難聴マウス |
|---|
| Outline of Final Research Achievements |
Recently, abnormalities in frequency discrimination have been observed in some patients with abnormal sound perception, but the cause of these abnormalities is unknown and there are no objective diagnostic criteria. We reported the mice with abnormal frequency discrimination and confirmed the predicted abnormal propagation of basal menbrane vibration from basal (high frequency) to apical (low frequency) rotation. We further elucidated the possible causes of this abnormality by simulating the cochlear model and suggested the possiblity that high frequency hearing loss model abnormally recognize the sound as a low frequency sound. Furthermore, by developing a technology to generate cochlear cells from iPS cells, they were able to reproduce the pathophysiology of hearing loss and succeeded in generating hair cells using the same method, which is expected to lead to clinical applications of inner ear cell therapy in the future.
|
| Free Research Field |
難聴
|
| Academic Significance and Societal Importance of the Research Achievements |
近年、周波数弁別の異常が散見されるがその原因は不明である。我々は遺伝子改変マウスの外有毛細胞の聴毛形成異常により、周波数弁別異常を持つ聴覚障害を発見した。同マウスは周波数弁別異常を持つ初めてのモデル動物であり、周波数弁別機構と聴覚神経系発達への影響を解明できる有用なモデルとなる。さらに我々はiPS細胞から蝸牛細胞を作製する技術開発により難聴病態を再現し、同様の方法で有毛細胞の作製を行った。周波数弁別異常マウスおよび同マウスiPS細胞由来有毛細胞を用いた生理学、分子生物学的解析により聴毛形成異常の機構や周波数弁別による聴覚神経系の適応発達の機構を解明しており、今後新しい内耳治療法が期待できる。
|
