Morphological analysis and gene therapy targeted the vestibular organ with Connexin 26 related hereditary deafness
| Project/Area Number |
26462567
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Otorhinolaryngology
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| Research Institution | Juntendo University |
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Principal Investigator |
OKADA Hiroko 順天堂大学, 医学部, 助教 (20433774)
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| Co-Investigator(Kenkyū-buntansha) |
神谷 和作 順天堂大学, 医学部, 准教授 (10374159)
飯塚 崇 順天堂大学, 医学部, 非常勤助教 (40372932)
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| Project Period (FY) |
2014-04-01 – 2017-03-31
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| Project Status |
Completed (Fiscal Year 2016)
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| Budget Amount *help |
¥4,940,000 (Direct Cost: ¥3,800,000、Indirect Cost: ¥1,140,000)
Fiscal Year 2016: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2014: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | 遺伝性難聴 / GJB2 / コネキシン26 / 前庭 / トランスジェニックマウス / 遺伝子治療 / アデノ随伴ウィルス / 前庭機能 / Gjb2 |
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| Outline of Final Research Achievements |
We analyzed the morphological and functional development of the vestibular organ in Cx26 transgenic mice between 0 days after birth (P0) and P140, which was compared with that of littermate control mice (non-Tg). The gross structure of the inner ear in non-transgenic and transgenic mice revealed no hydrops, no defects, no degeneration in either the cochlea or the vestibule throughout the postnatal period. Light microscopic observations in the sensory epithelium revealed normally developed and matured utricle macula, saccular macula, and ampulla in the transgenic mice, which were very similar to those of the nontransgenic mouse. The present study clearly demonstrated that postnatal development and maturation in the vestibular organ were morphologically and functionally completed in Cx26 transgenic mice. Furthermore, we examined the gene transfer with adeno associated virus (AAV) via semicircular canal and successfully delivered AAV into the vestibular cells.
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Report
(4 results)
Research Products
(20 results)
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[Journal Article] Deformation of the Outer Hair Cells and the Accumulation of Caveolin-2 in Connexin 26 Deficient Mice.2015
Author(s)
Anzai, T., Fukunaga, I., Hatakeyama, K., Fujimoto, A., Kobayashi, K., Nishikawa, A., Aoki, T., Noda, T., Minowa, O., Ikeda, K., and Kamiya, K
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Journal Title
PloS one
Volume: 10
Issue: 10
Pages: e0141258-e0141258
DOI
Related Report
Peer Reviewed / Open Access / Acknowledgement Compliant
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[Presentation] Differentiation of mouse iPS cell into Cx26-positive cell and formation of inter cellular Cx26-gap junction plaque2016
Author(s)
Ichiro Fukunaga, Kaori Hatakeyama,Toru Aoki, Atena Nishikawa, Ayumi Fujimoto, Katsuhisa Ikeda, Kazusaku Kamiya
Organizer
Association for Research in Otolaryngology (ARO), 39th Annual MidWinter Meeting
Place of Presentation
Manchester Grand Hyatt, San Diego, CA, USA
Year and Date
2016-02-20
Related Report
Int'l Joint Research
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[Presentation] Induction of iPS cell differentiation into Connexin26 positive cells with gap junction plaques using cochlear feeder cell2016
Author(s)
Ayumi Fujimoto, Ichiro Fukunaga, Kaori Hatakeyama, Toru Aoki, Atena Nishikawa, Katsuhisa Ikeda, Kazusaku Kamiya
Organizer
Association for Research in Otolaryngology (ARO), 39th Annual MidWinter Meeting
Place of Presentation
Manchester Grand Hyatt, San Diego, CA, USA
Year and Date
2016-02-20
Related Report
Int'l Joint Research
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[Presentation] Cochlear gap junction plaque, stabilized macromolecular complex composed of specific connexins2015
Author(s)
Kazusaku Kamiya, Ichiro Fukunaga, Kaori Hatakeyama, Toru Aoki, Ayumi Fujimoto, Atena Nishikawa ,Takashi Anzai, Osamu Minowa, Katsuhisa Ikeda
Organizer
52nd Inner Ear Biology Workshop
Place of Presentation
Campus of the Catholic University of Rome, Italy
Year and Date
2015-09-12
Related Report
Int'l Joint Research
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