• Search Research Projects
  • Search Researchers
  • How to Use
  1. Back to previous page

In vitro "virtual biopsy" of cochlear cells by using Wolfram syndrome patients derived hiPSCs

Research Project

Project/Area Number 15K15624
Research Category

Grant-in-Aid for Challenging Exploratory Research

Allocation TypeMulti-year Fund
Research Field Otorhinolaryngology
Research InstitutionKeio University

Principal Investigator

Fujioka Masato  慶應義塾大学, 医学部(信濃町), 講師 (70398626)

Co-Investigator(Kenkyū-buntansha) 小川 郁  慶應義塾大学, 医学部, 教授 (00169179)
大石 直樹  慶應義塾大学, 医学部, 講師 (10348740)
Project Period (FY) 2015-04-01 – 2018-03-31
Project Status Completed (Fiscal Year 2017)
Budget Amount *help
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2016: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2015: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Keywords疾患iPS創薬 / WFS1 / Wolfram症候群 / 遺伝性難聴 / コモンマーモセット / 橋渡し研究 / ヒトiPS細胞
Outline of Final Research Achievements

We identified the expression pattern of WFS1 gene in the primate cochlea using a small non-human primate common marmoset (Suzuki N, 2016). In marmosets, unlike rodents, WFS1 was also expressed in the basal cell of stria vascularis, where severe atrophy was reported in the temporal bone of Wolfram syndrome patients. Based on these findings, we generated WFS1 positive cochlear cells from human iPSCs and its function was analyzed.

Report

(4 results)
  • 2017 Annual Research Report   Final Research Report ( PDF )
  • 2016 Research-status Report
  • 2015 Research-status Report
  • Research Products

    (37 results)

All 2017 2016 2015 Other

All Int'l Joint Research (1 results) Journal Article (7 results) (of which Int'l Joint Research: 1 results,  Peer Reviewed: 7 results,  Open Access: 5 results,  Acknowledgement Compliant: 3 results) Presentation (27 results) (of which Int'l Joint Research: 11 results,  Invited: 7 results) Remarks (2 results)

  • [Int'l Joint Research] ノースウェスタン大学(米国)

    • Related Report
      2015 Research-status Report
  • [Journal Article] Prevalence of TECTA mutation in patients with mid-frequency sensorineural hearing loss.2017

    • Author(s)
      Yamamoto N, Mutai H, Namba K, Morita N, Masuda S, Nishi Y, Nakano A, Masuda S, Fujioka M, Kaga K, Ogawa K, Matsunaga T.
    • Journal Title

      Orphanet J Rare Dis

      Volume: 25;12(1) Issue: 1 Pages: 157-157

    • DOI

      10.1186/s13023-017-0708-z

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Cochlear Cell Modeling Using Disease-Specific iPSCs Unveils a Degenerative Phenotype and Suggests Treatments for Congenital Progressive Hearing Loss2017

    • Author(s)
      Makoto Hosoya, Masato Fujioka, Takefumi Sone, Satoshi Okamoto, Wado Akamatsu, Hideki Ukai, Hiroki R. Ueda, Kaoru Ogawa, Tatsuo Matsunaga, Hideyuki Okano
    • Journal Title

      Cell Reports

      Volume: 18 Issue: 1 Pages: 68-81

    • DOI

      10.1016/j.celrep.2016.12.020

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Expression pattern of wolframin, the WFS1 (Wolfram syndrome-1 gene) product, in common marmoset (Callithrix jacchus) cochlea.2016

    • Author(s)
      Suzuki N, Hosoya M, Oishi N, Okano H, Fujioka M, Ogawa K.
    • Journal Title

      Neuroreport.

      Volume: 3;27(11) Issue: 11 Pages: 833-6

    • DOI

      10.1097/wnr.0000000000000624

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Distinct Expression Patterns Of Causative Genes Responsible For Hereditary Progressive Hearing Loss In Non-Human Primate Cochlea.2016

    • Author(s)
      Hosoya M, Fujioka M, Ogawa K, Okano H
    • Journal Title

      Scientific Reports

      Volume: 26;6 Issue: 1 Pages: 22250-22250

    • DOI

      10.1038/srep22250

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Sustained Effect of Hyaluronic Acid in Subcutaneous Administration to the Cochlear Spiral Ganglion.2016

    • Author(s)
      Inagaki Y, Fujioka M, Kanzaki S, Watanabe K, Oishi N, Itakura G, Yasuda A, Shibata S, Nakamura M, Okano HJ, Okano H, Ogawa K.
    • Journal Title

      PLoS One in press

      Volume: 11 Issue: 4 Pages: e0153957-e0153957

    • DOI

      10.1371/journal.pone.0153957

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Overlapping expression of anion exchangers in the cochlea of a non-human primate suggests functional compensation.2016

    • Author(s)
      Hosoya M, Fujioka M, Kobayashi R, Okano H, Ogawa K.
    • Journal Title

      Neuroscience Research

      Volume: S0168-0102(16) Pages: 3000006-3000006

    • DOI

      10.1016/j.neures.2016.04.002

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] ERK2 mediates inner hair cell survival and decreases susceptibility to noise-induced hearing loss2015

    • Author(s)
      Kurioka T, Matsunobu T, Satoh Y, Niwa K, Endo S, Fujioka M, Shiotani A.
    • Journal Title

      Scientific Reports

      Volume: 18;5 Issue: 1 Pages: 16839-16839

    • DOI

      10.1038/srep16839

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] 両側進行性難聴の病態解明と新規治療法開発~iPS創薬の立場から~2017

    • Author(s)
      藤岡正人
    • Organizer
      第17回日本抗加齢医学会総会
    • Related Report
      2017 Annual Research Report
  • [Presentation] Cochlear cell modeling using disease-specific iPSCs unveiled a degenerative phenotype and treatments of a congenital progressive hearing loss2017

    • Author(s)
      Makoto Hosoya、Masato Fujioka , Tatsuo Matsunaga , Kaoru Ogawa ,
    • Organizer
      ENT World Congress IFOS Paris 2017
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] 『両側進行性難聴に対するiPS細胞研究~モデル動物不在の疾患への挑戦~』2017

    • Author(s)
      藤岡正人
    • Organizer
      BIOtech 第16回バイオ・ライフサイエンス研究展
    • Related Report
      2017 Annual Research Report
  • [Presentation] Cochlear Cell Modeling Using Disease-Specific iPSCs Unveils a Degenerative Phenotype and Suggests Treatments for Congenital Progressive Hearing Loss2017

    • Author(s)
      Masato Fujioka
    • Organizer
      8 th World Gene Convention-2017
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Pendred症候群患者特異的iPS細胞由来内耳細胞におけるPENDRINタンパク質の局在の検討2017

    • Author(s)
      佐伯翼・藤岡正人・細谷誠・西山崇経・松崎佐栄子・松永達雄・小川郁
    • Organizer
      第27回日本耳科学会総会・学術講演会
    • Related Report
      2017 Annual Research Report
  • [Presentation] EYA4遺伝子変異特異的疾患iPS細胞の樹立2017

    • Author(s)
      松崎佐栄子・藤岡正人・細谷誠・西山崇経・松崎佐栄子・松永達雄・小川郁
    • Organizer
      第27回日本耳科学会総会・学術講演会
    • Related Report
      2017 Annual Research Report
  • [Presentation] 内耳性難聴に対するiPS細胞を用いた創薬研究2017

    • Author(s)
      藤岡正人
    • Organizer
      第38回大阪耳疾患セミナー
    • Related Report
      2017 Annual Research Report
  • [Presentation] Predicting clinical effects of therapeutics by iPS-based in vitro model of genetic deafness: another promising approach of precision medicine2017

    • Author(s)
      Masato Fujioka
    • Organizer
      国際検討会 Presicion Medicine For Deafness
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] Therapeutics in genetic deafness by iPS-based in vitro model2017

    • Author(s)
      Masato Fujioka
    • Organizer
      臺大醫院演講
    • Related Report
      2017 Annual Research Report
    • Int'l Joint Research / Invited
  • [Presentation] 老化と聴力2017

    • Author(s)
      藤岡正人
    • Organizer
      百寿総合研究センターセミナー
    • Related Report
      2017 Annual Research Report
    • Invited
  • [Presentation] 両側進行性内耳性難聴の病態生理研究~疾患iPS創薬の立場から~2016

    • Author(s)
      藤岡正人・細谷誠・鈴木法臣・大石直樹・松崎佐栄子・小川郁
    • Organizer
      第26回日本耳科学会総会・学術講演会
    • Place of Presentation
      ホテル国際21(長野県長野市)
    • Year and Date
      2016-10-05
    • Related Report
      2016 Research-status Report
  • [Presentation] Minding species gaps in the cochlea: non-human primate and patient derived- hiPSC models for the study of hereditary hearing loss2016

    • Author(s)
      藤岡正人
    • Organizer
      IEB 2016
    • Place of Presentation
      モンペリエ,フランス
    • Year and Date
      2016-09-19
    • Related Report
      2016 Research-status Report
    • Int'l Joint Research
  • [Presentation] 母親にのみ新規<I>EYA4</I>遺伝子変異を認めた家族性難聴の1例2016

    • Author(s)
      藤岡正人・松永達雄・長谷部夏希・神崎晶・大石直樹・平賀良彦・鈴木法臣・松崎佐栄子・川浦光弘・小川郁
    • Organizer
      第78回耳鼻咽喉科臨床学会総会・学術講演会
    • Place of Presentation
      城山観光ホテル(鹿児島県鹿児島市)
    • Year and Date
      2016-06-23
    • Related Report
      2016 Research-status Report
  • [Presentation] Cochlear disease modeling and drug discovery by using human iPSC-based technologies2016

    • Author(s)
      Masato Fujioka
    • Organizer
      16th Japan-Korea Joint Meeting of Otorhinolaryngology-Head and Neck Surgery
    • Place of Presentation
      ハイアットリージェンシー東京(東京都新宿区)
    • Year and Date
      2016-03-29
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research / Invited
  • [Presentation] Translating back and forth in between benchside and bedside by hiPS based-technology and a primate model (invited speaker)2015

    • Author(s)
      Masato Fujioka
    • Organizer
      The 13rd. Japan-Taiwan Conference on Otolaryngology-Head and Neck Surgery,
    • Place of Presentation
      一橋大学一橋講堂(東京都千代田区)
    • Year and Date
      2015-12-02
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research / Invited
  • [Presentation] Expression pattern of Wolframin, the Wolfram syndrome 1 gene (WFS1) product, in Common Marmoset (Callithrix jacchus) inner ear.2015

    • Author(s)
      Noriomi Suzuki, Masato Fujioka, Makoto Hosoya, Kaoru Ogawa
    • Organizer
      The 13rd. Japan-Taiwan Conference on Otolaryngology-Head and Neck Surgery,
    • Place of Presentation
      一橋大学一橋講堂(東京都千代田区)
    • Year and Date
      2015-12-02
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] コモンマーモセット蝸牛におけるSLC26AおよびSLC4Aファミリー陰イオン交換輸送体の発現パターンの検討2015

    • Author(s)
      細谷誠 藤岡正人 小川郁
    • Organizer
      第60回日本聴覚医学会総会・学術講演会
    • Place of Presentation
      京王プラザホテル(東京都新宿区)
    • Year and Date
      2015-10-21
    • Related Report
      2015 Research-status Report
  • [Presentation] 9.4T MRIを用いたヒト固定側頭骨の高解像度描出のこころみ(第2報)2015

    • Author(s)
      藤岡正人 大石 直樹・栗原 渉・神崎 晶・小島 博己・小川 郁
    • Organizer
      第60回日本聴覚医学会総会・学術講演会
    • Place of Presentation
      京王プラザホテル(東京都新宿区)
    • Year and Date
      2015-10-21
    • Related Report
      2015 Research-status Report
  • [Presentation] コモンマーモセット蝸牛におけるCRYM遺伝子、CONNEXIN31遺伝子およびCOCH遺伝子の発現パターンの検討2015

    • Author(s)
      細谷 誠、藤岡 正人、小川 郁
    • Organizer
      日本耳科学会学術講演会
    • Place of Presentation
      長崎ブリックホール(長崎県長崎市)
    • Year and Date
      2015-10-08
    • Related Report
      2015 Research-status Report
  • [Presentation] 再生医学的アプローチと幹細胞医学による内耳性難聴の研究2015

    • Author(s)
      藤岡 正人
    • Organizer
      日本耳科学会学術講演会・聴覚生理研究会
    • Place of Presentation
      長崎ブリックホール(長崎県長崎市)
    • Year and Date
      2015-10-08
    • Related Report
      2015 Research-status Report
    • Invited
  • [Presentation] MYO15A新規遺伝子変異症例からの疾患特異的iPS細胞の樹立と解析2015

    • Author(s)
      藤岡正人、細谷 誠・松永 達雄・小川 郁・岡野 栄之
    • Organizer
      第38回日本神経科学大会
    • Place of Presentation
      Roma, Italy
    • Year and Date
      2015-09-14
    • Related Report
      2015 Research-status Report
  • [Presentation] Inflammatory and immune responses in the cochlea: perspective of basic research toward clinics2015

    • Author(s)
      Masato Fujioka
    • Organizer
      Inner Ear Biology Workshop - IEB2015
    • Place of Presentation
      Roma, Italy
    • Year and Date
      2015-09-12
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research / Invited
  • [Presentation] COMMON MARMOSET&;(CALLITHRIX JACCHUS): A USEFUL NON-HUMAN&#160;PRIMATE MODEL FOR HEARING AND AUDIOLOGY RESEARCH2015

    • Author(s)
      Noriomi Suzuki, Masato Fujioka, Makoto Hosoya, Kaoru Ogawa
    • Organizer
      Inner Ear Biology Workshop - IEB2015
    • Place of Presentation
      Roma, Italy
    • Year and Date
      2015-09-12
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Expression pattern of GRHL2, an age-related hearing impairment gene, in Common Marmoset (Callithrix jacchus) inner ear2015

    • Author(s)
      Makoto Hosoya, Noriomi Suzuki, Masato Fujioka, Kaoru Ogawa
    • Organizer
      Inner Ear Biology Workshop - IEB2015
    • Place of Presentation
      Roma, Italy
    • Year and Date
      2015-09-12
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] PENDRED症候群疾患特異的iPS細胞由来内耳細胞を用いた薬剤スクリーニング2015

    • Author(s)
      細谷 誠、藤岡 正人、岡本 理志、曽根 岳史、松永 達雄、赤松 和土、小川 郁、岡野 栄之
    • Organizer
      第36回日本炎症・再生医学会
    • Place of Presentation
      神戸国際会議場(兵庫県神戸市)
    • Year and Date
      2015-07-28
    • Related Report
      2015 Research-status Report
  • [Presentation] ヒトES/iPS細胞をソースとした高純度内耳有毛細胞誘導系と人工蝸牛感覚上皮シート作成のこころみ2015

    • Author(s)
      藤岡正人、細谷 誠、小川 郁、岡野 栄之
    • Organizer
      第36回日本炎症・再生医学会
    • Place of Presentation
      虎ノ門ヒルズフォーラム(東京都港区)
    • Year and Date
      2015-07-21
    • Related Report
      2015 Research-status Report
  • [Presentation] PENDRED症候群疾患特異的iPS細胞由来内耳細胞を用いた薬剤スクリーニング2015

    • Author(s)
      細谷誠、藤岡正人、松永達雄、小川郁
    • Organizer
      第116回日本耳鼻咽喉科学会総会・学術講演会
    • Place of Presentation
      国際フォーラム(東京都千代田区)
    • Year and Date
      2015-05-20
    • Related Report
      2015 Research-status Report
  • [Remarks] 慶應義塾大学医学部耳鼻咽喉科学教室研究紹介

    • Related Report
      2017 Annual Research Report
  • [Remarks] 慶應義塾大学医学部耳鼻咽喉科学教室研究紹介

    • Related Report
      2016 Research-status Report

URL: 

Published: 2015-04-16   Modified: 2022-01-27  

Information User Guide FAQ News Terms of Use Attribution of KAKENHI

Powered by NII kakenhi