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Analysis of Hedgehog signaling using Gorlin syndrome derived cells

Research Project

Project/Area Number 24592854
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Pathobiological dentistry/Dental radiology
Research InstitutionHyogo Medical University

Principal Investigator

NAKANO Yoshiro  兵庫医科大学, 医学部, 教授 (30360267)

Co-Investigator(Kenkyū-buntansha) TAMAOKI Tomoko (HASHIMOTO Tomoko)  兵庫医科大学, 医学部, 教授 (10172868)
KISHIMOTO Hiromitsu  兵庫医科大学, 医学部, 教授 (30291818)
NOGUCHI Kazuma  兵庫医科大学, 医学部, 准教授 (50309473)
Project Period (FY) 2012-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2014: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2013: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2012: ¥2,860,000 (Direct Cost: ¥2,200,000、Indirect Cost: ¥660,000)
KeywordsGorlin症候群 / Hedgehog signal / PTCH1 / 角化嚢胞性歯原性腫瘍 / ヘッジホッグ / 分化 / KCOT
Outline of Final Research Achievements

Hedgehog signaling plays pivotal roles in animal development and the control of adult organ homeostasis. Defect of Hedgehog receptor PTCH1 function causes Gorlin syndrome, which is a rare, dominantly inherited disease characterized by developmental anomalies and increased the risk of cancers. We have identified PTCH1 mutations in 15 Gorlin syndrome families. In order to understand the cellular events that are induced by the PTCH1 dysfunction, we have analyzed metabolites and the response against the DNA damages using Gorlin syndrome patient derived cells and non-syndromic cells. Furthermore, we established hereditary and sporadic keratocystic odontogenic tumor (KCOT) cell lines by introducing mutant CDK4, cyclin D1, and TRET. These cell lines show parakeratosis or nodule formation by increasing calcium concentration. Using these KCOT cell lines, it will be possible to analyze the mechanism of development of KCOT and the differences in sporadic or hereditary KCOT.

Report

(4 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Research-status Report
  • 2012 Research-status Report
  • Research Products

    (17 results)

All 2015 2014 2013 Other

All Journal Article (8 results) (of which Peer Reviewed: 6 results,  Open Access: 2 results,  Acknowledgement Compliant: 1 results) Presentation (8 results) Remarks (1 results)

  • [Journal Article] The Hippo pathway transcriptional co-activator, YAP, confers resistance to cisplatin in human oral squamous cell carcinoma.2015

    • Author(s)
      Yoshikawa K, Noguchi K, Nakano Y, Yamamura M, Takaoka K, Hashimoto-Tamaoki T, Kishimoto H.
    • Journal Title

      Int J Oncol

      Volume: 46 Issue: 6 Pages: 2364-2370

    • DOI

      10.3892/ijo.2015.2948

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Biallelic germline and somatic mutations in malignant mesothelioma: multiple mutations in transcription regulators including mSWI/SNF genes.2015

    • Author(s)
      Yoshikawa Y, Sato A, Tsujimura T, Otsuki T, Fukuoka K, Hasegawa S, Nakano T, Hashimoto-Tamaoki T.
    • Journal Title

      Int J Cancer

      Volume: 136 Issue: 3 Pages: 560-571

    • DOI

      10.1002/ijc.29015

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] チーム医療による口腔癌治療2014

    • Author(s)
      野口 一馬
    • Journal Title

      兵庫医科大学医学会雑誌

      Volume: 39 Pages: 53-58

    • Related Report
      2014 Annual Research Report
  • [Journal Article] Nevoid basal cell carcinoma syndrome with a unilateral giant ovarian fibroma in a Japanese 6-year-old girl.2014

    • Author(s)
      T. Jimbo, K. Masumoto, Y. Urita, H. Takayasu, T. Shinkai, T. Uesugi, C. Gotoh, N. Sakamoto, T. Sasaki, T. Oto, T. Fukushima, E. Noguchi, Y. Nakano.
    • Journal Title

      Eur J Pediatr

      Volume: 173 Issue: 5 Pages: 667-670

    • DOI

      10.1007/s00431-013-2200-7

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] 口腔扁平上皮癌の発生におけるHuman papillomavirus (HPV)感染の関与と診断法の検討.2013

    • Author(s)
      野口一馬,寺田友紀,中野芳朗,山村倫世,吉川恭平,頭司雄介,森寺邦康,高岡一樹,浦出雅裕,岸本裕充.
    • Journal Title

      日本口腔感染症学会雑誌

      Volume: 20 Pages: 70-76

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] 角化嚢胞性歯原性腫瘍細胞株の樹立と病態モデル作成の試み.2013

    • Author(s)
      野口一馬,中野芳朗,山村倫世,吉川恭平,浦出雅裕,岸本裕充.
    • Journal Title

      口腔組織培養学会誌

      Volume: 22 Pages: 27-34

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] ヘッジホッグ情報伝達の破綻による形態異常と腫瘍形成2013

    • Author(s)
      中野 芳朗
    • Journal Title

      兵医大医会誌

      Volume: 37 Pages: 29-34

    • Related Report
      2012 Research-status Report
  • [Journal Article] Functional analysis of Zyxin in cell migration and invasive potential of oral squamous cell carcinoma cells.2013

    • Author(s)
      Yamamura M, Noguchi K, Nakano Y, Segawa E at al.
    • Journal Title

      Int J Oncol

      Volume: 42(3) Issue: 3 Pages: 873-80

    • DOI

      10.3892/ijo.2013.1761

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Presentation] Hedgehog signaling and human disease2014

    • Author(s)
      Nakano Yoshiro, Noguchi Kazuma, Chiyo Hideaki, Pooh Ritsuko, Kishimoto Hiromitu, Tamaoki tomoko.
    • Organizer
      The 37th Annual Meeting of the Molecular Biology Society of Japan
    • Place of Presentation
      Yokohama
    • Year and Date
      2014-11-25 – 2014-11-27
    • Related Report
      2014 Annual Research Report
  • [Presentation] シスプラチン耐性口腔扁平上皮癌におけるHippo pathwayの関与の検討2014

    • Author(s)
      吉川 恭平,野口 一馬,山村 倫世,頭司 雄介,高岡 一樹,岸本 裕充
    • Organizer
      第51回日本口腔組織培養学会学術大会・総会
    • Place of Presentation
      小倉
    • Year and Date
      2014-11-15
    • Related Report
      2014 Annual Research Report
  • [Presentation] 口腔癌におけるTNM分類のための画像診断と外科的切除の実際2014

    • Author(s)
      野口 一馬,石蔵 礼一
    • Organizer
      第50回日本医学放射線学会秋季臨床大会
    • Place of Presentation
      神戸
    • Year and Date
      2014-09-26
    • Related Report
      2014 Annual Research Report
  • [Presentation] 次世代シークエンサーの地域医療教育への導入2014

    • Author(s)
      中野 芳朗, 玉置 知子[橋本], 久保 秀司, 吉川 良恵, 振津 かつみ, 若林 一郎, 今西 宏安, 高橋 敬子, 成瀬 均, 鈴木 敬一郎
    • Organizer
      第46回日本医学教育学会大会
    • Place of Presentation
      和歌山
    • Year and Date
      2014-07-18 – 2014-07-19
    • Related Report
      2014 Annual Research Report
  • [Presentation] 臨床遺伝学分野での臨床実習 遺伝カウンセリング、次世代シークエンサー解析の導入2014

    • Author(s)
      玉置 知子[橋本], 覚道 真理子, 佐藤 智佳, 振津 かつみ, 吉川 良恵, 久保 秀司, 中野 芳朗, 高橋 敬子, 成瀬 均, 鈴木 敬一郎
    • Organizer
      第46回日本医学教育学会大会
    • Place of Presentation
      和歌山
    • Year and Date
      2014-07-18 – 2014-07-19
    • Related Report
      2014 Annual Research Report
  • [Presentation] Greig症候群の家系例2014

    • Author(s)
      玉置 知子[橋本], 千代 豪昭, 夫 律子, 岸本 裕充, 覚道 真理子, 佐藤 智佳, 井上 佳代, 振津 かつみ, 三村 博子, 中野 芳朗
    • Organizer
      第38回日本遺伝カウンセリング学会学術集会
    • Place of Presentation
      東大阪
    • Year and Date
      2014-06-27
    • Related Report
      2014 Annual Research Report
  • [Presentation] Characterization of Golin syndrome-derived cells.2014

    • Author(s)
      Nakano Yoshiro, Noguchi Kazuma, Kishimoto Hiromitsu, Hashimoto-Tamaoki Tomoko.
    • Organizer
      Keystone Symposium Developmental Pathways and Cancer:Wnt, Notch and Hedgehog.
    • Place of Presentation
      Canada Banff
    • Related Report
      2013 Research-status Report
  • [Presentation] 基底細胞母斑症候群(Gorlin-Golz症候群)の家族例 30年にわたる遺伝カウンセリング2013

    • Author(s)
      玉置知子, 中野芳朗, 高橋千晶, 覚道真理子, 佐藤智佳, 三村博子, 千代豪昭
    • Organizer
      第37回日本遺伝カウンセリング学会学術集会
    • Place of Presentation
      川崎
    • Related Report
      2013 Research-status Report
  • [Remarks] 兵庫医科大学

    • URL

      http://www.hyo-med.ac.jp

    • Related Report
      2014 Annual Research Report

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Published: 2013-05-31   Modified: 2019-07-29  

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