Research for fibrous dysplasia using mutated GNAS1 transgenic mouse model

• Project/Area Number: 24592763
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Morphological basic dentistry
• Research Institution: Osaka University
• Principal Investigator: SATO Sunao 大阪大学, 歯学研究科(研究院), 講師 (70335660)
• Co-Investigator(Kenkyū-buntansha): ABE Makoto 大阪大学, 大学院歯学研究科, 講師 (40448105) ; KISHINO Mitsunobu 大阪大学, 大学院歯学研究科, 助教 (60346161) ; 宇佐美 悠 大阪大学, 歯学部附属病院, 助教 (80444579)
• Project Period (FY): 2012-04-01 – 2015-03-31
• Project Status: Completed (Fiscal Year 2014)
• Budget Amount: ¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000); Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000); Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000); Fiscal Year 2012: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
• Keywords: 線維性骨異形成症 / Fibrous dysplasia

Outline of Final Research Achievements

Fibrous dysplasia (FD) is common fibro-osseous lesions and activating point mutation of the alpha subunit of the stimulatory G protein gene (GNAS) causes FD. For FD patients, main medical treatment is surgical resection. Design of new effective ways of pharmacological intervention relies on appropriate FD animal model, however, to date there is no animal model suitable for reproduction of FD lesion. In this study, we produced cell line with GNAS point mutation and transgenic mice expressing GNAS point mutation with osteoblast specific. We examined the feature of these cell line and transgenic mice. Cell line with GNAS point mutation showed the similar gene expression with FD patients and transgenic mice had bone morphological change compared to wild type mice.
These results suggest that cell line with GNAS point mutation and transgenic mice with osteoblast specific GNAS point mutation are a useful FD model.

Research Products

• [Presentation] Establishment of therapeutic effect assessment animal model of fibrous dtsplasia (2014)
  • Author(s): Sato S, Noda Y, Oya O, Usami Y, Kishino M, Ogawa Y, Toyosawa S
  • Organizer: 17th International Congress on Oral Pathology and Medicine
  • Place of Presentation: Istanbul, Turkey
  • Year and Date: 2014-05-25 – 2014-05-30
• [Presentation] A case of spontaneous malignant transformation of fibrous dysplasia on the right mandible (2013)
  • Author(s): 豊澤悟、佐藤淳、岸野万伸、宇佐美悠、野田百合、松本由香、小川祐三
  • Organizer: 第24回日本臨床口腔病理学会総会・学術大会
  • Place of Presentation: 日本大学理工学部1号館6階CSTホール
• [Presentation] 下顎骨腫瘤の一例 (2012)
  • Author(s): 佐藤淳、岸野万伸、宇佐美悠、小川裕三、豊澤悟
  • Organizer: 第２３回日本臨床口腔病理学会総会・学術大会
  • Place of Presentation: 東京医科歯科大学
• [Book] 病理と臨床 2015年臨時増刊号（33巻） 病理診断クイックリファレンス 線維性骨異形成症 (2015)
  • Author(s): 佐藤淳、岸野万伸
  • Total Pages: 355
  • Publisher: 文光堂