A study to establish individualized medicine of cystic fibrosis using intestinal organoids derived from patients
Project/Area Number |
16K09392
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Gastroenterology
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Research Institution | Nagoya University |
Principal Investigator |
Ishiguro Hiroshi 名古屋大学, 総合保健体育科学センター, 教授 (90303651)
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Co-Investigator(Kenkyū-buntansha) |
相馬 義郎 国際医療福祉大学, 薬学部, 教授 (60268183)
山本 明子 名古屋大学, 総合保健体育科学センター, 准教授 (60402385)
洪 繁 慶應義塾大学, 医学部(信濃町), 准教授 (90402578)
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Co-Investigator(Renkei-kenkyūsha) |
NAKAKUKI Miyuki 名古屋大学, 総合保健体育科学センター, 研究員 (30578729)
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Project Period (FY) |
2016-04-01 – 2019-03-31
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Project Status |
Completed (Fiscal Year 2018)
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Budget Amount *help |
¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2018: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2017: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
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Keywords | 嚢胞性線維症 / CFTR / 腸管オルガノイド / オルガノイド / 遺伝子 / 細胞・組織 / 生理学 / 内科 |
Outline of Final Research Achievements |
Cystic fibrosis is caused by loss of CFTR function due to severe mutations in both alleles of the CFTR gene. It is an intractable disease that lumen of airway, intestine, pancreatic duct, etc. is obstructed with sticky mucus. To examine CFTR-dependent intestinal secretion, in the present study, organoids were established from small and large intestine and pancreatic duct of a mouse model of cystic fibrosis and samples of human large intestine. Principle investigator organizes the patient registry where 18 patients were registered during 2016-2018. When dele 16-17b-CFTR, the most frequent mutation in Japan, was expressed in CHO cells, maturation of CFTR protein was not sufficient and the mutant CFTR did not reach plasma membrane.
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Academic Significance and Societal Importance of the Research Achievements |
嚢胞性線維症はヨーロッパ人種に多くアジア人種では稀な疾患であるが、日本の患者は重症が多く2018年現在の平均生存期間は約23年である。原因となっている変異CFTRに対する分子治療薬の使用に向けて、基盤となるデータが必要とされている。本研究では、生体に近い条件で変異CFTRの性質を解析するために、患者の腸管粘膜サンプルからオルガノイドを作成し、CFTRに依存する腸液分泌を測定する方法を確立した。また、患者レジストリの事務局として蓄積した臨床データをもとにして診療の手引きを改訂し、主治医を含む医療関係者、患者とその家族に還元した。
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Report
(4 results)
Research Products
(37 results)
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[Journal Article] Ethnicity impacts the cystic fibrosis diagnosis: A note of caution.2017
Author(s)
Bosch B, Bilton D, Sosnay P, Raraigh KS, Mak DY, Ishiguro H, Gulmans V, Thomas M, Cuppens H, Amaral M, De Boeck K.
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Journal Title
J Cyst Fibros
Volume: 16
Issue: 4
Pages: 488-491
DOI
Related Report
Peer Reviewed / Int'l Joint Research
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[Presentation] Clinical characteristics, prognosis, and CFTR mutations of cystic fibrosis in Japan2018
Author(s)
Ishiguro H, Yamamoto A, Fujiki K, Nakakuki M, Taniguchi I, Kozawa Y, Sohma Y, Naruse S, Takeyama Y
Organizer
49th Annual Meeting of American Pancreatic Association
Related Report
Int'l Joint Research
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[Presentation] Nationwide epidemiological survey of cystic fibrosis in Japan2016
Author(s)
Yuka Usami, Akiko Yamamoto,Miyuki Nakakuki, Kotoyo Fujiki, Satoru Naruse Hiroshi, Hiroshi Ishiguro
Organizer
The 47th annual meeting of the Japan Pancreas Society, The 20th meeting of the International Association of Pancreatology, The 6th meeting of the Asian Oceanic Pancreatic Association
Place of Presentation
仙台
Year and Date
2016-08-04
Related Report
Int'l Joint Research
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[Presentation] Role of CFTR dysfunction in the pathogenesis of chronic pancreatitis2016
Author(s)
Akiko Yamamoto, Shiho Kondo, Kotoyo Fujiki, Miyuki Nakakuki,Yuka Kondo,Itsuka Taniguchi, Yuka Usami, Satoru Naruse, Hiroshi Ishiguro
Organizer
The 47th annual meeting of the Japan Pancreas Society, The 20th meeting of the International Association of Pancreatology, The 6th meeting of the Asian Oceanic Pancreatic Association
Place of Presentation
仙台
Year and Date
2016-08-04
Related Report
Int'l Joint Research
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[Presentation] Exocrine pancreatic function in Japanese patients with cystic fibrosis2016
Author(s)
Satoru Naruse, Shiho Kondo, Kotoyo Fujiki, Akiko Yamamoto, Miyuki Nakakuki, Toru Shimosegawa, Yoshifumi Takeyama, Hiroshi Ishiguro
Organizer
The 47th annual meeting of the Japan Pancreas Society, The 20th meeting of the International Association of Pancreatology, The 6th meeting of the Asian Oceanic Pancreatic Association
Place of Presentation
仙台
Year and Date
2016-08-04
Related Report
Int'l Joint Research
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[Book] 嚢胞性線維症の栄養ケア2018
Author(s)
藤木理代 編著、石黒 洋 監修
Total Pages
48
Publisher
名古屋大学消費生活協同組合印刷部
ISBN
9784909602015
Related Report
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