Elucidation of the pathophysiology of cleidocranial dysplasia using disease-specific iPS cells
| Project/Area Number |
16K20428
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| Research Category |
Grant-in-Aid for Young Scientists (B)
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| Allocation Type | Multi-year Fund |
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| Research Field |
Functional basic dentistry
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| Research Institution | Tokyo Dental College |
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Principal Investigator |
Saito Akiko 東京歯科大学, 歯学部, 助教 (90722835)
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| Project Period (FY) |
2016-04-01 – 2018-03-31
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| Project Status |
Completed (Fiscal Year 2017)
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| Budget Amount *help |
¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2017: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
Fiscal Year 2016: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
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| Keywords | 疾患iPS細胞 / 骨芽細胞分化 / 鎖骨頭蓋骨異形成症 / RUNX2 / 疾患特異的iPS細胞 / iPS細胞 |
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| Outline of Final Research Achievements |
We established cleidocranial dysplasia (CCD)-derived iPS cells (CCD-iPSCs) and also established Revertant-iPSCs in which RUNX2 mutation of CCD-iPSCs was corrected by CRISPR / Cas method. These iPSCs had pluripotency. In osteogenic induction analysis, expression of osteoblast differentiation markers such as ALP, OSX, OCN, and DLX5 was increased only in Revertant-iPSCs. In the rat calvarial bone defects transplantation experiment, the bone closure delay was observed in the CCD transplant group, and both bone volume and bone mineral content were lower than Revertant transplant group. From the above results, it was confirmed that the established CCD-iPSCs exhibited bone mineralization disorder accompanying osteoblast differentiation abnormality.
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Report
(3 results)
Research Products
(12 results)
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[Journal Article] Targeted reversion of induced pluripotent stem cells from patients with human cleidocranial dysplasia showed corrected osteoblastic differentiation both in vitro and in vivo.2018
Author(s)
Ooki A, Nakamura T, Onodera S, Hayashi K, Hasegawa D, Kato H, Onda T, Watanabe A, Kosaki K, Nishimura K, Ohtaka M, Nakanishi M, Sakamoto T, Yamaguchi A, Sueishi K, Azuma T
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Journal Title
Stem Cell Res Ther
Volume: 9
Issue: 1
Pages: 12-12
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Gorlin syndrome-derived induced pluripotent stem cells are hypersensitive to Hedgehog-mediated osteogenic induction.2017
Author(s)
Hasegawa D, Shino H, Onodera S, Nakamura T, Saito A, Onda T, Watanabe K, Nishimura K, Ohtaka M, Nakanishi M, Kosaki K, Yamaguchi A, Shibahara T, Azuma T
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Journal Title
PLos One
Volume: 12
Issue: 10
Pages: e0186879-e0186879
DOI
Related Report
Peer Reviewed / Open Access
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[Presentation] Analyses of osteogenic differentiation in iPS cells derived from cleidocranial dysplasia patients2016
Author(s)
Saito A, Oki A, Nakamura T, Onodera S, Shino H, Hasegawa D, Kosaki K, Onda T, Watanabe A, Shibahara T, Sueishi K, Azuma T
Organizer
The joint annual scientific meetings of the endocrine society of Australia, the society for reproductive biology and the Australian and new Zealand bone and mineral society
Place of Presentation
Gold Coast
Year and Date
2016-08-21
Related Report
Int'l Joint Research
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