2020 Fiscal Year Final Research Report
Odontogenic keratocyst model using iPSCs derived from patient with Golin syndrome
| Project/Area Number |
18K09753
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Review Section |
Basic Section 57060:Surgical dentistry-related
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| Research Institution | Tokyo Dental College |
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Principal Investigator |
Onodera Shoko 東京歯科大学, 歯学部, 講師 (90637662)
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| Co-Investigator(Kenkyū-buntansha) |
中村 貴 東京歯科大学, 歯学部, 講師 (80431948)
齋藤 暁子 東京歯科大学, 歯学部, 助教 (90722835)
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| Project Period (FY) |
2018-04-01 – 2021-03-31
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| Keywords | Gorlin症候群 / 疾患iPS細胞 / 遺伝子変異 / 歯原性角化嚢胞 |
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| Outline of Final Research Achievements |
We found that keratinocytes derived from G-OFiPSCs (GKCs) have increased expression of Hh target molecules. GKCs were irradiated and those cells showed high resistance to UV induced apoptosis. BCL2, known as anti-apoptotic molecule as well as Hh target, significantly increased in GKCs. Several molecules involved in DNA repair, cell cycle control, senescence, and genotoxic stress such as TP53, BRCA1 and GADD45A increased only in GKCs. GKCs are indicated to be resistant to UV irradiation by upregulating molecules which control DNA repair and genotoxic even under DNA damage caused by UV. The anti-apoptotic properties of GKCs may contribute BCC.
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| Free Research Field |
遺伝子変異性疾患
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| Academic Significance and Societal Importance of the Research Achievements |
Gorlin症候群（基底細胞母斑症候群）は日本人においては24万に1人の確立で発症する希少疾患であり、腫瘍性、嚢胞性病変の多発および骨形成異常を生じる。腫瘍性、嚢胞性病変は再発することも多いため、度重なる手術は患者のQOLを下げる原因となる。現在、根治的な治療はなく、外科的切除が第一選択となる。患者から樹立した疾患iPS細胞は、患者に生じる病態を実験上模すことができる可能性を有する細胞である。この細胞を用いて病態形成モデルを確立し、発症するメカニズムを検討することで、病気に対する薬剤などを探すことが期待できる。
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