Analysis of Gorlin syndrome derived benign or malignant tumor
| Project/Area Number |
15K11098
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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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| Research Field |
Pathobiological dentistry/Dental radiology
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| Research Institution | Hyogo Medical University |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
玉置 知子 (橋本知子) 兵庫医科大学, 医学部, 名誉教授 (10172868)
山西 清文 兵庫医科大学, 医学部, 教授 (10182586)
岸本 裕充 兵庫医科大学, 医学部, 教授 (30291818)
野口 一馬 兵庫医科大学, 医学部, 准教授 (50309473)
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| Project Period (FY) |
2015-04-01 – 2018-03-31
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| Project Status |
Completed (Fiscal Year 2017)
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| Budget Amount *help |
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2017: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2015: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
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| Keywords | Gorlin症候群 / 角化嚢胞性歯原性腫瘍 / hedgehog情報伝達 / PTCH1 / SMO / 基底細胞癌 / Hedgehog情報伝達系 / 分化誘導 / Hedgehog / 角化嚢胞性歯原性腫瘍（KCOT） / 分化 |
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| Outline of Final Research Achievements |
Gorlin syndrome is a dominantly inherited autosomal disease with a high frequency of tumor induction caused by the hedgehog receptor PTCH1. We analyzed PTCH1 and SMO alternation in Gorlin syndrome-associated tumors, such as malignant basal cell carcinoma, benign keratocystic odontogenic tumor (KCOT), and ovarian fibroma, revealing that the mutational status of PTCH1 or SMO itself does not account for differences in malignancy and tissue-specificity of these tumors.
KCOT is the most frequent benign tumor in Gorlin syndrome patients. We established KCOT cell lines from sporadic and Gorlin syndrome-derived KCOTs. Both lines showed stem and mesenchymal cell characteristics. However, in the presence of calcium, cells showed parakeratosis and epithelial or keratinocyte-related proteins were induced. These features were similar to those of in vivo KCOTs. Using these cell lines and 3-D hydrogel culture, we may be able to advance understanding of the nature and origin of KCOTs.
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Report
(4 results)
Research Products
(19 results)
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[Presentation] Hedgehog signaling and genetic diseases.2016
Author(s)
Yoshiro Nakano, Kazuma Noguchi, Hideaki Chiyo, Ritsuko Pooh, Hiromitsu Kishimoto, Tomoko Hashimoto-Tamaoki
Organizer
The 13th International Congress of Human Genetics
Place of Presentation
京都国際会議場（京都府京都市）
Related Report
Int'l Joint Research
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