The critical role for fibroblast in RDEB-SCC patients

Research Project

Project/Area Number	17K10223
Research Category	Grant-in-Aid for Scientific Research (C)
Allocation Type	Multi-year Fund
Section	一般
Research Field	Dermatology
Research Institution	Hokkaido University
Principal Investigator	Hata Hiroo 北海道大学, 医学研究院, 助教 (90399915)
Co-Investigator(Kenkyū-buntansha)	清水宏北海道大学, 医学研究院, 教授 (00146672) 柳輝希北海道大学, 大学病院, 助教 (50755973)
Project Period (FY)	2017-04-01 – 2020-03-31
Project Status	Completed (Fiscal Year 2019)
Budget Amount *help	¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000) Fiscal Year 2019: ¥650,000 (Direct Cost: ¥500,000、Indirect Cost: ¥150,000) Fiscal Year 2018: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000) Fiscal Year 2017: ¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000)
Keywords	表皮水疱症 / 創傷治癒 / 発癌 / 7型コラーゲン / 線維芽細胞 / 有棘細胞癌 / 皮膚腫瘍学
Outline of Final Research Achievements	In this study, we performed a functional analysis of fibroblasts in a patient with recessive dystrophic epidermolysis bullosa (RDEB) to elucidate the pathophysiology of "wound healing" and "squamous cell carcinoma" in RDEB. We have identified the expression of a specific molecule in RDEB patient fibroblasts and have clarified the molecular mechanism that the molecule is involved in "impaired wound healing" and "squamous cell carcinoma". First, We have observed the incidence of squamous cell carcinoma occurring in all RDEB patients (40 cases) at the Hokkaido University Hospital. Next, we analyzed the expression of molecules (TRIM29, keratin) involved in the progression of squamous cell carcinoma. We collected clinical specimens of squamous cell carcinoma in RDEB patients.
Academic Significance and Societal Importance of the Research Achievements	本研究成果の学術的意義として、7型コラーゲン欠損が創傷治癒と発癌機構に与える影響について検討した点がある。特に7型コラーゲン欠損線維芽細胞が、創傷治癒障害と有棘細胞癌の発生に関与するメカニズムに注目したことがあげられる。新規治療ターゲットとなる分子へのアプローチが可能となる可能性がある。社会的意義として劣性栄養障害型表皮水疱症患者の生活の質（QOL）向上に役立つ可能性があると思われる。