Production of a novel blistering disease model by regulating expression of collagen XVII
| Project/Area Number |
24390274
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Partial Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Dermatology
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| Research Institution | Hokkaido University |
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Principal Investigator |
Nishie Wataru 北海道大学, 医学(系)研究科(研究院), 准教授 (20443955)
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| Co-Investigator(Kenkyū-buntansha) |
UJIIE HIDEYUKI 北海道大学, 大学病院, 助教 (60374435)
SHINKUMA SATORU 北海道大学, 医学(系)研究科(研究院), 助教 (00613788)
NATSUGA KEN 北海道大学, 大学病院, 助教 (70645457)
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| Project Period (FY) |
2012-04-01 – 2016-03-31
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| Project Status |
Completed (Fiscal Year 2015)
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| Budget Amount *help |
¥17,940,000 (Direct Cost: ¥13,800,000、Indirect Cost: ¥4,140,000)
Fiscal Year 2015: ¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000)
Fiscal Year 2014: ¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000)
Fiscal Year 2013: ¥5,980,000 (Direct Cost: ¥4,600,000、Indirect Cost: ¥1,380,000)
Fiscal Year 2012: ¥6,760,000 (Direct Cost: ¥5,200,000、Indirect Cost: ¥1,560,000)
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| Keywords | 皮膚病態学 / 皮膚病理学 / 生体分子 / タンパク質 / 細胞・組織 / 酵素 / 自己免疫 |
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| Outline of Final Research Achievements |
Collagen XVII (COL17) is a hemidesmosomal molecule which keeps adhesion between the epidermis and dermis. Loss of CO17 expression due to mutation in the COL17A1 leads to blistering disease, epidermolysis bullosa (EB); in addition, autoimmunity to COL17 results in autoimmune blistering disorder bullous pemphigoid (BP). The purpose of this study is to produce a novel blistering model by regulating COL17 expression based on Tet-on system. Transgenic mice carrying human COL17 cDNA driven under the CMV promoter with Tet response element and Tet transactivator sequences driven under the K14 promoter were produced. Upon oral administration of doxycycline (DOX), the Tg mice started to express human COL17 in basal keratinocytes, following by production of IgG autoantibodies directing human COL17. The Tg mice must be useful tool for elucidating pathogenesis of EB and BP, as well as for establishing therapies for these orphan diseases.
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Report
(5 results)
Research Products
(14 results)
