2012 Fiscal Year Final Research Report
Generation of dominant dystrophic epidermolysis bullosa model mice using humanized COL7 mice
| Project/Area Number |
24659516
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| Research Category |
Grant-in-Aid for Challenging Exploratory Research
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| Allocation Type | Multi-year Fund |
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| Research Field |
Dermatology
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| Research Institution | Hokkaido University |
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Principal Investigator |
SHIMIZU Hiroshi 北海道大学, 大学院・医学研究科, 教授 (00146672)
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| Co-Investigator(Kenkyū-buntansha) |
SHINKUMA Satoru 北海道大学, 大学院・医学研究科, 助教 (00613788)
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| Project Period (FY) |
2012
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| Keywords | 表皮水疱症 / モデルマウス / ドミナントネガティブ効果 / グリシン置換変異 / 7型コラーゲン |
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| Research Abstract |
Dominant dystrophic epidermolysis bullosa (DDEB) is a clinically heterogeneous disorder characterized by blistering and scarring of the skin and mucous membranes in response to mechanical force. The DDEB phenotype arises because of a dominant negative effect of mutations in type VII collagen gene, resulting in the formation of structurally abnormal anchoring fibrils. There have not been DDEB model mouse. In thisstudy, we attempt to generate the DDEB model mice.
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