Project/Area Number |
13357008
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Research Category |
Grant-in-Aid for Scientific Research (A)
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Allocation Type | Single-year Grants |
Section | 展開研究 |
Research Field |
Dermatology
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Research Institution | HOKKAIDO UNIVERSITY |
Principal Investigator |
SHIMIZU Hiroshi Hokkaido Univ., Grad.School of Med., Prof., 大学院・医学研究科, 教授 (00146672)
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Co-Investigator(Kenkyū-buntansha) |
FURUICHI Yasuhiro GeneCare Research Institute Co.Ltd., Manager, 所長(研究職)
SHIMIZU Tadamichi Hokkaido University, Hospital, Lec., 医学部・歯学部附属病院, 講師 (70260396)
SAWAMURA Daisuke Hokkaido Univ., Grad.School of Med., Asso.Prof., 大学院・医学研究科, 助教授 (60196334)
MASUNAGA Takuji KOSE Corporation, Chief Researcher, 主任研究員
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Project Period (FY) |
2001 – 2003
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Keywords | Wound healing / epidermolysis bullosa / dystrophic / severe type / type VII collagen / protein supplement / gene therapy / epidermal transplantation |
Research Abstract |
Epidermolysis bullosa consists of a group of genetically determined skin disorders where minor trauma causes blisters and erosions in the skin and mucous membranes. Recent advance in epidermolysis bullosa research has identified several causative genes which encoding structural proteins for stability in adhesion of the epidermis and the dermis. However, there is no effective treatment for epidermolysis bullosa. The purposes of this study are to make a precise diagnosis of the patients with epidermolysis bullosa and to develop a protein therapy based on novel strategy, in which we produce defective recombinant protein and provide it to skin lesions of the patients. In this study we performed mutation analysis of many patients in Japan as well as foreign countries. Also, we treated patients with autologous epidermal sheet graft in Hokkaido University. Furthermore, we succeeded in purification of recombinant type VII collagen which majority of severe patients with epidermolysis bullosa lack in Japan. In this study, our mutation analysis of epidermolysis bullosa resulted in disclosing new findings about epidermolysis bullosa diagnostic and pathophysiological aspects. Although protein therapies using recombinant enzymes are ongoing in clinical practice, those using structural proteins has been never tried before. This study provide a great potential of protein therapy in the future.
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