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Bibliographic Record
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- Material Type
- 文書・図像類
- Author/Editor
- 樋口, 逸郎
- Author Heading
- Publication, Distribution, etc.
- Alternative Title
- Study on the development of treatments for collagen VI related myopathy
- Text Language Code
- jpn
- Subject Heading
- Target Audience
- 一般
- Note (General)
- 2009-2011年度科学研究費助成事業(科学研究費補助金(基盤研究(C)))研究成果報告書 課題番号：21591094 研究代表者：樋口逸郎 (鹿児島大学医学部・歯学部附属病院講師)6型コラーゲン関連疾患の治療法開発のためUllrich病やBethlemミオパチーを長期経過観察している。Bethlemミオパチーの歩行困難の主因は関節拘縮であり、整形外科的治療により歩行可能期間が延長した。非定型例発掘のため関節拘縮や過伸展を呈する診断未確定例について6型コラーゲンの３遺伝子および関連蛋白遺伝子について遺伝子解析を続けている。また、thrombomodulinがUllrich病線維芽細胞の接着能を改善し、6型コラーゲンの発現を増強することを解明した。We have been continuing a long-term follow-up of collagen VI related diseases as Ullrich disease and Bethlem myopathy. The main cause of difficulty in walking in Bethlem myopathy was joint contracture, and the ambulatory period was extended by the orthopedic treatment. We have been performing genetic analysis of genes of collagen VI and related proteins. We found that recombinant thrombomodulin can improve the adhesive capacity of fibroblasts and increase the expression of collagen VI in Ullrich disease.