{"created":"2023-06-19T09:10:43.848907+00:00","id":22263,"links":{},"metadata":{"_buckets":{"deposit":"2d82b470-24c9-4309-b4b9-18fcc6613339"},"_deposit":{"created_by":1,"id":"22263","owners":[1],"pid":{"revision_id":0,"type":"depid","value":"22263"},"status":"published"},"_oai":{"id":"oai:kumadai.repo.nii.ac.jp:00022263","sets":["343:347:348:349"]},"author_link":["96814","96813","96812"],"item_14_alternative_title_22":{"attribute_name":"その他の言語のタイトル","attribute_value_mlt":[{"subitem_alternative_title":"Transduction of full-length dystrophin to multiple skeletal muscles improves motor performance and lifespan in utrophin/dystrophin double knockout mice"}]},"item_14_alternative_title_23":{"attribute_name":"タイトル（ヨミ）","attribute_value_mlt":[{"subitem_alternative_title":"キンジストロフィー モデル マウス ノ コッカクキン エノ カンゼンチョウ dystmphin ドウニュウ ニ ヨル ウンドウ キノウ オヨビ ジュミョウ ノ カイゼン"}]},"item_14_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2008-03-25","bibliographicIssueDateType":"Issued"},"bibliographic_titles":[{}]}]},"item_14_creator_3":{"attribute_name":"別言語の著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Kawano, Ryoko"}],"nameIdentifiers":[{"nameIdentifier":"96814","nameIdentifierScheme":"WEKO"}]}]},"item_14_description_17":{"attribute_name":"フォーマット","attribute_value_mlt":[{"subitem_description":"application/pdf","subitem_description_type":"Other"},{"subitem_description":"application/pdf","subitem_description_type":"Other"},{"subitem_description":"text/plain","subitem_description_type":"Other"}]},"item_14_description_46":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"subitem_description":"学位論文(Thesis)","subitem_description_type":"Other"}]},"item_14_description_5":{"attribute_name":"内容記述","attribute_value_mlt":[{"subitem_description":"Duchenne muscular dystrophy (DMD) is a fatal progressive muscle wasting disease caused by defects in the dystrophin gene. No viral vector except the helper-dependent adenovirus vector (HDAdv) can package 14kb full-length dystrophin cDNA and HDAdv is considerably safer than old-generation adenovirus vectors due to the large-size deletion in its genome. I have generated HDAdv that carries myc-tagged murine full-length dystrophin cDNA (HDAdv-myc-mFLdys). I injected it into the multiple proximal muscles of 7-day-old utrophin/dystrophin double knockout mice (dko mice), which typically show symptoms quite similar to human DMD, because the proximal muscles are organs affected in DMD patients. Eight weeks after injections, the transduced dystrophin was widely expressed and I found a significant reduction of\ncentrally nucleated myofibers and the restoration of dystrophin associated proteins, β-dystroglycan (β-DG) and α-sarcoglycan (α-SG), as well as neuronal nitric oxide synthase(nNOS). The injected dko mice also showed an increase in body weight, an improvement in motor performances, and prolonged lifespan. Using HDAdv, I could\ntreat DMD model mice, even when the therapeutic gene was transferred into multiple skeletal muscles. These results suggest that multiple intramuscular administrations of HDAdv carrying full-length dystrophin may reduce symptoms and compensate for lost functions in DMD patients.","subitem_description_type":"Other"},{"subitem_description":"本研究では、dkoマウスの四肢近位筋および体幹の骨格筋にHDAdvを用いて完全長dystrophinを導入し、その治療効果を病理学的観点および運動機能の観点から評価、検討する。","subitem_description_type":"Other"}]},"item_14_publisher_36":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"熊本大学"}]},"item_14_subject_20":{"attribute_name":"日本十進分類法","attribute_value_mlt":[{"subitem_subject":"377.5","subitem_subject_scheme":"NDC"}]},"item_14_text_18":{"attribute_name":"形態","attribute_value_mlt":[{"subitem_text_value":"60937 bytes"},{"subitem_text_value":"3555291 bytes"},{"subitem_text_value":"97715 bytes"}]},"item_14_text_47":{"attribute_name":"資源タイプ・ローカル","attribute_value_mlt":[{"subitem_text_value":"博士論文"}]},"item_14_text_48":{"attribute_name":"資源タイプ・NII","attribute_value_mlt":[{"subitem_text_value":"Thesis or Dissertation"}]},"item_14_text_49":{"attribute_name":"資源タイプ・DCMI","attribute_value_mlt":[{"subitem_text_value":"text"}]},"item_14_text_50":{"attribute_name":"資源タイプ・ローカル表示コード","attribute_value_mlt":[{"subitem_text_value":"03"}]},"item_14_text_81":{"attribute_name":"学位番号","attribute_value_mlt":[{"subitem_text_value":"甲博医第1639号"}]},"item_14_version_type_19":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"河野, 亮子"}],"nameIdentifiers":[{"nameIdentifier":"96812","nameIdentifierScheme":"WEKO"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2020-03-02"}],"displaytype":"detail","filename":"22-1639.pdf","filesize":[{"value":"3.6 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"22-1639.pdf","url":"https://kumadai.repo.nii.ac.jp/record/22263/files/22-1639.pdf"},"version_id":"a414d4df-c27a-44ff-8a65-64b1bca05ecd"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Duchenne muscular dystrophy (DMD)","subitem_subject_scheme":"Other"},{"subitem_subject":"dystrophin","subitem_subject_scheme":"Other"},{"subitem_subject":"helper-dependent adenovirus vector","subitem_subject_scheme":"Other"},{"subitem_subject":"gene therapy","subitem_subject_scheme":"Other"},{"subitem_subject":"mdx mouse","subitem_subject_scheme":"Other"},{"subitem_subject":"dko mouse","subitem_subject_scheme":"Other"},{"subitem_subject":"nNOS","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"jpn"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"thesis","resourceuri":"http://purl.org/coar/resource_type/c_46ec"}]},"item_title":"筋ジストロフィーモデルマウスの骨格筋への完全長dystmphin導入による運動機能および寿命の改善","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"筋ジストロフィーモデルマウスの骨格筋への完全長dystmphin導入による運動機能および寿命の改善"}]},"item_type_id":"14","owner":"1","path":["349"],"pubdate":{"attribute_name":"公開日","attribute_value":"2014-08-12"},"publish_date":"2014-08-12","publish_status":"0","recid":"22263","relation_version_is_last":true,"title":["筋ジストロフィーモデルマウスの骨格筋への完全長dystmphin導入による運動機能および寿命の改善"],"weko_creator_id":"1","weko_shared_id":-1},"updated":"2023-06-19T18:55:00.647034+00:00"}