{"created":"2023-06-19T09:10:15.880419+00:00","id":21762,"links":{},"metadata":{"_buckets":{"deposit":"93ef363d-a825-49f7-97b5-2f62fde6c3a5"},"_deposit":{"created_by":1,"id":"21762","owners":[1],"pid":{"revision_id":0,"type":"depid","value":"21762"},"status":"published"},"_oai":{"id":"oai:kumadai.repo.nii.ac.jp:00021762","sets":["413:443"]},"author_link":["94242","94228","94232","94244","94230","94240","94239","94229","94243","94227","94241"],"item_16_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2008-05","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"5","bibliographicPageEnd":"831","bibliographicPageStart":"825","bibliographicVolumeNumber":"16","bibliographic_titles":[{"bibliographic_title":"Molecular Therapy"}]}]},"item_16_creator_3":{"attribute_name":"別言語の著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"河野, 亮子"}],"nameIdentifiers":[{"nameIdentifier":"94239","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"石崎, 雅俊"}],"nameIdentifiers":[{"nameIdentifier":"94240","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"前田, 寧"}],"nameIdentifiers":[{"nameIdentifier":"94241","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"内田, 友二"}],"nameIdentifiers":[{"nameIdentifier":"94242","nameIdentifierScheme":"WEKO"}]},{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"木村, 円","creatorNameLang":"ja"},{"creatorName":"キムラ, エン","creatorNameLang":"ja-Kana"},{"creatorName":"Kimura, En","creatorNameLang":"en"}],"familyNames":[{"familyName":"木村","familyNameLang":"ja"},{"familyName":"キムラ","familyNameLang":"ja-Kana"},{"familyName":"Kimura","familyNameLang":"en"}],"givenNames":[{"givenName":"円","givenNameLang":"ja"},{"givenName":"エン","givenNameLang":"ja-Kana"},{"givenName":"En","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"94243","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"内野, 誠"}],"nameIdentifiers":[{"nameIdentifier":"94244","nameIdentifierScheme":"WEKO"}]}]},"item_16_description_17":{"attribute_name":"フォーマット","attribute_value_mlt":[{"subitem_description":"application/pdf","subitem_description_type":"Other"}]},"item_16_description_46":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"subitem_description":"論文(Article)","subitem_description_type":"Other"}]},"item_16_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. No viral vector except the helper-dependent adenovirus vector (HDAdv) can package 14-kilobase (kb) full-length dystrophin complementary DNA (cDNA), and HDAdv is considerably safer than old-generation adenovirus vectors because of the large-size deletion in its genome. We have generated HDAdv that carries myc-tagged murine full-length dystrophin cDNA (HDAdv-myc-mFLdys). We injected it into 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 affected in DMD patients. Eight weeks after the injections, the transduced dystrophin was widely expressed, and we found a significant reduction in centrally nucleated myofibers and the restoration of the 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 performance, and a prolongation of life span. Using HDAdv, we could treat DMD model mice even by transferring the therapeutic gene into multiple skeletal muscles. Our results suggest that multiple intramuscular administrations of HDAdv carrying full-length dystrophin cDNA may reduce symptoms and compensate for lost functions in DMD patients.","subitem_description_type":"Other"}]},"item_16_description_77":{"attribute_name":"URL","attribute_value_mlt":[{"subitem_description":"http://www.nature.com/mt/journal/v16/n5/abs/mt200823a.html","subitem_description_type":"Other"}]},"item_16_publisher_36":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"Nature Publishing 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Therapy"}]},"item_16_source_id_9":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AA11475060","subitem_source_identifier_type":"NCID"}]},"item_16_subject_20":{"attribute_name":"日本十進分類法","attribute_value_mlt":[{"subitem_subject":"493.6","subitem_subject_scheme":"NDC"}]},"item_16_text_18":{"attribute_name":"形態","attribute_value_mlt":[{"subitem_text_value":"302252 bytes"}]},"item_16_text_47":{"attribute_name":"資源タイプ・ローカル","attribute_value_mlt":[{"subitem_text_value":"雑誌掲載論文"}]},"item_16_text_48":{"attribute_name":"資源タイプ・NII","attribute_value_mlt":[{"subitem_text_value":"Journal Article"}]},"item_16_text_49":{"attribute_name":"資源タイプ・DCMI","attribute_value_mlt":[{"subitem_text_value":"text"}]},"item_16_text_50":{"attribute_name":"資源タイプ・ローカル表示コード","attribute_value_mlt":[{"subitem_text_value":"01"}]},"item_16_text_78":{"attribute_name":"コメント","attribute_value_mlt":[{"subitem_text_value":"平成18～19年度科学研究費補助金(基盤研究(C)(2))研究成果報告書 課題番号:18590951 新世代アデノウイルスベクターとトランスポゼースによる進行性筋ジストロフィーの遺伝子治療 に掲載"}]},"item_16_version_type_19":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_type":"AM"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Kawano, Ryoko"}],"nameIdentifiers":[{"nameIdentifier":"94227","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Ishizaki, Masatoshi"}],"nameIdentifiers":[{"nameIdentifier":"94228","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Maeda, Yasushi"}],"nameIdentifiers":[{"nameIdentifier":"94229","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Uchida, Yuji"}],"nameIdentifiers":[{"nameIdentifier":"94230","nameIdentifierScheme":"WEKO"}]},{"creatorAffiliations":[{"affiliationNameIdentifiers":[{"affiliationNameIdentifier":"","affiliationNameIdentifierScheme":"ISNI","affiliationNameIdentifierURI":"http://www.isni.org/isni/"}],"affiliationNames":[{"affiliationName":"","affiliationNameLang":"ja"}]}],"creatorNames":[{"creatorName":"木村, 円","creatorNameLang":"ja"},{"creatorName":"キムラ, エン","creatorNameLang":"ja-Kana"},{"creatorName":"Kimura, En","creatorNameLang":"en"}],"familyNames":[{"familyName":"木村","familyNameLang":"ja"},{"familyName":"キムラ","familyNameLang":"ja-Kana"},{"familyName":"Kimura","familyNameLang":"en"}],"givenNames":[{"givenName":"円","givenNameLang":"ja"},{"givenName":"エン","givenNameLang":"ja-Kana"},{"givenName":"En","givenNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"94243","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Uchino, Makoto"}],"nameIdentifiers":[{"nameIdentifier":"94232","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":"Mol Ther_016_825.pdf","filesize":[{"value":"302.3 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"Mol Ther_016_825.pdf","url":"https://kumadai.repo.nii.ac.jp/record/21762/files/Mol Ther_016_825.pdf"},"version_id":"48d3d16d-1d14-47df-ad3c-93f5b364e06f"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"dystrophin","subitem_subject_scheme":"Other"},{"subitem_subject":"Duchenne muscular dystrophy","subitem_subject_scheme":"Other"},{"subitem_subject":"DMD","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"eng"}]},"item_resource_type":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"resourcetype":"journal article","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"Transduction of full-length dystrophin to multiple skeletal muscles improves motor performance and life span in utrophin/dystrophin double knockout mice","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Transduction of full-length dystrophin to multiple skeletal muscles improves motor performance and life span in utrophin/dystrophin double knockout mice"}]},"item_type_id":"16","owner":"1","path":["443"],"pubdate":{"attribute_name":"公開日","attribute_value":"2009-02-23"},"publish_date":"2009-02-23","publish_status":"0","recid":"21762","relation_version_is_last":true,"title":["Transduction of full-length dystrophin to multiple skeletal muscles improves motor performance and life span in utrophin/dystrophin double knockout mice"],"weko_creator_id":"1","weko_shared_id":-1},"updated":"2023-09-26T06:56:38.813516+00:00"}