{"created":"2023-06-20T16:49:33.332307+00:00","id":21798,"links":{},"metadata":{"_buckets":{"deposit":"60683404-136a-4075-997c-1ab4b6a19ad9"},"_deposit":{"created_by":3,"id":"21798","owners":[3],"pid":{"revision_id":0,"type":"depid","value":"21798"},"status":"published"},"_oai":{"id":"oai:kindai.repo.nii.ac.jp:00021798","sets":["14:923:1477:4678"]},"author_link":["37604"],"item_2_biblio_info_21":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2021-06-25","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1-2","bibliographicPageEnd":"11","bibliographicPageStart":"3","bibliographicVolumeNumber":"46","bibliographic_titles":[{"bibliographic_title":"近畿大学医学雑誌"},{"bibliographic_title":"Medical Journal of Kindai University","bibliographic_titleLang":"en"}]}]},"item_2_description_33":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"[抄録]アルツハイマー病，パーキンソン病，筋萎縮性側索硬化症，脊髄小脳変性症などの神経変性疾患は，有効な治療法に乏しい難病である．１９９０年代からの分子遺伝学的解析技術の進展によって多くの遺伝性疾患の原因遺伝子が同定され，従来からの病理学的知見と併せて，様々なタンパク質のミスフォールディング・凝集により神経変性が引き起こされるという広く普遍的な発症分子メカニズムが考えられるようになった．筆者らは，遺伝性疾患であるポリグルタミン病をモデルとして研究を進め，ペプチド QBP１ が異常伸長ポリグルタミンタンパク質の異常βシート構造転移・凝集を阻害し，神経変性を抑制することを明らかにした．続いて，低分子化合物スクリーニングによりアルギニンの凝集阻害活性を見出し，ポリグルタミン病モデルショウジョウバエ，マウスへの有効性を明らかにし，現在脊髄小脳失調症患者に対する医師主導治験を行っている．また，分子シャペロンやオートファジー系タンパク質分解機構などタンパク質恒常性（プロテオスターシス）維持機構の応用による治療法開発も行ってきた．一方，遺伝子非翻訳領域内のリピート配列異常伸長を原因とするノンコーディングリピート病の１つ SCA３１ について，リピート RNA に結合する TDP -４３ が RNA シャペロンとして，神経変性を抑制することを見出した．このような研究により，近い将来に神経変性疾患が克服できることを期待している．","subitem_description_type":"Abstract"}]},"item_2_description_41":{"attribute_name":"フォーマット","attribute_value_mlt":[{"subitem_description":"application/pdf","subitem_description_type":"Other"}]},"item_2_identifier_registration":{"attribute_name":"ID登録","attribute_value_mlt":[{"subitem_identifier_reg_text":"10.15100/00021773","subitem_identifier_reg_type":"JaLC"}]},"item_2_publisher_14":{"attribute_name":"出版者 名前","attribute_value_mlt":[{"subitem_publisher":"近畿大学医学会"}]},"item_2_source_id_22":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"03858367","subitem_source_identifier_type":"ISSN"}]},"item_2_text_7":{"attribute_name":"著者(英)","attribute_value_mlt":[{"subitem_text_language":"en","subitem_text_value":"Nagai,Yoshitaka"}]},"item_2_text_8":{"attribute_name":"著者 所属","attribute_value_mlt":[{"subitem_text_value":"近畿大学医学部内科学教室脳神経内科部門"}]},"item_2_text_9":{"attribute_name":"著者所属(翻訳)","attribute_value_mlt":[{"subitem_text_value":"Department of Neurology,Faculty of Medicine,Kindai University"}]},"item_2_version_type_12":{"attribute_name":"版","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_be7fb7dd8ff6fe43","subitem_version_type":"NA"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"永井, 義隆"},{"creatorName":"ナガイ, ヨシタカ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2021-06-30"}],"displaytype":"detail","filename":"AN00063584-20210625-0003.pdf","filesize":[{"value":"1.3 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"AN00063584-20210625-0003","url":"https://kindai.repo.nii.ac.jp/record/21798/files/AN00063584-20210625-0003.pdf"},"version_id":"adc98d46-19c7-42f6-b407-18310f478f3d"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"神経変性疾患","subitem_subject_scheme":"Other"},{"subitem_subject":"ポリグルタミン病","subitem_subject_scheme":"Other"},{"subitem_subject":"ノンコーディングリピート病","subitem_subject_scheme":"Other"},{"subitem_subject":"タンパク質ミスフォールディング・凝集","subitem_subject_scheme":"Other"},{"subitem_subject":"RAN 翻訳","subitem_subject_scheme":"Other"},{"subitem_subject":"分子標的治療法","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":"departmental bulletin paper","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"〈総説〉神経変性疾患克服への展望","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"〈総説〉神経変性疾患克服への展望"},{"subitem_title":"Toward developing therapy for neurodegenerative diseases","subitem_title_language":"en"}]},"item_type_id":"2","owner":"3","path":["4678"],"pubdate":{"attribute_name":"公開日","attribute_value":"2021-05-20"},"publish_date":"2021-05-20","publish_status":"0","recid":"21798","relation_version_is_last":true,"title":["〈総説〉神経変性疾患克服への展望"],"weko_creator_id":"3","weko_shared_id":3},"updated":"2023-06-20T19:25:48.538420+00:00"}