{"created":"2023-07-27T06:51:49.443257+00:00","id":45398,"links":{},"metadata":{"_buckets":{"deposit":"5cd6ba9a-7b2f-4043-a40c-0a5bc841f20f"},"_deposit":{"created_by":18,"id":"45398","owners":[18],"pid":{"revision_id":0,"type":"depid","value":"45398"},"status":"published"},"_oai":{"id":"oai:kanazawa-u.repo.nii.ac.jp:00045398","sets":["2812:2813:2822"]},"author_link":["467","78917"],"item_9_biblio_info_8":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2012-05-21","bibliographicIssueDateType":"Issued"},"bibliographicPageStart":"4p.","bibliographicVolumeNumber":"2009-2011","bibliographic_titles":[{"bibliographic_title":"平成23(2011)年度 科学研究費補助金 若手研究(B) 研究成果報告書"},{"bibliographic_title":"2011 Fiscal Year Final Research Report","bibliographic_titleLang":"en"}]}]},"item_9_creator_33":{"attribute_name":"著者別表示","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{}],"nameIdentifiers":[{},{}]}]},"item_9_description_21":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Wiskott-Aldrich syndrome protein(WAS)症例26例について、血清IgA分子の糖鎖異常、血清IgG-IgA免疫複合体量の解析を行った。その結果、1. WAS症例では、糖鎖異常IgAおよびIgG-IgA免疫複合体量が年齢依存性に、また、自己免疫疾患を合併している症例で有意に増加していること、2.骨髄移植により、糖鎖異常IgAの量は減少し、糸球体沈着IgAの程度および腎組織障害が改善することが判明した。これらの結果から、IgA糖鎖異常は、WASに合併する自己免疫疾患の病態に深く関与している可能性が示唆された。","subitem_description_type":"Abstract"},{"subitem_description":"We evaluated the role of aberrant IgA production in the development of autoimmunity and glomerulonephritis in 26 patients with WAS. We found that serum galactose-deficient IgA levels and the levels of CIC containing IgA and IgG increased in WAS patients as an age dependent manner. Interestingly, these levels significantly increased in WAS patients with autoimmune manifestations. Furthermore, IgA-dominant immune deposits in the renal mesangium and glomerular injury in an XLT patient were significantly reduced after BMT. Galactose-deficient IgA levels were also markedly decreased after BMT. These findings indicate that mutations in WAS may be associated with aberrant IgA production and the development of autoimmune diseases including IgAN. Furthermore, immune reconstitution by BMT could correct aberrant IgA induced autoimmune diseases. These findings indicate that aberrant IgA production due to mutations in the WAS gene may be critically involved in the development of autoimmune diseases and glomerulonephritis.","subitem_description_type":"Abstract"}]},"item_9_description_22":{"attribute_name":"内容記述","attribute_value_mlt":[{"subitem_description":"研究課題/領域番号:21790972, 研究期間(年度):2009-2011","subitem_description_type":"Other"},{"subitem_description":"出典：研究課題「WAS腎症をモデルとしたIgA腎症の発症機構に関する分子免疫学的解析」課題番号21790972\n（KAKEN：科学研究費助成事業データベース（国立情報学研究所）） \n（https://kaken.nii.ac.jp/report/KAKENHI-PROJECT-21790972/21790972seika/）を加工して作成","subitem_description_type":"Other"}]},"item_9_description_5":{"attribute_name":"提供者所属","attribute_value_mlt":[{"subitem_description":"金沢大学附属病院小児科","subitem_description_type":"Other"}]},"item_9_identifier_registration":{"attribute_name":"ID登録","attribute_value_mlt":[{"subitem_identifier_reg_text":"10.24517/00051735","subitem_identifier_reg_type":"JaLC"}]},"item_9_relation_28":{"attribute_name":"関連URI","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_text":"https://kaken.nii.ac.jp/search/?qm=10401902"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://kaken.nii.ac.jp/search/?qm=10401902","subitem_relation_type_select":"URI"}},{"subitem_relation_name":[{"subitem_relation_name_text":"https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-21790972/"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-21790972/","subitem_relation_type_select":"URI"}},{"subitem_relation_name":[{"subitem_relation_name_text":"https://kaken.nii.ac.jp/report/KAKENHI-PROJECT-21790972/21790972seika/"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://kaken.nii.ac.jp/report/KAKENHI-PROJECT-21790972/21790972seika/","subitem_relation_type_select":"URI"}}]},"item_9_version_type_25":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_ab4af688f83e57aa","subitem_version_type":"AM"}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2018-07-20"}],"displaytype":"detail","filename":"ME-PR-SHIMIZU-M-kaken 2012-4p.pdf","filesize":[{"value":"227.3 kB"}],"format":"application/pdf","licensetype":"license_11","mimetype":"application/pdf","url":{"label":"ME-PR-SHIMIZU-M-kaken 2012-4p.pdf","url":"https://kanazawa-u.repo.nii.ac.jp/record/45398/files/ME-PR-SHIMIZU-M-kaken 2012-4p.pdf"},"version_id":"32ad066d-bc57-4a72-b067-15199911df64"}]},"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":"research report","resourceuri":"http://purl.org/coar/resource_type/c_18ws"}]},"item_title":"WAS腎症をモデルとしたIgA腎症の発症機構に関する分子免疫学的解析","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"WAS腎症をモデルとしたIgA腎症の発症機構に関する分子免疫学的解析"},{"subitem_title":"Molecular immunological analysis of the pathogenesis of Wiskott Aldrich syndrome associated IgA nephropathy","subitem_title_language":"en"}]},"item_type_id":"9","owner":"18","path":["2822"],"pubdate":{"attribute_name":"公開日","attribute_value":"2018-07-20"},"publish_date":"2018-07-20","publish_status":"0","recid":"45398","relation_version_is_last":true,"title":["WAS腎症をモデルとしたIgA腎症の発症機構に関する分子免疫学的解析"],"weko_creator_id":"18","weko_shared_id":-1},"updated":"2023-07-27T13:27:25.564476+00:00"}