{"created":"2023-07-27T06:51:25.068479+00:00","id":44835,"links":{},"metadata":{"_buckets":{"deposit":"42c8d23b-530b-4d0a-9973-5f8e76281627"},"_deposit":{"created_by":18,"id":"44835","owners":[18],"pid":{"revision_id":0,"type":"depid","value":"44835"},"status":"published"},"_oai":{"id":"oai:kanazawa-u.repo.nii.ac.jp:00044835","sets":["2812:2813:2825"]},"author_link":["2881","20436"],"item_9_biblio_info_8":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2009-04-10","bibliographicIssueDateType":"Issued"},"bibliographicPageStart":"6p.","bibliographicVolumeNumber":"2007-2008","bibliographic_titles":[{"bibliographic_title":"平成20(2008)年度 科学研究費補助金 基盤研究(C) 研究成果報告書"},{"bibliographic_title":"2008 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":"X-SCIDは通常、T細胞ならびにNK細胞を欠損し、複合免疫不全症の病態を示すが、本研究ではOmenn症候群様の異常T細胞増殖と重症皮膚病変を示した症例の病態を解析した。その結果、皮膚に浸潤するT細胞には共通γ鎖遺伝子に第二変異が起こっていることが確認された。これまでの研究でも、第二変異あるいは遺伝子変異の復帰により増殖したクローンが病態を修飾している症例を経験しており、本症例はこれまでの一連の研究と関連し、わずかなクローンに生じた新たな変異が臨床症状に多大な影響を及ぼすという、原発性免疫不全症ならではの現象を確認することとなった。","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:19591244, 研究期間(年度):2007-2008","subitem_description_type":"Other"},{"subitem_description":"出典：「複合免疫不全症の多様な臨床像とその背景となる分子生物学的基盤に関する研究」研究成果報告書　課題番号19591244\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-19591244/19591244seika/)を加工して作成","subitem_description_type":"Other"}]},"item_9_identifier_registration":{"attribute_name":"ID登録","attribute_value_mlt":[{"subitem_identifier_reg_text":"10.24517/00051174","subitem_identifier_reg_type":"JaLC"}]},"item_9_publisher_17":{"attribute_name":"公開者","attribute_value_mlt":[{"subitem_publisher":"金沢大学医薬保健研究域医学系"}]},"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=40210281"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://kaken.nii.ac.jp/search/?qm=40210281","subitem_relation_type_select":"URI"}},{"subitem_relation_name":[{"subitem_relation_name_text":"https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-19591244/"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-19591244/","subitem_relation_type_select":"URI"}},{"subitem_relation_name":[{"subitem_relation_name_text":"https://kaken.nii.ac.jp/report/KAKENHI-PROJECT-19591244/19591244seika/"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://kaken.nii.ac.jp/report/KAKENHI-PROJECT-19591244/19591244seika/","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":"2018-06-22"}],"displaytype":"detail","filename":"HO-PR-YACHIE-A-kaken 2009-6p.pdf","filesize":[{"value":"515.9 kB"}],"format":"application/pdf","licensetype":"license_11","mimetype":"application/pdf","url":{"label":"HO-PR-YACHIE-A-kaken 2009-6p.pdf","url":"https://kanazawa-u.repo.nii.ac.jp/record/44835/files/HO-PR-YACHIE-A-kaken 2009-6p.pdf"},"version_id":"94c34065-bae3-442d-a399-ade4fb7b5fc3"}]},"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":"複合免疫不全症の多様な臨床像とその背景となる分子生物学的基盤に関する研究","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"複合免疫不全症の多様な臨床像とその背景となる分子生物学的基盤に関する研究"},{"subitem_title":"Molecular mechanisms underlying the complex clinical features of combined immunodeficiency disorders","subitem_title_language":"en"}]},"item_type_id":"9","owner":"18","path":["2825"],"pubdate":{"attribute_name":"公開日","attribute_value":"2018-06-22"},"publish_date":"2018-06-22","publish_status":"0","recid":"44835","relation_version_is_last":true,"title":["複合免疫不全症の多様な臨床像とその背景となる分子生物学的基盤に関する研究"],"weko_creator_id":"18","weko_shared_id":-1},"updated":"2023-07-27T14:14:52.993224+00:00"}