{"created":"2024-10-30T09:40:08.792572+00:00","id":2007627,"links":{},"metadata":{"_buckets":{"deposit":"6e249345-9934-4e42-9fc6-aea0e42f6470"},"_deposit":{"created_by":7,"id":"2007627","owners":[7],"pid":{"revision_id":0,"type":"depid","value":"2007627"},"status":"published"},"_oai":{"id":"oai:tokushima-u.repo.nii.ac.jp:02007627","sets":["1713853213384:1713853296295:1716267876683:1716268551628"]},"author_link":["1556","209","1548","1542","1521"],"item_10001_alternative_title_1":{"attribute_name":"タイトル別表記","attribute_value_mlt":[{"subitem_alternative_title":"ホモ接合型SFTPA1変異により特発性肺線維症患者のⅡ型肺胞上皮細胞のnecroptosisが亢進する","subitem_alternative_title_language":"ja"},{"subitem_alternative_title":"A mutation in SFTPA1 and pulmonary fibrosis","subitem_alternative_title_language":"en"}]},"item_10001_biblio_info_7":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2019-10-10","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"12","bibliographicPageEnd":"2735","bibliographicPageStart":"2724","bibliographicVolumeNumber":"216","bibliographic_titles":[{"bibliographic_title":"Journal of Experimental Medicine","bibliographic_titleLang":"en"}]}]},"item_10001_description_5":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Idiopathic pulmonary fibrosis (IPF) is a fatal disease characterized by scattered fibrotic lesions in the lungs. The pathogenesis and genetic basis of IPF remain poorly understood. Here, we show that a homozygous missense mutation in SFTPA1 caused IPF in a consanguineous Japanese family. The mutation in SFTPA1 disturbed the secretion of SFTPA1 protein. Sftpa1 knock-in (Sftpa1-KI) mice that harbored the same mutation as patients spontaneously developed pulmonary fibrosis that was accelerated by influenza virus infection. Sftpa1-KI mice showed increased necroptosis of alveolar epithelial type II (AEII) cells with phosphorylation of IRE1α leading to JNK-mediated up-regulation of Ripk3. The inhibition of JNK ameliorated pulmonary fibrosis in Sftpa1-KI mice, and overexpression of Ripk3 in Sftpa1-KI mice treated with a JNK inhibitor worsened pulmonary fibrosis. These findings provide new insight into the mechanisms of IPF in which a mutation in SFTPA1 promotes necroptosis of AEII cells through JNK-mediated up-regulation of Ripk3, highlighting the necroptosis pathway as a therapeutic target for IPF.","subitem_description_language":"en","subitem_description_type":"Abstract"}]},"item_10001_publisher_8":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"Rockefeller University Press","subitem_publisher_language":"en"}]},"item_10001_rights_15":{"attribute_name":"権利情報","attribute_value_mlt":[{"subitem_rights":"© 2019 Takezaki et al. This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms/). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 International license, as described at https://creativecommons.org/licenses/by-nc-sa/4.0/).","subitem_rights_language":"en"}]},"item_10001_source_id_9":{"attribute_name":"収録物ID","attribute_value_mlt":[{"subitem_source_identifier":"15409538","subitem_source_identifier_type":"ISSN"},{"subitem_source_identifier":"00221007","subitem_source_identifier_type":"ISSN"},{"subitem_source_identifier":"AA12119243","subitem_source_identifier_type":"NCID"},{"subitem_source_identifier":"AA00697559","subitem_source_identifier_type":"NCID"}]},"item_10001_version_type_20":{"attribute_name":"出版タイプ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_be7fb7dd8ff6fe43","subitem_version_type":"NA"}]},"item_1714461018643":{"attribute_name":"報告番号","attribute_value_mlt":[{"subitem_dissertationnumber":"甲第3426号"}]},"item_1714461102074":{"attribute_name":"学位名","attribute_value_mlt":[{"subitem_degreename":"博士（医学）","subitem_degreename_language":"ja"}]},"item_1714461118377":{"attribute_name":"学位授与年月日","attribute_value_mlt":[{"subitem_dategranted":"2020-04-23"}]},"item_1714461137393":{"attribute_name":"学位授与機関","attribute_value_mlt":[{"subitem_degreegrantor":[{"subitem_degreegrantor_language":"ja","subitem_degreegrantor_name":"徳島大学"}]}]},"item_1715043197608":{"attribute_name":"アクセス権","attribute_value_mlt":[{"subitem_access_right":"open access"}]},"item_1718868208704":{"attribute_name":"備考","attribute_value_mlt":[{"subitem_textarea_language":"ja","subitem_textarea_value":"内容要旨・審査要旨・論文本文の公開\n本論文は，著者Akio Takezakiの学位論文として提出され，学位審査・授与の対象となっている。\n学位授与者所属 : 徳島大学大学院医科学教育部（医学専攻）"}]},"item_1718868303842":{"attribute_name":"学位記番号","attribute_value_mlt":[{"subitem_text_language":"ja","subitem_text_value":"甲医第1457号"}]},"item_1722929371688":{"attribute_name":"出版社版DOI","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_language":"ja","subitem_relation_name_text":"10.1084/jem.20182351"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://doi.org/10.1084/jem.20182351","subitem_relation_type_select":"DOI"}}]},"item_1723180141928":{"attribute_name":"EID","attribute_value_mlt":[{"subitem_identifier_type":"URI","subitem_identifier_uri":"362480"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"竹﨑, 彰夫","creatorNameLang":"ja"},{"creatorName":"タケザキ, アキオ","creatorNameLang":"ja-Kana"},{"creatorName":"Takezaki, Akio","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"九十九, 伸一","creatorNameLang":"ja"}],"nameIdentifiers":[{"nameIdentifier":"1556","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"セトグチ, ヤスヒロ","creatorNameLang":"ja"},{"creatorName":"セトグチ, ヤスヒロ","creatorNameLang":"ja-Kana"},{"creatorName":"Setoguchi, Yasuhiro","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"Ledford, Julie G.","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"後東, 久嗣","creatorNameLang":"ja"}],"nameIdentifiers":[{"nameIdentifier":"209","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"ホソミチ, カズヨシ","creatorNameLang":"ja"},{"creatorName":"ホソミチ, カズヨシ","creatorNameLang":"ja-Kana"},{"creatorName":"Hosomichi, Kazuyoshi","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"上原, 久典","creatorNameLang":"ja"}],"nameIdentifiers":[{"nameIdentifier":"1548","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"西岡, 安彦","creatorNameLang":"ja"}],"nameIdentifiers":[{"nameIdentifier":"1542","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"安友, 康二","creatorNameLang":"ja"}],"nameIdentifiers":[{"nameIdentifier":"1521","nameIdentifierScheme":"WEKO"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_access","date":[{"dateType":"Available","dateValue":"2020-05-14"}],"displaytype":"detail","filename":"k3426_abstract_review.pdf","filesize":[{"value":"213 KB"}],"format":"application/pdf","mimetype":"application/pdf","url":{"objectType":"abstract","url":"https://tokushima-u.repo.nii.ac.jp/record/2007627/files/k3426_abstract_review.pdf"},"version_id":"3076e882-7654-4320-9161-07b5eaeec5dd"},{"accessrole":"open_access","date":[{"dateType":"Available","dateValue":"2020-05-14"}],"displaytype":"detail","filename":"k3426_fulltext.pdf","filesize":[{"value":"3.81 MB"}],"format":"application/pdf","mimetype":"application/pdf","url":{"objectType":"fulltext","url":"https://tokushima-u.repo.nii.ac.jp/record/2007627/files/k3426_fulltext.pdf"},"version_id":"6379fcc2-a429-458c-b7ec-c24cf5b87cf6"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"idiopathic pulmonary fibrosis","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"SFTPA1 mutation","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"typeⅡ alveolar epithelial cell","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"necroptosis","subitem_subject_language":"en","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":"doctoral thesis","resourceuri":"http://purl.org/coar/resource_type/c_db06"}]},"item_title":"A homozygous SFTPA1 mutation drives necroptosis of typeⅡ alveolar epithelial cells in patients with idiopathic pulmonary fibrosis","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"A homozygous SFTPA1 mutation drives necroptosis of typeⅡ alveolar epithelial cells in patients with idiopathic pulmonary fibrosis","subitem_title_language":"en"}]},"item_type_id":"40001","owner":"7","path":["1716268551628"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2020-05-14"},"publish_date":"2020-05-14","publish_status":"0","recid":"2007627","relation_version_is_last":true,"title":["A homozygous SFTPA1 mutation drives necroptosis of typeⅡ alveolar epithelial cells in patients with idiopathic pulmonary fibrosis"],"weko_creator_id":"7","weko_shared_id":-1},"updated":"2024-10-30T09:40:19.125622+00:00"}