{"created":"2023-06-19T11:44:32.430278+00:00","id":14495,"links":{},"metadata":{"_buckets":{"deposit":"01977890-80fb-475d-84d4-51e6f4a47dec"},"_deposit":{"created_by":15,"id":"14495","owners":[15],"pid":{"revision_id":0,"type":"depid","value":"14495"},"status":"published"},"_oai":{"id":"oai:mie-u.repo.nii.ac.jp:00014495","sets":["334:627:1692670486782"]},"author_link":[],"item_8_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2020-06-04","bibliographicIssueDateType":"Issued"}}]},"item_8_description_14":{"attribute_name":"フォーマット","attribute_value_mlt":[{"subitem_description":"application/pdf","subitem_description_type":"Other"}]},"item_8_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"骨形成因子2型受容体(BMPR2) 変異は、肺動脈性肺高血圧の主要な遺伝的危険因子である。BMPR2変異保有患者は、予後が不良であるが、機序は不明である。マウスの肺高血圧(PH)モデルでは、高度のPH作成は困難であったが、近年ゲノム編集により、容易にラットの遺伝子操作が可能となり、進行性ラットPHモデルを用い検討が可能となった。BMPR2遺伝子変異ラットでは、BMPR2遺伝子に1塩基挿入によるミスセンス変異が確認された。変異ラットのPHは早期には野生型と同等であったが、変異ラットの生存率は低かった。ゲノム編集により、BMPR2変異の意義をPHの発症初期から進行期まで縦断的に検討が可能であった。","subitem_description_type":"Abstract"},{"subitem_description":" Mutations in bone morphogenetic protein receptor type-2(BMPR2) are the main genetic risk factor for developing pulmonary arterial hypertension (PAH). Recent studies have shown that PAH patients with BMPR2 mutation present earlier with severe hemodynamic compromise and worse transplant-free survival than those without mutations. The mechanisms underlying the difference in clinical phenotype in BMPR2 mutation carriers remain unclear. We tested the hypothesis that rats with a CRISPR/Cas9-mediated monoallelic BMPR2 mutation develop more severe pulmonary hypertension (PH) leading to poor survival than those without the mutation. For the first time using CRISPR/Cas9, this study demonstrates that rats with BMPR2 mutation develop MCT-PH similarly to wildtype until day21 but had poor survival thereafter and warrants further studies into underlying mechanism directing towards precision medicine in PAH targeted therapy for patients with BMPR2 mutations.","subitem_description_type":"Abstract"}]},"item_8_description_5":{"attribute_name":"内容記述","attribute_value_mlt":[{"subitem_description":"2017年度～2019年度科学研究費補助金(基盤研究(C))研究成果報告書","subitem_description_type":"Other"}]},"item_8_description_64":{"attribute_name":"科研費番号","attribute_value_mlt":[{"subitem_description":"17K10140","subitem_description_type":"Other"}]},"item_8_publisher_30":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"三重大学"}]},"item_8_text_31":{"attribute_name":"出版者（ヨミ）","attribute_value_mlt":[{"subitem_text_value":"ミエダイガク"}]},"item_8_text_65":{"attribute_name":"資源タイプ（三重大）","attribute_value_mlt":[{"subitem_text_value":"Kaken / 科研費報告書"}]},"item_8_version_type_15":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"澤田, 博文","creatorNameLang":"ja"},{"creatorName":"サワダ, ヒロフミ","creatorNameLang":"ja-Kana"},{"creatorName":"Sawada, Hirohumi","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"丸山, 一男","creatorNameLang":"ja"},{"creatorName":"マルヤマ, カズオ","creatorNameLang":"ja-Kana"},{"creatorName":"Maruyama, Kazuo","creatorNameLang":"en"}]},{"creatorNames":[{"creatorName":"三谷, 義英","creatorNameLang":"ja"},{"creatorName":"ミタニ, ヨシヒデ","creatorNameLang":"ja-Kana"},{"creatorName":"Mitani, Yoshihide","creatorNameLang":"en"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2022-01-19"}],"displaytype":"detail","filename":"2021RP0029.pdf","filesize":[{"value":"295.8 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"2021RP0029","url":"https://mie-u.repo.nii.ac.jp/record/14495/files/2021RP0029.pdf"},"version_id":"5072ce07-ae84-4f95-a116-22516626e3ca"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"肺高血圧","subitem_subject_scheme":"Other"},{"subitem_subject":"動物モデル","subitem_subject_scheme":"Other"},{"subitem_subject":"遺伝子変異","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":"research report","resourceuri":"http://purl.org/coar/resource_type/c_18ws"}]},"item_title":"BMPR2遺伝子改変ラットを用いた肺高血圧血管病変形成機序の解明","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"BMPR2遺伝子改変ラットを用いた肺高血圧血管病変形成機序の解明","subitem_title_language":"ja"},{"subitem_title":"Role of BMPR2 mutation in pulmonary Hypertension in Rats ","subitem_title_language":"en"}]},"item_type_id":"8","owner":"15","path":["1692670486782"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2022-01-19"},"publish_date":"2022-01-19","publish_status":"0","recid":"14495","relation_version_is_last":true,"title":["BMPR2遺伝子改変ラットを用いた肺高血圧血管病変形成機序の解明"],"weko_creator_id":"15","weko_shared_id":-1},"updated":"2023-11-09T05:32:09.865981+00:00"}