{"created":"2023-05-15T16:29:57.781073+00:00","id":915,"links":{},"metadata":{"_buckets":{"deposit":"78f23d75-8032-46e5-8cc7-97dcc01747ec"},"_deposit":{"created_by":2,"id":"915","owners":[2],"pid":{"revision_id":0,"type":"depid","value":"915"},"status":"published"},"_oai":{"id":"oai:nagasaki-u.repo.nii.ac.jp:00000915","sets":["8:9"]},"author_link":["4350","4348","4353","4349","4351","4352","4346","4347"],"item_2_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2020-06-23","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"12","bibliographicPageEnd":"2639","bibliographicPageStart":"2631","bibliographicVolumeNumber":"4","bibliographic_titles":[{"bibliographic_title":"Blood Advances"}]}]},"item_2_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Thrombomodulin functions as an anticoagulant through thrombin binding and protein C activation. We herein report the first case of hereditary functional thrombomodulin deficiency presenting with recurrent subcutaneous hemorrhage and old cerebral infarction. The patient had a homozygous substitution of glycine by aspartate at amino acid residue 412 (Gly412Asp) in the thrombin-binding domain of the thrombomodulin gene (designated thrombomodulin-Nagasaki). In vitro assays using a recombinant thrombomodulin with the same mutation as the patient showed a total lack of thrombin binding and activation of protein C and thrombin-activatable fibrinolysis inhibitor (TAFI). Marked clinical and laboratory improvement was obtained with recombinant human soluble thrombomodulin therapy.","subitem_description_type":"Abstract"}]},"item_2_description_63":{"attribute_name":"引用","attribute_value_mlt":[{"subitem_description":"Blood advances, 4(12), pp.2631-2639; 2020","subitem_description_type":"Other"}]},"item_2_publisher_33":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"American Society of Hematology"}]},"item_2_relation_12":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_type":"isIdenticalTo","subitem_relation_type_id":{"subitem_relation_type_id_text":"10.1182/bloodadvances.2019001155","subitem_relation_type_select":"DOI"}}]},"item_2_rights_13":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"c 2020 by The American Society of Hematology"}]},"item_2_source_id_7":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"24739529","subitem_source_identifier_type":"ISSN"}]},"item_2_source_id_8":{"attribute_name":"EISSN","attribute_value_mlt":[{"subitem_source_identifier":"24739537","subitem_source_identifier_type":"ISSN"}]},"item_2_version_type_16":{"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":"Okada, Masahiko"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Tominaga, Norio"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Honda, Goichi"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Nishioka, Junji"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Akita, Nobuyuki"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Hayashi, Tatsuya"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Suzuki, Koji"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Moriuchi, Hiroyuki"}],"nameIdentifiers":[{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2020-12-18"}],"displaytype":"detail","filename":"BloAdv4_2631.pdf","filesize":[{"value":"1.5 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"BloAdv4_2631.pdf","url":"https://nagasaki-u.repo.nii.ac.jp/record/915/files/BloAdv4_2631.pdf"},"version_id":"582f179a-31c7-42a7-b01c-609dcd928973"}]},"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":"journal article","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"A case of thrombomodulin mutation causing defective thrombin binding with absence of protein C and TAFI activation","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"A case of thrombomodulin mutation causing defective thrombin binding with absence of protein C and TAFI activation"}]},"item_type_id":"2","owner":"2","path":["9"],"pubdate":{"attribute_name":"公開日","attribute_value":"2020-07-01"},"publish_date":"2020-07-01","publish_status":"0","recid":"915","relation_version_is_last":true,"title":["A case of thrombomodulin mutation causing defective thrombin binding with absence of protein C and TAFI activation"],"weko_creator_id":"2","weko_shared_id":-1},"updated":"2023-05-16T04:11:21.830110+00:00"}