{"_buckets": {"deposit": "c784bb31-4868-4ea8-bf4f-7488783cad73"}, "_deposit": {"id": "21351", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "21351"}, "status": "published"}, "_oai": {"id": "oai:soar-ir.repo.nii.ac.jp:00021351", "sets": ["462"]}, "author_link": ["109398", "109399", "109400", "109401", "109402", "109403"], "item_1628147817048": {"attribute_name": "出版タイプ", "attribute_value_mlt": [{"subitem_version_resource": "http://purl.org/coar/version/c_ab4af688f83e57aa", "subitem_version_type": "AM"}]}, "item_6_biblio_info_6": {"attribute_name": "書誌情報", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2020-01-20", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "2", "bibliographicPageEnd": "197", "bibliographicPageStart": "190", "bibliographicVolumeNumber": "42", "bibliographic_titles": [{"bibliographic_title": "INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY"}]}]}, "item_6_description_20": {"attribute_name": "抄録", "attribute_value_mlt": [{"subitem_description": "Introduction: Congenital fibrinogen disorders are classified as afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia. However, difficulties are associated with discriminating between dysfibrinogenemia, hypofibrinogenemia, and hypodysfibrinogenemia using routine analyses. We previously reported a heterozygous variant fibrinogen (γA289V; Kanazawa III) as hypodysfibrinogenemia; however, the same variant had previously been described as hypofibrinogenemia. To clarify the production of γA289V fibrinogen, we expressed recombinant γA289V (r-γA289V) fibrinogen and compared it with wild-type (WT) and adjacent recombinant variant fibrinogens. Methods: Target mutations were introduced into a fibrinogen γ-chain expression vector by site-directed mutagenesis, and the vector was then transfected into Chinese hamster ovary cells to produce recombinant fibrinogen. Fibrinogen was purified from the plasma of the proposita, and culture media and fibrinogen functions were analyzed using fibrin polymerization, plasmin protection, and FXIIIa-catalyzed fibrinogen cross-linking. Results: The fibrinogen concentration ratio of the culture media to cell lysates was markedly lower for r-γA289V fibrinogen than for WT. Because the secretion of recombinant γF290L (r-γF290L) fibrinogen was similar to WT, we compared r-γF290L fibrinogen functions with WT. The fibrin polymerization of Kanazawa III plasma (K-III) fibrinogen was significantly weaker than normal plasma fibrinogen. Moreover, K-III fibrinogen showed a markedly reduced “D:D” interaction. However, all functions of r-γF290L fibrinogen were similar to WT. An in silico analysis confirmed the above results. Conclusion: The present results demonstrated that γA289 is crucial for the γ-module structure, and the γA289V substitution markedly reduced fibrinogen secretion. Moreover, K-III fibrinogen showed markedly reduced fibrin polymerization and “D:D” interactions. γA289V fibrinogen was confirmed as hypodysfibrinogenemia.", "subitem_description_type": "Abstract"}]}, "item_6_description_30": {"attribute_name": "資源タイプ（コンテンツの種類）", "attribute_value_mlt": [{"subitem_description": "Article", "subitem_description_type": "Other"}]}, "item_6_description_5": {"attribute_name": "引用", "attribute_value_mlt": [{"subitem_description": "INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY.42(2):190-197(2020)", "subitem_description_type": "Other"}]}, "item_6_link_3": {"attribute_name": "信州大学研究者総覧へのリンク", "attribute_value_mlt": [{"subitem_link_text": "Taira, Chiaki", "subitem_link_url": "https://soar-rd.shinshu-u.ac.jp/profile/ja.upAVgekh.html"}, {"subitem_link_text": "Higuchi, Yumiko", "subitem_link_url": "https://soar-rd.shinshu-u.ac.jp/profile/ja.OhfpgCkh.html"}, {"subitem_link_text": "Okumura, Nobuo", "subitem_link_url": "https://soar-rd.shinshu-u.ac.jp/profile/ja.OVnmgCkh.html"}]}, "item_6_link_67": {"attribute_name": "WoS", "attribute_value_mlt": [{"subitem_link_text": "Web of Science", "subitem_link_url": "http://gateway.isiknowledge.com/gateway/Gateway.cgi?\u0026GWVersion=2\u0026SrcAuth=ShinshuUniv\u0026SrcApp=ShinshuUniv\u0026DestLinkType=FullRecord\u0026DestApp=WOS\u0026KeyUT=000508596500001"}]}, "item_6_publisher_4": {"attribute_name": "出版者", "attribute_value_mlt": [{"subitem_publisher": "WILEY"}]}, "item_6_relation_47": {"attribute_name": "PubMed", "attribute_value_mlt": [{"subitem_relation_name": [{"subitem_relation_name_text": "31957968"}], "subitem_relation_type_id": {"subitem_relation_type_id_text": "https://www.ncbi.nlm.nih.gov/pubmed/31957968", "subitem_relation_type_select": "PMID"}}]}, "item_6_relation_48": {"attribute_name": "DOI", "attribute_value_mlt": [{"subitem_relation_name": [{"subitem_relation_name_text": "10.1111/ijlh.13152"}], "subitem_relation_type_id": {"subitem_relation_type_id_text": "https://doi.org/10.1111/ijlh.13152", "subitem_relation_type_select": "DOI"}}]}, "item_6_rights_62": {"attribute_name": "権利", "attribute_value_mlt": [{"subitem_rights": "This is the peer reviewed version of the following article: [Kaido, T, Yoda, M, Kamijo, T, Taira, C, Higuchi, Y, Okumura, N. Heterozygous variant fibrinogen γA289V (Kanazawa III) was confirmed as hypodysfibrinogenemia by plasma and recombinant fibrinogens. Int J Lab Hematol. 2020; 42: 190© 197.], which has been published in final form at https://doi.org/10.1111/ijlh.13152. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions."}]}, "item_6_select_64": {"attribute_name": "著者版フラグ", "attribute_value_mlt": [{"subitem_select_item": "author"}]}, "item_6_source_id_35": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "1751-5521", "subitem_source_identifier_type": "PISSN"}]}, "item_6_source_id_39": {"attribute_name": "NII ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "1751-553X", "subitem_source_identifier_type": "EISSN"}]}, "item_6_source_id_40": {"attribute_name": "書誌レコードID", "attribute_value_mlt": [{"subitem_source_identifier": "AA12801851", "subitem_source_identifier_type": "NCID"}]}, "item_6_text_69": {"attribute_name": "wosonly authkey", "attribute_value_mlt": [{"subitem_text_value": "\"D; D\" interaction; dysfibrinogenemia; fibrinogen; hypodysfibrinogenemia; gamma-module"}]}, "item_6_text_70": {"attribute_name": "wosonly keywords", "attribute_value_mlt": [{"subitem_text_value": "POLYMERIZATION; GAMMA"}]}, "item_6_textarea_68": {"attribute_name": "wosonly abstract", "attribute_value_mlt": [{"subitem_textarea_value": "Introduction Congenital fibrinogen disorders are classified as afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia. However, difficulties are associated with discriminating between dysfibrinogenemia, hypofibrinogenemia, and hypodysfibrinogenemia using routine analyses. We previously reported a heterozygous variant fibrinogen (gamma A289V; Kanazawa III) as hypodysfibrinogenemia; however, the same variant had previously been described as hypofibrinogenemia. To clarify the production of gamma A289V fibrinogen, we expressed recombinant gamma A289V (r-gamma A289V) fibrinogen and compared it with wild-type (WT) and adjacent recombinant variant fibrinogens. Methods Target mutations were introduced into a fibrinogen gamma-chain expression vector by site-directed mutagenesis, and the vector was then transfected into Chinese hamster ovary cells to produce recombinant fibrinogen. Fibrinogen was purified from the plasma of the proposita, and culture media and fibrinogen functions were analyzed using fibrin polymerization, plasmin protection, and FXIIIa-catalyzed fibrinogen cross-linking. Results The fibrinogen concentration ratio of the culture media to cell lysates was markedly lower for r-gamma A289V fibrinogen than for WT. Because the secretion of recombinant gamma F290L (r-gamma F290L) fibrinogen was similar to WT, we compared r-gamma F290L fibrinogen functions with WT. The fibrin polymerization of Kanazawa III plasma (K-III) fibrinogen was significantly weaker than normal plasma fibrinogen. Moreover, K-III fibrinogen showed a markedly reduced \"D:D\" interaction. However, all functions of r-gamma F290L fibrinogen were similar to WT. An in silico analysis confirmed the above results. Conclusion The present results demonstrated that gamma A289 is crucial for the gamma-module structure, and the gamma A289V substitution markedly reduced fibrinogen secretion. Moreover, K-III fibrinogen showed markedly reduced fibrin polymerization and \"D:D\" interactions. gamma A289V fibrinogen was confirmed as hypodysfibrinogenemia."}]}, "item_creator": {"attribute_name": "著者", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Kaido, Takahiro", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "109398", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Yoda, Masahiro", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "109399", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Kamijo, Tomu", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "109400", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Taira, Chiaki", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "109401", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Higuchi, Yumiko", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "109402", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Okumura, Nobuo", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "109403", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "ファイル情報", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2021-01-20"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "INTERNATIONALJOURNALOFLABORATORYHEMATOLOGY42-2_190.pdf", "filesize": [{"value": "771.4 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensefree": "This is the peer reviewed version of the following article: [Kaido, T, Yoda, M, Kamijo, T, Taira, C, Higuchi, Y, Okumura, N. Heterozygous variant fibrinogen γA289V (Kanazawa III) was confirmed as hypodysfibrinogenemia by plasma and recombinant fibrinogens. Int J Lab Hematol. 2020; 42: 190© 197.], which has been published in final form at https://doi.org/10.1111/ijlh.13152. 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