{"created":"2021-03-01T06:07:40.101487+00:00","id":3835,"links":{},"metadata":{"_buckets":{"deposit":"c7c77236-f329-461b-be6d-58253adcc191"},"_deposit":{"id":"3835","owners":[],"pid":{"revision_id":0,"type":"depid","value":"3835"},"status":"published"},"_oai":{"id":"oai:soar-ir.repo.nii.ac.jp:00003835","sets":["461:462"]},"author_link":["7398","7399","7400","7401","7402","7403","7404","7405","7406"],"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":"2012-01","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"90","bibliographicPageStart":"87","bibliographicVolumeNumber":"23","bibliographic_titles":[{"bibliographic_title":"BLOOD COAGULATION & FIBRINOLYSIS"}]}]},"item_6_description_20":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"A heterozygous patient with dysfibrinogenemia with slight bleeding and no thrombotic complications was diagnosed with fibrinogen Kyoto VI (K-VI). To elucidate the genetic mutation(s) and characterize the variant protein, we performed the following experiments and compared with identical and similar variants that have already been reported. The proposita's PCR-amplified DNA was analyzed by sequencing and her purified plasma fibrinogen underwent SDS-PAGE followed by immunoblotting, fibrin polymerization, and scanning electron microscopic observation of fibrin clot and fibers. Sequence analyses showed that K-VI fibrinogen substituted W (TGG) for terminal codon (TAG), resulting in 12 amino acid elongation 462-473 (WSPIRRFLLFCM) in the B beta-chain. Protein analyses indicated that the presence of some albumin-binding variant fibrinogens and a dimeric molecule of variant fibrinogens reduced fibrin polymerization, with a thinner fiber and aberrant fibrin network. These results are almost the same as for the identical variant of Magdeburg, however, different from the similar variant of Osaka VI [ 12 amino acid elongation 462-473 (KSPIRRFLLFCM) in the B beta-chain] in the presence of variant forms and clot structure. We speculate the side-chain difference at 462 residues, W in K-VI, K in Osaka VI, and/or the difference in the presence of disulfide bridged forms of variant fibrinogens, led to the notable difference in the fibrin bundle network. Although a strong evolutional and structural association between B beta-chain and gamma-chain molecules is established, the corresponding recombinant 15 residue elongation variants of the fibrinogen gamma-chain showed reduced assembly and secretion.","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":"BLOOD COAGULATION & FIBRINOLYSIS. 23(1):87-90 (2012)","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":"Okumura, Nobuo","subitem_link_url":"http://soar-rd.shinshu-u.ac.jp/profile/ja.OVnmgCkh.html"},{"subitem_link_text":"Honda, Takayuki","subitem_link_url":"http://soar-rd.shinshu-u.ac.jp/profile/ja.WeDUHFkh.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?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000298627400016"}]},"item_6_publisher_4":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"LIPPINCOTT WILLIAMS & WILKINS"}]},"item_6_relation_47":{"attribute_name":"PubMed","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_text":"22001526"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://pubmed.ncbi.nlm.nih.gov/22001526","subitem_relation_type_select":"PMID"}}]},"item_6_relation_48":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_text":"10.1097/MBC.0b013e32834cb243"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://doi.org/10.1097/MBC.0b013e32834cb243","subitem_relation_type_select":"DOI"}}]},"item_6_rights_62":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"This is a non-final version of an article published in final form in BLOOD COAGULATION & FIBRINOLYSIS. 23(1):87-90 (2012)"}]},"item_6_source_id_35":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"0957-5235","subitem_source_identifier_type":"PISSN"}]},"item_6_source_id_40":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AA10794937","subitem_source_identifier_type":"NCID"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Okumura,  Nobuo","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"7398","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Terasawa,  Fumiko","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"7399","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Takezawa,  Yuka","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"7400","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Hirota-Kawadobora,  Masako","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"7401","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Inaba,  Tohru","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"7402","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Fujita,  Naohisa","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"7403","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Saito,  Masazumi","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"7404","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Sugano,  Mitsutoshi","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"7405","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Honda,  Takayuki","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"7406","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":"2015-09-24"}],"displaytype":"detail","filename":"Heterozygous_B_beta-chain_C-terminal_12_amino_acid_elongation_variant.pdf","filesize":[{"value":"303.4 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"Heterozygous_B_beta-chain_C-terminal_12_amino_acid_elongation_variant.pdf","url":"https://soar-ir.repo.nii.ac.jp/record/3835/files/Heterozygous_B_beta-chain_C-terminal_12_amino_acid_elongation_variant.pdf"},"version_id":"bbc48225-7fd6-4274-bc70-b18f2dad04f6"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"albumin-binding form","subitem_subject_scheme":"Other"},{"subitem_subject":"B beta-chain","subitem_subject_scheme":"Other"},{"subitem_subject":"dysfibrinogen","subitem_subject_scheme":"Other"},{"subitem_subject":"variant dimeric fibrinogen","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":"journal article","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia","subitem_title_language":"en"}]},"item_type_id":"6","owner":"1","path":["462"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2013-02-04"},"publish_date":"2013-02-04","publish_status":"0","recid":"3835","relation_version_is_last":true,"title":["Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia"],"weko_creator_id":"1","weko_shared_id":-1},"updated":"2022-12-14T04:39:56.414721+00:00"}