{"created":"2021-03-01T06:24:29.306824+00:00","id":21350,"links":{},"metadata":{"_buckets":{"deposit":"4e57dea7-d4b9-4130-a041-78ca3c23b285"},"_deposit":{"id":"21350","owners":[],"pid":{"revision_id":0,"type":"depid","value":"21350"},"status":"published"},"_oai":{"id":"oai:soar-ir.repo.nii.ac.jp:00021350","sets":["461:462"]},"author_link":["109393","109394","109395","109396","109397"],"item_1628147817048":{"attribute_name":"出版タイプ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_6_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2018-01-22","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageStart":"320","bibliographicVolumeNumber":"19","bibliographic_titles":[{"bibliographic_title":"INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES"}]}]},"item_6_description_20":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Hereditary fibrinogen Aα-chain amyloidosis (Aα-chain amyloidosis) is a type of autosomal dominant systemic amyloidosis caused by mutations in fibrinogen Aα-chain gene (FGA). Patients with Aα-chain amyloidosis have been mainly reported in Western countries but have been rarely reported in Asia, with only five patients with Aα-chain amyloidosis being reported in Korea, China, and Japan. Clinically, the most prominent manifestation in Asian patients with Aα-chain amyloidosis is progressive nephropathy caused by excessive amyloid deposition in the glomeruli, which is similar to that observed in patients with Aα-chain amyloidosis in Western countries. In molecular features in Asian Aα-chain amyloidosis, the most common variant, E526V, was found in only one Chinese kindred, and other four kindred each had a different variant, which have not been identified in other countries. These variants are located in the C-terminal region (amino acid residues 517?555) of mature Aα-chain, which was similar to that observed in patients with Aα-chain amyloidosis in other countries. The precise number of Asian patients with Aα-chain amyloidosis is unclear. However, patients with Aα-chain amyloidosis do exist in Asian countries, and the majority of these patients may be diagnosed with other types of systemic amyloidosis.","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 MOLECULAR SCIENCES.19:320(2018)","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":"Yazaki, Masahide","subitem_link_url":"https://soar-rd.shinshu-u.ac.jp/profile/ja.gpTauakh.html"},{"subitem_link_text":"Yoshinaga, Tsuneaki","subitem_link_url":"https://soar-rd.shinshu-u.ac.jp/profile/ja.yVTmbVym.html"},{"subitem_link_text":"Sekijima, Yoshiki","subitem_link_url":"https://soar-rd.shinshu-u.ac.jp/profile/ja.WhLNuakh.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?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000424407200310"}]},"item_6_publisher_4":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"MDPI"}]},"item_6_relation_47":{"attribute_name":"PubMed","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_text":"29361747"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://www.ncbi.nlm.nih.gov/pubmed/29361747","subitem_relation_type_select":"PMID"}}]},"item_6_relation_48":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_name":[{"subitem_relation_name_text":"10.3390/ijms19010320"}],"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://doi.org/10.3390/ijms19010320","subitem_relation_type_select":"DOI"}}]},"item_6_rights_62":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"© 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/)."}]},"item_6_source_id_35":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"1422-0067","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":"AA12038549","subitem_source_identifier_type":"NCID"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Yazaki, Masahide","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"109393","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Yoshinaga, Tsuneaki","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"109394","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Sekijima, Yoshiki","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"109395","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Kametani, Fuyuki","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"109396","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Okumura, Nobuo","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"109397","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":"2020-05-15"}],"displaytype":"detail","filename":"ijms-19-00320-Yazaki.pdf","filesize":[{"value":"1.6 MB"}],"format":"application/pdf","licensefree":"© 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"ijms-19-00320-Yazaki.pdf","url":"https://soar-ir.repo.nii.ac.jp/record/21350/files/ijms-19-00320-Yazaki.pdf"},"version_id":"12125e30-13cb-4ec8-a87f-1b534eaa4f47"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"fibrinogen Aα-chain amyloidosis","subitem_subject_scheme":"Other"},{"subitem_subject":"hereditary renal amyloidosis","subitem_subject_scheme":"Other"},{"subitem_subject":"Ostertag-type amyloidosis","subitem_subject_scheme":"Other"},{"subitem_subject":"laser microdissection","subitem_subject_scheme":"Other"},{"subitem_subject":"proteomics analysis","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":"Hereditary Fibrinogen Aα-Chain Amyloidosis in Asia: Clinical and Molecular Characteristics","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Hereditary Fibrinogen Aα-Chain Amyloidosis in Asia: Clinical and Molecular Characteristics","subitem_title_language":"en"}]},"item_type_id":"6","owner":"1","path":["462"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2020-05-15"},"publish_date":"2020-05-15","publish_status":"0","recid":"21350","relation_version_is_last":true,"title":["Hereditary Fibrinogen Aα-Chain Amyloidosis in Asia: Clinical and Molecular Characteristics"],"weko_creator_id":"1","weko_shared_id":-1},"updated":"2022-12-14T04:19:00.913761+00:00"}