{"_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"]}, "item_6_biblio_info_6": {"attribute_name": "\u66f8\u8a8c\u60c5\u5831", "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": "\u6284\u9332", "attribute_value_mlt": [{"subitem_description": "Hereditary fibrinogen A\u03b1-chain amyloidosis (A\u03b1-chain amyloidosis) is a type of autosomal dominant systemic amyloidosis caused by mutations in fibrinogen A\u03b1-chain gene (FGA). Patients with A\u03b1-chain amyloidosis have been mainly reported in Western countries but have been rarely reported in Asia, with only five patients with A\u03b1-chain amyloidosis being reported in Korea, China, and Japan. Clinically, the most prominent manifestation in Asian patients with A\u03b1-chain amyloidosis is progressive nephropathy caused by excessive amyloid deposition in the glomeruli, which is similar to that observed in patients with A\u03b1-chain amyloidosis in Western countries. In molecular features in Asian A\u03b1-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\u2013555) of mature A\u03b1-chain, which was similar to that observed in patients with A\u03b1-chain amyloidosis in other countries. The precise number of Asian patients with A\u03b1-chain amyloidosis is unclear. However, patients with A\u03b1-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": "\u8cc7\u6e90\u30bf\u30a4\u30d7\uff08\u30b3\u30f3\u30c6\u30f3\u30c4\u306e\u7a2e\u985e\uff09", "attribute_value_mlt": [{"subitem_description": "Article", "subitem_description_type": "Other"}]}, "item_6_description_5": {"attribute_name": "\u5f15\u7528", "attribute_value_mlt": [{"subitem_description": "INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES.19:320(2018)", "subitem_description_type": "Other"}]}, "item_6_link_3": {"attribute_name": "\u4fe1\u5dde\u5927\u5b66\u7814\u7a76\u8005\u7dcf\u89a7\u3078\u306e\u30ea\u30f3\u30af", "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?\u0026GWVersion=2\u0026SrcAuth=ShinshuUniv\u0026SrcApp=ShinshuUniv\u0026DestLinkType=FullRecord\u0026DestApp=WOS\u0026KeyUT=000424407200310"}]}, "item_6_publisher_4": {"attribute_name": "\u51fa\u7248\u8005", "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": "\u6a29\u5229", "attribute_value_mlt": [{"subitem_rights": "\u00a9 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_select_64": {"attribute_name": "\u8457\u8005\u7248\u30d5\u30e9\u30b0", "attribute_value_mlt": [{"subitem_select_item": "publisher"}]}, "item_6_source_id_35": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "1422-0067", "subitem_source_identifier_type": "ISSN"}]}, "item_6_source_id_39": {"attribute_name": "NII ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "1422-0067", "subitem_source_identifier_type": "ISSN"}]}, "item_6_source_id_40": {"attribute_name": "\u66f8\u8a8c\u30ec\u30b3\u30fc\u30c9ID", "attribute_value_mlt": [{"subitem_source_identifier": "AA12038549", "subitem_source_identifier_type": "NCID"}]}, "item_6_text_69": {"attribute_name": "wosonly authkey", "attribute_value_mlt": [{"subitem_text_value": "fibrinogen A-chain amyloidosis; hereditary renal amyloidosis; Ostertag-type amyloidosis; laser microdissection; proteomics analysis"}]}, "item_6_text_70": {"attribute_name": "wosonly keywords", "attribute_value_mlt": [{"subitem_text_value": "STEM-CELL TRANSPLANTATION; ALPHA-CHAIN; RENAL AMYLOIDOSIS; TRANSTHYRETIN AMYLOIDOSIS; LIVER-TRANSPLANTATION; SYSTEMIC AMYLOIDOSIS; GENE; VARIANT; MUTATION; THERAPY"}]}, "item_6_textarea_68": {"attribute_name": "wosonly abstract", "attribute_value_mlt": [{"subitem_textarea_value": "Hereditary fibrinogen A-chain amyloidosis (A-chain amyloidosis) is a type of autosomal dominant systemic amyloidosis caused by mutations in fibrinogen A-chain gene (FGA). Patients with A-chain amyloidosis have been mainly reported in Western countries but have been rarely reported in Asia, with only five patients with A-chain amyloidosis being reported in Korea, China, and Japan. Clinically, the most prominent manifestation in Asian patients with A-chain amyloidosis is progressive nephropathy caused by excessive amyloid deposition in the glomeruli, which is similar to that observed in patients with A-chain amyloidosis in Western countries. In molecular features in Asian A-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 A-chain, which was similar to that observed in patients with A-chain amyloidosis in other countries. The precise number of Asian patients with A-chain amyloidosis is unclear. However, patients with A-chain amyloidosis do exist in Asian countries, and the majority of these patients may be diagnosed with other types of systemic amyloidosis."}]}, "item_creator": {"attribute_name": "\u8457\u8005", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Yazaki, Masahide"}], "nameIdentifiers": [{"nameIdentifier": "109393", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Yoshinaga, Tsuneaki"}], "nameIdentifiers": [{"nameIdentifier": "109394", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Sekijima, Yoshiki"}], "nameIdentifiers": [{"nameIdentifier": "109395", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Kametani, Fuyuki"}], "nameIdentifiers": [{"nameIdentifier": "109396", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Okumura, Nobuo"}], "nameIdentifiers": [{"nameIdentifier": "109397", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "\u30d5\u30a1\u30a4\u30eb\u60c5\u5831", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2020-05-15"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "ijms-19-00320-Yazaki.pdf", "filesize": [{"value": "1.6 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensefree": "\u00a9 2018 by the authors. 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