{"_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": ["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. 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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": "著者版フラグ", "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": "EISSN"}]}, "item_6_source_id_39": {"attribute_name": "NII 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_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. 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