Item type |
学術雑誌論文 / Journal Article(1) |
公開日 |
2020-05-15 |
タイトル |
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タイトル |
Hereditary Fibrinogen Aα-Chain Amyloidosis in Asia: Clinical and Molecular Characteristics |
言語 |
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言語 |
eng |
DOI |
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関連識別子 |
https://doi.org/10.3390/ijms19010320 |
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関連名称 |
10.3390/ijms19010320 |
キーワード |
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主題 |
fibrinogen Aα-chain amyloidosis, hereditary renal amyloidosis, Ostertag-type amyloidosis, laser microdissection, proteomics analysis |
資源タイプ |
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資源 |
http://purl.org/coar/resource_type/c_6501 |
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タイプ |
journal article |
著者 |
Yazaki, Masahide
Yoshinaga, Tsuneaki
Sekijima, Yoshiki
Kametani, Fuyuki
Okumura, Nobuo
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信州大学研究者総覧へのリンク |
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氏名 |
Yazaki, Masahide |
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URL |
https://soar-rd.shinshu-u.ac.jp/profile/ja.gpTauakh.html |
信州大学研究者総覧へのリンク |
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氏名 |
Yoshinaga, Tsuneaki |
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URL |
https://soar-rd.shinshu-u.ac.jp/profile/ja.yVTmbVym.html |
信州大学研究者総覧へのリンク |
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氏名 |
Sekijima, Yoshiki |
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URL |
https://soar-rd.shinshu-u.ac.jp/profile/ja.WhLNuakh.html |
信州大学研究者総覧へのリンク |
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氏名 |
Okumura, Nobuo |
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URL |
https://soar-rd.shinshu-u.ac.jp/profile/ja.OVnmgCkh.html |
出版者 |
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出版者 |
MDPI |
引用 |
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内容記述 |
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES.19:320(2018) |
書誌情報 |
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
巻 19,
号 1,
p. 320,
発行日 2018-01-22
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抄録 |
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内容記述 |
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. |
資源タイプ(コンテンツの種類) |
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内容記述 |
Article |
ISSN |
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収録物識別子タイプ |
EISSN |
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収録物識別子 |
1422-0067 |
書誌レコードID |
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収録物識別子タイプ |
NCID |
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収録物識別子 |
AA12038549 |
PubMed |
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関連識別子 |
https://www.ncbi.nlm.nih.gov/pubmed/29361747 |
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関連名称 |
29361747 |
権利 |
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権利情報 |
© 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/). |
出版タイプ |
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出版タイプ |
VoR |
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出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
WoS |
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URL |
http://gateway.isiknowledge.com/gateway/Gateway.cgi?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000424407200310 |