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  1. 050 医学部, 大学院医学系研究科
  2. 0501 学術論文

Novel heterozygous dysfibrinogenemia, Sumida (A alpha C472S), showed markedly impaired lateral aggregation of protofibrils and mildly lower functional fibrinogen levels

http://hdl.handle.net/10091/18412
95fe0dc8-79bb-407b-a1bc-3ae41c5ca661
名前 / ファイル ライセンス アクション
Novel_heterozygous_dysfibrinogenemia.pdf Novel_heterozygous_dysfibrinogenemia.pdf (817.6 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2015-06-02
タイトル
言語 en
タイトル Novel heterozygous dysfibrinogenemia, Sumida (A alpha C472S), showed markedly impaired lateral aggregation of protofibrils and mildly lower functional fibrinogen levels
言語
言語 eng
キーワード
主題Scheme Other
主題 Fibrinogen
キーワード
主題Scheme Other
主題 Dysfibrinogenemia
キーワード
主題Scheme Other
主題 Lateral aggregation of protofibrils
キーワード
主題Scheme Other
主題 Fibrin fiber
キーワード
主題Scheme Other
主題 Clot lysis
資源タイプ
資源 http://purl.org/coar/resource_type/c_6501
タイプ journal article
著者 Ikeda, Minami

× Ikeda, Minami

WEKO 6141

en Ikeda, Minami

Search repository
Arai, Shinpei

× Arai, Shinpei

WEKO 6142

en Arai, Shinpei

Search repository
Mukai, Saki

× Mukai, Saki

WEKO 6143

en Mukai, Saki

Search repository
Takezawa, Yuka

× Takezawa, Yuka

WEKO 6144

en Takezawa, Yuka

Search repository
Terasawa, Fumiko

× Terasawa, Fumiko

WEKO 6145

en Terasawa, Fumiko

Search repository
Okumura, Nobuo

× Okumura, Nobuo

WEKO 6146

en Okumura, Nobuo

Search repository
信州大学研究者総覧へのリンク
氏名 Okumura, Nobuo
URL http://soar-rd.shinshu-u.ac.jp/profile/ja.OVnmgCkh.html
出版者
出版者 PERGAMON-ELSEVIER SCIENCE LTD
引用
内容記述タイプ Other
内容記述 THROMBOSIS RESEARCH. 135(4):710-717 (2015)
書誌情報 THROMBOSIS RESEARCH

巻 135, 号 4, p. 710-717, 発行日 2015-04
抄録
内容記述タイプ Abstract
内容記述 Introduction: We encountered a 6-year-old girl with systemic lupus erythematosus. Although no bleeding or thrombotic tendency was detected, routine coagulation screening tests revealed slightly lower plasma fibrinogen levels, as determined by functional and antigenic measurements (functional/antigenic ratio=0.857), suggesting hypodysfibrinogenemia. Materials and methods: DNA sequence and functional analyses were performed on purified plasma fibrinogen, and recombinant variant fibrinogen was synthesized in Chinese hamster ovary cells based on the results obtained. Results: DNA sequencing revealed a heterozygous A alpha C472S substitution (mature protein residue number) in the alpha C-domain. A alpha C472S fibrinogen indicated the presence of additional disulfide-bonded molecules, and markedly impaired lateral aggregation of protofibrils in spite of slightly lower functional plasma fibrinogen levels. Scanning electron microscopic observations showed a thin fiber fibrin clot, and t-PA and plasminogen-mediated clot lysis was similar to that of a normal control. Recombinant variant fibrinogen-producing cells demonstrated that destruction of the A alpha 442C-472C disulfide bond did not prevent the synthesis or secretion of fibrinogen, whereas the variant A alpha chain of the secreted protein was degraded faster than that of the normal control. Conclusion: Our results suggest that A alpha C472S fibrinogen may cause dysfibrinogenemia, but not hypofibrinogenemia. The destruction and steric hindrance of the alpha C-domain of variant fibrinogen led to the impaired lateral aggregation of protofibrils and t-PA and plasminogen-mediated fibrinolysis, as well as several previously reported variants located in the alpha C-domain, and demonstrated the presence of disulfide-bonded molecules.
資源タイプ(コンテンツの種類)
内容記述タイプ Other
内容記述 Article
ISSN
収録物識別子タイプ PISSN
収録物識別子 0049-3848
書誌レコードID
収録物識別子タイプ NCID
収録物識別子 AA00863148
PubMed
関連識別子
識別子タイプ PMID
関連識別子 https://pubmed.ncbi.nlm.nih.gov/25613923
関連名称
関連名称 25613923
DOI
関連識別子
識別子タイプ DOI
関連識別子 https://doi.org/10.1016/j.thromres.2015.01.013
関連名称
関連名称 10.1016/j.thromres.2015.01.013
権利
権利情報 © 2015, Elsevier. Licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International http://creativecommons.org/licenses/by-nc-nd/4.0/
出版タイプ
出版タイプ AM
出版タイプResource http://purl.org/coar/version/c_ab4af688f83e57aa
WoS
表示名 Web of Science
URL http://gateway.isiknowledge.com/gateway/Gateway.cgi?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000351086000024
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