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Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia
http://hdl.handle.net/10091/16227
http://hdl.handle.net/10091/162270393bed7-ee27-4292-95ea-d5e10b8fdfd0
名前 / ファイル | ライセンス | アクション |
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Item type | 学術雑誌論文 / Journal Article(1) | |||||||||||||||||||||||
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公開日 | 2013-02-04 | |||||||||||||||||||||||
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タイトル | Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia | |||||||||||||||||||||||
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言語 | eng | |||||||||||||||||||||||
DOI | ||||||||||||||||||||||||
関連識別子 | https://doi.org/10.1097/MBC.0b013e32834cb243 | |||||||||||||||||||||||
関連名称 | 10.1097/MBC.0b013e32834cb243 | |||||||||||||||||||||||
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主題 | albumin-binding form, B beta-chain, dysfibrinogen, variant dimeric fibrinogen | |||||||||||||||||||||||
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資源 | http://purl.org/coar/resource_type/c_6501 | |||||||||||||||||||||||
タイプ | journal article | |||||||||||||||||||||||
著者 |
Okumura, Nobuo
× Okumura, Nobuo
× Terasawa, Fumiko
× Takezawa, Yuka
× Hirota-Kawadobora, Masako
× Inaba, Tohru
× Fujita, Naohisa
× Saito, Masazumi
× Sugano, Mitsutoshi
× Honda, Takayuki
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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 | |||||||||||||||||||||||
信州大学研究者総覧へのリンク | ||||||||||||||||||||||||
氏名 | Honda, Takayuki | |||||||||||||||||||||||
URL | http://soar-rd.shinshu-u.ac.jp/profile/ja.WeDUHFkh.html | |||||||||||||||||||||||
出版者 | ||||||||||||||||||||||||
出版者 | LIPPINCOTT WILLIAMS & WILKINS | |||||||||||||||||||||||
引用 | ||||||||||||||||||||||||
内容記述 | BLOOD COAGULATION & FIBRINOLYSIS. 23(1):87-90 (2012) | |||||||||||||||||||||||
書誌情報 |
BLOOD COAGULATION & FIBRINOLYSIS 巻 23, 号 1, p. 87-90, 発行日 2012-01 |
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内容記述 | A heterozygous patient with dysfibrinogenemia with slight bleeding and no thrombotic complications was diagnosed with fibrinogen Kyoto VI (K-VI). To elucidate the genetic mutation(s) and characterize the variant protein, we performed the following experiments and compared with identical and similar variants that have already been reported. The proposita's PCR-amplified DNA was analyzed by sequencing and her purified plasma fibrinogen underwent SDS-PAGE followed by immunoblotting, fibrin polymerization, and scanning electron microscopic observation of fibrin clot and fibers. Sequence analyses showed that K-VI fibrinogen substituted W (TGG) for terminal codon (TAG), resulting in 12 amino acid elongation 462-473 (WSPIRRFLLFCM) in the B beta-chain. Protein analyses indicated that the presence of some albumin-binding variant fibrinogens and a dimeric molecule of variant fibrinogens reduced fibrin polymerization, with a thinner fiber and aberrant fibrin network. These results are almost the same as for the identical variant of Magdeburg, however, different from the similar variant of Osaka VI [ 12 amino acid elongation 462-473 (KSPIRRFLLFCM) in the B beta-chain] in the presence of variant forms and clot structure. We speculate the side-chain difference at 462 residues, W in K-VI, K in Osaka VI, and/or the difference in the presence of disulfide bridged forms of variant fibrinogens, led to the notable difference in the fibrin bundle network. Although a strong evolutional and structural association between B beta-chain and gamma-chain molecules is established, the corresponding recombinant 15 residue elongation variants of the fibrinogen gamma-chain showed reduced assembly and secretion. | |||||||||||||||||||||||
資源タイプ(コンテンツの種類) | ||||||||||||||||||||||||
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収録物識別子タイプ | PISSN | |||||||||||||||||||||||
収録物識別子 | 0957-5235 | |||||||||||||||||||||||
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収録物識別子タイプ | NCID | |||||||||||||||||||||||
収録物識別子 | AA10794937 | |||||||||||||||||||||||
PubMed | ||||||||||||||||||||||||
識別子タイプ | PMID | |||||||||||||||||||||||
関連識別子 | https://pubmed.ncbi.nlm.nih.gov/22001526 | |||||||||||||||||||||||
関連名称 | 22001526 | |||||||||||||||||||||||
権利 | ||||||||||||||||||||||||
権利情報 | This is a non-final version of an article published in final form in BLOOD COAGULATION & FIBRINOLYSIS. 23(1):87-90 (2012) | |||||||||||||||||||||||
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出版タイプ | AM | |||||||||||||||||||||||
出版タイプResource | http://purl.org/coar/version/c_ab4af688f83e57aa | |||||||||||||||||||||||
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URL | http://gateway.isiknowledge.com/gateway/Gateway.cgi?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000298627400016 |
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Okumura, Nobuo, Terasawa, Fumiko, Takezawa, Yuka, Hirota-Kawadobora, Masako, Inaba, Tohru, Fujita, Naohisa, Saito, Masazumi, Sugano, Mitsutoshi, Honda, Takayuki, 2012, Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia: LIPPINCOTT WILLIAMS & WILKINS, 87–90 p.
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