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

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/10091/16227
0393bed7-ee27-4292-95ea-d5e10b8fdfd0
名前 / ファイル ライセンス アクション
Heterozygous_B_beta-chain_C-terminal_12_amino_acid_elongation_variant.pdf Heterozygous_B_beta-chain_C-terminal_12_amino_acid_elongation_variant.pdf (303.4 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2013-02-04
タイトル
タイトル Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia
言語
言語 eng
DOI
関連識別子 https://doi.org/10.1097/MBC.0b013e32834cb243
関連名称 10.1097/MBC.0b013e32834cb243
キーワード
主題 albumin-binding form, B beta-chain, dysfibrinogen, variant dimeric fibrinogen
資源タイプ
資源 http://purl.org/coar/resource_type/c_6501
タイプ journal article
著者 Okumura, Nobuo

× Okumura, Nobuo

en Okumura, Nobuo

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Terasawa, Fumiko

× Terasawa, Fumiko

en Terasawa, Fumiko

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Takezawa, Yuka

× Takezawa, Yuka

en Takezawa, Yuka

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Hirota-Kawadobora, Masako

× Hirota-Kawadobora, Masako

en Hirota-Kawadobora, Masako

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Inaba, Tohru

× Inaba, Tohru

en Inaba, Tohru

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Fujita, Naohisa

× Fujita, Naohisa

en Fujita, Naohisa

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Saito, Masazumi

× Saito, Masazumi

en Saito, Masazumi

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Sugano, Mitsutoshi

× Sugano, Mitsutoshi

en Sugano, Mitsutoshi

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Honda, Takayuki

× Honda, Takayuki

en 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
抄録
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)
ISSN
収録物識別子タイプ PISSN
収録物識別子 0957-5235
書誌レコードID
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)
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
URL http://gateway.isiknowledge.com/gateway/Gateway.cgi?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000298627400016
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Cite as

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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