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We have already reported impaired fibrin polymerization in a variant fibrinogen (gammaArg275Cys), the Cys being located in the D:D interface. Since this substitution occurred in a heterozygous individual, interpretation of the functional analysis was complicated. We tried to resolve this complication by synthesizing a recombinant variant fibrinogen. Methods: A variant gamma-chain expression plasmid was transfected into Chinese hamster ovary cells expressing normal human fibrinogen Aalpha- and Bbeta-chains. The recombinant variant fibrinogen (gamma275C) was purified using an immunoaffinity column, and we compared its structure and functions with those of normal recombinant fibrinogen (gamma275R) and plasma variant fibrinogen. Results: Mass analyses showed the existence of disulfide-linked Cys in both patient and recombinant variant fibrinogens. Functional analyses indicated that both fibrin polymerization and gamma-gamma dimer formation were markedly impaired in the variant fibrinogen. The impairments were much more pronounced in gamma275C than in plasma variant fibrinogen. In addition, scanning electron microscopic observation of fibrin clots made from gamma275C revealed less dense fibrin fiber bundles and larger fiber diameter than in those made from gamma275R, and also the existence of many aberrant fibrin fibers with tapered ends. Conclusion: These results indicate that gammaArg275 has an important residue affecting the structure and function of the,gamma-chain C-terminal domain. However, the variant D:D interface can interact with that of the normal fibrinogen existing in a heterozygous patient with dysfibrinogenemia."}]}, "item_creator": {"attribute_name": "著者", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Ishikawa,  S", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8465", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Hirota-Kawadobora,  M", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8466", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Tozuka,  M", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8467", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Ishii,  K", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8468", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Terasawa,  F", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8469", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Okumura,  N", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "8470", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "ファイル情報", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2015-09-24"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "JTH-2003-00426R-FIG.pdf", "filesize": [{"value": "855.6 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_note", "mimetype": "application/pdf", "size": 855600.0, "url": {"label": "JTH-2003-00426R-FIG.pdf", "url": "https://soar-ir.repo.nii.ac.jp/record/3990/files/JTH-2003-00426R-FIG.pdf"}, "version_id": "72ed9100-55e4-4941-92f2-164c2ed6059a"}]}, "item_language": {"attribute_name": "言語", "attribute_value_mlt": [{"subitem_language": "eng"}]}, "item_resource_type": {"attribute_name": "資源タイプ", "attribute_value_mlt": [{"resourcetype": "journal article", "resourceuri": "http://purl.org/coar/resource_type/c_6501"}]}, "item_title": "Recombinant fibrinogen, gamma 275Arg -\u003e Cys, exhibits formation of disulfide bond with cysteine and severely impaired D : D interactions", "item_titles": {"attribute_name": "タイトル", "attribute_value_mlt": [{"subitem_title": "Recombinant fibrinogen, gamma 275Arg -\u003e Cys, exhibits formation of disulfide bond with cysteine and severely impaired D : D interactions", "subitem_title_language": "en"}]}, "item_type_id": "6", "owner": "1", "path": ["462"], "permalink_uri": "http://hdl.handle.net/10091/1009", "pubdate": {"attribute_name": "PubDate", "attribute_value": "2008-03-27"}, "publish_date": "2008-03-27", "publish_status": "0", "recid": "3990", "relation": {}, "relation_version_is_last": true, "title": ["Recombinant fibrinogen, gamma 275Arg -\u003e Cys, exhibits formation of disulfide bond with cysteine and severely impaired D : D interactions"], "weko_shared_id": -1}
  1. 050 医学部, 大学院医学系研究科
  2. 0501 学術論文

Recombinant fibrinogen, gamma 275Arg -> Cys, exhibits formation of disulfide bond with cysteine and severely impaired D : D interactions

http://hdl.handle.net/10091/1009
http://hdl.handle.net/10091/1009
0dbaea3f-a500-4e41-88c9-3151b57ba3fc
名前 / ファイル ライセンス アクション
JTH-2003-00426R-FIG.pdf JTH-2003-00426R-FIG.pdf (855.6 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2008-03-27
タイトル
言語 en
タイトル Recombinant fibrinogen, gamma 275Arg -> Cys, exhibits formation of disulfide bond with cysteine and severely impaired D : D interactions
言語
言語 eng
資源タイプ
資源 http://purl.org/coar/resource_type/c_6501
タイプ journal article
著者 Ishikawa, S

× Ishikawa, S

WEKO 8465

en Ishikawa, S

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Hirota-Kawadobora, M

× Hirota-Kawadobora, M

WEKO 8466

en Hirota-Kawadobora, M

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Tozuka, M

× Tozuka, M

WEKO 8467

en Tozuka, M

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Ishii, K

× Ishii, K

WEKO 8468

en Ishii, K

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Terasawa, F

× Terasawa, F

WEKO 8469

en Terasawa, F

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Okumura, N

× Okumura, N

WEKO 8470

en Okumura, N

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信州大学研究者総覧へのリンク
氏名 Okumura, N
URL http://soar-rd.shinshu-u.ac.jp/profile/ja.OVnmgCkh.html
出版者
出版者 BLACKWELL PUBL LTD
引用
内容記述タイプ Other
内容記述 JOURNAL OF THROMBOSIS AND HAEMOSTASIS. 2(3): 468-475 (2004)
書誌情報 JOURNAL OF THROMBOSIS AND HAEMOSTASIS

巻 2, 号 3, p. 468-475, 発行日 2004-03
内容記述
内容記述タイプ Other
内容記述 This is an electronic version of an Article published in Journal of Thrombosis and Haemostasis 2004; 2(3): 468-475
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 1538-7933
書誌レコードID
収録物識別子タイプ NCID
収録物識別子 AA11811179
DOI
識別子タイプ DOI
関連識別子 https://doi.org/10.1111/j.1538-7836.2004.00600.x
関連名称 10.1111/j.1538-7836.2004.00600.x
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=000220289400015
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