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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}
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/10091/10090dbaea3f-a500-4e41-88c9-3151b57ba3fc
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 | 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× Hirota-Kawadobora, M× Tozuka, M× Ishii, K× Terasawa, F× 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 |
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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 |