WEKO3
アイテム
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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. 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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\u0027s 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. Blood Coagul Fibrinolysis 23:87-90 (C) 2011 Wolters Kluwer Health vertical bar Lippincott Williams \u0026 Wilkins."}]}, "item_creator": {"attribute_name": "著者", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Okumura, Nobuo", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7398", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Terasawa, Fumiko", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7399", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Takezawa, Yuka", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7400", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Hirota-Kawadobora, Masako", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7401", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Inaba, Tohru", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7402", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Fujita, Naohisa", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7403", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Saito, Masazumi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7404", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Sugano, Mitsutoshi", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7405", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Honda, Takayuki", "creatorNameLang": "en"}], "nameIdentifiers": [{"nameIdentifier": "7406", "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": "Heterozygous_B_beta-chain_C-terminal_12_amino_acid_elongation_variant.pdf", "filesize": [{"value": "303.4 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_note", "mimetype": "application/pdf", "size": 303400.0, "url": {"label": "Heterozygous_B_beta-chain_C-terminal_12_amino_acid_elongation_variant.pdf", "url": "https://soar-ir.repo.nii.ac.jp/record/3835/files/Heterozygous_B_beta-chain_C-terminal_12_amino_acid_elongation_variant.pdf"}, "version_id": "bbc48225-7fd6-4274-bc70-b18f2dad04f6"}]}, "item_keyword": {"attribute_name": "キーワード", "attribute_value_mlt": [{"subitem_subject": "albumin-binding form", "subitem_subject_scheme": "Other"}, {"subitem_subject": "B beta-chain", "subitem_subject_scheme": "Other"}, {"subitem_subject": "dysfibrinogen", "subitem_subject_scheme": "Other"}, {"subitem_subject": "variant dimeric fibrinogen", "subitem_subject_scheme": "Other"}]}, "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": "Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia", "item_titles": {"attribute_name": "タイトル", "attribute_value_mlt": [{"subitem_title": "Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia", "subitem_title_language": "en"}]}, "item_type_id": "6", "owner": "1", "path": ["462"], "permalink_uri": "http://hdl.handle.net/10091/16227", "pubdate": {"attribute_name": "PubDate", "attribute_value": "2013-02-04"}, "publish_date": "2013-02-04", "publish_status": "0", "recid": "3835", "relation": {}, "relation_version_is_last": true, "title": ["Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia"], "weko_shared_id": -1}
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 | |||||
タイトル | ||||||
言語 | en | |||||
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 | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | albumin-binding form | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | B beta-chain | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | dysfibrinogen | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | 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× 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 | ||||||
内容記述タイプ | Other | |||||
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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抄録 | ||||||
内容記述タイプ | Abstract | |||||
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) | ||||||
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 | 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 | |||||
DOI | ||||||
識別子タイプ | DOI | |||||
関連識別子 | https://doi.org/10.1097/MBC.0b013e32834cb243 | |||||
関連名称 | 10.1097/MBC.0b013e32834cb243 | |||||
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 | ||||||
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=000298627400016 |