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

gamma 375W fibrinogen-synthesizing CHO cells indicate the accumulation of variant fibrinogen within endoplasmic reticulum

http://hdl.handle.net/10091/17618
http://hdl.handle.net/10091/17618
1c35e957-de78-4411-844f-820645348aa2
名前 / ファイル ライセンス アクション
gamma_375W_fibrinogen-synthesizing_CHO_cells.pdf gamma_375W_fibrinogen-synthesizing_CHO_cells.pdf (884.1 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2014-06-20
タイトル
タイトル gamma 375W fibrinogen-synthesizing CHO cells indicate the accumulation of variant fibrinogen within endoplasmic reticulum
言語
言語 eng
DOI
関連識別子 https://doi.org/10.1016/j.thromres.2013.10.033
関連名称 10.1016/j.thromres.2013.10.033
キーワード
主題 Endoplasmic reticulum, Fibrinogen storage disease, Fibrinogen gamma-chain mutation, Hepatic endoplasmic reticulum storage disease, Hypofibrinogenemia
資源タイプ
資源 http://purl.org/coar/resource_type/c_6501
タイプ journal article
著者 Kobayashi, Tamaki

× Kobayashi, Tamaki

en Kobayashi, Tamaki

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Arai, Shinpei

× Arai, Shinpei

en Arai, Shinpei

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Ogiwara, Naoko

× Ogiwara, Naoko

en Ogiwara, Naoko

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Takezawa, Yuka

× Takezawa, Yuka

en Takezawa, Yuka

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Nanya, Mai

× Nanya, Mai

en Nanya, Mai

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Terasawa, Fumiko

× Terasawa, Fumiko

en Terasawa, Fumiko

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Okumura, Nobuo

× Okumura, Nobuo

en Okumura, Nobuo

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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
出版者
出版者 PERGAMON-ELSEVIER SCIENCE LTD
引用
内容記述 THROMBOSIS RESEARCH. 133(1):101-107 (2014)
書誌情報 THROMBOSIS RESEARCH

巻 133, 号 1, p. 101-107, 発行日 2014-01
抄録
内容記述 Background: Hepatic endoplasmic reticulum (ER) storage disease (HERSD) associated with hypofibrinogenemia has been reported in patients with four types of heterozygous gamma-chain variant fibrinogen in the C terminal region. Of interest, substitution of gamma R375W induced hypofibrinogenemia and HERSD, whereas gamma R375G induced dysfibrinogenemia. Objectives: To analyze the synthesis of variant fibrinogen and morphological characteristics, we established variant fibrinogen-producing cells and compared them with wild-type fibrinogen-synthesizing cells. Methods: The fibrinogen gamma-chain expression vectors coding gamma 375W and gamma 375G were altered by oligonucleotide-directed mutagenesis and transfected into Chinese hamster ovary (CHO) cells. Synthesis of fibrinogen (media and cell lysates) was measured by ELISA for each cloned cell line and morphological characteristics were observed by immunofluorescence and transmission electron microscopy. Results: The medium/cell lysate fibrinogen ratio of gamma 375W-CHO cells was markedly lower than that of the normal cells and gamma 375G-CHO cells. Immunostaining with anti-fibrinogen antibody showed only gamma 375W-CHO cells, but revealed two types of cells containing cytoplasmic inclusion bodies, scattered large-granular bodies and fibrous forms. Observation by confocal microscopy indicated that both inclusion bodies were colocalized with fibrinogen and ER-membrane protein; furthermore, transmission electron microscopic observation demonstrated dilatation of the ER by large-granular inclusion bodies and fibrous forms filled with regularly structured fibular materials within the dilated ER. Conclusion: These results demonstrated that assembled and non-secreted gamma 375W fibrinogen was accumulated in the dilated ER and aggregated variant fibrinogen was seen as regularly structured fibular materials, which was similar to the fingerprint-like pattern observed at inclusion bodies in patients' hepatocytes affected with HERSD.
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 0049-3848
書誌レコードID
収録物識別子タイプ NCID
収録物識別子 AA00863148
PubMed
識別子タイプ PMID
関連識別子 https://pubmed.ncbi.nlm.nih.gov/24210681
関連名称 24210681
権利
権利情報 Copyright© 2013 Elsevier Ltd.
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=000328911900018
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