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

The fibrous form of intracellular inclusion bodies in recombinant variant fibrinogen-producing cells is specific to the hepatic fibrinogen storage disease-inducible variant fibrinogen

http://hdl.handle.net/10091/00022105
http://hdl.handle.net/10091/00022105
dc933457-9b2b-4e79-ada1-7161c375474c
名前 / ファイル ライセンス アクション
INTERNATIONALJOURNALOFHEMATOLOGY105_758.pdf INTERNATIONALJOURNALOFHEMATOLOGY105_758.pdf (1.7 MB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2020-05-15
タイトル
言語 en
タイトル The fibrous form of intracellular inclusion bodies in recombinant variant fibrinogen-producing cells is specific to the hepatic fibrinogen storage disease-inducible variant fibrinogen
言語
言語 eng
キーワード
主題Scheme Other
主題 Intracellular inclusion bodies
キーワード
主題Scheme Other
主題 Hypofibrinogenemia
キーワード
主題Scheme Other
主題 Fibrinogen storage disease
キーワード
主題Scheme Other
主題 Immunofluorescence
キーワード
主題Scheme Other
主題 Recombinant fibrinogen
資源タイプ
資源 http://purl.org/coar/resource_type/c_6501
タイプ journal article
著者 Arai, Shinpei

× Arai, Shinpei

WEKO 109380

en Arai, Shinpei

Search repository
Ogiwara, Naoko

× Ogiwara, Naoko

WEKO 109381

en Ogiwara, Naoko

Search repository
Mukai, Saki

× Mukai, Saki

WEKO 109382

en Mukai, Saki

Search repository
Takezawa, Yuka

× Takezawa, Yuka

WEKO 109383

en Takezawa, Yuka

Search repository
Sugano, Mitsutoshi

× Sugano, Mitsutoshi

WEKO 109384

en Sugano, Mitsutoshi

Search repository
Honda, Takayuki

× Honda, Takayuki

WEKO 109385

en Honda, Takayuki

Search repository
Okumura, Nobuo

× Okumura, Nobuo

WEKO 109386

en Okumura, Nobuo

Search repository
信州大学研究者総覧へのリンク
氏名 Arai, Shinpei
URL https://soar-rd.shinshu-u.ac.jp/profile/ja.uCnFPUym.html
信州大学研究者総覧へのリンク
氏名 Honda, Takayuki
URL https://soar-rd.shinshu-u.ac.jp/profile/ja.WeDUHFkh.html
信州大学研究者総覧へのリンク
氏名 Okumura, Nobuo
URL https://soar-rd.shinshu-u.ac.jp/profile/ja.OVnmgCkh.html
出版者
出版者 SPRINGER JAPAN KK
引用
内容記述タイプ Other
内容記述 INTERNATIONAL JOURNAL OF HEMATOLOGY.105:758-768(2017)
書誌情報 INTERNATIONAL JOURNAL OF HEMATOLOGY

巻 105, p. 758-768, 発行日 2017-02-04
抄録
内容記述タイプ Abstract
内容記述 Fibrinogen storage disease (FSD) is a rare disorder that is characterized by the accumulation of fibrinogen in hepatocytes and induces liver injury. Six mutations in the γC domain (γG284R, γT314P, γD316N, the deletion of γG346-Q350, γG366S, and γR375W) have been identified for FSD. Our group previously established γ375W fibrinogen-producing Chinese hamster ovary (CHO) cells and observed aberrant large granular and fibrous forms of intracellular inclusion bodies. The aim of this study was to investigate whether fibrous intracellular inclusion bodies are specific to FSD-inducible variant fibrinogen. Thirteen expression vectors encoding the variant γ-chain were stably or transiently transfected into CHO cells expressing normal fibrinogen Aα- and Bβ-chains or HuH-7 cells, which were then immunofluorescently stained. Six CHO and HuH-7 cell lines that transiently produced FSD-inducible variant fibrinogen presented the fibrous (3.2?22.7 and 2.1?24.5%, respectively) and large granular (5.4?25.5 and 7.7?23.9%) forms of intracellular inclusion bodies. Seven CHO and HuH-7 cell lines that transiently produced FSD-non-inducible variant fibrinogen only exhibit the large granular form. These results demonstrate that transiently transfected variant fibrinogen-producing CHO cells and inclusion bodies of the fibrous form may be useful in non-invasive screening for FSD risk factors for FSD before its onset.
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 0925-5710
書誌レコードID
収録物識別子タイプ NCID
収録物識別子 AA10797094
PubMed
識別子タイプ PMID
関連識別子 https://www.ncbi.nlm.nih.gov/pubmed/28161763
関連名称 28161763
DOI
識別子タイプ DOI
関連識別子 https://doi.org/10.1007/s12185-017-2185-5
関連名称 10.1007/s12185-017-2185-5
権利
権利情報 This is a post-peer-review, pre-copyedit version of an article published in INTERNATIONAL JOURNAL OF HEMATOLOGY. The final authenticated version is available online at: https://doi.org/10.1007/s12185-017-2185-5
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=000401324100008
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