{"created":"2023-05-15T15:57:47.102228+00:00","id":27067,"links":{},"metadata":{"_buckets":{"deposit":"20ab4a1b-1379-4cc9-8255-b981b3dc62d2"},"_deposit":{"created_by":3,"id":"27067","owners":[3],"pid":{"revision_id":0,"type":"depid","value":"27067"},"status":"published"},"_oai":{"id":"oai:shinshu.repo.nii.ac.jp:00027067","sets":["2668"]},"author_link":["105120","105118","105115","105114","105117","105116","105121","105119"],"item_20002_biblio_info_27":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2014-01","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"107","bibliographicPageStart":"101","bibliographicVolumeNumber":"133","bibliographic_titles":[{"bibliographic_title":"THROMBOSIS RESEARCH"}]}]},"item_20002_creator_3":{"attribute_name":"作成者(その他言語)","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Kobayashi, Tamaki"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Arai, Shinpei"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Ogiwara, Naoko"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Takezawa, Yuka"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Nanya, Mai"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Terasawa, Fumiko"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Okumura, Nobuo"}],"nameIdentifiers":[{}]}]},"item_20002_date_19":{"attribute_name":"日付","attribute_value_mlt":[{"subitem_date_issued_datetime":"2015-09-24","subitem_date_issued_type":"Created"}]},"item_20002_description_14":{"attribute_name":"内容記述","attribute_value_mlt":[{"subitem_description":"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.","subitem_description_type":"Other"}]},"item_20002_description_20":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"subitem_description":"Article","subitem_description_type":"Other"}]},"item_20002_description_22":{"attribute_name":"その他の資源識別子","attribute_value_mlt":[{"subitem_description":"THROMBOSIS RESEARCH. 133(1):101-107 (2014)","subitem_description_type":"Other"}]},"item_20002_full_name_16":{"attribute_name":"公開者(その他言語)","attribute_value_mlt":[{"nameIdentifiers":[{}],"names":[{"name":"PERGAMON-ELSEVIER, SCIENCE LTD"}]}]},"item_20002_identifier_23":{"attribute_name":"資源識別子URI","attribute_value_mlt":[{"subitem_identifier_type":"HDL","subitem_identifier_uri":"http://hdl.handle.net/10091/17618"}]},"item_20002_relation_31":{"attribute_name":"PubMed番号","attribute_value_mlt":[{"subitem_relation_type":"isVersionOf","subitem_relation_type_id":{"subitem_relation_type_id_text":"http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search&DB=pubmed&term=24210681","subitem_relation_type_select":"PMID"}}]},"item_20002_relation_32":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_type":"isVersionOf","subitem_relation_type_id":{"subitem_relation_type_id_text":"http://dx.doi.org/10.1016/j.thromres.2013.10.033","subitem_relation_type_select":"DOI"}}]},"item_20002_rights_50":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"Copyright© 2013 Elsevier Ltd."}]},"item_20002_source_id_25":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"0049-3848","subitem_source_identifier_type":"ISSN"}]},"item_20002_source_id_26":{"attribute_name":"処理レコードID(総合目録DB)","attribute_value_mlt":[{"subitem_source_identifier":"AA00863148","subitem_source_identifier_type":"NCID"}]},"item_20002_version_type_51":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_ab4af688f83e57aa","subitem_version_type":"AM"}]},"item_access_right":{"attribute_name":"アクセス権","attribute_value_mlt":[{"subitem_access_right":"metadata only access","subitem_access_right_uri":"http://purl.org/coar/access_right/c_14cb"}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"url":{"url":"https://soar-ir.repo.nii.ac.jp/?action=repository_action_common_download&item_id=3721&item_no=1&attribute_id=65&file_no=1"}}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Endoplasmic reticulum","subitem_subject_scheme":"Other"},{"subitem_subject":"Fibrinogen storage disease","subitem_subject_scheme":"Other"},{"subitem_subject":"Fibrinogen gamma-chain mutation","subitem_subject_scheme":"Other"},{"subitem_subject":"Hepatic endoplasmic reticulum storage disease","subitem_subject_scheme":"Other"},{"subitem_subject":"Hypofibrinogenemia","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":"gamma 375W fibrinogen-synthesizing CHO cells indicate the accumulation of variant fibrinogen within endoplasmic reticulum","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"gamma 375W fibrinogen-synthesizing CHO cells indicate the accumulation of variant fibrinogen within endoplasmic reticulum"}]},"item_type_id":"20002","owner":"3","path":["2668"],"pubdate":{"attribute_name":"公開日","attribute_value":"2020-09-01"},"publish_date":"2020-09-01","publish_status":"0","recid":"27067","relation_version_is_last":true,"title":["gamma 375W fibrinogen-synthesizing CHO cells indicate the accumulation of variant fibrinogen within endoplasmic reticulum"],"weko_creator_id":"3","weko_shared_id":3},"updated":"2023-05-15T22:11:15.937048+00:00"}