{"_buckets": {"deposit": "d8c31bb8-1f0f-4606-b852-a609c44f207a"}, "_deposit": {"id": "2051", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "2051"}, "status": "published"}, "_oai": {"id": "oai:niigata-u.repo.nii.ac.jp:00002051", "sets": ["454", "473"]}, "item_5_biblio_info_6": {"attribute_name": "書誌情報", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2003-04", "bibliographicIssueDateType": "Issued"}, "bibliographicIssueNumber": "4", "bibliographicPageEnd": "415", "bibliographicPageStart": "409", "bibliographicVolumeNumber": "35", "bibliographic_titles": [{"bibliographic_title": "Journal of Molecular and Cellular Cardiology"}, {"bibliographic_title": "Journal of Molecular and Cellular Cardiology", "bibliographic_titleLang": "en"}]}]}, "item_5_description_4": {"attribute_name": "抄録", "attribute_value_mlt": [{"subitem_description": "Andersen’s syndrome, which is characterized by periodic paralysis, cardiac arrhythmias and dysmorphic features, is a hereditary disease, and missense mutations of KCNJ2, which encodes an inward rectifying potassium channel, have been reported recently. We performed clinical and molecular analyses of a patient with Andersen’s syndrome, and found a novel mutation (G215D) of KCNJ2. Twelve lead electrocardiography revealed a long QT interval and frequent premature ventricular contractions, and polymorphic ventricular tachycardia was induced by programmed electrical stimulation. Use of a conventional whole-cell patch-clamp system with COS7 cells demonstrated that the G215D mutant was non functional, and that co-expression of wild type (WT)- and mutant-KCNJ2 shows a dominant negative effect on both inward and outward currents. We performed confocal laser scanning microscopy to assess the cellular trafficking of WT- and mutant-KCNJ2 subunits tagged with yellow fluorescent protein (YFP) and cyan fluorescent protein (CFP), respectively. Tagging with the YFP did not affect the channel function of WT-KCNJ2 and both proteins showed similar plasma membrane fluorescence patterns. Furthermore, the result of fluorescence resonance energy transfer (FRET) studies at the plasma membrane region suggested that both YFP-tagged WT- and CFP-tagged mutant-KCNJ2 combine to construct a hetero-multimer of the potassium channel. In conclusion, the G215D mutant of KCNJ2 is distributed normally in the plasma membrane, but exhibits a dominant negative effect and reduces the Kir2.1 current, presumably due to hetero-multimer construction.", "subitem_description_type": "Abstract"}]}, "item_5_full_name_3": {"attribute_name": "著者別名", "attribute_value_mlt": [{"nameIdentifiers": [{"nameIdentifier": "6785", "nameIdentifierScheme": "WEKO"}], "names": [{"name": "塙, 晴雄"}]}]}, "item_5_publisher_7": {"attribute_name": "出版者", "attribute_value_mlt": [{"subitem_publisher": "Elsevier Science Ltd."}]}, "item_5_relation_14": {"attribute_name": "DOI", "attribute_value_mlt": [{"subitem_relation_type_id": {"subitem_relation_type_id_text": "info:doi/10.1016/S0022-2828(03)00046-4", "subitem_relation_type_select": "DOI"}}]}, "item_5_select_19": {"attribute_name": "著者版フラグ", "attribute_value_mlt": [{"subitem_select_item": "author"}]}, "item_5_source_id_11": {"attribute_name": "書誌レコードID", "attribute_value_mlt": [{"subitem_source_identifier": "AA00702819", "subitem_source_identifier_type": "NCID"}]}, "item_5_source_id_9": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "00222828", "subitem_source_identifier_type": "ISSN"}]}, "item_creator": {"attribute_name": "著者", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Hosaka, Yukio"}], "nameIdentifiers": [{"nameIdentifier": "6776", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Hanawa, Haruo"}], "nameIdentifiers": [{"nameIdentifier": "6777", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Washizuka, Takashi"}], "nameIdentifiers": [{"nameIdentifier": "6778", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Chinushi, Masaomi"}], "nameIdentifiers": [{"nameIdentifier": "6779", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Yamashita, Fumio"}], "nameIdentifiers": [{"nameIdentifier": "6780", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Yoshida, Tsuyoshi"}], "nameIdentifiers": [{"nameIdentifier": "6781", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Komura, Satoru"}], "nameIdentifiers": [{"nameIdentifier": "6782", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Watanabe, Hiroshi"}], "nameIdentifiers": [{"nameIdentifier": "6783", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Aizawa, Yoshifusa"}], "nameIdentifiers": [{"nameIdentifier": "6784", "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": "2019-07-29"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "JMCC all.pdf", "filesize": [{"value": "235.8 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 235800.0, "url": {"label": "JMCC all.pdf", "url": "https://niigata-u.repo.nii.ac.jp/record/2051/files/JMCC all.pdf"}, "version_id": "e1b23eb4-8a3d-47c4-a91d-3bcd91dcbffe"}]}, "item_keyword": {"attribute_name": "キーワード", "attribute_value_mlt": [{"subitem_subject": "Andersen\u0027s syndrome", "subitem_subject_scheme": "Other"}, {"subitem_subject": "mutation", "subitem_subject_scheme": "Other"}, {"subitem_subject": "KCNJ2", "subitem_subject_scheme": "Other"}, {"subitem_subject": "potassium channel", "subitem_subject_scheme": "Other"}, {"subitem_subject": "long QT", "subitem_subject_scheme": "Other"}, {"subitem_subject": "ventricular tachycardia", "subitem_subject_scheme": "Other"}, {"subitem_subject": "patch-clamp", "subitem_subject_scheme": "Other"}, {"subitem_subject": "confocal laser scanning microscopy", "subitem_subject_scheme": "Other"}, {"subitem_subject": "trafficking", "subitem_subject_scheme": "Other"}, {"subitem_subject": "fluorescence resonance energy transfer(FRET)", "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": "Function, Subcellular Localization and Assembly of a Novel Mutation of KCNJ2 in Andersen\u0027s Syndrome", "item_titles": {"attribute_name": "タイトル", "attribute_value_mlt": [{"subitem_title": "Function, Subcellular Localization and Assembly of a Novel Mutation of KCNJ2 in Andersen\u0027s Syndrome"}, {"subitem_title": "Function, Subcellular Localization and Assembly of a Novel Mutation of KCNJ2 in Andersen\u0027s Syndrome", "subitem_title_language": "en"}]}, "item_type_id": "5", "owner": "1", "path": ["454", "473"], "permalink_uri": "http://hdl.handle.net/10191/4874", "pubdate": {"attribute_name": "公開日", "attribute_value": "2007-06-04"}, "publish_date": "2007-06-04", "publish_status": "0", "recid": "2051", "relation": {}, "relation_version_is_last": true, "title": ["Function, Subcellular Localization and Assembly of a Novel Mutation of KCNJ2 in Andersen\u0027s Syndrome"], "weko_shared_id": null}