{"created":"2023-07-27T06:30:06.448575+00:00","id":14986,"links":{},"metadata":{"_buckets":{"deposit":"03bc28ad-c80a-44e2-b29f-143acfb6719e"},"_deposit":{"created_by":3,"id":"14986","owners":[3],"pid":{"revision_id":0,"type":"depid","value":"14986"},"status":"published"},"_oai":{"id":"oai:kanazawa-u.repo.nii.ac.jp:00014986","sets":["4281:4291:4312"]},"author_link":["86","21465","85909","69343","34"],"item_4_biblio_info_8":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2000-01-01","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"7","bibliographicPageEnd":"438","bibliographicPageStart":"432","bibliographicVolumeNumber":"38","bibliographic_titles":[{"bibliographic_title":"化学と生物 = Kagaku to Seibutsu"}]}]},"item_4_creator_33":{"attribute_name":"著者別表示","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Sai, Yoshimichi"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"Tamai, Ikumi"}],"nameIdentifiers":[{},{}]},{"creatorNames":[{"creatorName":"Tsuji, Akira"}],"nameIdentifiers":[{},{},{}]}]},"item_4_description_21":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"有機カチオンを輸送するトランスポーターOCTN1のファミ リーとしてヒト腎臓より単離されたOCTN2は, ミトコンド リア内での長鎖脂肪酸のβ酸化に必須なカルニチンをNa+ 依存的に各組織細胞内に輸送するカルニチントランスポータ ーとして機能する. 全身性カルニチン欠乏症患者や, その疾 患モデル動物として発見されたJVSマウスでは, 腎尿細管で のカルニチン再吸収が行なわれず, 血中および組織中カルニ チン濃度が著しく低下している. そのために脂肪酸代謝に支 障をきたし, カルニチンの投与を続けない限り, 心筋症, 骨格 筋症, 脂肪肝などの症状により死亡に至る. 両者の病態に共 通してOCTN2遺伝子の変異によるカルニチン輸送能の損 失が発症の原因になっていることが明らかとなってきた.","subitem_description_type":"Abstract"}]},"item_4_description_5":{"attribute_name":"提供者所属","attribute_value_mlt":[{"subitem_description":"金沢大学附属病院薬剤部","subitem_description_type":"Other"}]},"item_4_identifier_registration":{"attribute_name":"ID登録","attribute_value_mlt":[{"subitem_identifier_reg_text":"10.24517/00014973","subitem_identifier_reg_type":"JaLC"}]},"item_4_publisher_17":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"日本農芸化学会 = Japan Society for Bioscience, Biotechnology, and 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許可を得て登録"}]},"item_4_source_id_11":{"attribute_name":"NCID","attribute_value_mlt":[{"subitem_source_identifier":"AN00037573","subitem_source_identifier_type":"NCID"}]},"item_4_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"0453-073X","subitem_source_identifier_type":"ISSN"}]},"item_4_version_type_25":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"崔, 吉道"}],"nameIdentifiers":[{},{},{},{}]},{"creatorNames":[{"creatorName":"玉井, 郁巳"}],"nameIdentifiers":[{},{},{},{}]},{"creatorNames":[{"creatorName":"辻, 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Transporter, A Major Factor on Systemic Carnitine Deficiency Syndrome","subitem_title_language":"en"}]},"item_type_id":"4","owner":"3","path":["4312"],"pubdate":{"attribute_name":"公開日","attribute_value":"2017-10-04"},"publish_date":"2017-10-04","publish_status":"0","recid":"14986","relation_version_is_last":true,"title":["有機カチオン/カルニチントランスポーターと疾患"],"weko_creator_id":"3","weko_shared_id":3},"updated":"2023-07-27T08:51:26.322518+00:00"}