{"created":"2023-06-20T15:47:51.899377+00:00","id":2391,"links":{},"metadata":{"_buckets":{"deposit":"f080db15-259d-456f-b797-78ee99f8d12b"},"_deposit":{"created_by":4,"id":"2391","owners":[4],"pid":{"revision_id":0,"type":"depid","value":"2391"},"status":"published"},"_oai":{"id":"oai:hama-med.repo.nii.ac.jp:00002391","sets":["1:11"]},"author_link":[],"item_3_biblio_info_5":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"1983-12-30","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"6","bibliographicPageEnd":"293","bibliographicPageStart":"289","bibliographicVolumeNumber":"27","bibliographic_titles":[{"bibliographic_title":"生物物理化学"}]}]},"item_3_description_9":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"乳酸脱水素酵素(EC.1.1.1.27,LDH)はAおよびBの二つのサブユニットの4量体として各組織に存在する.この二つのサブユニットのlocusはそれぞれ異なっており,Aが染色体11,Bが12の短鎖に存在することが知られており,サブユニット欠損の始めての症例はBサブユニット欠損症として1971年に北村らによって報告された1).その後,我々は運動後のミオグロビン尿を主訴とした患者にAサブユニット欠損症を見出し2),ここにAおよびBそれぞれのサブユニット欠損症がそろつて我が国で見出されたこととなった. この二つのサブユニット欠損症は,各組織での両サブユニットの産生量が異なること,また両サブユニットの持つ生理的活性が異なることから,臨床的には症状の発現をまったく異にしている.いづれの症例も,LDHが解糖系の酵素であることから,それぞれのサブユニット産生の多い臓器での糖代謝異常による臨床症状が期待されるが,臨床症状の著明なのはAサブユニット欠損症であり,Bサブユニット欠損症は臨床症状を示さない. ここではこれら二つのサブユニット欠損症を対比しながらLDHの生理機能の1部に言及する.また赤血球を用いたスクリーニングより,この欠損症の保因者がかなりの頻度に存在することが判明していることから,日常の検査データの評価上の問題点にも併せて言及したい.","subitem_description_type":"Abstract"}]},"item_3_publisher_6":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"日本電気泳動学会"}]},"item_3_relation_28":{"attribute_name":"出版社DOI","attribute_value_mlt":[{"subitem_relation_type":"isIdenticalTo","subitem_relation_type_id":{"subitem_relation_type_id_text":"10.2198/sbk.27.289","subitem_relation_type_select":"DOI"}}]},"item_3_rights_7":{"attribute_name":"権利","attribute_value_mlt":[{"subitem_rights":"日本電気泳動学会"}]},"item_3_source_id_19":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"00319082","subitem_source_identifier_type":"ISSN"}]},"item_3_source_id_20":{"attribute_name":"EISSN","attribute_value_mlt":[{"subitem_source_identifier":"13499785","subitem_source_identifier_type":"ISSN"}]},"item_3_version_type_32":{"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":"菅野, 剛史"}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2018-08-27"}],"displaytype":"detail","filename":"Seibutubuturi27_6-289.pdf","filesize":[{"value":"3.9 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"Seibutubuturi27_6-289.pdf","url":"https://hama-med.repo.nii.ac.jp/record/2391/files/Seibutubuturi27_6-289.pdf"},"version_id":"90a47964-d819-4978-b206-5d90343a4866"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"lactate dehydrogenase (LDH),","subitem_subject_scheme":"Other"},{"subitem_subject":"A-subunit,","subitem_subject_scheme":"Other"},{"subitem_subject":"B-sunit,","subitem_subject_scheme":"Other"},{"subitem_subject":"subunit deficiency","subitem_subject_scheme":"Other"}]},"item_language":{"attribute_name":"言語","attribute_value_mlt":[{"subitem_language":"jpn"}]},"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":"LDHサブユニット欠損症","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"LDHサブユニット欠損症","subitem_title_language":"ja"}]},"item_type_id":"3","owner":"4","path":["11"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2013-08-27"},"publish_date":"2013-08-27","publish_status":"0","recid":"2391","relation_version_is_last":true,"title":["LDHサブユニット欠損症"],"weko_creator_id":"4","weko_shared_id":-1},"updated":"2023-08-02T05:00:45.951783+00:00"}