{"created":"2023-06-19T10:28:26.993819+00:00","id":592,"links":{},"metadata":{"_buckets":{"deposit":"6fe9f51f-fa1b-462c-bd5f-b22dde9ccc37"},"_deposit":{"created_by":25,"id":"592","owners":[25],"pid":{"revision_id":0,"type":"depid","value":"592"},"status":"published"},"_oai":{"id":"oai:kwmed.repo.nii.ac.jp:00000592","sets":["1709617079800:35:186:429"]},"author_link":["119884","119885"],"item_1694495855422":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_version_type":"VoR"}]},"item_3_biblio_info_12":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"1987","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"1","bibliographicPageEnd":"61","bibliographicPageStart":"44","bibliographicVolumeNumber":"13","bibliographic_titles":[{"bibliographic_title":"川崎医学会誌","bibliographic_titleLang":"ja"},{"bibliographic_title":"Kawasaki medical journal","bibliographic_titleLang":"en"}]}]},"item_3_description_8":{"attribute_name":"記事種別(日)","attribute_value_mlt":[{"subitem_description":"原著","subitem_description_language":"ja","subitem_description_type":"Other"}]},"item_3_identifier_14":{"attribute_name":"URL","attribute_value_mlt":[{"subitem_identifier_type":"URI","subitem_identifier_uri":"http://igakkai.kms-igakkai.com/wp/wp-content/uploads/1987/KMJ13(1)44-61.1987.pdf"}]},"item_3_relation_20":{"attribute_name":"DOI","attribute_value_mlt":[{"subitem_relation_type_id":{"subitem_relation_type_id_text":"https://doi.org/10.11482/KMJ-J13(1)44 ","subitem_relation_type_select":"DOI"}}]},"item_3_source_id_1":{"attribute_name":"雑誌書誌ID","attribute_value_mlt":[{"subitem_source_identifier":"AN00045593","subitem_source_identifier_type":"NCID"},{"subitem_source_identifier":"AN12940574","subitem_source_identifier_type":"NCID"}]},"item_3_source_id_19":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"0386-5924","subitem_source_identifier_type":"PISSN"},{"subitem_source_identifier":"2758-089X","subitem_source_identifier_type":"EISSN"}]},"item_3_text_6":{"attribute_name":"著者所属(日)","attribute_value_mlt":[{"subitem_text_language":"ja","subitem_text_value":"川崎町科大学大学院生化学系I"}]},"item_3_text_7":{"attribute_name":"著者所属(英)","attribute_value_mlt":[{"subitem_text_language":"en","subitem_text_value":"Graduate Course of Biochemistry, Kawasaki Medical School"}]},"item_3_textarea_10":{"attribute_name":"抄録(日)","attribute_value_mlt":[{"subitem_textarea_language":"ja","subitem_textarea_value":"遺伝性楕円赤血球症25例(HE),遺伝性球状赤血球症5例(HS)および遺伝性有口赤血球症5例(H.St.)における溶血の病因について,それらのspectrinの生化学的性状,膜機能および細胞形態の立場から検討した.HE症例では,その細胞形態によって2群に分類された.第1はrod shape type(rod型細胞が15%以下の群),第2はnon-rod type(rod型細胞が5%以下の群)で,前者は16例,後者は9例であった.溶血型の多くはnon-rod typeであった.さらに典型的な溶血型では,その楕円赤血球にstomatocytic changeが加わっていた.一方,膜機能Na-influxから溶血型をみると9例中8例に,Na-influxの亢進が認められた.rod型細胞を遠心法により集め,そのrod型細胞のcell ageとNa-influxについて検討した.結果はrod型細胞は,ovalocyteおよびdiscocyteよりさらにageingの進行した細胞と考えられた.また,Na-influxに関しては何ら正常赤血球と有意な差を示さなかった.しかし,このrod型細胞より作られたTriton shellでは,明らかにmechanical stabilityの低下が認められた.溶血型HE症例のうちで,膜蛋白band 4.2欠損例が発見され,この症例ではspectrin α-IV domainの等電点にも異常が検出された.その他のHE,HSおよびH.St.症例におけるspectrinの検討では,各domain,dimer-dimer associationおよび耐熱安定性には異常を認めなかった."}]},"item_3_textarea_11":{"attribute_name":"抄録(英)","attribute_value_mlt":[{"subitem_textarea_language":"en","subitem_textarea_value":"The pathogenesis of increased hemolysis was studied on spectrin biochemistry, membrane functions and morphology in hereditary elliptocytosis (25), spherocytosis (5) and stomatocytosis. Based on morphological features, the HE patients were classified into two major groups; those with a rod shape type (>15%) of elliptocytosis (16), and those with a non-rod type (<5%) of elliptocytosis. Most of cases with overt hemolysis were detected among the non-rod type cases. Overt hemolysis typically tended to be accompanied with stomatocytic changes, which appear to be superimposed on elliptic changes. Sodium influx increased in 8 of 9 HE patients with overt hemolysis. Rod shaped elliptocytes were collected by the centrifugation method, and these cells were examined on the cell age and sodium influx. By this method, it was concluded that the cell age of rod shaped elliptocytes was older than ovalocytes and discocytes, and that no significant difference was detected between rod shaped elliptocytes and normal discocytes in sodium influx. On the other hand, instability of these rod shaped elliptocytes in triton shells was detected under the mechanical shaking. The membrane protein band 4.2 deficiency was detected in a case of hemolytic HE, in which abnormal isoelectric point of spectrin α-IV domain was observed. Spectrin abnormality in domain composition, dimer-dimer association, and thermal stability were not observed in other cases with HE, HS, H.St. patients studied."}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"神崎, 暁郎","creatorNameLang":"ja"},{"creatorName":"カンザキ, アキオ","creatorNameLang":"ja-Kana"}],"nameIdentifiers":[{}]},{"creatorNames":[{"creatorName":"Kanzaki, Akio","creatorNameLang":"en"}],"nameIdentifiers":[{}]}]},"item_files":{"attribute_name":"ファイル情報","attribute_type":"file","attribute_value_mlt":[{"accessrole":"open_date","date":[{"dateType":"Available","dateValue":"2017-01-23"}],"displaytype":"detail","filename":"KJ00009816299.pdf","filesize":[{"value":"8.4 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"KJ00009816299.pdf","objectType":"fulltext","url":"https://kwmed.repo.nii.ac.jp/record/592/files/KJ00009816299.pdf"},"version_id":"6a3d384e-6c18-43c3-b8e1-0be90598d59c"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"Hereditary elliptocytosis","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"Spectrin","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"Cytoskeleton","subitem_subject_language":"en","subitem_subject_scheme":"Other"},{"subitem_subject":"Sodium influx","subitem_subject_language":"en","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":"赤血球膜異常症における膜蛋白の病態に関する研究 : 特に遺伝性楕円赤血球症について","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"赤血球膜異常症における膜蛋白の病態に関する研究 : 特に遺伝性楕円赤血球症について","subitem_title_language":"ja"},{"subitem_title":"Studies on Red Cell Membrane Disorders with Membrane Protein Abnormalities : Special Reference to Hereditary Elliptocytosis","subitem_title_language":"en"}]},"item_type_id":"3","owner":"25","path":["429"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2017-01-23"},"publish_date":"2017-01-23","publish_status":"0","recid":"592","relation_version_is_last":true,"title":["赤血球膜異常症における膜蛋白の病態に関する研究 : 特に遺伝性楕円赤血球症について"],"weko_creator_id":"25","weko_shared_id":-1},"updated":"2023-10-06T00:39:26.074317+00:00"}