{"created":"2023-05-15T09:02:45.621559+00:00","id":3627,"links":{},"metadata":{"_buckets":{"deposit":"b148bb48-77a2-43ca-a28a-7dbf78d5cf0c"},"_deposit":{"created_by":3,"id":"3627","owners":[3],"pid":{"revision_id":0,"type":"depid","value":"3627"},"status":"published"},"_oai":{"id":"oai:hirosaki.repo.nii.ac.jp:00003627","sets":["557:561:643"]},"author_link":["11833","11835","11834"],"item_3_biblio_info_8":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2007-11-29","bibliographicIssueDateType":"Issued"},"bibliographicIssueNumber":"Supplement","bibliographicPageEnd":"S88","bibliographicPageStart":"S82","bibliographicVolumeNumber":"59","bibliographic_titles":[{"bibliographic_title":"弘前医学"}]}]},"item_3_description_17":{"attribute_name":"フォーマット","attribute_value_mlt":[{"subitem_description":"application/pdf","subitem_description_type":"Other"}]},"item_3_description_5":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"Congenital Hyperinsulinism in Infancy （HI） is a potentially-lethal condition of neonates and during\nearly childhood. For many years the pathophysiology of this disorder was unknown. Recent advances in genetics,\nhistopathology and molecule physiology have now revealed the causes of HI in a large cohort of patients. From defects\nin ion channel subunit genes to lesions in the control of pancreatic B-cell metabolism and anaplerosis, the causes of\nHI are both varied and numerous. However, in all cases they appear to share a common target protein - the ATPsensitive\nK-channel. The function of these channels is not only critical to the control of healthy normal insulin-secreting\ncell function, but “activating” defects in these channels lead to permanent neonatal diabetes and type 2 diabetes. HI\ncan therefore arise through “channelopathies” of K-ATP channels: HI-KATP through gene defects in ABCC8 and\nKCNJ11 （Ch11.p15）; or as a result of “metabolopathies” through defects in the genes encoding glucokinase HI-GK\n（GCK, Ch.7p15-p13）, glutamate dehydrogenase HI-GDH （GLUD1, Ch.10q23.3） and Short-chain L-3-hydroxyacyl-CoA\ndehydrogenase HI-SCHAD（ HADHSC, Ch.4q22-q26）. Advances in the integration of genetic medicine and cell biology\nhave provided key insights into the causes of HI, and this has been of key importance to the defi nition of pathogenesis.\nHowever, medical therapy for HI remains largely unchanged due to the availability of limited agents that are selective\nand specifi c for the termination of insulin release from β-cells. CHI can be a devastating disease, and in this review\nfocuses upon the relationship between the basis of HI and current / future therapies, including stem cells.","subitem_description_type":"Abstract"}]},"item_3_description_7":{"attribute_name":"引用","attribute_value_mlt":[{"subitem_description":"弘前医学. 59(Suppl.), 2007, p.S82-S88","subitem_description_type":"Other"}]},"item_3_publisher_35":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"弘前大学大学院医学研究科・弘前医学会"}]},"item_3_radio_42":{"attribute_name":"資源タイプ","attribute_value_mlt":[{"subitem_radio_item":"Article"}]},"item_3_source_id_11":{"attribute_name":"書誌レコードID","attribute_value_mlt":[{"subitem_source_identifier":"AN00211444","subitem_source_identifier_type":"NCID"}]},"item_3_source_id_9":{"attribute_name":"ISSN","attribute_value_mlt":[{"subitem_source_identifier":"0439-1721","subitem_source_identifier_type":"ISSN"}]},"item_3_subject_19":{"attribute_name":"日本十進分類法","attribute_value_mlt":[{"subitem_subject":"491.4","subitem_subject_scheme":"NDC"}]},"item_3_subject_23":{"attribute_name":"NIIサブジェクト","attribute_value_mlt":[{"subitem_subject":"基礎医学","subitem_subject_scheme":"Other"}]},"item_3_text_4":{"attribute_name":"著者所属","attribute_value_mlt":[{"subitem_text_value":"Faculty of Life Sciences, University of Manchester"},{"subitem_text_value":"Faculty of Life Sciences, University of Manchester"},{"subitem_text_value":"Faculty of Life Sciences, University of Manchester"}]},"item_3_version_type_18":{"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":"Cosgrove, Karen E."}],"nameIdentifiers":[{"nameIdentifier":"11833","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Shepherd, Ruth M."}],"nameIdentifiers":[{"nameIdentifier":"11834","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Dunne, Mark J."}],"nameIdentifiers":[{"nameIdentifier":"11835","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":"2018-03-13"}],"displaytype":"detail","filename":"HirosakiMedJ_59_S82.pdf","filesize":[{"value":"713.9 kB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"HirosakiMedJ_59_S82.pdf","url":"https://hirosaki.repo.nii.ac.jp/record/3627/files/HirosakiMedJ_59_S82.pdf"},"version_id":"a5d3b019-e1d7-4156-bb6d-28a82ff6df6c"}]},"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":"Congenital hyperinsulinism: From bench to bedside","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"Congenital hyperinsulinism: From bench to bedside"}]},"item_type_id":"3","owner":"3","path":["643"],"pubdate":{"attribute_name":"公開日","attribute_value":"2009-09-18"},"publish_date":"2009-09-18","publish_status":"0","recid":"3627","relation_version_is_last":true,"title":["Congenital hyperinsulinism: From bench to bedside"],"weko_creator_id":"3","weko_shared_id":-1},"updated":"2023-05-16T06:42:48.518870+00:00"}