{"created":"2021-03-01T06:12:31.792670+00:00","id":5943,"links":{},"metadata":{"_buckets":{"deposit":"2cc7e6cc-7f00-4ce3-8164-d2989e13bc50"},"_deposit":{"id":"5943","owners":[],"pid":{"revision_id":0,"type":"depid","value":"5943"},"status":"published"},"_oai":{"id":"oai:nagoya.repo.nii.ac.jp:00005943","sets":["329:333:334:737"]},"author_link":["15802","15803","15804","15805","15806","15807"],"item_1615768549627":{"attribute_name":"出版タイプ","attribute_value_mlt":[{"subitem_version_resource":"http://purl.org/coar/version/c_970fb48d4fbd8a85","subitem_version_type":"VoR"}]},"item_9_biblio_info_6":{"attribute_name":"書誌情報","attribute_value_mlt":[{"bibliographicIssueDates":{"bibliographicIssueDate":"2003-12","bibliographicIssueDateType":"Issued"},"bibliographicPageEnd":"11","bibliographicPageStart":"1","bibliographicVolumeNumber":"47","bibliographic_titles":[{"bibliographic_title":"Environmental Medicine : annual report of the Research Institute of Environmental Medicine, Nagoya University","bibliographic_titleLang":"en"}]}]},"item_9_description_4":{"attribute_name":"抄録","attribute_value_mlt":[{"subitem_description":"As a structural component of mammalian cell membranes, cholesterol is essential for increasing the rigidity of the lipid bilayer and thus decreasing membrane fluidity and permeability. Cholesterol or its precursors are also essential for the synthesis of a wide variety of molecules that affect diverse physiological functions including nutrient absorption, vitamin balance, stress responses, reproductive function and osmoregulation. Hence, an elaborate regulatory system involving more than 100 genes has evolved to maintain the concentration of cholesterol within narrow limits. During the past 10 years, a number of defects in the late stages of the biosynthetic pathway have been associated with diseases. These diseases are linked with the accumulation of a variety of intermediate precursors which will in many cases at least partially compensate for the associated hypocholesterolemia. One such condition is desmosterolosis, an autosomal recessive disorder which results from mutations in the 3β-hydroxysterol-Δ^24-reductase gene. The associated pathology underscores the essential roles of cholesterol not only in the maintenance of normal cell functioning but also during ontogeny. The discovery of this and other post-squalene genetic disorders provides a new tool to better understand the essential roles of cholesterol in homeostasis.","subitem_description_language":"en","subitem_description_type":"Abstract"}]},"item_9_description_5":{"attribute_name":"内容記述","attribute_value_mlt":[{"subitem_description":"国立情報学研究所で電子化したコンテンツを使用している。","subitem_description_language":"ja","subitem_description_type":"Other"}]},"item_9_identifier_60":{"attribute_name":"URI","attribute_value_mlt":[{"subitem_identifier_type":"HDL","subitem_identifier_uri":"http://hdl.handle.net/2237/7557"}]},"item_9_identifier_registration":{"attribute_name":"ID登録","attribute_value_mlt":[{"subitem_identifier_reg_text":"10.18999/envm.47.1","subitem_identifier_reg_type":"JaLC"}]},"item_9_publisher_32":{"attribute_name":"出版者","attribute_value_mlt":[{"subitem_publisher":"Research Institute of Environmental Medicine, Nagoya University","subitem_publisher_language":"en"}]},"item_9_select_15":{"attribute_name":"著者版フラグ","attribute_value_mlt":[{"subitem_select_item":"publisher"}]},"item_9_source_id_7":{"attribute_name":"ISSN（print）","attribute_value_mlt":[{"subitem_source_identifier":"0287-0517","subitem_source_identifier_type":"PISSN"}]},"item_9_text_14":{"attribute_name":"フォーマット","attribute_value_mlt":[{"subitem_text_value":"application/pdf"}]},"item_access_right":{"attribute_name":"アクセス権","attribute_value_mlt":[{"subitem_access_right":"open access","subitem_access_right_uri":"http://purl.org/coar/access_right/c_abf2"}]},"item_creator":{"attribute_name":"著者","attribute_type":"creator","attribute_value_mlt":[{"creatorNames":[{"creatorName":"Zadworny, David","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"15802","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Sarkar, Devanand","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"15803","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Imai, Tsuneo","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"15804","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Mirza, Rusella","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"15805","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Kambe, Fukushi","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"15806","nameIdentifierScheme":"WEKO"}]},{"creatorNames":[{"creatorName":"Seo, Hisao","creatorNameLang":"en"}],"nameIdentifiers":[{"nameIdentifier":"15807","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":"2018-02-19"}],"displaytype":"detail","filename":"1-11.pdf","filesize":[{"value":"1.5 MB"}],"format":"application/pdf","licensetype":"license_note","mimetype":"application/pdf","url":{"label":"1-11.pdf","objectType":"fulltext","url":"https://nagoya.repo.nii.ac.jp/record/5943/files/1-11.pdf"},"version_id":"02fb95fe-cf44-4fe7-ba25-de0fe6b2671a"}]},"item_keyword":{"attribute_name":"キーワード","attribute_value_mlt":[{"subitem_subject":"3β-hydroxysterol-Δ^24-reductase(DHCR24)","subitem_subject_scheme":"Other"},{"subitem_subject":"desmosterolosis","subitem_subject_scheme":"Other"},{"subitem_subject":"cholesterol metabolism","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":"departmental bulletin paper","resourceuri":"http://purl.org/coar/resource_type/c_6501"}]},"item_title":"The Involvement of 3β-hydroxysterol-Δ^24-reductase, A Post-squalene Enzyme in Cholesterol Biosynthesis, in Desmosterolosis","item_titles":{"attribute_name":"タイトル","attribute_value_mlt":[{"subitem_title":"The Involvement of 3β-hydroxysterol-Δ^24-reductase, A Post-squalene Enzyme in Cholesterol Biosynthesis, in Desmosterolosis","subitem_title_language":"en"}]},"item_type_id":"9","owner":"1","path":["737"],"pubdate":{"attribute_name":"PubDate","attribute_value":"2007-04-04"},"publish_date":"2007-04-04","publish_status":"0","recid":"5943","relation_version_is_last":true,"title":["The Involvement of 3β-hydroxysterol-Δ^24-reductase, A Post-squalene Enzyme in Cholesterol Biosynthesis, in Desmosterolosis"],"weko_creator_id":"1","weko_shared_id":-1},"updated":"2023-01-26T06:18:46.208129+00:00"}