{"_buckets": {"deposit": "d312fabd-03e3-449f-8b5e-e7bbeb55c296"}, "_deposit": {"id": "25095", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "25095"}, "status": "published"}, "_oai": {"id": "oai:nagoya.repo.nii.ac.jp:00025095"}, "item_10_biblio_info_6": {"attribute_name": "\u66f8\u8a8c\u60c5\u5831", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2017-10", "bibliographicIssueDateType": "Issued"}, "bibliographicPageEnd": "9", "bibliographicPageStart": "3", "bibliographicVolumeNumber": "88", "bibliographic_titles": [{"bibliographic_title": "JOURNAL OF DERMATOLOGICAL SCIENCE"}]}]}, "item_10_description_4": {"attribute_name": "\u6284\u9332", "attribute_value_mlt": [{"subitem_description": "Research on the genetic abnormalities and pathogenetic processes of ichthyoses\nhas progressed remarkably, and many causative genes and molecules have been\nidentified in ichthyoses and ichthyosis syndromes. Most of the genes/molecules\ncausative of ichthyosis are associated with the barrier function of the stratum\ncorneum, and defects in the skin barrier play important roles in the pathogenesis of\nvarious ichthyosis phenotypes.\nIt has been elucidated that, of the ichthyosis-causative genes, ABCA12, ALOXE3,\nALOX12B, CYP4F22, CERS3, ABHD5, PNPLA1 and ELOVL4 work in the formation\nof the corneocyte lipid envelope (CLE), a structure that is essential to sound skin\nbarrier function. The CLE mostly consists of ultra-long-chain (ULC) ceramides\nderived from ULC-acylceramide (EOS; a combination of esterified \u03c9-hydroxy fatty\nacids and sphingosines).\nIn this review, I shed light on the synthesis, metabolism and transport of\nepidermal ceramides, especially on ULC-acylceramide and the processes of CLE\nformation. In addition, I summarize the pathogeneses of various ichthyoses and\nichthyosis syndromes from the aspects of abnormal synthesis of ULC-acylceramide\nand malformation of the CLE.\nInvestigations on the pathomechanisms of ichthyoses have provided novel\nknowledge on the synthesis and metabolism of ceramides in the epidermis.\nConversely, research on the dynamics of epidermal ceramides has contributed to the\nelucidation of the pathogenesis of ichthyoses.\nAdvances in our understanding of the biology of epidermal lipids and the disease\npathogeneses of ichthyoses and ichthyosis syndromes promise to provide clues for\nthe development of effective therapies for ichthyosis patients in the near future.", "subitem_description_type": "Abstract"}]}, "item_10_identifier_60": {"attribute_name": "URI", "attribute_value_mlt": [{"subitem_identifier_type": "DOI", "subitem_identifier_uri": "http://doi.org/10.1016/j.jdermsci.2017.06.002"}, {"subitem_identifier_type": "HDL", "subitem_identifier_uri": "http://hdl.handle.net/2237/27314"}]}, "item_10_publisher_32": {"attribute_name": "\u51fa\u7248\u8005", "attribute_value_mlt": [{"subitem_publisher": "Elsevier"}]}, "item_10_rights_12": {"attribute_name": "\u6a29\u5229", "attribute_value_mlt": [{"subitem_rights": "\u00a9 2017. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/"}]}, "item_10_select_15": {"attribute_name": "\u8457\u8005\u7248\u30d5\u30e9\u30b0", "attribute_value_mlt": [{"subitem_select_item": "author"}]}, "item_10_source_id_7": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "0923-1811", "subitem_source_identifier_type": "ISSN"}]}, "item_creator": {"attribute_name": "\u8457\u8005", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Akiyama, Masashi"}], "nameIdentifiers": [{"nameIdentifier": "74862", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "\u30d5\u30a1\u30a4\u30eb\u60c5\u5831", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2018-10-01"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": 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"Akiyama_M_J_Dermatol_Sci_main_text.pdf \u30d5\u30a1\u30a4\u30eb\u516c\u958b\u65e5:2018/10/01", "url": "https://nagoya.repo.nii.ac.jp/record/25095/files/Akiyama_M_J_Dermatol_Sci_main_text.pdf"}, "version_id": "0a5b977d-558a-4994-acaa-64b28d0b6612"}]}, "item_keyword": {"attribute_name": "\u30ad\u30fc\u30ef\u30fc\u30c9", "attribute_value_mlt": [{"subitem_subject": "acylceramide", "subitem_subject_scheme": "Other"}, {"subitem_subject": "ceramide", "subitem_subject_scheme": "Other"}, {"subitem_subject": "corneocyte lipid envelope", "subitem_subject_scheme": "Other"}, {"subitem_subject": "EOS", "subitem_subject_scheme": "Other"}, {"subitem_subject": "ichthyosis syndrome", "subitem_subject_scheme": "Other"}]}, "item_language": {"attribute_name": "\u8a00\u8a9e", "attribute_value_mlt": [{"subitem_language": "eng"}]}, "item_resource_type": {"attribute_name": "\u8cc7\u6e90\u30bf\u30a4\u30d7", "attribute_value_mlt": [{"resourcetype": "journal article", "resourceuri": 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