{"_buckets": {"deposit": "f2768ded-78b7-4af6-b66e-984202b3bd95"}, "_deposit": {"id": "7185", "owners": [], "pid": {"revision_id": 0, "type": "depid", "value": "7185"}, "status": "published"}, "_oai": {"id": "oai:soar-ir.repo.nii.ac.jp:00007185", "sets": ["849"]}, "author_link": ["17375", "17376", "17377", "17378", "17379", "17380", "17381", "17382"], "item_1628147817048": {"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_6_biblio_info_6": {"attribute_name": "書誌情報", "attribute_value_mlt": [{"bibliographicIssueDates": {"bibliographicIssueDate": "2012", "bibliographicIssueDateType": "Issued"}, "bibliographicPageStart": "Article ID 371691", "bibliographic_titles": [{"bibliographic_title": "PPAR RESEARCH"}]}]}, "item_6_description_19": {"attribute_name": "内容記述", "attribute_value_mlt": [{"subitem_description": "信州大学博士（医学）・学位論文・平成24年5月14日授与（乙第1148号）・若林雅人", "subitem_description_type": "Other"}]}, "item_6_description_20": {"attribute_name": "抄録", "attribute_value_mlt": [{"subitem_description": "To examine fatty acid accumulation and its toxic effects in cells, we analyzed skin fibroblasts from six patients with mitochondrial trifunctional protein deficiency, who had abnormalities in the second through fourth reactions in fatty acid beta-oxidation system. We found free fatty acid accumulation, enhanced three acyl-CoA dehydrogenases, catalyzing the first reaction in the beta-oxidation system and being assumed to have normal activities in these patients, and PPAR alpha activation that was confirmed in the experiments using MK886, a PPAR alpha specific antagonist and fenofibrate, a PPAR alpha specific agonist. These novel findings suggest that the fatty acid accumulation and the resulting PPAR alpha activation are major causes of the increase in the beta-oxidation ability as probable compensation for fatty acid metabolism in the patients\u0027 fibroblasts, and that enhanced cell proliferation and increased oxidative stress due to the PPAR alpha activation relate to the development of specific clinical features such as hypertrophic cardiomyopathy, slight hepatomegaly, and skeletal myopathy. Additionally, significant suppression of the PPAR alpha activation by means of MK886 treatment is assumed to provide a new method of treating this deficiency.", "subitem_description_type": "Abstract"}]}, "item_6_description_30": {"attribute_name": "資源タイプ（コンテンツの種類）", "attribute_value_mlt": [{"subitem_description": "Article", "subitem_description_type": "Other"}]}, "item_6_description_5": {"attribute_name": "引用", "attribute_value_mlt": [{"subitem_description": "PPAR Research. Volume 2012 (2012), Article ID 371691, 7 pages", "subitem_description_type": "Other"}]}, "item_6_link_67": {"attribute_name": "WoS", "attribute_value_mlt": [{"subitem_link_text": "Web of Science", "subitem_link_url": "http://gateway.isiknowledge.com/gateway/Gateway.cgi?\u0026GWVersion=2\u0026SrcAuth=ShinshuUniv\u0026SrcApp=ShinshuUniv\u0026DestLinkType=FullRecord\u0026DestApp=WOS\u0026KeyUT=000305018500001"}]}, "item_6_publisher_4": {"attribute_name": "出版者", "attribute_value_mlt": [{"subitem_publisher": "HINDAWI PUBLISHING CORPORATION"}]}, "item_6_relation_48": {"attribute_name": "DOI", "attribute_value_mlt": [{"subitem_relation_name": [{"subitem_relation_name_text": "10.1155/2012/371691"}], "subitem_relation_type_id": {"subitem_relation_type_id_text": "https://doi.org/10.1155/2012/371691", "subitem_relation_type_select": "DOI"}}]}, "item_6_rights_62": {"attribute_name": "権利", "attribute_value_mlt": [{"subitem_rights": "Copyright © 2012 Wakabayashi Masato et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cite"}]}, "item_6_select_64": {"attribute_name": "著者版フラグ", "attribute_value_mlt": [{"subitem_select_item": "publisher"}]}, "item_6_source_id_35": {"attribute_name": "ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "1687-4757", "subitem_source_identifier_type": "ISSN"}]}, "item_6_source_id_39": {"attribute_name": "NII ISSN", "attribute_value_mlt": [{"subitem_source_identifier": "1687-4757", "subitem_source_identifier_type": "ISSN"}]}, "item_6_source_id_40": {"attribute_name": "書誌レコードID", "attribute_value_mlt": [{"subitem_source_identifier": "AA12156341", "subitem_source_identifier_type": "NCID"}]}, "item_creator": {"attribute_name": "著者", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "Wakabayashi,  Masato"}], "nameIdentifiers": [{"nameIdentifier": "17375", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Kamijo,  Yuji"}], "nameIdentifiers": [{"nameIdentifier": "17376", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Nakajima,  Takero"}], "nameIdentifiers": [{"nameIdentifier": "17377", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Tanaka,  Naoki"}], "nameIdentifiers": [{"nameIdentifier": "17378", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Sugiyama,  Eiko"}], "nameIdentifiers": [{"nameIdentifier": "17379", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Tian,  Yangyang"}], "nameIdentifiers": [{"nameIdentifier": "17380", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Kimura,  Takefumi"}], "nameIdentifiers": [{"nameIdentifier": "17381", "nameIdentifierScheme": "WEKO"}]}, {"creatorNames": [{"creatorName": "Aoyama,  Toshifumi"}], "nameIdentifiers": [{"nameIdentifier": "17382", "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": "2015-09-25"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "H24Otsu1148_Wakabayashi.pdf", "filesize": [{"value": "1.3 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_note", "mimetype": "application/pdf", "size": 1300000.0, "url": {"label": "H24Otsu1148_Wakabayashi.pdf", "url": "https://soar-ir.repo.nii.ac.jp/record/7185/files/H24Otsu1148_Wakabayashi.pdf"}, "version_id": "510b051e-7dbc-4777-8268-82bdb6595eff"}]}, "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": "Fatty Acid Accumulation and Resulting PPAR alpha Activation in Fibroblasts due to Trifunctional Protein Deficiency", "item_titles": {"attribute_name": "タイトル", "attribute_value_mlt": [{"subitem_title": "Fatty Acid Accumulation and Resulting PPAR alpha Activation in Fibroblasts due to Trifunctional Protein Deficiency", "subitem_title_language": "en"}]}, "item_type_id": "6", "owner": "1", "path": ["849"], "permalink_uri": "http://hdl.handle.net/10091/16851", "pubdate": {"attribute_name": "PubDate", "attribute_value": "2013-03-26"}, "publish_date": "2013-03-26", "publish_status": "0", "recid": "7185", "relation": {}, "relation_version_is_last": true, "title": ["Fatty Acid Accumulation and Resulting PPAR alpha Activation in Fibroblasts due to Trifunctional Protein Deficiency"], "weko_shared_id": -1}