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Fatty Acid Accumulation and Resulting PPAR alpha Activation in Fibroblasts due to Trifunctional Protein Deficiency
http://hdl.handle.net/10091/16851
http://hdl.handle.net/10091/168515e9acb68-0fc2-4e9a-ad0a-561b8448e62a
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
H24Otsu1148_Wakabayashi.pdf (1.3 MB)
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| Item type | 学術雑誌論文 / Journal Article(1) | |||||
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| 公開日 | 2013-03-26 | |||||
| タイトル | ||||||
| タイトル | Fatty Acid Accumulation and Resulting PPAR alpha Activation in Fibroblasts due to Trifunctional Protein Deficiency | |||||
| 言語 | en | |||||
| 言語 | ||||||
| 言語 | eng | |||||
| DOI | ||||||
| 識別子タイプ | DOI | |||||
| 関連識別子 | https://doi.org/10.1155/2012/371691 | |||||
| 関連名称 | 10.1155/2012/371691 | |||||
| 資源タイプ | ||||||
| 資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
| 資源タイプ | journal article | |||||
| 著者 |
Wakabayashi, Masato
× Wakabayashi, Masato× Kamijo, Yuji× Nakajima, Takero× Tanaka, Naoki× Sugiyama, Eiko× Tian, Yangyang× Kimura, Takefumi× Aoyama, Toshifumi |
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| 出版者 | ||||||
| 出版者 | HINDAWI PUBLISHING CORPORATION | |||||
| 引用 | ||||||
| 内容記述タイプ | Other | |||||
| 内容記述 | PPAR Research. Volume 2012 (2012), Article ID 371691, 7 pages | |||||
| 書誌情報 |
PPAR RESEARCH p. Article ID 371691, 発行日 2012 |
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| 内容記述 | ||||||
| 内容記述タイプ | Other | |||||
| 内容記述 | 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 | |||||
| 抄録 | ||||||
| 内容記述タイプ | Abstract | |||||
| 内容記述 | 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' 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. | |||||
| 資源タイプ（コンテンツの種類） | ||||||
| 内容記述タイプ | Other | |||||
| 内容記述 | Article | |||||
| ISSN | ||||||
| 収録物識別子タイプ | ISSN | |||||
| 収録物識別子 | 1687-4757 | |||||
| 書誌レコードID | ||||||
| 収録物識別子タイプ | NCID | |||||
| 収録物識別子 | AA12156341 | |||||
| 権利 | ||||||
| 権利情報 | 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 | |||||
| 出版タイプ | ||||||
| 出版タイプ | VoR | |||||
| 出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |||||
| WoS | ||||||
| 表示名 | Web of Science | |||||
| URL | http://gateway.isiknowledge.com/gateway/Gateway.cgi?&GWVersion=2&SrcAuth=ShinshuUniv&SrcApp=ShinshuUniv&DestLinkType=FullRecord&DestApp=WOS&KeyUT=000305018500001 | |||||
