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Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency
Joint Authors
Kimura, Takefumi
Sugiyama, Eiko
Kamijo, Yuji
Nakajima, Takero
Aoyama, Toshifumi
Yangyang, Tian
Wakabayashi, Masato
Tanaka, Naoki
Source
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-7, 7 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-05-08
Country of Publication
Egypt
No. of Pages
7
Main Subjects
Natural & Life Sciences (Multidisciplinary)
Biology
Abstract EN
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 β-oxidation system.
We found free fatty acid accumulation, enhanced three acyl-CoA dehydrogenases, catalyzing the first reaction in the β-oxidation system and being assumed to have normal activities in these patients, and PPARα activation that was confirmed in the experiments using MK886, a PPARα specific antagonist and fenofibrate, a PPARα specific agonist.
These novel findings suggest that the fatty acid accumulation and the resulting PPARα activation are major causes of the increase in the β-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 PPARα activation relate to the development of specific clinical features such as hypertrophic cardiomyopathy, slight hepatomegaly, and skeletal myopathy.
Additionally, significant suppression of the PPARα activation by means of MK886 treatment is assumed to provide a new method of treating this deficiency.
American Psychological Association (APA)
Wakabayashi, Masato& Kamijo, Yuji& Nakajima, Takero& Tanaka, Naoki& Sugiyama, Eiko& Yangyang, Tian…[et al.]. 2012. Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency. PPAR Research،Vol. 2012, no. 2012, pp.1-7.
https://search.emarefa.net/detail/BIM-466783
Modern Language Association (MLA)
Wakabayashi, Masato…[et al.]. Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency. PPAR Research No. 2012 (2012), pp.1-7.
https://search.emarefa.net/detail/BIM-466783
American Medical Association (AMA)
Wakabayashi, Masato& Kamijo, Yuji& Nakajima, Takero& Tanaka, Naoki& Sugiyama, Eiko& Yangyang, Tian…[et al.]. Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency. PPAR Research. 2012. Vol. 2012, no. 2012, pp.1-7.
https://search.emarefa.net/detail/BIM-466783
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-466783