Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency

Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency

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,...

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Bibliographic Details
Main Authors: Masato Wakabayashi, Yuji Kamijo, Takero Nakajima, Naoki Tanaka, Eiko Sugiyama, Tian Yangyang, Takefumi Kimura, Toshifumi Aoyama
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:PPAR Research
Online Access:http://dx.doi.org/10.1155/2012/371691
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