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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Detalhes bibliográficos
Main Authors: Wakabayashi, Masato, Kamijo, Yuji, Nakajima, Takero, Tanaka, Naoki, Sugiyama, Eiko, Yangyang, Tian, Kimura, Takefumi, Aoyama, Toshifumi
Formato: Artigo
Idioma:Inglês
Publicado em: Hindawi Publishing Corporation 2012
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3357605/
https://ncbi.nlm.nih.gov/pubmed/22654897
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1155/2012/371691
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