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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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Detalles Bibliográficos
Autores principales:	Wakabayashi, Masato, Kamijo, Yuji, Nakajima, Takero, Tanaka, Naoki, Sugiyama, Eiko, Yangyang, Tian, Kimura, Takefumi, Aoyama, Toshifumi
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Hindawi Publishing Corporation 2012
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3357605/ https://www.ncbi.nlm.nih.gov/pubmed/22654897 http://dx.doi.org/10.1155/2012/371691

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