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Mitochondrial morphology, bioenergetics and proteomic responses in fatty acid oxidation disorders

Mutations in nuclear genes encoding for mitochondrial proteins very long-chain acyl-CoA dehydrogenase (VLCAD) and trifunctional protein (TFP) cause rare autosomal recessive disorders. Studies in fibroblasts derived from patients with mutations in VLCAD and TFP exhibit mitochondrial defects. To gain...

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Detalles Bibliográficos
Autores principales: Raimo, Serena, Zura-Miller, Gabriella, Fezelinia, Hossein, Spruce, Lynn A., Zakopoulos, Iordanis, Mohsen, Al-Walid, Vockley, Jerry, Ischiropoulos, Harry
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7970426/
https://www.ncbi.nlm.nih.gov/pubmed/33725513
http://dx.doi.org/10.1016/j.redox.2021.101923