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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Pubblicato in:Redox Biol
Autori principali: Raimo, Serena, Zura-Miller, Gabriella, Fezelinia, Hossein, Spruce, Lynn A., Zakopoulos, Iordanis, Mohsen, Al-Walid, Vockley, Jerry, Ischiropoulos, Harry
Natura: Artigo
Lingua:Inglês
Pubblicazione: Elsevier 2021
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Research Paper
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC7970426/
https://ncbi.nlm.nih.gov/pubmed/33725513
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.redox.2021.101923
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