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OxPhos defects cause hypermetabolism and reduce lifespan in cells and in patients with mitochondrial diseases

Patients with primary mitochondrial oxidative phosphorylation (OxPhos) defects present with fatigue and multi-system disorders, are often lean, and die prematurely, but the mechanistic basis for this clinical picture remains unclear. By integrating data from 17 cohorts of patients with mitochondrial...

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Detalles Bibliográficos
Autores principales: Sturm, Gabriel, Karan, Kalpita R., Monzel, Anna S., Santhanam, Balaji, Taivassalo, Tanja, Bris, Céline, Ware, Sarah A., Cross, Marissa, Towheed, Atif, Higgins-Chen, Albert, McManus, Meagan J., Cardenas, Andres, Lin, Jue, Epel, Elissa S., Rahman, Shamima, Vissing, John, Grassi, Bruno, Levine, Morgan, Horvath, Steve, Haller, Ronald G., Lenaers, Guy, Wallace, Douglas C., St-Onge, Marie-Pierre, Tavazoie, Saeed, Procaccio, Vincent, Kaufman, Brett A., Seifert, Erin L., Hirano, Michio, Picard, Martin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9837150/
https://www.ncbi.nlm.nih.gov/pubmed/36635485
http://dx.doi.org/10.1038/s42003-022-04303-x