| Literature DB >> 27374853 |
Delia Yubero1, Raquel Montero2, Miguel A Martín3, Julio Montoya4, Antonia Ribes5, Manuela Grazina6, Eva Trevisson7, Juan Carlos Rodriguez-Aguilera8, Iain P Hargreaves9, Leonardo Salviati7, Plácido Navas8, Rafael Artuch10, Cristina Jou2, Cecilia Jimenez-Mallebrera2, Andres Nascimento2, Belén Pérez-Dueñas2, Carlos Ortez1, Federico Ramos1, Jaume Colomer1, Mar O'Callaghan2, Mercè Pineda1, Angels García-Cazorla2, Carmina Espinós11, Angels Ruiz12, Alfons Macaya13, Anna Marcé-Grau13, Judit Garcia-Villoria5, Angela Arias5, Sonia Emperador4, Eduardo Ruiz-Pesini4, Ester Lopez-Gallardo4, Viruna Neergheen9, Marta Simões6, Luisa Diogo14, Alberto Blázquez8, Adrián González-Quintana15, Aitor Delmiro8, Cristina Domínguez-González8, Joaquín Arenas8, M Teresa García-Silva8, Elena Martín15, Pilar Quijada15, Aurelio Hernández-Laín15, María Morán8, Eloy Rivas Infante11, Rainiero Ávila Polo11, Carmen Paradas Lópe11, Juan Bautista Lorite11, Eva M Martínez Fernández11, Ana B Cortés11, Ana Sánchez-Cuesta11, Maria V Cascajo16, María Alcázar16, Gloria Brea-Calvo16.
Abstract
We evaluated the coenzyme Q₁₀ (CoQ) levels in patients who were diagnosed with mitochondrial oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders (n=72). Data from the 72 cases in this study revealed that 44.4% of patients showed low CoQ concentrations in either their skeletal muscle or skin fibroblasts. Our findings suggest that secondary CoQ deficiency is a common finding in OXPHOS and non-OXPHOS disorders. We hypothesize that cases of CoQ deficiency associated with OXPHOS defects could be an adaptive mechanism to maintain a balanced OXPHOS, although the mechanisms explaining these deficiencies and the pathophysiological role of secondary CoQ deficiency deserves further investigation.Entities:
Keywords: Coenzyme Q(10); Mitochondrial respiratory chain; Muscle biopsy; Oxidative phosphorylation disorders
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Year: 2016 PMID： 27374853 DOI： 10.1016/j.mito.2016.06.007
Source DB: PubMed Journal: Mitochondrion ISSN： 1567-7249 Impact factor: 4.160