Secondary coenzyme Q 10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders
2016; Elsevier BV; Volume: 30; Linguagem: Inglês
10.1016/j.mito.2016.06.007
ISSN1872-8278
AutoresDèlia Yubero, Raquel Montero, Miguel Á. Martín, Julio Montoya, Antònia Ribes, Manuela Grazina, Eva Trevisson, Juan Carlos Rodríguez‐Aguilera, Iain P. Hargreaves, Leonardo Salviati, Plácido Navas, Rafael Artuch, Cristina Jou, C. Jiménez-Mallebrera, A. Nascimento, Belén Pérez‐Dueñas, C. Ortez, Federico Ramos, Jaume Colomer, Mar O’Callaghan, Mercè Pineda, Àngels García‐Cazorla, Carmen Espinós, Angels Ruiz, Alfons Macaya, Anna Marcé‐Grau, Judit García‐Villoria, Ángela Arias, Sonia Emperador, Eduardo Ruiz‐Pesini, Ester López‐Gallardo, Viruna Neergheen, Marta Simões, Luísa Diogo, Alberto Blázquez, Adrián González-Quintana, Aitor Delmiro, Cristina Domínguez‐González, Joaquı́n Arenas, M.T. García‐Silva, Elena Martín, Pilar Quijada, Aurelio Hernández‐Laín, María Morán, Eloy Rivas, Rainiero Ávila Polo, Carmen Paradas Lópe, Juan Bautista Lorite, Eva M. Martínez Fernández, Ana Cortés, Ana Sánchez‐Cuesta, María V. Cascajo, María Alcázar‐Fabra, Gloria Brea‐Calvo,
Tópico(s)Mitochondrial Function and Pathology
ResumoWe 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.
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