Secondary coenzyme Q10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders

Yubero, D. ; Montero, R. ; Martín, M. A. ; Montoya, J. (Universidad de Zaragoza) ; Ribes, A. ; Grazina, M. ; Trevisson, E. ; Rodriguez-Aguilera, J. ; Hargreaves, I. P. ; Salviati, L. ; Navas, P. ; Artuch, R. ; Jou, C. ; Jimenez-Mallebrera, C. ; Nascimento, A. ; Pérez-Dueñas, B. ; Ortez, C. ; Ramos, F. ; Colomer, J. ; O''Callaghan, M. ; Pineda, M. ; García-Cazorla, A. ; Espinós, C. ; Ruiz, A. ; Macaya, A. ; Marcé-Grau, A. ; Garcia-Villoria, J. ; Arias, A. ; Emperador, S. ; Ruiz-Pesini, E. (Universidad de Zaragoza) ; Lopez-Gallardo, E. (Universidad de Zaragoza) ; Neergheen, V. ; Simões, M. ; Diogo, L. ; Blázquez, A. ; González-Quintana, A. ; Delmiro, A. ; Domínguez-González, C. ; Arenas, J. ; García-Silva, M. T. ; Martín, E. ; Quijada, P. ; Hernández-Laín, A. ; Morán, M. ; Rivas Infante, E. ; Ávila Polo, R. ; Paradas Lópe, C. ; Bautista Lorite, J. ; Martínez Fernández, E. M. ; Cortés, A. B. ; Sánchez-Cuesta, A. ; Cascajo, M. V. ; Alcázar, M. ; Brea-Calvo, G.

Resumen: 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.

Idioma: Inglés

DOI: 10.1016/j.mito.2016.06.007

Año: 2016

Publicado en: MITOCHONDRION 30 (2016), 51-58
ISSN: 1567-7249

Factor impacto JCR: 3.704 (2016)

Categ. JCR: GENETICS & HEREDITY rank: 51 / 165 = 0.309 (2016) - Q2 - T1
Categ. JCR: CELL BIOLOGY rank: 82 / 188 = 0.436 (2016) - Q2 - T2

Factor impacto SCIMAGO: 1.852 - Molecular Medicine (Q1) - Cell Biology (Q2) - Molecular Biology (Q2)

Tipo y forma: Article (Published version)

Área (Departamento): Area Histología (Dpto. Anatom.Histolog.Humanas)
Área (Departamento): Área Bioquímica y Biolog.Mole. (Dpto. Bioq.Biolog.Mol. Celular)