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

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

doi:10.1016/j.mito.2016.06.007

000130565 001__ 130565
000130565 005__ 20240126184244.0
000130565 0247_ $$2doi$$a10.1016/j.mito.2016.06.007
000130565 0248_ $$2sideral$$a95666
000130565 037__ $$aART-2016-95666
000130565 041__ $$aeng
000130565 100__ $$aYubero, D.
000130565 245__ $$aSecondary coenzyme Q10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders
000130565 260__ $$c2016
000130565 5203_ $$aWe 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.
000130565 540__ $$9info:eu-repo/semantics/openAccess$$aAll rights reserved$$uhttp://www.europeana.eu/rights/rr-f/
000130565 590__ $$a3.704$$b2016
000130565 591__ $$aGENETICS & HEREDITY$$b51 / 165 = 0.309$$c2016$$dQ2$$eT1
000130565 591__ $$aCELL BIOLOGY$$b82 / 188 = 0.436$$c2016$$dQ2$$eT2
000130565 592__ $$a1.852$$b2016
000130565 593__ $$aMolecular Medicine$$c2016$$dQ1
000130565 593__ $$aCell Biology$$c2016$$dQ2
000130565 593__ $$aMolecular Biology$$c2016$$dQ2
000130565 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000130565 700__ $$aMontero, R.
000130565 700__ $$aMartín, M. A.
000130565 700__ $$0(orcid)0000-0003-1770-6299$$aMontoya, J.$$uUniversidad de Zaragoza
000130565 700__ $$aRibes, A.
000130565 700__ $$aGrazina, M.
000130565 700__ $$aTrevisson, E.
000130565 700__ $$aRodriguez-Aguilera, J.
000130565 700__ $$aHargreaves, I. P.
000130565 700__ $$aSalviati, L.
000130565 700__ $$aNavas, P.
000130565 700__ $$aArtuch, R.
000130565 700__ $$aJou, C.
000130565 700__ $$aJimenez-Mallebrera, C.
000130565 700__ $$aNascimento, A.
000130565 700__ $$aPérez-Dueñas, B.
000130565 700__ $$aOrtez, C.
000130565 700__ $$aRamos, F.
000130565 700__ $$aColomer, J.
000130565 700__ $$aO''Callaghan, M.
000130565 700__ $$aPineda, M.
000130565 700__ $$aGarcía-Cazorla, A.
000130565 700__ $$aEspinós, C.
000130565 700__ $$aRuiz, A.
000130565 700__ $$aMacaya, A.
000130565 700__ $$aMarcé-Grau, A.
000130565 700__ $$aGarcia-Villoria, J.
000130565 700__ $$aArias, A.
000130565 700__ $$0(orcid)0000-0001-5964-6138$$aEmperador, S.
000130565 700__ $$0(orcid)0000-0002-0269-7337$$aRuiz-Pesini, E.$$uUniversidad de Zaragoza
000130565 700__ $$0(orcid)0000-0002-3217-1424$$aLopez-Gallardo, E.$$uUniversidad de Zaragoza
000130565 700__ $$aNeergheen, V.
000130565 700__ $$aSimões, M.
000130565 700__ $$aDiogo, L.
000130565 700__ $$aBlázquez, A.
000130565 700__ $$aGonzález-Quintana, A.
000130565 700__ $$aDelmiro, A.
000130565 700__ $$aDomínguez-González, C.
000130565 700__ $$aArenas, J.
000130565 700__ $$aGarcía-Silva, M. T.
000130565 700__ $$aMartín, E.
000130565 700__ $$aQuijada, P.
000130565 700__ $$aHernández-Laín, A.
000130565 700__ $$aMorán, M.
000130565 700__ $$aRivas Infante, E.
000130565 700__ $$aÁvila Polo, R.
000130565 700__ $$aParadas Lópe, C.
000130565 700__ $$aBautista Lorite, J.
000130565 700__ $$aMartínez Fernández, E. M.
000130565 700__ $$aCortés, A. B.
000130565 700__ $$aSánchez-Cuesta, A.
000130565 700__ $$aCascajo, M. V.
000130565 700__ $$aAlcázar, M.
000130565 700__ $$aBrea-Calvo, G.
000130565 7102_ $$11003$$2443$$aUniversidad de Zaragoza$$bDpto. Anatom.Histolog.Humanas$$cArea Histología
000130565 7102_ $$11002$$2060$$aUniversidad de Zaragoza$$bDpto. Bioq.Biolog.Mol. Celular$$cÁrea Bioquímica y Biolog.Mole.
000130565 773__ $$g30 (2016), 51-58$$pMitochondrion$$tMITOCHONDRION$$x1567-7249
000130565 8564_ $$s156942$$uhttps://zaguan.unizar.es/record/130565/files/texto_completo.pdf$$yVersión publicada
000130565 8564_ $$s2658335$$uhttps://zaguan.unizar.es/record/130565/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
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000130565 951__ $$a2024-01-26-18:10:20
000130565 980__ $$aARTICLE

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