000130501 001__ 130501 000130501 005__ 20240125162931.0 000130501 0247_ $$2doi$$a10.1016/j.mito.2013.04.001 000130501 0248_ $$2sideral$$a81822 000130501 037__ $$aART-2013-81822 000130501 041__ $$aeng 000130501 100__ $$aMontero, R. 000130501 245__ $$aCoenzyme Q10 deficiency in mitochondrial DNA depletion syndromes 000130501 260__ $$c2013 000130501 5203_ $$aWe evaluated coenzyme Q10 (CoQ) levels in patients studied under suspicion of mitochondrial DNA depletion syndromes (MDS) (n = 39). CoQ levels were quantified by HPLC, and the percentage of mtDNA depletion by quantitative real-time PCR. A high percentage of MDS patients presented with CoQ deficiency as compared to other mitochondrial patients (Mann–Whitney-U test: p = 0.001). Our findings suggest that MDS are frequently associated with CoQ deficiency, as a possible secondary consequence of disease pathophysiology. Assessment of muscle CoQ status seems advisable in MDS patients since the possibility of CoQ supplementation may then be considered as a candidate therapy. 000130501 540__ $$9info:eu-repo/semantics/openAccess$$aAll rights reserved$$uhttp://www.europeana.eu/rights/rr-f/ 000130501 590__ $$a3.524$$b2013 000130501 591__ $$aGENETICS & HEREDITY$$b52 / 164 = 0.317$$c2013$$dQ2$$eT1 000130501 591__ $$aCELL BIOLOGY$$b85 / 184 = 0.462$$c2013$$dQ2$$eT2 000130501 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion 000130501 700__ $$aGrazina, M. 000130501 700__ $$0(orcid)0000-0002-3217-1424$$aLópez-Gallardo, E.$$uUniversidad de Zaragoza 000130501 700__ $$0(orcid)0000-0003-1770-6299$$aMontoya, J.$$uUniversidad de Zaragoza 000130501 700__ $$aBriones, P. 000130501 700__ $$aNavarro-Sastre, A. 000130501 700__ $$aLand, J. M. 000130501 700__ $$aHargreaves, I. P. 000130501 700__ $$aArtuch, R. 000130501 700__ $$adel, Mar O. 000130501 700__ $$aJou, C. 000130501 700__ $$aJimenez, C. 000130501 700__ $$aBuján, N. 000130501 700__ $$aPineda, M. 000130501 700__ $$aGarcía-Cazorla, A. 000130501 700__ $$aNascimento, A. 000130501 700__ $$aPerez-Dueñas, B. 000130501 700__ $$0(orcid)0000-0002-0269-7337$$aRuiz-Pesini, E.$$uUniversidad de Zaragoza 000130501 700__ $$aFratter, C. 000130501 700__ $$aSalviati, L. 000130501 700__ $$aSimões, M. 000130501 700__ $$aMendes, C. 000130501 700__ $$aJoãoSantos, M. 000130501 700__ $$aDiogo, L. 000130501 700__ $$aGarcia, P. 000130501 700__ $$aNavas, P. 000130501 7102_ $$11002$$2060$$aUniversidad de Zaragoza$$bDpto. Bioq.Biolog.Mol. Celular$$cÁrea Bioquímica y Biolog.Mole. 000130501 773__ $$g13, 4 (2013), 337-341$$pMitochondrion$$tMITOCHONDRION$$x1567-7249 000130501 8564_ $$s189049$$uhttps://zaguan.unizar.es/record/130501/files/texto_completo.pdf$$yVersión publicada 000130501 8564_ $$s2853691$$uhttps://zaguan.unizar.es/record/130501/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada 000130501 909CO $$ooai:zaguan.unizar.es:130501$$particulos$$pdriver 000130501 951__ $$a2024-01-25-15:15:51 000130501 980__ $$aARTICLE