doi:10.1016/j.mito.2013.04.001engMontero, R.Grazina, M.López-Gallardo, E.Montoya, J.Briones, P.Navarro-Sastre, A.Land, J. M.Hargreaves, I. P.Artuch, R.del, Mar O.Jou, C.Jimenez, C.Buján, N.Pineda, M.García-Cazorla, A.Nascimento, A.Perez-Dueñas, B.Ruiz-Pesini, E.Fratter, C.Salviati, L.Simões, M.Mendes, C.JoãoSantos, M.Diogo, L.Garcia, P.Navas, P.Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromesART-2013-81822We 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.2013http://zaguan.unizar.es/record/13050110.1016/j.mito.2013.04.001http://zaguan.unizar.es/record/130501oai:zaguan.unizar.es:130501MITOCHONDRION 13, 4 (2013), 337-341All rights reservedhttp://www.europeana.eu/rights/rr-f/info:eu-repo/semantics/closedAccess