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Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromes

2013; Elsevier BV; Volume: 13; Issue: 4 Linguagem: Inglês

10.1016/j.mito.2013.04.001

1872-8278

Raquel Montero, Manuela Grazina, Ester López‐Gallardo, Julio Montoya, Paz Briones, Aleix Navarro‐Sastre, John M. Land, Iain P. Hargreaves, Rafael Artuch, María del Mar O’Callaghan, Cristina Jou, C. Jiménez-Mallebrera, Núria Bujan, Mercè Pineda, Àngels García‐Cazorla, A. Nascimento, Belén Pérez‐Dueñas, Eduardo Ruiz‐Pesini, Carl Fratter, Leonardo Salviati, Marta Simões, Cândida Mendes, Maria João Santos, Luísa Diogo, Paula Garcia, Plácido Navas,

Mitochondrial Function and Pathology

We 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.