Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromes

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...

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Bibliographic Details
Main Author: Montero, Raquel (author)
Other Authors: Grazina, Manuela (author), López-Gallardo, Ester (author), Montoya, Julio (author), Briones, Paz (author), Navarro-Sastre, Aleix (author), Land, John M. (author), Hargreaves, Iain P. (author), Artuch, Rafael (author), O'Callaghan, Maria del Mar (author), Jou, Cristina (author), Jimenez, Cecilia (author), Buján, Nuria (author), Pineda, Mercè (author), García-Cazorla, Angels (author), Nascimento, Andrés (author), Perez-Dueñas, Belen (author), Ruiz-Pesini, Eduardo (author), Fratter, Carl (author), Salviati, Leonardo (author), Simões, Marta (author), Mendes, Cândida (author), Santos, Maria João (author), Diogo, Luísa (author), Garcia, Paula (author), Navas, Plácido (author)
Format: article
Language:eng
Published: 2013
Subjects:
Mitochondrial DNA depletion syndrome
Coenzyme Q10 deficiency
Mitochondrial disorders
Online Access:http://hdl.handle.net/10316/27281
Country:Portugal
Oai:oai:estudogeral.sib.uc.pt:10316/27281
Description
Summary: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.

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