Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10883073 | Mitochondrion | 2013 | 5 Pages |
Abstract
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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Authors
Raquel Montero, Manuela Grazina, Ester López-Gallardo, Julio Montoya, Paz Briones, Aleix Navarro-Sastre, John M. Land, Iain P. Hargreaves, Rafael Artuch, Coenzyme Q10 deficiency study group. Coenzyme Q10 deficiency study group.,