期刊
MITOCHONDRION
卷 13, 期 4, 页码 337-341出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.mito.2013.04.001
关键词
Mitochondrial DNA depletion syndrome; Coenzyme Q(10) deficiency; Mitochondrial disorders
资金
- Instituto de Salud Carlos III, Ministerio de Ciencia e Innovacion, Spain (FIS) [PI11/00078, PI10/00662, PI11/01301, PI08/0307, PI08/0663, PI07/00184]
- The Centro de Investigaciones Biomedicas en Red de Enfermedades Raras (CIBERER) by Departamento de Ciencia, Tecnologia y Universidad del Gobierno de Aragon y Fondo Social Europeo (Grupo B33)
- Asociacion de Enfermos de Patologia Mitocondrial (AEPMI)
- Department of Health's NIHR Biomedical Research Centres
We evaluated coenzyme Q(10) (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. (c) 2013 Elsevier B.V. and Mitochondria Research Society. All rights reserved.
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