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Title

Secondary coenzyme Q10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders

AuthorsYubero, Delia; Montero, Raquel; Martín, Miguel A.; Rodríguez-Aguilera, Juan Carlos ; Navas, Plácido ; Artuch, Rafael; García-Cazorla, Angels; Rivas Infante, Eloy; Cascajo Almenara, M. V. ; Alcázar-Fabra, María; Brea-Calvo, Gloria
KeywordsMitochondrial respiratory chain
Oxidative phosphorylation disorders
Coenzyme Q10
Muscle biopsy
Issue Date2016
PublisherElsevier
CitationMitochondrion 30: 51-58 (2016)
AbstractWe evaluated the coenzyme Q₁₀ (CoQ) levels in patients who were diagnosed with mitochondrial oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders (n = 72). Data from the 72 cases in this study revealed that 44.4% of patients showed low CoQ concentrations in either their skeletal muscle or skin fibroblasts. Our findings suggest that secondary CoQ deficiency is a common finding in OXPHOS and non-OXPHOS disorders. We hypothesize that cases of CoQ deficiency associated with OXPHOS defects could be an adaptive mechanism to maintain a balanced OXPHOS, although the mechanisms explaining these deficiencies and the pathophysiological role of secondary CoQ deficiency deserves further investigation.
DescriptionCoQ deficiency study group: et al.
URIhttp://hdl.handle.net/10261/163399
Identifiersdoi: 10.1016/j.mito.2016.06.007
e-issn: 1872-8278
issn: 1567-7249
Appears in Collections:(CABD) Artículos
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