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Title: Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromes
Authors: Montero, Raquel 
Grazina, Manuela 
López-Gallardo, Ester 
Montoya, Julio 
Briones, Paz 
Navarro-Sastre, Aleix 
Land, John M. 
Hargreaves, Iain P. 
Artuch, Rafael 
O'Callaghan, Maria del Mar 
Jou, Cristina 
Jimenez, Cecilia 
Buján, Nuria 
Pineda, Mercè 
García-Cazorla, Angels 
Nascimento, Andrés 
Perez-Dueñas, Belen 
Ruiz-Pesini, Eduardo 
Fratter, Carl 
Salviati, Leonardo 
Simões, Marta
Mendes, Cândida 
Santos, Maria João 
Diogo, Luisa 
Garcia, Paula 
Navas, Plácido 
Keywords: Mitochondrial DNA depletion syndrome; Coenzyme Q10 deficiency; Mitochondrial disorders
Issue Date: Jul-2013
Publisher: Elsevier
Citation: MONTERO, Raquel [et. al] - Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromes. "Mitochondrion". ISSN 1567-7249. Vol. 13 Nº. 4 (2013) p. 337-341
Serial title, monograph or event: Mitochondrion
Volume: 13
Issue: 4
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.
ISSN: 1567-7249
DOI: 10.1016/j.mito.2013.04.001
Rights: openAccess
Appears in Collections:FMUC Medicina - Artigos em Revistas Internacionais
I&D CNC - Artigos em Revistas Internacionais

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