Coenzyme Q10 deficiency in mitochondrial DNA depletion syndromes.
Mitochondrion
; 13(4): 337-41, 2013 Jul.
Article
em En
| MEDLINE
| ID: mdl-23583954
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Ataxia
/
Ubiquinona
/
Miopatias Mitocondriais
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Debilidade Muscular
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Doenças Mitocondriais
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Erros Inatos do Metabolismo
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Doenças Musculares
Tipo de estudo:
Diagnostic_studies
Limite:
Adolescent
/
Adult
/
Child
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Child, preschool
/
Female
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Humans
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Infant
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Male
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Newborn
Idioma:
En
Ano de publicação:
2013
Tipo de documento:
Article