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Closed Access item Analysis of Coenzyme Q10 in muscle and fibroblasts for the diagnosis of CoQ10 deficiency syndromes

Authors:Montero, Raquel
Sánchez-Alcázar, José Antonio
Briones, Paz
Rodríguez-Hernández, Ángeles
Cordero, Mario D.
Trevisson, Eva
Salviati, Leonardo
Pineda, Mercé
García-Cazorla, Angels
Navas, Plácido
Artuch, Rafael
Keywords:Biological markers, Electron Transport, Fibroblasts, Mitochondria, Ubiquinone, Coenzyme Q10
Issue Date:20-Mar-2008
Canadian Society of Clinical Chemists
Citation:Clinical Biochemistry - New York 41(9): 697-700 (2008)
Abstract:OBJECTIVES: To study CoQ(10) concentrations in muscle and fibroblast from 6 patients with a CoQ(10) deficiency syndrome. DESIGN AND METHODS: CoQ(10) was quantified by HPLC with electrochemical detection. RESULTS: Four out of the 6 cases showed muscle CoQ(10) deficiency plus a reduction of mitochondrial respiratory chain enzyme activities. All cases showed decreased CoQ(10) values in fibroblasts when compared with controls. CONCLUSIONS: Biochemical study of CoQ(10) in both muscle and fibroblasts seems advisable to demonstrate the deficiency in all patients.
Description:4 páginas, 2 tablas.
Publisher version (URL):http://dx.doi.org/10.1016/j.clinbiochem.2008.03.007
Appears in Collections:(CABD) Artículos

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