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

AuthorsMontero, 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
KeywordsBiological markers
Electron Transport
Coenzyme Q10
Issue Date20-Mar-2008
Canadian Society of Clinical Chemists
CitationClinical Biochemistry - New York 41(9): 697-700 (2008)
AbstractOBJECTIVES: 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.
Description4 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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