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

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

To study CoQ(10) concentrations in muscle and fibroblast from 6 patients with a CoQ(10) deficiency syndrome. CoQ(10) was quantified by HPLC with electrochemical detection. Four out of the 6 cases showed muscle CoQ(10) deficiency plus a reduction of mitochondrial respiratory chain enzyme activities....

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Veröffentlicht in:Clinical biochemistry 2008-06, Vol.41 (9), p.697-700
Hauptverfasser: Montero, Raquel, Sánchez-Alcázar, José Antonio, Briones, Paz, Hernández, Angeles Rodríguez, Cordero, Mario D, Trevisson, Eva, Salviati, Leonardo, Pineda, Mercé, García-Cazorla, Angels, Navas, Plácido, Artuch, Rafael
Format: Artikel
Sprache:eng
Schlagworte:
Adolescent
Adult
Biomarkers - metabolism
Child
Child, Preschool
Electron Transport - physiology
Fibroblasts - enzymology
Humans
Infant
Infant, Newborn
Middle Aged
Mitochondria, Muscle - enzymology
Mitochondria, Muscle - genetics
Muscle, Skeletal - enzymology
Syndrome
Ubiquinone - analogs & derivatives
Ubiquinone - analysis
Ubiquinone - biosynthesis
Ubiquinone - deficiency
Ubiquinone - genetics
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Zusammenfassung:To study CoQ(10) concentrations in muscle and fibroblast from 6 patients with a CoQ(10) deficiency syndrome. CoQ(10) was quantified by HPLC with electrochemical detection. 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. Biochemical study of CoQ(10) in both muscle and fibroblasts seems advisable to demonstrate the deficiency in all patients.
ISSN:0009-9120
1873-2933
DOI:10.1016/j.clinbiochem.2008.03.007