Plasma and thrombocyte levels of coenzyme Q10in children with Smith-Lemli-Opitz syndrome (SLOS) and the influence of HMG-CoA reductase inhibitors
In: BioFactors, Jg. 32 (2008), S. 191-197
Online
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Zugriff:
Introduction: SLOS is caused by a defect of cholesterol synthesis. HMG-CoA reductase inhibitors have been shown to improve biochemical parameters in this condition, but they have also been associated with CoQ 10 deficiency in patients with hypercholesterolemia. The aim of this study was to analyse plasma and intracellular CoQ 10 levels in SLOS patients and to determine the influence of HMG-CoA reductase inhibitors. Methods: Plasma concentrations of CoQ 10 and vitamin E were measured in 14 patients, intracellular CoQ 10 levels were determined in platelets of 10 patients with SLOS and compared to controls. Results: Plasma CoQ 10 and vitamin E levels were significantly lower in SLOS patients. This difference equalised after adjustment to cholesterol concentrations. Treatment with simvastatin did not influence CoQ 10 levels and redox status. Platelet CoQ 10 concentrations were similar between patients and controls but there were striking differences in the CoQ 10 redox status with a decrease of oxidised CoQ 10 . Conclusion: Decreased concentrations of plasma CoQ 10 and vitamin E in SLOS patients are due to a diminished carrier capacity. The higher percentage of reduced CoQ 10 in platelets points to an up-regulation of mitochondrial protection mechanisms. Further studies are needed to evaluate a possible benefit of CoQ 10 supplementation in SLOS patients.
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Plasma and thrombocyte levels of coenzyme Q10in children with Smith-Lemli-Opitz syndrome (SLOS) and the influence of HMG-CoA reductase inhibitors
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Autor/in / Beteiligte Person: | Niklowitz, Petra ; Hoffmann, Georg F. ; Andler, Werner ; Haas, Dorothea ; Menke, Thomas |
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Zeitschrift: | BioFactors, Jg. 32 (2008), S. 191-197 |
Veröffentlichung: | Wiley, 2008 |
Medientyp: | unknown |
ISSN: | 1872-8081 (print) ; 0951-6433 (print) |
DOI: | 10.1002/biof.5520320123 |
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