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Issue title: The Fifth Conference of the International CoQ10 Association, Kobe 2007 – 50th anniversary of CoQ10 discovery
Article type: Research Article
Authors: Haas, Dorothea | Niklowitz, Petra | Hoffmann, Georg F. | Andler, Werner | Menke, Thomas
Affiliations: Department of General Pediatrics, Division of Inborn Metabolic Diseases, University Hospital Heidelberg, Heidelberg, Germany | Vestische Kinderklinik Datteln, University Witten/Herdecke, Datteln, Germany
Note: [] Address for correspondence: Dr.~Dorothea Haas, University Hospital for Pediatric and Adolescent Medicine, Department of General Pediatrics, Division of Inborn Metabolic Diseases, Im Neuenheimer Feld 153, D-69120 Heidelberg, Germany. Tel.: +49 6221 5639330; Fax: +49 6221 565565; E-mail: [email protected]
Abstract: 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.
Keywords: Smith-Lemli-Opitz syndrome, cholesterol biosynthesis, Coenzyme Q[TeX:] _{10}, HMG-CoA reductase inhibitors
Journal: BioFactors, vol. 32, no. 1-4, pp. 191-197, 2008
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