Coenzyme Q10 deficiency in patients with hereditary hemochromatosis |
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| Authors: | Manuela R. Martinefski María F. Yamasato María B. Di Carlo Jorge R. Daruich Valeria P. Tripodi |
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| Affiliation: | 1. Universidad de Buenos Aires, Facultad de Farmacia y Bioquímica, Departamento de Tecnología Farmacéutica, Buenos Aires, Argentina;2. Universidad de Buenos Aires, Facultad de Medicina, División de Gastroenterología, Sección Hepatología, Hospital de Clínicas José de San Martin, Buenos Aires, Argentina;3. Universidad de Buenos Aires, Facultad de Farmacia y Bioquímica, Departamento de Bioquímica Clínica, Hospital de Clínicas José de San Martin, Buenos Aires, Argentina;4. Consejo Nacional de Investigaciones Científicas y Técnicas, CONICET, Argentina;1. Chemistry Department, Faculty of Science, Menoufia University, Egypt;2. Tropical Medicine Department, Faculty of Medicine, Menoufia University, Egypt;3. Internal Medicine Department, Faculty of Medicine, Menoufia University, Egypt;4. Chemistry Department, College of Education for Pure Science, Ibn Al-Haitham, University of Baghdad, Iraq;5. Pathological Analysis Department, College of Science, Basrah University, Iraq;6. Medical Biochemistry and Molecular Biology Department, Faculty of Medicine, Menoufia University, Egypt;1. Le Bonheur Children’s Hospital, USA;2. Department of Pediatrics, Division of Gastroenterology, University of Tennessee Health Sciences Center, USA;3. Department of Pediatrics, Division of Infectious Diseases, USA;4. Department of Pathology, University of Tennessee Health Sciences Center. Memphis, TN, USA;5. University of Louisville, School of Medicine, USA;1. Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China;2. Department of Neurology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guang Dong, China;3. Department of Neurology, The First People’s Hospital of Zhongshan City, Zhongshan, Guang Dong, China;1. Department of Gastroenterology, Army Specialty Medical Center, Army Medical University, No. 10 Changjiangzhi Road, Chongqing 400042, PR China;2. Department of Gastroenterology, The Chinese People''s Liberation Army 962 Hospital, Harbin, PR China;1. Department of Digestive Diseases, Edouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France;2. University Claude Bernard Lyon 1, Lyon, France;3. Ramsay Générale de Santé, Clinique de la Sauvegarde, Lyon, France;4. Department of Pediatric Hepatogastroenterology and Nutrition, and Centre National de Référence de l’Atrésie des Voies Biliaires et des Cholestases Génétiques, Femme-Mère-Enfant Hospital, Hospices Civils de Lyon, Lyon, France;5. Department of Anesthesiology, Femme-Mère-Enfant Hospital, Hospices Civils de Lyon, Lyon, France |
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| Abstract: | ![]()
AimHereditary hemochromatosis (HH) is a group of inherited disorders that causes a slow and progressive iron deposition in diverse organs, particularly in the liver. Iron overload induces oxidative stress and tissue damage. Coenzyme Q10 (CoQ10) is a cofactor in the electron-transport chain of the mitochondria, but it is also a potent endogenous antioxidant. CoQ10 interest has recently grown since various studies show that CoQ10 supplementation may provide protective and safe benefits in mitochondrial diseases and oxidative stress disorders. In the present study we sought to determine CoQ10 plasma level in patients recently diagnosed with HH and to correlate it with biochemical, genetic, and histological features of the disease. Methods: Plasma levels of CoQ10, iron, ferritin, transferrin and vitamins (A, C and E), liver tests (transaminases, alkaline phosphatase and bilirubin), and histology, as well as three HFE gene mutations (H63D, S654C and C282Y), were assessed in thirty-eight patients (32 males, 6 females) newly diagnosed with HH without treatment and in twenty-five age-matched normolipidemic healthy subjects with no HFE gene mutations (22 males, 3 females) and without clinical or biochemical signs of iron overload or liver diseases.ResultsPatients with HH showed a significant decrease in CoQ10 levels respect to control subjects (0.31 ± 0.03 µM vs 0.70 ± 0.06 µM, p < 0.001, respectively) independently of the genetic mutation, cirrhosis, transferrin saturation, ferritin level or markers of hepatic dysfunction. Although a decreasing trend in CoQ10 levels was observed in patients with elevated iron levels, no correlation was found between both parameters in patients with HH. Vitamins C and A levels showed no changes in HH patients. Vitamin E was significantly decreased in HH patients (21.1 ± 1.3 µM vs 29.9 ± 2.5 µM, p < 0.001, respectively), but no correlation was observed with CoQ10 levels. Conclusion: The decrease in CoQ10 levels found in HH patients suggests that CoQ10 supplementation could be a safe intervention strategy complementary to the traditional therapy to ameliorate oxidative stress and further tissue damage induced by iron overload. |
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| Keywords: | Coenzyme Q10 Oxidative stress Ubiquinone Hemochromatosis Iron overload, supplementation |
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