Affiliations: Department of Biochemistry, Faculty of Medicine,
Siriraj Hospital, Mahidol University, Bangkok, Thailand | Department of Medicine, Faculty of Medicine, Siriraj
Hospital, Mahidol University, Bangkok, Thailand | Thalassemia Research Center, Institute of Science and
Technology for Research and Development, Mahidol University, Bangkok,
Thailand
Note: [] Address for correspondence: Ruchaneekorn W. Kalpravidh,
Department of Biochemistry, Faculty of Medicine, Siriraj Hospital, Mahidol
University, Bangkok 10700, Thailand. Tel.: +66 2419 7581; Fax: +66 2411 1428;
E-mail: sirkp@mahidol.ac.th
Abstract: Thalassemia is a group of genetic disorders resulting from different
mutations in the globin gene complex and leading to an imbalance in globin
synthesis. Unmatched globin chains are less stable and susceptible to
oxidation. Patients with β-thalassemia/HbE are prone to increased
oxidative stress as indicated by increased lipid peroxidation product,
malondialdehyde (MDA), partly because of the presence of iron in the form of
heme and hemichromes released from excess globin chains and excess iron
deposition in various tissues. The level of antioxidant such as glutathione is
markedly decreased while activities of antioxidant enzymes including superoxide
dismutase (SOD), catalase, and glutathione peroxidase (GSH-Px) are increased.
We have recently found that the levels of coenzyme Q_{10}
(CoQ_{10}) are also very low in thalassemia. We therefore
evaluated the oxidative stress and the antioxidants in these patients before
and after supplementation with 100 mg CoQ_{10} daily for 6
months. The results showed that the plasma level of CoQ_{10}
significantly increased and the oxidative stress decreased as the level of MDA
declined. The administration of CoQ_{10} led to significant
improvement of biochemical parameters of antioxidant enzymes. The antioxidant
supplementation will be beneficial for thalassemia patients as adjunct therapy
to increase their quality of life.
Keywords: β-thalassemia/Hb E, oxidative stress, antioxidant, coenzyme Q[TeX:] _{10}
Journal: BioFactors, vol. 25, no. 1-4, pp. 225-234, 2005
