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Oxidative Stress in β-thalassaemia and Sickle Cell Disease

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Journal Redox Biol
Date 2015 Aug 19
PMID 26285072
Citations 69
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Abstract

Sickle cell disease and β-thalassaemia are inherited haemoglobinopathies resulting in structural and quantitative changes in the β-globin chain. These changes lead to instability of the generated haemoglobin or to globin chain imbalance, which in turn impact the oxidative environment both intracellularly and extracellularly. The ensuing oxidative stress and the inability of the body to adequately overcome it are, to a large extent, responsible for the pathophysiology of these diseases. This article provides an overview of the main players and control mechanisms involved in the establishment of oxidative stress in these haemoglobinopathies.

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References
1.
Hamer I, WATTIAUX R . Deleterious effects of xanthine oxidase on rat liver endothelial cells after ischemia/reperfusion. Biochim Biophys Acta. 1995; 1269(2):145-52. DOI: 10.1016/0167-4889(95)00111-5. View

2.
Ghaffari S . Oxidative stress in the regulation of normal and neoplastic hematopoiesis. Antioxid Redox Signal. 2008; 10(11):1923-40. PMC: 2932538. DOI: 10.1089/ars.2008.2142. View

3.
Shalev O, Repka T, Goldfarb A, Grinberg L, Abrahamov A, Olivieri N . Deferiprone (L1) chelates pathologic iron deposits from membranes of intact thalassemic and sickle red blood cells both in vitro and in vivo. Blood. 1995; 86(5):2008-13. View

4.
Schaer D, Buehler P, Alayash A, Belcher J, Vercellotti G . Hemolysis and free hemoglobin revisited: exploring hemoglobin and hemin scavengers as a novel class of therapeutic proteins. Blood. 2012; 121(8):1276-84. PMC: 3578950. DOI: 10.1182/blood-2012-11-451229. View

5.
Ramirez J, Schaad O, Durual S, Cossali D, Docquier M, Beris P . Growth differentiation factor 15 production is necessary for normal erythroid differentiation and is increased in refractory anaemia with ring-sideroblasts. Br J Haematol. 2008; 144(2):251-62. DOI: 10.1111/j.1365-2141.2008.07441.x. View