Oxidative stress in β-thalassaemia and sickle cell disease

• Published in: Redox biology Vol. 6; pp. 226 - 239
• Main Authors: Voskou, S, Aslan, M, Fanis, P, Phylactides, M, Kleanthous, M
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier 01-12-2015
• Subjects: Anemia, Sickle Cell - blood; Anemia, Sickle Cell - pathology; Anion Exchange Protein 1, Erythrocyte - chemistry; Anion Exchange Protein 1, Erythrocyte - metabolism; beta-Globins - metabolism; beta-Thalassemia - blood; beta-Thalassemia - pathology; Erythrocytes - metabolism; Erythrocytes - pathology; Heme - chemistry; Heme - metabolism; Hemeproteins - chemistry; Hemeproteins - metabolism; Humans; Iron - metabolism; Iron Overload - blood; Iron Overload - pathology; Oxidative Stress; Phosphatidylserines - chemistry; Phosphatidylserines - metabolism; Protein Stability; Reactive Oxygen Species - metabolism; Reperfusion Injury - blood; Reperfusion Injury - pathology; Review; Thrombophilia - blood; Thrombophilia - pathology
• ISSN: 2213-2317; 2213-2317
• DOI: 10.1016/j.redox.2015.07.018

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.