Oxidative stress in β-thalassaemia and sickle cell disease.
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.
The Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus.
S
S
Voskou
Akdeniz University, Faculty of Medicine, Department of Medical Biochemistry, Antalya, Turkey.
M
M
Aslan
The Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus.
P
P
Fanis
The Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus. Electronic address: mphylact@cing.ac.cy.
M
M
Phylactides
The Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus.
M
M
Kleanthous
D000755:Anemia, Sickle Cell / Q000097:blood* / Q000473:pathology; D001457:Anion Exchange Protein 1, Erythrocyte / Q000737:chemistry / Q000378:metabolism; D004912:Erythrocytes / Q000378:metabolism / Q000473:pathology; D006418:Heme / Q000737:chemistry / Q000378:metabolism; D006420:Hemeproteins / Q000737:chemistry / Q000378:metabolism; D006801:Humans; D007501:Iron / Q000378:metabolism; D019190:Iron Overload / Q000097:blood* / Q000473:pathology; D018384:Oxidative Stress; D010718:Phosphatidylserines / Q000737:chemistry / Q000378:metabolism; D055550:Protein Stability; D017382:Reactive Oxygen Species / Q000378:metabolism; D015427:Reperfusion Injury / Q000097:blood* / Q000473:pathology; D019851:Thrombophilia / Q000097:blood* / Q000473:pathology; D055544:beta-Globins / Q000378:metabolism*; D017086:beta-Thalassemia / Q000097:blood* / Q000473:pathology
D001457:Anion Exchange Protein 1, Erythrocyte; D006420:Hemeproteins; D010718:Phosphatidylserines; D017382:Reactive Oxygen Species; C454201:SLC4A1 protein, human; D055544:beta-Globins; C001332:hemichrome; D006418:Heme; D007501:Iron