Oxidative stress and β-thalassemic erythroid cells behind the molecular defect.

摘要: β-thalassemia is a worldwide distributed monogenic red cell disorder, characterized by the absence or reduced β-globin chain synthesis. Despite extensive knowledge of molecular defects causing β-thalassemia, less known about mechanisms responsible for associated ineffective erythropoiesis and survival, which sustain anemia β-thalassemia. The unbalance alpha-gamma presence pathological free iron promote severe membrane oxidative stress, results in abnormal β-thalassemic features. These cells are precociously removed macrophage system through two mechanisms: removal phosphatidylserine positive natural occurring antibody produced against abnormally clustered protein band 3. In present review we will discuss changes homeostasis related to stress its connection with production microparticles malaria infection. reactive oxygen species (ROS) also involved still partially pathways. Novel cytoprotective systems such as ASHP, eIF2α, peroxiredoxin-2 have been suggested be important ROS erythropoiesis. Finally, major vitro vivo studies antioxidants