Oxidative Damage and Erythrocyte Membrane Transport Abnormalities in Thalassemias

摘要: Oxidative damage induced by free globin chains has been implicated in the pathogenesis of membrane abnormalities observed a and B thalassemia. We have evaluated transport Na+ K+ erythrocytes patients with thalassemias as well two experimental models that use normal human red blood cells, one for thalassemia (methylhydrazine treatment, like) /3 thalacurmia (phenylhydrazine like). With exception Na-K pump, similar alterations were thalassemia-like erythrocytes. These were: increased KCl cotransport, Na-Li countertransport reduced Na-K-Cl HEREAS MOLECULAR DEFECTS responsible decreased chain synthesis described great detail, imposed presence or p is complex less characterized. Morphologic, biochemical, metabolic changes erythrocyte contribute to premature destruction thalassemic Thus, represents an important mechanism leading anemia thala~semias."~ Previous studies shown several lipids increase binding skeleton' decrease thiol^.^" are characterized different skeleton protein cell deformability features.6s7 Many these suggestive oxidative damage?.' One features distinguishes severe from dehydration? minor cells (RBCs) lose potassium (K+) after incubation serumg autolo