Kelebihan Rantai Α pada Talasemia β

Abstract

Beta - thalassemia is an inherited human disorder which is characterized by a deficient production of hemoglobin βchains and an attendant accumulation of structurally normal α chains. Oxidative damage induced by free globinchains has been implicated in the pathogenesis of the membrane abnormalities observed in β thalassemia. Morphologic,biochemical and metabolic changes of the erythrocyte membrane contribute to the premature destruction of thalassemicerythrocytes. This destruction might be ameliorated by increasing the intracellular proteolysis of the excess α subunits.Invitro study shows degradation of globin α chains in thalassemic reticulocyte is did by proteasome.