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  • CHARACTERIZATION AND COMPARISON OF THE RED-BLOOD-CELL MEMBRANE DAMAGE IN SEVERE HUMAN ALPHA-THALASSEMIA AND BETA-THALASSEMIA BLOOD Advani, R., Sorenson, S., Shinar, E., LANDE, W., Rachmilewitz, E., Schrier, S. L. 1992; 79 (4): 1058-1063

    Abstract:

    The aim of the present work was to understand the pathophysiology of the severe human thalassemias as represented by beta-thalassemia intermedia and hemoglobin (Hb) H (alpha-thalassemia) disease. We have previously shown that the material properties of the red blood cell (RBC) and its membrane differ in severe alpha- and beta-thalassemia, and we now show that this difference is probably caused by accumulation of alpha-globin chains at the cytoskeleton in beta-thalassemia, whereas beta-globin chains are associated with the cytoskeleton in alpha-thalassemia. In both alpha- and beta-thalassemia, some of these globin chains have become oxidized as evidenced by loss of the free thiols. Furthermore, there is similar evidence of oxidation of protein 4.1 in beta-thalassemia, whereas beta-spectrin appears to be subject to oxidation in alpha-thalassemia. These observations support the idea that the association of partly oxidized globin chains with the cytoskeleton results in oxidation of adjacent skeletal proteins. The abnormality of protein 4.1 in beta-thalassemia is consistent with a prior observation, and is also in accord with the known importance of protein 4.1 in maintenance of membrane stability, a property that is abnormal in beta-thalassemic membranes.

    View details for Web of Science ID A1992HD66000031

    View details for PubMedID 1737089

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