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Characterization and comparison of the red blood cell membrane damage in
severe human alpha- and beta-thalassemia
R Advani, S Sorenson, E Shinar, W Lande, E Rachmilewitz and SL Schrier
Department of Hematology, Stanford University School of Medicine, CA.
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.
Volume 79,
Issue 4,
pp. 1058-1063,
02/15/1992
Copyright © 1992 by The American Society of Hematology
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