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Erythrocyte membrane skeleton abnormalities in severe beta-thalassemia
E Shinar, O Shalev, EA Rachmilewitz and SL Schrier
The protein composition of ghosts, inside-out vesicles (IOV), and membrane
skeletons (MS) of erythrocytes (RBC) from splenectomized (spx) and
nonsplenectomized (non-spx) patients with beta-thalassemia major and
beta-thalassemia intermedia was determined. Ghosts from spx thalassemia
intermedia patients had a significant increase in their globin content
(which was mostly heme reactive) and contained extra polypeptides in the
protein 4.2 to 5 and 6-globin areas. The Triton- extracted MS from all of
the thalassemic patients showed two major abnormalities: they retained up
to twice the amount of protein 3 when compared with controls; they had a
significant increase in their globin content, the concentration of which
was independent of their protein 3 content. Analysis of the IOV revealed no
differences between those prepared from normal controls and those of the
patients. MS from spx thalassemia intermedia patients were grossly abnormal
when examined by scanning electron microscopy and they exhibited aggregates
of material that on transmission electron microscopy suggested the presence
of globin precipitates. We propose that, although the integral protein
composition, as reflected in the IOV, from severely affected beta-
thalassemics is intact, their MS assembly is deranged. The altered skeletal
structure of thalassemic RBC could result from attachment of denatured
globin to the skeleton components. These abnormalities may contribute to
the premature cell death seen in severe beta-thalassemia.
Volume 70,
Issue 1,
pp. 158-164,
07/01/1987
Copyright © 1987 by The American Society of Hematology
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