Erythrocyte Membrane Skeleton Abnormalities in Severe 1-Thalassemia

The protein composition of ghosts. inside-out vesicles (by). and membrane skeletons (MS) of erythrocytes (ABC) from splenectomized (spx) and nonsplenectomized (nonspx) patients with $-thalassemia major and $-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: (a) they retained up to twice the amount of protein 3 when compared with controls; (b) they had a significant increase in their globin content. the concentration of which was independent of their protein 3 content. Analysis of the lOV revealed no differences between those prepared from