Characterization and comparison of the red blood cell membrane damage in severe human alpha- and beta-thalassemia
作者： R Advani , S Sorenson , E Shinar , W Lande , E Rachmilewitz
DOI: 10.1182/BLOOD.V79.4.1058.1058
关键词:
摘要: The aim of the present work was to understand pathophysiology severe human thalassemias as represented by beta-thalassemia intermedia and hemoglobin (Hb) H (alpha-thalassemia) disease. We have previously shown that material properties red blood cell (RBC) its membrane differ in alpha- beta-thalassemia, we now show this difference is probably caused accumulation alpha-globin chains at cytoskeleton whereas beta-globin are associated with thalassemia. In both some these globin become oxidized evidenced loss free thiols. Furthermore, there similar evidence oxidation protein 4.1 beta-spectrin appears be subject alpha-thalassemia. These observations support idea association partly results adjacent skeletal proteins. abnormality consistent a prior observation, also accord known importance maintenance stability, property abnormal beta-thalassemic membranes.
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