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镰状细胞疾病中与基质相关的βs珠蛋白链的优先结合。

Preferential binding of beta s globin chains associated with stroma in sickle cell disorders.

作者信息

Bank A, Mears G, Weiss R, O'Donnell J V, Natta C

出版信息

J Clin Invest. 1974 Oct;54(4):805-9. doi: 10.1172/JCI107820.

Abstract

Sickle cell anemia (SS) is associated with abnormalities of the red cell membrane and decreased red cell deformability. The present study assesses globin chain binding to stroma in SS, sickle cell trait (AS), and nonsickling (AA) cells. The results indicate that there is preferential binding of newly synthesized beta(S) globin to red cell stroma in SS cells and preferential binding of beta(S) to stroma compared to beta(A) in AS cells. These studies show that beta(S) globin binding to stroma accompanies the membrane abnormalities in SS and AS patients.

摘要

镰状细胞贫血(SS)与红细胞膜异常及红细胞变形性降低有关。本研究评估了SS、镰状细胞性状(AS)和非镰状细胞(AA)细胞中珠蛋白链与基质的结合情况。结果表明,在SS细胞中,新合成的β(S)珠蛋白优先与红细胞基质结合;在AS细胞中,与β(A)相比,β(S)优先与基质结合。这些研究表明,在SS和AS患者中,β(S)珠蛋白与基质的结合伴随着膜异常。

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