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正常与异常人类红细胞的血影蛋白膜骨架:综述

The spectrin membrane skeleton of normal and abnormal human erythrocytes: a review.

作者信息

Goodman S R, Shiffer K

出版信息

Am J Physiol. 1983 Mar;244(3):C121-41. doi: 10.1152/ajpcell.1983.244.3.C121.

Abstract

The erythrocyte membrane skeleton composed of spectrin, actin, and several other proteins is essential for the maintenance of the erythrocyte shape, reversible deformability, and membrane structural integrity in addition to controlling the lateral mobility of integral membrane proteins. In this review, we shall give an historical development of the current model of the erythrocyte membrane skeleton. We will then describe how the experimental technology developed to study the normal membrane skeleton has paved the way for the recent identification of alterations of skeletal protein interactions in hereditary spherocytosis, hereditary elliptocytosis, and hereditary pyropoikilocytosis. We will conclude with a discussion of some of the more exciting and promising directions for future research that are currently being initiated in this vanguard field of cell biology.

摘要

由血影蛋白、肌动蛋白和其他几种蛋白质组成的红细胞膜骨架,除了控制整合膜蛋白的侧向流动性外,对于维持红细胞的形状、可逆变形性和膜结构完整性至关重要。在这篇综述中,我们将阐述当前红细胞膜骨架模型的历史发展。然后,我们将描述为研究正常膜骨架而开发的实验技术如何为最近鉴定遗传性球形红细胞增多症、遗传性椭圆形红细胞增多症和遗传性热异形红细胞增多症中骨架蛋白相互作用的改变铺平了道路。我们将以讨论细胞生物学这一前沿领域目前正在开展的一些更令人兴奋和有前景的未来研究方向作为总结。

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