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地中海贫血综合征患者的红细胞膜变形性

Erythrocyte membrane deformability in patients with thalassemia syndromes.

作者信息

Athanasiou G, Zoubos N, Missirlis Y

机构信息

Department of Mechanical Engineering, University of Patras, Greece.

出版信息

Nouv Rev Fr Hematol (1978). 1991;33(1):15-20.

PMID:1945820
Abstract

The purpose of this study was to measure the erythrocyte membrane's elasticity in patients with thalassemia syndromes. These syndromes have been well investigated regarding the problems associated with the hemoglobin solution. A few studies dealing with the whole cells' flexibility have also been published. However, there are extremely few studies regarding the elastic behaviour of the membrane of these cells which plays a significant role in the viability of these erythrocytes especially when they transverse the small capillaries of the spleen. The micropipette aspiration method was used for this purpose. Cells from patients with thalassemia syndromes were aspirated into micropipettes of internal diameter of 1.2-1.6 microns. The slope of the curve relating the length of the aspirated tongue to the negative pressure was measured to give the elastic shear modulus. This modulus was compared with the one of the normal cells. Inclusions (Heinz bodies) were produced artificially in normal cells and their elastic shear modulus was also measured. The results show that the elastic shear modulus measured in homozygous beta-thalassemia was 33% higher than the normal one. The elastic shear modulus in cells with artificial Heinz bodies was also higher than the normal one and its value increased with the number of the inclusion Heinz bodies. The elastic shear modulus in heterozygous beta-thalassemia was slightly higher than the normal one. The apparent stiffening of the membrane of the homozygous cells might be partially due to the "constraining effect" of the inclusions to the membrane's ability to deform freely.

摘要

本研究的目的是测量地中海贫血综合征患者红细胞膜的弹性。关于与血红蛋白溶液相关的问题,这些综合征已得到充分研究。也有一些关于全细胞柔韧性的研究发表。然而,关于这些细胞的膜的弹性行为的研究极少,而这种弹性行为在这些红细胞的存活中起着重要作用,尤其是当它们穿过脾脏的小毛细血管时。为此采用了微量移液器抽吸法。将地中海贫血综合征患者的细胞吸入内径为1.2 - 1.6微米的微量移液器中。测量抽吸舌长度与负压相关曲线的斜率,以得出弹性剪切模量。将该模量与正常细胞的模量进行比较。在正常细胞中人工产生包涵体(海因茨小体),并测量其弹性剪切模量。结果表明,纯合β地中海贫血中测得的弹性剪切模量比正常情况高33%。含有人工海因茨小体的细胞中的弹性剪切模量也高于正常情况,且其值随包涵体海因茨小体数量的增加而增加。杂合β地中海贫血中的弹性剪切模量略高于正常情况。纯合细胞的膜明显变硬可能部分归因于包涵体对膜自由变形能力的“限制作用”。

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Erythrocyte membrane deformability in patients with thalassemia syndromes.地中海贫血综合征患者的红细胞膜变形性
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The viscosity of red blood cell membranes in patients with beta-thalassaemia.
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Deformability of the erythrocyte membrane in patients with myelodysplastic syndromes.骨髓增生异常综合征患者红细胞膜的可变形性
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Alterations and pathology of thalassemic red cells: comparison between alpha- and beta-thalassemia.地中海贫血红细胞的改变与病理:α地中海贫血与β地中海贫血的比较
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