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对正常人、血小板无力症患者和伯-苏二氏血小板的质膜进行的冷冻断裂研究。

Freeze-fracture studies on the plasma membranes of normal human, thrombasthenic, and Bernard-Soulier platelets.

作者信息

Chevalier J, Nurden A T, Thiery J M, Savariau E, Caen J P

出版信息

J Lab Clin Med. 1979 Aug;94(2):232-45.

PMID:572392
Abstract

The description of severe molecular deficiencies of different membrane glycoproteins in thrombasthenic and Bernard-Soulier platelets has led us to investigate the intramembrane organization of their plasma membranes using freeze-fracture electron microscopy. An initial examination of the cleaved plasma membranes of freeze-fractured normal human platelets revealed randomly distributed MAPs on the fracture faces of both the outer and inner phospholipid leaflets of the bilayer. Particle densities of 925 +/- 52/micrometer22 on the EF and 427 +/- 29/micrometer2 on the PF were calculated with a computer-linked picture analyzer. The particle size was heterogeneous on both fracture faces, and the number of particles decreased exponentially in the 5 to 13 nm size range. Examination of the platelets of three thrombasthenic patients revealed a low particle density (36% to 69% of the normal range) on the PF of the cleaved plasma membrane and a reduced particle coefficient between the two fracture faces (PF/EF). This abnormality was accompanied by a preferential decrease in the larger sized particles. In marked contrast 8 to 13 nm particles predominated on both fracture faces of the plasma membranes of the platelets of a Bernard-Soulier patient, and a greater concentration of particles on the PF rather than the EF was uniquely observed. The results further define the modified structure of thrombasthenic and Bernard-Soulier platelet plasma membranes and suggest a structural heterogeneity within the total MAP population of the membranes of normal human platelets.

摘要

对血小板无力症和伯纳德 - 索利尔血小板中不同膜糖蛋白严重分子缺陷的描述,促使我们使用冷冻断裂电子显微镜研究其质膜的膜内组织结构。对冷冻断裂的正常人血小板的裂解质膜进行初步检查发现,双层膜外层和内层磷脂小叶的断裂面上存在随机分布的膜相关蛋白(MAPs)。使用计算机连接的图像分析仪计算得出,EF面上的颗粒密度为925±52/μm²,PF面上的颗粒密度为427±29/μm²。两个断裂面上的颗粒大小均不均一,且在5至13nm尺寸范围内颗粒数量呈指数下降。对三名血小板无力症患者的血小板进行检查发现,裂解质膜的PF面上颗粒密度较低(为正常范围的36%至69%),且两个断裂面之间的颗粒系数(PF/EF)降低。这种异常伴随着较大尺寸颗粒的优先减少。与之形成鲜明对比的是,一名伯纳德 - 索利尔患者血小板质膜的两个断裂面上8至13nm的颗粒占主导地位,并且独特地观察到PF面上的颗粒浓度高于EF面。这些结果进一步明确了血小板无力症和伯纳德 - 索利尔血小板质膜的改变结构,并表明正常人血小板膜中总MAP群体内存在结构异质性。

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