Physical, chemical and functional changes following platelet activation in normal and "giant" platelets.
Physical, chemical and functional changes following platelet activation in normal and "giant" platelets.
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正常和“巨型”血小板中血小板活化后的物理、化学和功能变化。
DOI：
10.1055/s-0038-1652658
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
J. Milton
中科院分区：
文献类型：
--
作者：
M. Frojmovic;J. Milton
健康人供体中未活化的椎间盘细胞的平均体积约为6.0微米3（范围3.8-7.5微米3），而从患有遗传性“巨大”血小板综合征的供体中获得的形状相似的椎间盘细胞的平均值要么正常（一个Bernard-Soulier综合征（BSS）和一个患有蒙特利尔血小板综合征（MPS）的家庭的所有五个成员，或者平均而言，高达正常值的两倍（范围6.4-13.8微米3）。这种明显的异质性由于更一致和显著的观察结果而变得复杂，即BSS和MPS血小板在活化后都经历了无限期延长的有缺陷的高容量形状变化，与ADP加入正常血小板后1-5秒内可测量的短暂高容量变化相反。这表明，在正常和“巨型”血小板的高容量的形状变化是伴随着外部化质膜表面积的增加，最可能的来源是表面连接的小管系统。在2/3的同胞MPS供体的血小板中未检测到膜糖蛋白I异常。这些膜的变化，改变血小板功能的精确关系进行了比较，为正常和“巨”血小板，但在很大程度上仍有待实验确定。早期形状变化似乎与早期显微镜测量的聚集（PA）密切相关，PA和浊度测量的大聚集通常表现为正常至“巨”血小板升高。
Unactivated discocytes in healthy human donors have mean volumes of approximately 6.0 microns3 (range 3.8-7.5 microns3), while mean values for similarly-shaped discocytes obtained from donors with the hereditary "giant" platelet syndromes were either normal (one Bernard-Soulier syndrome (BSS) and all five members of a family with the Montreal platelet syndrome (MPS) or, on average, up to twice normal (range 6.4-13.8 microns3). This apparent heterogeneity is complicated by the much more consistent and significant observation that both BSS and MPS platelets undergo a defective hypervolumetric shape change following activation which is prolonged indefinitely, in contrast to a transient hypervolumetric change measureable in 1-5 s following ADP addition to normal platelets. It is suggested that the hypervolumetric shape change in both normal and "giant" platelets is accompanied by an increase in externalized plasma membrane surface area, with the most probable source being surface-connected canalicular system. Membrane glycoprotein I abnormalities were not detectable in platelets for 2/3 sibling MPS donors. The precise relation of these membrane changes to altered platelet functions is compared for normal and "giant" platelets, but largely remains to be experimentally determined. Early shape change appears tightly associated with early microscopically-measured aggregation (PA), with both PA and turbidimetrically-measured macroaggregation generally appearing normal to elevated for "giant" platelets.