Specific protein and glycoprotein deficiencies in platelets isolated from two patients with the gray platelet syndrome.
Specific protein and glycoprotein deficiencies in platelets isolated from two patients with the gray platelet syndrome.
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DOI：
10.1182/blood.v59.4.709.bloodjournal594709
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发表时间：
1982-04
期刊：
影响因子：
20.3
通讯作者：
A. Nurden;T. Kunicki;D. Dupuis;C. Soria;J. Caen
中科院分区：
文献类型：
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作者：
A. Nurden;T. Kunicki;D. Dupuis;C. Soria;J. Caen
灰色血小板综合征是一种罕见的遗传性血小板疾病，以电子显微镜观察到的α - ha颗粒缺失为特征。用sds -聚丙烯酰胺凝胶电泳（SDS-PAGE）对2例患者的血小板糖蛋白组成进行分析，发现几种高分子量糖蛋白的碳水化合物染色减少或缺失。在乳酸过氧化物酶催化的碘化过程中，主要的周期性酸席夫（PAS）染色膜糖蛋白被正常检测到，并被125I正常标记。通过单或二维SDS-PAGE和考马斯蓝染色对灰色血小板的蛋白质组成进行分析，发现血小板反应蛋白（血小板反应蛋白）明显缺失，血小板纤维蛋白原和白蛋白浓度明显降低，2种低分子量多肽水平严重降低，与血小板因子4和β -血小板球蛋白在SDS-PAGE上的迁移速度相同。在经过凝血酶诱导的释放反应的正常人血小板中观察到与灰色血小板相似的SDS-PAGE图谱。使用兔抗人血小板抗体制备的交叉免疫电泳和使用单特异性抗血清的火箭免疫电泳分析灰色血小板蛋白证实了上述发现，并显示viir:Ag因子和冷不溶性球蛋白的严重缺陷。相反，通常检测到细胞质蛋白因子XIII（亚基A）。我们的研究提供了进一步的证据，证明循环灰色血小板特异性地缺乏或显著降低α颗粒蛋白的浓度。
The gray platelet syndrome is a rare inherited platelet disorder characterized by the absence of alp ha-granules as observed by electron microscopy. Analysis of the glycoprotein composition of the platelets of 2 such patients by SDS-polyacrylamide gel electrophoresis (SDS-PAGE) revealed decreased or absent staining for carbohydrate of several high molecular weight glycoproteins. The major periodic acid Schiff (PAS) staining membrane glycoproteins were normally detected and were normally labeled with 125I during lactoperoxidase-catalyzed iodination. Analysis of the protein composition of gray platelets by single or two-dimensional SDS-PAGE followed by Coomassie blue staining revealed an apparent absence of GP Ig (thrombospondin), markedly reduced platelet fibrinogen and albumin concentrations, and severely reduced levels of 2 low molecular weight polypeptides exhibiting identical rates of migration on SDS-PAGE as platelet factor 4 and beta-thromboglobulin. SDS-PAGE profiles similar to those of the gray platelets were observed with normal human platelets that had undergone the release reaction induced by thrombin. Analysis of gray platelet proteins by crossed immunoelectrophoresis using a rabbit anti-human platelet antibody preparation and rocket immunoelectrophoresis using monospecific antisera confirmed the above findings and showed additional severe deficiencies of factor VIIIR:Ag and cold-insoluble globulin. In contrast, factor XIII (subunit A), a cytoplasmic protein, was normally detected. Our studies provide further evidence that circulating gray platelets specifically lack, or have markedly decreased concentrations of, the alpha-granule proteins.