Giltay J C, Leeksma O C, Breederveld C, van Mourik J A
Blood. 1987 Mar;69(3):809-12.
Glanzmann's thrombasthenia is a bleeding disorder, inherited in an autosomal recessive way and characterized by an absence or deficiency of the platelet glycoprotein (GP) IIb/IIIa complex. Recently, we and others demonstrated that cultured human umbilical vein endothelial cells synthesized a membrane protein complex similar to the platelet GP IIb/IIIa complex. In this article, we demonstrate that endothelial cells isolated from the umbilical vein of a newborn with Glanzmann's thrombasthenia, as compared with normal endothelial cells, show no difference in their ability to synthesize and express this GP IIb/IIIa complex. Our results indicate that Glanzmann's thrombasthenia is not accompanied by an "endotheliopathy."
血小板无力症是一种遗传性出血性疾病，以常染色体隐性方式遗传，其特征是血小板糖蛋白（GP）IIb/IIIa复合物缺失或缺乏。最近，我们和其他人证明，培养的人脐静脉内皮细胞合成了一种类似于血小板GP IIb/IIIa复合物的膜蛋白复合物。在本文中，我们证明，与正常内皮细胞相比，从患有血小板无力症的新生儿脐静脉分离出的内皮细胞在合成和表达这种GP IIb/IIIa复合物的能力上没有差异。我们的结果表明，血小板无力症不伴有“内皮病变”。
