Sporn L A, Marder V J, Wagner D D
Cell. 1986 Jul 18;46(2):185-90. doi: 10.1016/0092-8674(86)90735-x.
von Willebrand factor (vWf) secreted constitutively by human endothelial cells was compared to that released from Weibel-Palade bodies after stimulation. The majority of constitutively secreted molecules were dimeric and contained both pro-vWf and mature subunits. In contrast, the vWf released by the calcium ionophore A23187 or thrombin consisted of only very large multimers of mature subunits. The large multimers are known to be more active in in vitro platelet binding assays, and their absence in vivo results in a bleeding disorder. Endothelial cells therefore concentrate a special subclass of very large and biologically potent vWf multimers in Weibel-Palade bodies, presumably available for release in response to vascular injury.
将人内皮细胞组成性分泌的血管性血友病因子（vWf）与刺激后从魏尔-帕拉德小体释放的vWf进行了比较。组成性分泌的大多数分子是二聚体，同时包含前vWf和成熟亚基。相比之下，钙离子载体A23187或凝血酶释放的vWf仅由成熟亚基的非常大的多聚体组成。已知这些大多聚体在体外血小板结合试验中更具活性，而它们在体内的缺失会导致出血性疾病。因此，内皮细胞将一类特殊的非常大且具有生物活性的vWf多聚体集中在魏尔-帕拉德小体中，推测可在血管损伤时释放。
