Gebrane-Younes J, Drouet L, Caen J P, Orcel L
Service central d'Anatomie et Cytologie pathologiques, Faculté de Médecine Saint-Antoine, Paris.
Biol Struct Morphog. 1988;1(2):58-62.
Originally described in vascular endothelial cells, Weibel-Palade bodies were considered as specific of this cellular type, as they have never been reported elsewhere. Weibel-Palade bodies serve as storage granules for von Willebrand factor which is stored in microtubular form. Besides endothelial cells von Willebrand factor is also synthetized by bone marrow megakaryocytes. Von Willebrand factor has been located in alpha-granules of megakaryocytes and blood platelets. We describe true Weibel-Palade bodies in pig megakaryocytes, and also alpha-granules which look like an evolutionary form of Weibel-Palade bodies. Von Willebrand Factor is most likely stored in microtubular form in these two types of structure. This is supported by the absence of microtubules in these granules in cells obtained from pigs homozygous for the von Willebrand disease (lacking totally this protein).
最初在血管内皮细胞中被描述的魏尔-帕拉德小体，曾被认为是这种细胞类型所特有的，因为从未在其他地方有过相关报道。魏尔-帕拉德小体作为血管性血友病因子的储存颗粒，以微管形式储存。除了内皮细胞外，血管性血友病因子也由骨髓巨核细胞合成。血管性血友病因子已定位在巨核细胞和血小板的α颗粒中。我们在猪巨核细胞中描述了真正的魏尔-帕拉德小体，以及看起来像是魏尔-帕拉德小体进化形式的α颗粒。血管性血友病因子很可能以微管形式储存在这两种结构中。这一点得到了支持，即来自血管性血友病纯合猪（完全缺乏这种蛋白质）的细胞中，这些颗粒中没有微管。
