Weibel-Palade bodies in pig megakaryocytes.

J Gebrane-Younes, L Drouet, J P Caen, L Orcel
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Abstract

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).

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猪巨核细胞的韦贝尔-帕拉德小体。
最初在血管内皮细胞中被描述,Weibel-Palade小体被认为是这种细胞类型的特异性,因为它们从未在其他地方报道过。韦贝尔-帕拉德体作为血管性血友病因子的储存颗粒,以微管形式储存。除内皮细胞外,血管性血友病因子也可由骨髓巨核细胞合成。血管性血友病因子位于巨核细胞和血小板的α颗粒中。我们描述了猪巨核细胞中真正的Weibel-Palade小体,以及看起来像Weibel-Palade小体进化形式的α -颗粒。在这两种结构中,血管性血友病因子最有可能以微管形式储存。从血管性血友病纯合子猪细胞中获得的这些颗粒中缺乏微管(完全缺乏这种蛋白质)支持了这一点。
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