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血管性血友病因子在血小板α颗粒内部结构中的偏心定位,该结构类似于魏尔-帕拉德小体。

Eccentric localization of von Willebrand factor in an internal structure of platelet alpha-granule resembling that of Weibel-Palade bodies.

作者信息

Cramer E M, Meyer D, le Menn R, Breton-Gorius J

出版信息

Blood. 1985 Sep;66(3):710-3.

PMID:3875375
Abstract

Immunogold staining was used to study the ultrastructural distribution of von Willebrand factor (vWF) in unstimulated platelets. vWF was detected in the alpha-granules with a specific eccentric distribution pattern opposite the nucleoids. Similar findings were obtained with a polyclonal antibody or a pool of monoclonal antibodies to human vWF. This labeling coincided with the presence of tubular structures located at the periphery of the alpha-granules. These structures were better visualized on platelets treated for standard electron microscopy: they formed a group of one to four tubules ranging from 200 A to 250 A in diameter. They closely resembled the internal tubular structures found in Weibel-Palade bodies, which are the storage organelles of vWF in endothelial cells.

摘要

免疫金染色法用于研究未受刺激血小板中血管性血友病因子(vWF)的超微结构分布。在α颗粒中检测到vWF,其具有与拟核相对的特定偏心分布模式。使用针对人vWF的多克隆抗体或单克隆抗体池也获得了类似的结果。这种标记与位于α颗粒周边的管状结构的存在一致。在经过标准电子显微镜处理的血小板上,这些结构更清晰可见:它们形成一组一到四个直径在200埃到250埃之间的小管。它们与在魏尔-帕拉德小体中发现的内部管状结构非常相似,而魏尔-帕拉德小体是内皮细胞中vWF的储存细胞器。

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