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血管性血友病因子的生理功能取决于其在内皮细胞韦贝尔-帕拉德小体中的管状储存。

The physiological function of von Willebrand's factor depends on its tubular storage in endothelial Weibel-Palade bodies.

作者信息

Michaux Grégoire, Abbitt Kate B, Collinson Lucy M, Haberichter Sandra L, Norman Keith E, Cutler Daniel F

机构信息

Department of Biochemistry, University College London, Gower Street, London WC1E 6BT, United Kingdom.

出版信息

Dev Cell. 2006 Feb;10(2):223-32. doi: 10.1016/j.devcel.2005.12.012.

Abstract

Weibel-Palade bodies are the 1-5 microm long rod-shaped storage organelles of endothelial cells. We have investigated the determinants and functional significance of this shape. We find that the folding of the hemostatic protein von Willebrand's factor (VWF) into tubules underpins the rod-like shape of Weibel-Palade bodies. Further, while the propeptide and the N-terminal domains of mature VWF are sufficient to form tubules, their maintenance relies on a pH-dependent interaction between the two. We show that the tubular conformation of VWF is essential for a rapid unfurling of 100 microm long, platelet-catching VWF filaments when exposed to neutral pH after exocytosis in cell culture and in living blood vessels. If tubules are disassembled prior to exocytosis, then short or tangled filaments are released and platelet recruitment is reduced. Thus, a 100-fold compaction of VWF into tubules determines the unique shape of Weibel-Palade bodies and is critical to this protein's hemostatic function.

摘要

魏尔-帕拉德小体是内皮细胞中长度为1 - 5微米的杆状储存细胞器。我们研究了这种形状的决定因素及其功能意义。我们发现止血蛋白血管性血友病因子(VWF)折叠成小管是魏尔-帕拉德小体呈杆状的基础。此外,虽然成熟VWF的前肽和N端结构域足以形成小管,但它们的维持依赖于两者之间pH依赖性的相互作用。我们表明,VWF的管状构象对于在细胞培养和活体血管中胞吐后暴露于中性pH值时快速展开100微米长的、捕捉血小板的VWF细丝至关重要。如果在胞吐之前小管被拆解,那么就会释放出短的或缠结的细丝,血小板募集也会减少。因此,VWF压缩100倍形成小管决定了魏尔-帕拉德小体的独特形状,并且对该蛋白的止血功能至关重要。

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