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血管性血友病因子

von Willebrand factor.

作者信息

Ruggeri Z M, Ware J

机构信息

Roon Research Laboratory for Arteriosclerosis and Thrombosis, Department of Molecular and Experimental Medicine, Scripps Research Institute, La Jolla, California 92037.

出版信息

FASEB J. 1993 Feb 1;7(2):308-16. doi: 10.1096/fasebj.7.2.8440408.

Abstract

von Willebrand factor is a multimeric glycoprotein essential for the normal arrest of bleeding after tissue injury (hemostasis). The molecule is present in blood, both in plasma and inside platelets, as well as in endothelial cells and the subendothelial matrix of the vessel wall. Through multiple functional domains, von Willebrand factor mediates the attachment of platelets to exposed tissues, where discontinuity of the vascular endothelium occurs, and the subsequent platelet aggregation leading to the formation of platelet thrombi. The crucial role of von Willebrand factor in platelet function is particularly apparent when hemodynamic conditions create blood flow with high shear stress, as in capillaries under physiologic conditions or in stenosed and partially occluded arteries in disease states. The involvement of von Willebrand factor in the processes that lead to acute thrombosis has attracted considerable interest on the molecular and functional biology of the protein. Indeed, understanding the mechanisms and structural bases for von Willebrand factor function may result in new and effective approaches to anti-thrombotic intervention.

摘要

血管性血友病因子是一种多聚体糖蛋白,对于组织损伤后正常的止血过程至关重要。该分子存在于血液中,包括血浆和血小板内部,也存在于血管内皮细胞和血管壁的内皮下基质中。通过多个功能域,血管性血友病因子介导血小板与暴露组织的附着,此处血管内皮存在连续性中断,随后血小板聚集导致血小板血栓形成。当血流动力学条件产生高剪切应力的血流时,如生理条件下的毛细血管或疾病状态下狭窄和部分闭塞的动脉中,血管性血友病因子在血小板功能中的关键作用尤为明显。血管性血友病因子参与导致急性血栓形成的过程,这引起了对该蛋白分子和功能生物学的极大兴趣。事实上,了解血管性血友病因子功能的机制和结构基础可能会带来新的有效抗血栓干预方法。

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