Regulation of plasma von Willebrand factor.
Regulation of plasma von Willebrand factor.
复制标题
血浆von Willebrand因子的调节。
DOI：
10.12688/f1000research.13056.1
复制
发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Desch KC
中科院分区：
文献类型：
--
作者：
Desch KC
血管性血友病因子（VWF）是一种多聚体血浆糖蛋白，在血液凝固过程中起核心作用。通过其特定功能域、血管壁、凝血因子VIII和血小板受体之间的相互作用，VWF通过与血小板结合并将因子VIII传递到血管损伤部位来维持止血。在健康人群中，血浆VWF水平变化很大。VWF的重要作用体现在血浆VWF浓度正态分布的极端情况下，VWF水平低的个体更有可能出现皮肤粘膜出血。相反，VWF水平高的人患静脉血栓栓塞性疾病、中风和冠状动脉疾病的风险更高。本报告将总结我们对健康和有症状人群中VWF血浆变异的环境影响和遗传控制的理解的最新进展，并将强调目前推动这一研究领域的未解问题。
Von Willebrand factor (VWF) is a multimeric plasma glycoprotein that plays a central role in the initiation of blood coagulation. Through interactions between its specific functional domains, the vascular wall, coagulation factor VIII, and platelet receptors, VWF maintains hemostasis by binding to platelets and delivering factor VIII to the sites of vascular injury. In the healthy human population, plasma VWF levels vary widely. The important role of VWF is illustrated by individuals at the extremes of the normal distribution of plasma VWF concentrations where individuals with low VWF levels are more likely to present with mucocutaneous bleeding. Conversely, people with high VWF levels are at higher risk for venous thromboembolic disease, stroke, and coronary artery disease. This report will summarize recent advances in our understanding of environmental influences and the genetic control of VWF plasma variation in healthy and symptomatic populations and will also highlight the unanswered questions that are currently driving this field of study.