Hassan Md Imtaiyaz, Saxena Aditya, Ahmad Faizan
Centre for Interdisciplinary Research in Basic Sciences, Jamia Millia Islamia, Jamia Nagar, New Delhi 110025, India.
Blood Coagul Fibrinolysis. 2012 Jan;23(1):11-22. doi: 10.1097/MBC.0b013e32834cb35d.
von Willebrand factor (VWF) is a long plasma protein that contains many domains and each domain has its own function. VWF exists in a multimeric form and performs varieties of functions in the human body, including thrombus formation and blood coagulation. The crystal structures of three subdomains are known, and, interestingly, all three domains share identical three-dimensional fold with α-β-α sandwiched model. VWF is directly associated with different types of von Willebrand disease. In this review, our aim is to gather recent developments on structure and functions of VWF and its clinical relevance.
血管性血友病因子（VWF）是一种长链血浆蛋白，包含多个结构域，每个结构域都有其自身的功能。VWF以多聚体形式存在，在人体中发挥多种功能，包括血栓形成和血液凝固。已知三个亚结构域的晶体结构，有趣的是，所有这三个结构域都具有相同的三维折叠结构，即α-β-α夹心模型。VWF与不同类型的血管性血友病直接相关。在本综述中，我们的目的是收集有关VWF结构与功能及其临床相关性的最新进展。
