血管性血友病因子
因子(编程语言)
医学
计算机科学
血小板
内科学
程序设计语言
作者
Klytaimnistra Kiouptsi,Christoph Reinhardt
出处
期刊:Sub-cellular biochemistry
日期:2020-01-01
卷期号:: 437-464
被引量:19
标识
DOI:10.1007/978-3-030-41769-7_18
摘要
Von Willebrand factorVon Willebrand Factor (VWF) (VWF) and coagulationCoagulation factorCoagulation factor VIII (FVIII) VIII (FVIII) circulate as a complex in plasma and have a major role in the hemostatic system. VWFVon Willebrand Factor (VWF) has a dual role in hemostasisHemostasis. It promotes plateletPlatelet adhesion by anchoring the plateletsPlatelet to the subendothelial matrix of damaged vessels and it protects FVIII from proteolytic degradation. Moreover, VWFVon Willebrand Factor (VWF) is an acute phaseProteins proteinAcute phase protein that has multiple roles in vascular inflammationInflammation and is massively secreted from Weibel-Palade bodies upon endothelial cell activation. Activated FVIII on the other hand, together with coagulationCoagulation factor IX forms the tenase complex, an essential feature of the propagation phase of coagulationCoagulation on the surface of activated plateletsPlatelet. VWFVon Willebrand Factor (VWF) deficiency, either quantitative or qualitative, results in von Willebrand disease (VWD), the most common bleeding disorder. The deficiency of FVIII is responsible for HemophiliaHemophilia A, an X-linked bleeding disorder. Here, we provide an overview on the role of theVon Willebrand Factor (VWF) VWF-FVIII interaction in vascular physiology.
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