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Factor VIII and von Willebrand Factor
Thrombosis and Haemostasis, 1998IntroducationFactor VIII and von Willebrand factor are plasma glycoproteins whose deficiency or structural defects cause hemophilia A and von Willebrand disease, respectively (1). These diseases are the most common inherited bleeding disorders of man. Factor VIII and vWF are synthesized by different cell types and circulate in plasma as a tightly bound
Vlot, A.J. +3 more
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Von Willebrand factor multimer patterns in von Willebrand's disease
British Journal of Haematology, 1983Summary. The von Willebrand factor antigen (factor VIII‐related antigen, VIIIR:Ag) multimer pattern has been analysed by SDS‐agarose electrophoresis of plasmas from 116 patients (47 families) with von Willebrand's disease. In addition to previously recognized patterns, a subclassification was established between plasmas that had a type Ia pattern ...
L W, Hoyer +5 more
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von Willebrand factor and von Willebrand disease.
[Rinsho ketsueki] The Japanese journal of clinical hematology, 2017von Willebrand factor (VWF) has two major roles in hemostasis, as a form of molecular glue which functions in platelet plug formation and as a protective transporter for coagulation factor VIII (FVIII). VWF shows a multimeric chain structure composed of 270 kDa subunits containing binding domains for FVIII, platelet and collagens. Biosynthesis, storage,
Taei, Matsui, Jiharu, Hamako
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Von Willebrand factor/factor VIII concentrates in the treatment of von Willebrand disease
Blood Coagulation & Fibrinolysis, 2009Therapy for von Willebrand disease (VWD) aims to restore the hemostatic function conferred by von Willebrand factor (VWF), which facilitates platelet adhesion and aggregation, and serves to increase potentially low coagulation factor VIII (FVIII) in plasma. In patients unresponsive to desmopressin (DDAVP), the preferred treatment is with plasma-derived
Javier, Batlle +4 more
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von Willebrand Factor, von Willebrand Factor-Cleaving Protease, and Shear Stress
Cardiovascular & Hematological Agents in Medicinal Chemistry, 2007von Willebrand factor (VWF) is a multimeric plasma glycoprotein (GP) involved in platelet adhesion at the site of vascular damage, which acts as a bridge between the injured subendothelium and the platelet receptors. The multimeric structure of VWF allows it to support multiple interactions with platelets and endothelial components under high shear ...
P, Perutelli, A C, Molinari
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Von Willebrand factor processing
Hämostaseologie, 2017SummaryVon Willebrand factor (VWF) is a multimeric glycoprotein essential for primary haemostasis that is produced only in endothelial cells and megakaryocytes. Key to VWF’s function in recruitment of platelets to the site of vascular injury is its multimeric structure.
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Thrombosis and von Willebrand Factor
2015One of the key players in both hemostasis and thrombosis is von Willebrand factor (vWF), which demonstrates a duality between these two processes. Thrombus is structured by numerous elements, including endothelial cells, platelets, plasma proteins and shear stress alteration.
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von Willebrand Factor and Aging
Seminars in Thrombosis and Hemostasis, 2014von Willebrand factor (VWF) plays critical roles in initiating primary hemostasis and extending the half-life of coagulation factor VIII in circulation. VWF levels increase with age and elevated levels are associated with an increased risk of venous thromboembolism and cardiovascular disease (CVD).
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Von Willebrand factor and fibrinogen
Current Opinion in Cell Biology, 1993Progress in understanding the structure and function of von Willebrand factor and fibrinogen has provided new insights into the mechanisms of hemostasis and thrombosis. This, in turn, has generated concepts that may become applicable to the diagnosis and treatment of occlusive vascular diseases and bleeding disorders, both congenital and acquired.
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The von Willebrand Factor Gene and Genetics of von Willebrand's Disease
Mayo Clinic Proceedings, 1991The von Willebrand factor (vWF) gene spans 178 kilobases in the human genome, is interrupted by 51 introns, and has been localized to human chromosome 12p12----12pter. In addition, a pseudogene that duplicates the midportion of the vWF gene has been identified on chromosome 22.
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