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von Willebrand factor to the rescue
Blood, 2009Abstractvon Willebrand factor (VWF) is a large multimeric adhesive glycoprotein with complex roles in thrombosis and hemostasis. Abnormalities in VWF give rise to a variety of bleeding complications, known as von Willebrand disease (VWD), the most common inherited bleeding disorder in humans.
Simon F, De Meyer +2 more
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Platelet von Willebrand Factor
Mayo Clinic Proceedings, 1991von Willebrand factor (vWF) circulates in the blood in two distinct compartments. One, plasma vWF, is synthesized and released from endothelial cells; the second, synthesized by megakaryocytes, circulates in platelets primarily stored in the alpha granules.
H R, Gralnick +6 more
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von Willebrand's factor and von Willebrand's disease.
Current opinion in hematology, 1997von Willebrand's factor is required for platelet adhesion to subendothelium, and for normal factor VIII survival in the circulation. These functions require the assembly of von Willebrand's factor into multimers that exhibit properly regulated binding to platelet glycoprotein lb. Recent studies suggest that the propeptide of von Willebrand's factor may
T, Matsushita, Z, Dong, J E, Sadler
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[Von Willebrand factor and Von Willebrand disease].
Nederlands tijdschrift voor geneeskunde, 2014Von Willebrand factor (VWF) is a multimeric adhesive protein that binds platelets to exposed subendothelium and carries factor VIII in the circulation. VWD is classified into three major subtypes, distinguished by a quantitative deficiency of VWF (type 1: partial deficiency; type 3: complete deficiency) or qualitative defects of VWF (type 2A, 2B, 2M ...
Paul W M, Verhezen +2 more
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von Willebrand factor and von Willebrand disease.
Blood, 1987Progress has occurred in the past several years in the understanding of the structure and function of von Willebrand factor (vWF). This multimeric glycoprotein exhibits a dual role, that of mediating platelet adhesion and aggregation onto thrombogenic surfaces, and that of functioning as carrier in plasma for the factor VIII procoagulant protein.
Z M, Ruggeri, T S, Zimmerman
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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 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 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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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/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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