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Acquired von Willebrand Syndrome: Its Pathophysiology, Laboratory Features and Management
Acquired von Willebrand syndrome is a bleeding disorder associated with various underlying diseases. The clinical manifestations are similar to...
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Molecular Genetics of Type 2 von Willebrand Disease
Type 2 von Willebrand disease (VWD) is characterized by a wide heterogeneity of functional and structural defects. These abnormalities cause either...
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Molecular and Cellular Biology of von Willebrand Factor
von Willebrand factor (VWF) is a plasma protein that performs 2 main functions in hemostasis: it mediates platelet adhesion to the injured vessel...
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von Willebrand Factor—Cleaving Protease and Upshaw-Schulman Syndrome
Vascular endothelial cell (EC)-produced plasma von Willebrand factor (vWF) plays a critical role in primary hemostasis through its action of...
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The von Willebrand factor collagen-binding activity assay: clinical application
A collagen type III based collagen-binding assay was developed for measuring the functional activity of the von Willebrand factor. The assay had a...
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Association of the 3467C>T mutation (T1156M) in the von Willebrand's factor gene with dominant type 1 von Willebrand's disease
Type 1 is the most frequent form of von Willebrand's disease, which is characterized by a quantitative partial deficiency of von Willebrand's...
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Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS): The New Thinking
TTP and HUS are two disorders with many similarities. Though their first descriptions appeared at different time in history, there has been a trend...
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Autosomal recessive inheritance of von Willebrand factor-cleaving protease deficiency
A child with chronic relapsing thrombotic thrombocytopenic purpura (TTP/HUS) had recurrent thrombocytopenia, microangiopathic hemolytic anemia with...
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Recombinant von Willebrand factor: Potential Therapeutic Use
Human von Willebrand factor (vWF) produced by recombinant technology offers a new perspective in treatment of von Willebrand disease (vWD). Several...
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Characterization of von Willebrand factor in primary pulmonary hypertension
The aim of this study was to determine the value of von Willebrand factor (vWF), a well-characterized endothelial cell protein secretion, as a marker...
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Laboratory diagnosis of von Willebrand disease
Von Willebrand disease is the most-common inherited bleeding disorder, including both quantitative (types 1 and 3) and qualitative (type 2) defects...
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Collagen covalently immobilized onto plastic surfaces simplifies measurement of von Willebrand factor-collagen binding activity
Human collagen type III was immobilized covalently via activated carbohydrate moieties onto hydrazine-treated microtiter plates which could be used...
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Proteolytic processing of von Willebrand factor subunit: Heterogeneity in type-IIA von Willebrand disease
Type IIA von Willebrand disease (vWD) is a heterogeneous disorder for which two different pathogenetic mechanisms have been proposed: increased...
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The von Willebrand factor
Von Willebrand factor (vWf) is a multimeric and multivalent adhesive protein which is essential for platelet adhesion to subendothelium and for...