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The role of VWF in the immunogenicity of FVIII
Thrombosis Research, 2008Up to 33% of patients with severe haemophilia A develop inhibitory antibodies to factor VIM (FVIII) that can significantly impair treatment with FVIII. The plasma protein von Willebrand factor (VWF) binds to FVIII and is known to be important for the functioning of FVIII.
Sébastien, Lacroix-Desmazes +3 more
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Blood, 2013
Abstract Von Willebrand disease is a bleeding disorder with reduced or abnormal function of VWF. While VWF has at least three functions (binding to FVIII, platelet GPIb, and collagen), the function of VWF has been quantified primarily using techniques in which ristocetin promotes the binding of VWF to platelet GPIb. It is recognized that
Robert R. Montgomery +5 more
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Abstract Von Willebrand disease is a bleeding disorder with reduced or abnormal function of VWF. While VWF has at least three functions (binding to FVIII, platelet GPIb, and collagen), the function of VWF has been quantified primarily using techniques in which ristocetin promotes the binding of VWF to platelet GPIb. It is recognized that
Robert R. Montgomery +5 more
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Oxidative stress on VWF proteolysis
Blood, 2010In this issue of Blood , Chen and colleagues demonstrate that the rate of cleaving VWF by ADAMTS13 is significantly slowed when the residue Met1606 in the VWF A2 domain is oxidized.[1][1] The finding adds a new dimension to the complexity of regulating VWF cleavage and links the rate of ...
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Sugar targets VWF for the chop
Blood, 2010In this issue of Blood , McGrath et al provide new insights into the influence of glycosylation on VWF proteolysis by ADAMTS13.[1][1] Von Willebrand factor (VWF) is synthesized in vascular endothelial cells and megakaryocytes and undergoes extensive processing prior to secretion.
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Thrombosis and Haemostasis, 1987
SummaryNine monoclonal antibodies (MAb, coded ESvWF 1-5, 7-10) to human von Willebrand’s factor (vWf) have been studied for their labelling characteristics with 125I and their ability to demonstrate vWf multimers by autoradiography after discontinuous SDS electrophoresis on agarose and agarose/acrylamide gels in plasma from normal and von Willebrand’s ...
M S, Enayat +4 more
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SummaryNine monoclonal antibodies (MAb, coded ESvWF 1-5, 7-10) to human von Willebrand’s factor (vWf) have been studied for their labelling characteristics with 125I and their ability to demonstrate vWf multimers by autoradiography after discontinuous SDS electrophoresis on agarose and agarose/acrylamide gels in plasma from normal and von Willebrand’s ...
M S, Enayat +4 more
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Hämostaseologie, 1996
ZusammenfassungDie immunologische Bestimmung der von-Willebrand-Faktor-Konzentration ist ein wichtiger Baustein in der Strategie zur Diagnostik des von-Willebrand-Syn-droms. Mit dem hier vorgestellten Test, STA LIA vWF (Boehringer Mannheim GmbH) kann diese Bestimmung in der Routineanalytik innerhalb von 10 Minuten an den Gerinnungssystemen STA und STA ...
Ute Reimann, K.-E. Stötzer
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ZusammenfassungDie immunologische Bestimmung der von-Willebrand-Faktor-Konzentration ist ein wichtiger Baustein in der Strategie zur Diagnostik des von-Willebrand-Syn-droms. Mit dem hier vorgestellten Test, STA LIA vWF (Boehringer Mannheim GmbH) kann diese Bestimmung in der Routineanalytik innerhalb von 10 Minuten an den Gerinnungssystemen STA und STA ...
Ute Reimann, K.-E. Stötzer
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Biologicals, 2015
ADAMTS13 is a metalloproteinase that cleaves von Willebrand factor (VWF) into smaller multimers in vivo. This cleavage creates both the typical multimeric size distribution and the characteristic triplet band distribution of VWF. Here we analysed ADAMTS13 content, VWF multimeric size distribution and VWF triplet structure in five commercial VWF/factor ...
Christoph, Kannicht +4 more
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ADAMTS13 is a metalloproteinase that cleaves von Willebrand factor (VWF) into smaller multimers in vivo. This cleavage creates both the typical multimeric size distribution and the characteristic triplet band distribution of VWF. Here we analysed ADAMTS13 content, VWF multimeric size distribution and VWF triplet structure in five commercial VWF/factor ...
Christoph, Kannicht +4 more
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Science's STKE, 2001
A hallmark of thrombotic thrombocytopenic purpura (TTP) is destruction of platelets and erythrocytes. It is thought that the greatly reduced proteolytic cleavage of von Willebrand factor [(VWF), a protein that is important for proper blood clotting] seen in TTP patients, provides a clue to the etiology of the disease.
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A hallmark of thrombotic thrombocytopenic purpura (TTP) is destruction of platelets and erythrocytes. It is thought that the greatly reduced proteolytic cleavage of von Willebrand factor [(VWF), a protein that is important for proper blood clotting] seen in TTP patients, provides a clue to the etiology of the disease.
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