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The disulfide bond Cys2724-Cys2774 in the C-terminal cystine knot domain of von Willebrand factor is critical for its dimerization and secretion
BackgroundType 3 von Willebrand disease (VWD) exhibits severe hemorrhagic tendency with complicated pathogenesis. The C-terminal cystine knot (CTCK)...
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Von Willebrand Factor and Platelet Aggregation: from Bench to Clinical Practice
Purpose of ReviewThis review describes von Willebrand factor (VWF)-mediated platelet function in inherited and acquired bleeding disorders, and...
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Molecular and clinical profile of type 2 von Willebrand disease in Iran: a thirteen-year experience
Type 2 von Willebrand disease (VWD) is the most common congenital bleeding disorder, with variable bleeding tendency and a complex laboratory...
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Vwf K1362A resulted in failure of protein synthesis in mice
Von Willebrand factor (VWF) is synthesized in megakaryocytes and endothelial cells (ECs) and has two main roles: to carry and protect coagulation...
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Emerging new therapeutic antibody derivatives for cancer treatment
Monoclonal antibodies constitute a promising class of targeted anticancer agents that enhance natural immune system functions to suppress cancer cell...
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The role of von Willebrand factor in thrombotic microangiopathy
Thrombotic microangiopathy (TMA) is caused by thrombus formation in the microvasculature. The disease spectrum of TMA includes, amongst others,...
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Gastrointestinal Bleeding in Native and Prosthetic Valve Disease
Gastrointestinal bleeding with severe aortic stenosis was originally described in the 1950s by Heyde, although for years, the association was...
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Thrombotic thrombocytopenic purpura
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe...
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Mutations in the D1 domain of von Willebrand factor impair their propeptide-dependent multimerization, intracellular trafficking and secretion
We identified three novel mutations (p.Gly39Arg, p.Lys157Glu, p.Cys379Gly) and one previously known mutation (p.Asp141Asn) in the von Willebrand...
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A new ER-specific photosensitizer unravels 1O2-driven protein oxidation and inhibition of deubiquitinases as a generic mechanism for cancer PDT
Photosensitizers (PS) are ideally devoid of any activity in the absence of photoactivation, and rely on molecular oxygen for the formation of singlet...
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Von Willebrand Disease: Range of the Disease, and Management
Von Willebrand disease is the most common autosomal inherited bleeding disorder. It is caused by quantitative or qualitative defects of the von...
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The unfolded von Willebrand factor response in bloodstream: the self-association perspective
von Willebrand factor (vWF) is a multimeric glycoprotein essential for hemostasis after vascular injury, which modulates platelet-surface and...
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Elevated Von Willebrand factor propeptide for the diagnosis of thrombotic microangiopathy and for predicting a poor outcome
Thrombotic microangiopathy (TMA) is associated with vascular endothelial cell injury and is sometimes linked with poor outcome. Von Willebrand factor...
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Characterization of antibodies for quantitative determination of spiggin protein levels in male and female three-spined stickleback (Gasterosteus aculeatus)
Spiggin is an adhesive glycoprotein produced in the kidney of sticklebacks during the breeding season and is subsequently secreted into the urinary...
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von Willebrand factor in CHD and stroke: Relationships and therapeutic implications
Atherosclerotic cardiovascular disease (CVD), which includes coronary heart disease (CHD) and stroke, is now the most common cause of death in the...
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The effects of colloid solutions on hemostasis
PurposeColloid solutions are widely used to prevent or to correct hypovolemia in surgical patients. Although more efficacious than crystalloids, they...
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New Insight into the Molecular Basis of Hemophilia A
Since publication of the sequence of the factor VIII gene ( F8 ) in 1984, a large number of mutations that cause hemophilia A (HA) have been...