Clinical and molecular predictors of thrombocytopenia and risk of bleeding in patients with von Willebrand disease type 2B: a cohort study of 67 patients

AB Federici, PM Mannucci, G Castaman… - Blood, The Journal …, 2009 - ashpublications.org
Type 2B von Willebrand disease (VWD2B) is caused by an abnormal von Willebrand factor
(VWF) with increased affinity for the platelet receptor glycoprotein Ib-α (GPIb-α) that may …

Genetic background of von Willebrand disease: history, current state, and future perspectives

J Zolkova, J Sokol, T Simurda… - … in thrombosis and …, 2020 - thieme-connect.com
Sequencing of the gene encoding for von Willebrand factor (VWF) has brought new insight
into the physiology of VWF as well as its pathophysiology in the context of von Willebrand …

[HTML][HTML] Purified A2 domain of von Willebrand factor binds to the active conformation of von Willebrand factor and blocks the interaction with platelet glycoprotein Ibα

C Martin, LD Morales, MA Cruz - Journal of thrombosis and haemostasis, 2007 - Elsevier
Background: von Willebrand factor (VWF) does not interact with circulating platelets unless it
is induced to expose the binding site for platelet glycoprotein (GP) Ibα in the A1 domain by …

Impaired megakaryocytopoiesis in type 2B von Willebrand disease with severe thrombocytopenia

P Nurden, N Debili, W Vainchenker, R Bobe… - Blood, 2006 - ashpublications.org
In type 2B von Willebrand disease, there is spontaneous binding of mutated von Willebrand
factor (VWF) multimers to platelets. Here we report a family in which severe …

Genetics of type 2B von Willebrand disease:“true 2B,”“tricky 2B,” or “not 2B.” What are the modifiers of the phenotype?

M Othman, EJ Favaloro - Seminars in thrombosis and …, 2008 - thieme-connect.com
Type 2B von Willebrand disease (VWD) is a qualitative type of VWD with a unique feature
among VWD types, resulting from an increased binding of von Willebrand factor (VWF) to its …

[HTML][HTML] von Willebrand factor A1 domain can adequately substitute for A3 domain in recruitment of flowing platelets to collagen

A Bonnefoy, RA Romijn, PAH Vandervoort… - Journal of Thrombosis …, 2006 - Elsevier
Summary Background: Binding of von Willebrand factor (VWF) to platelet GPIbα and to
collagen is attributed to VWF A1 and A3 domains, respectively. Objectives: Using VWF, VWF …

Frequency of platelet type versus type 2B von Willebrand disease

A Hamilton, M Ozelo, J Leggo, C Notley… - Thrombosis and …, 2011 - thieme-connect.com
Less than 50 patients are reported with platelet type von Willebrand disease (PT-VWD)
worldwide. Several reports have discussed the diagnostic challenge of this disease versus …

Characterization of collagen thin films for von Willebrand factor binding and platelet adhesion

RR Hansen, AA Tipnis, TC White-Adams, JA Di Paola… - Langmuir, 2011 - ACS Publications
Von Willebrand factor (VWF) binding and platelet adhesion to subendothelial collagens are
initial events in thrombus formation at sites of vascular injury. These events are often studied …

Misfolding of vWF to pathologically disordered conformations impacts the severity of von Willebrand disease

A Tischer, P Madde, L Moon-Tasson, M Auton - Biophysical journal, 2014 - cell.com
The primary hemostatic von Willebrand factor (vWF) functions to sequester platelets from
rheological blood flow and mediates their adhesion to damaged subendothelium at sites of …

Shear stress accumulation enhances von willebrand factor-induced platelet p-selectin translocation in a PI3K/Akt pathway-dependent manner

J Fang, X Sun, S Liu, P Yang, J Lin, J Feng… - Frontiers in Cell and …, 2021 - frontiersin.org
Platelet adhesion and activation through the interaction of von Willebrand factor (VWF) with
platelet glycoprotein (GP) Ibα are the early key events in hemostasis and thrombosis …