Von Willebrand disease mutation spectrum and associated mutation mechanisms

A de Jong, J Eikenboom - Thrombosis research, 2017 - Elsevier
Von Willebrand disease (VWD) is a bleeding disorder that is mainly caused by mutations in
the multimeric protein von Willebrand factor (VWF). These mutations may lead to …

[HTML][HTML] Developments in the diagnostic procedures for von Willebrand disease

A De Jong, J Eikenboom - Journal of Thrombosis and Haemostasis, 2016 - Elsevier
Von Willebrand disease (VWD) is the most common inherited bleeding disorder but its
diagnosis can be challenging due to the heterogeneity of the disease. VWD is mainly …

[HTML][HTML] Endothelial characteristics in healthy endothelial colony forming cells; generating a robust and valid ex vivo model for vascular disease

S de Boer, M Bowman, C Notley, A Mo, P Lima… - Journal of Thrombosis …, 2020 - Elsevier
Background Endothelial colony forming cells (ECFCs) derived from peripheral blood can be
used to analyze the pathophysiology of vascular diseases ex vivo. However, heterogeneity …

[HTML][HTML] Angiogenic characteristics of blood outgrowth endothelial cells from patients with von Willebrand disease

DJ Groeneveld, T van Bekkum, RJ Dirven… - Journal of Thrombosis …, 2015 - Elsevier
Summary Background Endothelial von Willebrand factor (VWF) inhibits angiogenesis.
Accordingly, blood outgrowth endothelial cells (BOECs) isolated from von Willebrand …

Characterization of large in-frame von Willebrand factor deletions highlights differing pathogenic mechanisms

A Cartwright, SJ Webster, A de Jong, RJ Dirven… - Blood …, 2020 - ashpublications.org
Copy number variation (CNV) is known to cause all von Willebrand disease (VWD) types,
although the associated pathogenic mechanisms involved have not been extensively …

Von Willebrand disease: from in vivo to in vitro disease models

S de Boer, J Eikenboom - Hemasphere, 2019 - journals.lww.com
Von Willebrand factor (VWF) plays an essential role in primary hemostasis and is exclusively
synthesized and stored in endothelial cells and megakaryocytes. Upon vascular injury, VWF …

Fifth Åland Island conference on von Willebrand disease

E Berntorp, A Ågren, L Aledort, M Blombäck… - …, 2018 - Wiley Online Library
The fifth Åland Island meeting on von Willebrand disease (VWD) was held on the Åland
Islands, Finland, from 22 to 24 September 2016—90 years after the first case of VWD was …

The current understanding of molecular pathogenesis of quantitative von willebrand disease, types 1 and 3

H Yadegari, J Oldenburg - Hämostaseologie, 2020 - thieme-connect.com
Von Willebrand disease (VWD), the most prevalent congenital bleeding disorder, arises from
deficiencies in quantity or quality of von Willebrand factor (VWF). The quantitative …

Advancing multimer analysis of von Willebrand factor by single-molecule AFM imaging

A Löf, G König, S Schneppenheim… - PLoS …, 2019 - journals.plos.org
The formation of hemostatic plugs at sites of vascular injury crucially involves the multimeric
glycoprotein von Willebrand factor (VWF). VWF multimers are linear chains of N-terminally …

A relação do sistema sanguíneo ABO com o Fator de von Willebrand eo seu pró-peptídeo (FVWpp)

DF Valadares - 2020 - teses.usp.br
O Fator de von Willebrand (FVW) é uma proteína envolvida na hemostasia, responsável por
mediar a adesão e agregação plaquetára em eventos de lesão vascular. Essa proteína é …