von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends

PJ Lenting, OD Christophe… - Blood, The Journal of the …, 2015 - ashpublications.org
To understand the placement of a certain protein in a physiological system and the
pathogenesis of related disorders, it is not only of interest to determine its function but also …

von Willebrand factor: more than a regulator of hemostasis and thrombosis

GP Luo, B Ni, X Yang, YZ Wu - Acta haematologica, 2012 - karger.com
Abstract von Willebrand factor (vWF) was first identified as an adhesive glycoprotein
involved in hemostasis by Zimmermann in 1971. Since then, vWF has been shown to play a …

Proteomic screen identifies IGFBP7 as a novel component of endothelial cell-specific Weibel-Palade bodies

D Van Breevoort, EL van Agtmaal… - Journal of proteome …, 2012 - ACS Publications
Vascular endothelial cells contain unique storage organelles, designated Weibel-Palade
bodies (WPBs), that deliver inflammatory and hemostatic mediators to the vascular lumen in …

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 …

Cellular and molecular basis of von Willebrand disease: studies on blood outgrowth endothelial cells

RD Starke, KE Paschalaki, CEF Dyer… - Blood, The Journal …, 2013 - ashpublications.org
Von Willebrand disease (VWD) is a heterogeneous bleeding disorder caused by decrease
or dysfunction of von Willebrand factor (VWF). A wide range of mutations in the VWF gene …

Analysis of the storage and secretion of von Willebrand factor in blood outgrowth endothelial cells derived from patients with von Willebrand disease

JW Wang, EAM Bouwens, MC Pintao… - Blood, The Journal …, 2013 - ashpublications.org
Patients with von Willebrand disease (VWD) are often heterozygous for a missense mutation
in the von Willebrand factor (VWF) gene. Investigating the pathogenic features of VWF …

[HTML][HTML] Blocking von Willebrand factor for treatment of cutaneous inflammation

C Hillgruber, AK Steingräber, B Pöppelmann… - Journal of Investigative …, 2014 - Elsevier
Von Willebrand factor (VWF), a key player in hemostasis, is increasingly recognized as a
proinflammatory protein. Here, we found a massive accumulation of VWF in skin biopsies of …

[HTML][HTML] Weibel–Palade bodies: a window to von Willebrand disease

KM Valentijn, J Eikenboom - Journal of Thrombosis and Haemostasis, 2013 - Elsevier
Summary Weibel–Palade bodies (WPBs) are the storage organelles for von Willebrand
factor (VWF) in endothelial cells. VWF forms multimers that assemble into tubular structures …

von Willebrand factor is dimerized by protein disulfide isomerase

S Lippok, K Kolšek, A Löf, D Eggert… - Blood, The Journal …, 2016 - ashpublications.org
Multimeric von Willebrand factor (VWF) is essential for primary hemostasis. The biosynthesis
of VWF high-molecular-weight multimers requires spatial separation of each step because of …

Endothelial Colony Forming Cells in the spotlight, insights into the pathophysiology of von Willebrand Disease and rare bleeding disorders.

SNJ Laan, BG Lenderink, JCJ Eikenboom… - Journal of Thrombosis …, 2024 - Elsevier
Endothelial cells deliver a vital contribution to the maintenance of hemostasis by constituting
an anatomical as well as functional barrier between the blood and the rest of the body. Apart …