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 …

STXBP1 encephalopathy: A neurodevelopmental disorder including epilepsy

H Stamberger, M Nikanorova, MH Willemsen… - Neurology, 2016 - AAN Enterprises
Objective: To give a comprehensive overview of the phenotypic and genetic spectrum of
STXBP1 encephalopathy (STXBP1-E) by systematically reviewing newly diagnosed and …

[HTML][HTML] Exocytosis of Weibel–Palade bodies: how to unpack a vascular emergency kit

M Schillemans, E Karampini, M Kat… - Journal of Thrombosis and …, 2019 - Elsevier
The blood vessel wall has a number of self‐healing properties, enabling it to minimize blood
loss and prevent or overcome infections in the event of vascular trauma. Endothelial cells …

Weibel Palade bodies: unique secretory organelles of endothelial cells that control blood vessel homeostasis

J Naß, J Terglane, V Gerke - Frontiers in Cell and Developmental …, 2021 - frontiersin.org
Vascular endothelial cells produce and release compounds regulating vascular tone, blood
vessel growth and differentiation, plasma composition, coagulation and fibrinolysis, and also …

[HTML][HTML] Genetic regulation of plasma von Willebrand factor levels in health and disease

LL Swystun, D Lillicrap - Journal of Thrombosis and Haemostasis, 2018 - Elsevier
Plasma levels of the multimeric glycoprotein von Willebrand factor (VWF) constitute a
complex quantitative trait with a continuous distribution and wide range in the normal …

Trafficking in blood vessel development

CR Francis, EJ Kushner - Angiogenesis, 2022 - Springer
Blood vessels demonstrate a multitude of complex signaling programs that work in concert
to produce functional vasculature networks during development. A known, but less widely …

Regulation of VWF expression, and secretion in health and disease

Y Xiang, J Hwa - Current opinion in hematology, 2016 - journals.lww.com
VWF biosynthesis, maturation, and secretion is a complex process, which mandates tight
regulation. Significant progress has been made in our understandings of VWF expression …

GDP/GTP exchange factor MADD drives activation and recruitment of secretory Rab GTPases to Weibel-Palade bodies

M Kat, PE Bürgisser, H Janssen, IM De Cuyper… - Blood …, 2021 - ashpublications.org
Abstract von Willebrand factor (VWF) is an essential hemostatic protein that is synthesized
and secreted by endothelial cells and stored in Weibel-Palade bodies (WPBs). The …

Proximity proteomics of endothelial Weibel-Palade bodies identifies novel regulator of von Willebrand factor secretion

A Holthenrich, HCA Drexler, T Chehab… - Blood, The Journal …, 2019 - ashpublications.org
Weibel-Palade bodies (WPB) are unique secretory organelles of endothelial cells that store
factors regulating vascular hemostasis and local inflammation. Endothelial activation …

Stimulated release of intraluminal vesicles from Weibel-Palade bodies

J Streetley, AV Fonseca, J Turner… - Blood, The Journal …, 2019 - ashpublications.org
Abstract Weibel-Palade bodies (WPBs) are secretory granules that contain von Willebrand
factor and P-selectin, molecules that regulate hemostasis and inflammation, respectively …