von Willebrand factor, Jedi knight of the bloodstream

TA Springer - Blood, The Journal of the American Society of …, 2014 - ashpublications.org
When blood vessels are cut, the forces in the bloodstream increase and change character.
The dark side of these forces causes hemorrhage and death. However, von Willebrand …

Spectrum of the Mutations in B ernard–S oulier Syndrome

A Savoia, S Kunishima, D De Rocco, B Zieger… - Human …, 2014 - Wiley Online Library
Bernard–Soulier syndrome (BSS) is a rare autosomal recessive bleeding disorder
characterized by defects of the GPIb‐IX‐V complex, a platelet receptor for von Willebrand …

Platelet GPIbα is a mediator and potential interventional target for NASH and subsequent liver cancer

M Malehmir, D Pfister, S Gallage, M Szydlowska… - Nature medicine, 2019 - nature.com
Non-alcoholic fatty liver disease ranges from steatosis to non-alcoholic steatohepatitis
(NASH), potentially progressing to cirrhosis and hepatocellular carcinoma (HCC). Here, we …

Flow-induced elongation of von Willebrand factor precedes tension-dependent activation

H Fu, Y Jiang, D Yang, F Scheiflinger, WP Wong… - Nature …, 2017 - nature.com
Von Willebrand factor, an ultralarge concatemeric blood protein, must bind to platelet GPIbα
during bleeding to mediate hemostasis, but not in the normal circulation to avoid thrombosis …

Activation of von Willebrand factor via mechanical unfolding of its discontinuous autoinhibitory module

NA Arce, W Cao, AK Brown, ER Legan… - Nature …, 2021 - nature.com
Von Willebrand factor (VWF) activates in response to shear flow to initiate hemostasis, while
aberrant activation could lead to thrombosis. Above a critical shear force, the A1 domain of …

Platelet clearance via shear-induced unfolding of a membrane mechanoreceptor

W Deng, Y Xu, W Chen, DS Paul, AK Syed… - Nature …, 2016 - nature.com
Mechanisms by which blood cells sense shear stress are poorly characterized. In platelets,
glycoprotein (GP) Ib–IX receptor complex has been long suggested to be a shear sensor …

Activated αIIbβ3 on platelets mediates flow-dependent NETosis via SLC44A2

A Constantinescu-Bercu, L Grassi, M Frontini… - Elife, 2020 - elifesciences.org
Platelet-neutrophil interactions are important for innate immunity, but also contribute to the
pathogenesis of deep vein thrombosis, myocardial infarction and stroke. Here we report that …

A biophysical view on von Willebrand factor activation

A Löf, JP Müller, MA Brehm - Journal of cellular physiology, 2018 - Wiley Online Library
The process of hemostatic plug formation at sites of vascular injury crucially relies on the
large multimeric plasma glycoprotein von Willebrand factor (VWF) and its ability to recruit …

Primary haemostasis: newer insights

MC Berndt, P Metharom, RK Andrews - Haemophilia, 2014 - Wiley Online Library
At the same time as biophysical and omics approaches are drilling deeper into the
molecular details of platelets and other blood cells, as well as their receptors and …

Identification of a juxtamembrane mechanosensitive domain in the platelet mechanosensor glycoprotein Ib-IX complex

W Zhang, W Deng, L Zhou, Y Xu… - Blood, The Journal …, 2015 - ashpublications.org
Abstract How glycoprotein (GP) Ib-IX complex on the platelet surface senses the blood flow
through its binding to the plasma protein von Willebrand factor (VWF) and transmits a signal …