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 …

The road to lysosome‐related organelles: Insights from Hermansky‐Pudlak syndrome and other rare diseases

SL Bowman, J Bi‐Karchin, L Le, MS Marks - Traffic, 2019 - Wiley Online Library
Lysosome‐related organelles (LROs) comprise a diverse group of cell type‐specific,
membrane‐bound subcellular organelles that derive at least in part from the endolysosomal …

Structure and function of the polymeric mucins in airways mucus

DJ Thornton, K Rousseau, MA McGuckin - Annu. Rev. Physiol., 2008 - annualreviews.org
The airways mucus gel performs a critical function in defending the respiratory tract against
pathogenic and environmental challenges. In normal physiology, the secreted mucins, in …

[HTML][HTML] Role of von Willebrand factor in the haemostasis

F Peyvandi, I Garagiola, L Baronciani - Blood Transfusion, 2011 - ncbi.nlm.nih.gov
© SIMTI Servizi Srl von Willebrand factor (VWF) is an adhesive and multimeric glycoprotein
that found its historical origin in 1924, when the Finnish physician Erik von Willebrand first …

The role of autophagy in vascular biology

SC Nussenzweig, S Verma, T Finkel - Circulation research, 2015 - Am Heart Assoc
There is increasing interest in the role of autophagic flux in maintaining normal vessel wall
biology and a growing suspicion that autophagic dysregulation may be a common pathway …

Functional architecture of Weibel-Palade bodies

KM Valentijn, JE Sadler, JA Valentijn… - Blood, The Journal …, 2011 - ashpublications.org
Abstract Weibel-Palade bodies (WPBs) are elongated secretory organelles specific to
endothelial cells that contain von Willebrand factor (VWF) and a variety of other proteins that …

Autophagy regulates endothelial cell processing, maturation and secretion of von Willebrand factor

T Torisu, K Torisu, IH Lee, J Liu, D Malide, CA Combs… - Nature medicine, 2013 - nature.com
Endothelial secretion of von Willebrand factor (VWF) from intracellular organelles known as
Weibel-Palade bodies (WPBs) is required for platelet adhesion to the injured vessel wall …

Lysosome-related organelles: unusual compartments become mainstream

MS Marks, HFG Heijnen, G Raposo - Current opinion in cell biology, 2013 - Elsevier
Lysosome-related organelles (LROs) comprise a group of cell type-specific subcellular
compartments with unique composition, morphology and structure that share some features …

[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 …

Unwinding the von Willebrand factor strings puzzle

K De Ceunynck, SF De Meyer… - Blood, The Journal of …, 2013 - ashpublications.org
Abstract von Willebrand factor (VWF) is amongst others synthesized by endothelial cells and
stored as ultra-large (UL) VWF multimers in Weibel-Palade bodies. Although UL-VWF is …