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 …

Steered molecular dynamics simulation in rational drug design

PC Do, EH Lee, L Le - Journal of Chemical Information and …, 2018 - ACS Publications
Conventional de novo drug design is time consuming, laborious, and resource intensive. In
recent years, emerging in silico approaches have been proven to be critical to accelerate the …

Cysteines as redox molecular switches and targets of disease

A Fra, ED Yoboue, R Sitia - Frontiers in molecular neuroscience, 2017 - frontiersin.org
Thiol groups can undergo numerous modifications, making cysteine a unique molecular
switch. Cysteine plays structural and regulatory roles as part of proteins or glutathione …

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 …

Orchestration of primary hemostasis by platelet and endothelial lysosome-related organelles

E Karampini, R Bierings, J Voorberg - … , thrombosis, and vascular …, 2020 - Am Heart Assoc
Megakaryocyte-derived platelets and endothelial cells store their hemostatic cargo in α-and
δ-granules and Weibel-Palade bodies, respectively. These storage granules belong to the …

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] 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 …

Comparative Proteomics Reveals the Potential Targets of BcNoxR, a Putative Regulatory Subunit of NADPH Oxidase of Botrytis cinerea

H Li, Z Zhang, C He, G Qin… - Molecular Plant-Microbe …, 2016 - Am Phytopath Society
The NADPH oxidase (NOX) complex has been shown to play a crucial role in stress
response and in the virulence of various fungal pathogens. The underlying molecular …

Disulfide bonds in protein folding and stability

MJ Feige, I Braakman, LM Hendershot - 2018 - books.rsc.org
Disulfide Bonds in Protein Folding and Stability | Oxidative Folding of Proteins: Basic
Principles, Cellular Regulation and Engineering | Books Gateway | Royal Society of Chemistry …