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Chemigenetic Ca2+ indicators report elevated Ca2+ levels in endothelial Weibel-Palade bodies.
PLoS One
January 2025
Institute of Medical Biochemistry, Center for Molecular Biology of Inflammation, University of Muenster, Muenster, Germany.
Weibel-Palade bodies (WPB) are secretory organelles exclusively found in endothelial cells and among other cargo proteins, contain the hemostatic von-Willebrand factor (VWF). Stimulation of endothelial cells results in exocytosis of WPB and release of their cargo into the vascular lumen, where VWF unfurls into long strings of up to 1000 µm and recruits platelets to sites of vascular injury, thereby mediating a crucial step in the hemostatic response. The function of VWF is strongly correlated to its structure; in order to fulfill its task in the vascular lumen, VWF has to undergo a complex packing/processing after translation into the ER.
View Article and Find Full Text PDFHow Electricity Prevents Us from Bleeding to Death.
Rev Physiol Biochem Pharmacol
January 2025
Institute of Medical Sciences, University of Aberdeen, Aberdeen, Scotland, UK.
Rapid tissue repair is also needed in the event of damage to blood vessels. Most of the essential steps that prevent us from bleeding to death involve the functions of Von Willebrand factor (VWF) and many of these are dependent on electrical forces.
View Article and Find Full Text PDFVon Willebrand Factor in Weibel-Palade Bodies of Endothelial Cells Involved in the Immune Response to Hirame Novirhabdovirus Infection in a Teleost Paralichthys olivaceus.
J Fish Dis
December 2024
Laboratory of Pathology and Immunology of Aquatic Animals, KLMME, Ocean University of China, Qingdao, China.
von Willebrand factor (vWF) is a large multimeric sialoglycoprotein that plays key roles in normal haemostasis, inflammation regulation, angiogenesis and cancer metastasis in mammals. The gene, protein sequences and functions of vWF in flounder Paralichthys olivaceus (PovWF) were analysed in this study. PovWF possesses an 8550-bp open reading frame (ORF) that encodes a 2849 amino acid protein.
View Article and Find Full Text PDFThe severe von Willebrand disease variant p.M771V leads to impaired anterograde trafficking of von Willebrand factor in patient-derived and base-edited endothelial colony-forming cells.
J Thromb Haemost
November 2024
Department of Hematology, Erasmus University Medical Centre, Rotterdam, The Netherlands. Electronic address:
Background: von Willebrand disease (VWD) is the most common inherited bleeding disorder caused by quantitative or qualitative defects in von Willebrand factor (VWF). The p.M771V VWF variant leads to a severe bleeding phenotype in homozygous patients.
View Article and Find Full Text PDFRalB uncoupled exocyst mediates endothelial Weibel-Palade body exocytosis.
bioRxiv
September 2024
Section of Hematology, Yale Cancer Center, Yale University School of Medicine, New Haven, CT, USA.
Ras-like (Ral) GTPases play essential regulatory roles in many cellular processes, including exocytosis. Cycling between GDP- and GTP-bound states, Ral GTPases function as molecular switches and regulate effectors, specifically the multi-subunit tethering complex exocyst. Here, we show that Ral isoform RalB controls regulated exocytosis of Weibel-Palade bodies (WPBs), the specialized endothelial secretory granules that store hemostatic protein von Willebrand factor.
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