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The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story

von Willebrand factor (VWF) plays key roles in both primary and secondary hemostasis by capturing platelets and chaperoning clotting factor VIII, respectively. It is stored within the Weibel–Palade bodies (WPBs) of endothelial cells as a highly prothrombotic protein, and its release is thus necessar...

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Detalles Bibliográficos
Autores principales: Nightingale, T, Cutler, D
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BlackWell Publishing Ltd 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4255685/
https://www.ncbi.nlm.nih.gov/pubmed/23809123
http://dx.doi.org/10.1111/jth.12225
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author Nightingale, T
Cutler, D
author_facet Nightingale, T
Cutler, D
author_sort Nightingale, T
collection PubMed
description von Willebrand factor (VWF) plays key roles in both primary and secondary hemostasis by capturing platelets and chaperoning clotting factor VIII, respectively. It is stored within the Weibel–Palade bodies (WPBs) of endothelial cells as a highly prothrombotic protein, and its release is thus necessarily under tight control. Regulating the secretion of VWF involves multiple layers of cellular machinery that act together at different stages, leading to the exocytic fusion of WPBs with the plasma membrane and the consequent release of VWF. This review aims to provide a snapshot of the current understanding of those components, in particular the members of the Rab family, acting in the increasingly complex story of VWF secretion.
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spelling pubmed-42556852014-12-08 The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story Nightingale, T Cutler, D J Thromb Haemost State of the Art von Willebrand factor (VWF) plays key roles in both primary and secondary hemostasis by capturing platelets and chaperoning clotting factor VIII, respectively. It is stored within the Weibel–Palade bodies (WPBs) of endothelial cells as a highly prothrombotic protein, and its release is thus necessarily under tight control. Regulating the secretion of VWF involves multiple layers of cellular machinery that act together at different stages, leading to the exocytic fusion of WPBs with the plasma membrane and the consequent release of VWF. This review aims to provide a snapshot of the current understanding of those components, in particular the members of the Rab family, acting in the increasingly complex story of VWF secretion. BlackWell Publishing Ltd 2013-06 2013-06-30 /pmc/articles/PMC4255685/ /pubmed/23809123 http://dx.doi.org/10.1111/jth.12225 Text en Copyright © 2013 International Society on Thrombosis and Haemostasis http://creativecommons.org/licenses/by/3.0/ This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle State of the Art
Nightingale, T
Cutler, D
The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story
title The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story
title_full The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story
title_fullStr The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story
title_full_unstemmed The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story
title_short The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story
title_sort secretion of von willebrand factor from endothelial cells; an increasingly complicated story
topic State of the Art
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4255685/
https://www.ncbi.nlm.nih.gov/pubmed/23809123
http://dx.doi.org/10.1111/jth.12225
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