A new look at an old body: molecular determinants of Weibel-Palade body composition and von Willebrand factor exocytosis

Sophie Hordijk, Tom Carter, Ruben Bierings*

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

3 Citations (Scopus)
18 Downloads (Pure)

Abstract

Endothelial cells, forming a monolayer along blood vessels, intricately regulate vascular hemostasis, inflammatory responses, and angiogenesis. A key determinant of these functions is the controlled secretion of Weibel-Palade bodies (WPBs), which are specialized endothelial storage organelles housing a presynthesized pool of the hemostatic protein von Willebrand factor and various other hemostatic, inflammatory, angiogenic, and vasoactive mediators. This review delves into recent mechanistic insights into WPB biology, including the biogenesis that results in their unique morphology, the acquisition of intraluminal vesicles and other cargo, and the contribution of proton pumps to organelle acidification. Additionally, in light of a number of proteomic approaches to unravel the regulatory networks that control WPB formation and secretion, we provide a comprehensive overview of the WPB exocytotic machinery, including their molecular and cellular mechanisms.

Original languageEnglish
Pages (from-to)1290-1303
Number of pages14
JournalJournal of Thrombosis and Haemostasis
Volume22
Issue number5
Early online date1 Feb 2024
DOIs
Publication statusPublished - May 2024

Bibliographical note

Publisher Copyright: © 2024 The Author(s)

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