Lifecycle of Weibel-Palade bodies

Marjon Mourik; Jeroen Eikenboom

doi:10.5482/HAMO-16-07-0021

Hämostaseologie, Inhaltsverzeichnis

Hamostaseologie 2017; 37(01): 13-24
DOI: 10.5482/HAMO-16-07-0021

Plenary lecture

Schattauer GmbH

Lifecycle of Weibel-Palade bodies

Lebenszyklus von Weibel-Palade-Körperchen

Autor*innen

Marjon Mourik

¹Department of Plasma Proteins, Sanquin-AMC Landsteiner Laboratory, Amsterdam, The Netherlands
Jeroen Eikenboom

²Department of Thrombosis and Haemostasis, Leiden University Medical Center, Leiden, The Netherlands

Abstract

Summary

Weibel-Palade bodies (WPBs) are rod or cigar-shaped secretory organelles that are formed by the vascular endothelium. They contain a diverse set of proteins that either function in haemostasis, inflammation, or angiogenesis. Biogenesis of the WPB occurs at the Golgi apparatus in a process that is dependent on the main component of the WPB, the haemostatic protein von Willebrand Factor (VWF). During this process the organelle is directed towards the regulated secretion pathway by recruiting the machinery that responds to exocytosis stimulating agonists. Upon maturation in the periphery of the cell the WPB recruits Rab27A which regulates WPB secretion. To date several signaling pathways have been found to stimulate WPB release. These signaling pathways can trigger several secretion modes including single WPB release and multigranular exocytosis. In this review we will give an overview of the WPB lifecycle from biogenesis to secretion and we will discuss several deficiencies that affect the WPB lifecycle.

Zusammenfassung

Weibel-Palade-Körperchen (WPK) sind längsovale sekretorische Organellen, die vom Gefäßendothel gebildet werden. Sie enthalten diverse Proteine, die an der Blutstillung, Entzündung bzw. Angiogenese beteiligt sind. Die Biogenese der WPK erfolgt im Golgi-Apparat. Der Prozess hängt vom Hämostase-Protein von-Willebrand-Faktor (VWF) ab, der den Hauptbestandteil der WPK bildet. Während dieses Prozesses werden die Organellen in Richtung des regulierten Sekretionspfades geleitet, indem der Mechanismus aktiviert wird, der durch Stimulation von Agonisten auf die Exozytose reagiert. Bei Reifung in der Zellperipherie rekrutiert das WPK Rab27A, das die WPK-Sekretion reguliert. Bisher sind mehrere Signalwege bekannt, die die WPK-Freisetzung stimulieren. Die Signalwege können verschiedene Sekretionsmodi auslösen, wie beispielsweise die einzelne WPK-Freisetzung und die multigranuläre Exozytose. Dieser Artikel enthält eine Übersicht über den WPK-Lebenszyklus von der Biogenese bis zur Sekretion. Wir besprechen außerdem mehrere Mängel, die den WPK-Lebenszyklus betreffen.

Keywords

Weibel-Palade body - biogenesis - secretion

Schlüsselwörter

Weibel-Palade-Körperchen - Biogenese - Sekretion

Volltext

Referenzen

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