title: Lifecycle of Weibel-Palade bodies.
abstract: 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.
authors.forename: Marjon
authors.initials: M
authors.lastname: Mourik
authors.affiliation: Jeroen Eikenboom, Leiden University Medical Center, Department of Thrombosis and Haemostasis, C7-61, P.O. Box 9600, 2300 RC Leiden, The Netherlands, Tel: +31 71 526 4906, E-Mail: H.C.J.Eikenboom@lumc.nl.
authors.forename: Jeroen
authors.initials: J
authors.lastname: Eikenboom
mesh_terms: D000818:Animals; D042783:Endothelial Cells / Q000276:immunology*; D005089:Exocytosis / Q000276:immunology*; D006801:Humans; D007113:Immunity, Innate / Q000276:immunology; D008955:Models, Cardiovascular; D018448:Models, Immunological; D015398:Signal Transduction / Q000276:immunology*; D020302:Weibel-Palade Bodies / Q000276:immunology*; D020691:rab GTP-Binding Proteins / Q000276:immunology*; D000075745:rab27 GTP-Binding Proteins; D014841:von Willebrand Factor / Q000276:immunology*
chemical_list: D000075745:rab27 GTP-Binding Proteins; D014841:von Willebrand Factor; C066694:RAB27A protein, human; D020691:rab GTP-Binding Proteins
keywords: Weibel-Palade body; biogenesis; secretion