title: Biogenesis of Weibel-Palade bodies.
abstract: Weibel-Palade bodies (WPBs) are the lysosome-related secretory organelles of endothelial cells. Their content protein von Willebrand factor, plays a key role in haemostasis, whilst P-selectin in the membranes is critical in the initiation of inflammation. Biogenesis of these rod-shaped structures is driven by von Willebrand factor, since its heterologous expression leads to formation of organelles morphologically indistinguishable from bona fide WPBs. The two main membrane proteins of WPBs, CD63 and P-selectin, have complex itineraries controlled largely by cytoplasmic targeting signals. We are only just beginning to understand the way in which these three proteins come together to form mature WPBs.
authors.affiliation: MRC Laboratory for Molecular Cell Biology, Cell Biology Unit, University College London, Gower Street, WC1E 6BT, London, UK.
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Hannah
authors.forename: Ross
authors.initials: R
authors.lastname: Williams
authors.forename: Jasber
authors.initials: J
authors.lastname: Kaur
authors.forename: Lindsay J
authors.initials: LJ
authors.lastname: Hewlett
authors.forename: Daniel F
authors.initials: DF
authors.lastname: Cutler
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D015703:Antigens, CD / Q000378:metabolism; D006801:Humans; D008969:Molecular Sequence Data; D019007:P-Selectin / Q000378:metabolism; D010980:Platelet Membrane Glycoproteins / Q000378:metabolism; D060149:Tetraspanin 30; D020302:Weibel-Palade Bodies / Q000502:physiology*; D014841:von Willebrand Factor / Q000235:genetics* / Q000378:metabolism*
chemical_list: D015703:Antigens, CD; C554586:CD63 protein, human; D019007:P-Selectin; D010980:Platelet Membrane Glycoproteins; D060149:Tetraspanin 30; D014841:von Willebrand Factor