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W eibel– P alade bodies: a window to von W illebrand disease
Author(s) -
Valentijn K. M.,
Eikenboom J.
Publication year - 2013
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1111/jth.12160
Subject(s) - von willebrand factor , platelet , von willebrand disease , hemostasis , secretion , organelle , chemistry , microbiology and biotechnology , immunology , biology , medicine , biochemistry
Summary W eibel– P alade bodies ( WPB s) are the storage organelles for von W illebrand factor ( VWF ) in endothelial cells. VWF forms multimers that assemble into tubular structures in WPB s. Upon demand, VWF is secreted into the blood circulation, where it unfolds into strings that capture platelets during the onset of primary hemostasis. Numerous mutations affecting VWF lead to the bleeding disorder von W illebrand disease. This review reports the recent findings on the effects of VWF mutations on the biosynthetic pathway of VWF and its storage in WPB s. These new findings have deepened our understanding of VWF synthesis, storage, secretion, and function.