Weibel-Palade bodies: a window to von Willebrand disease

J Thromb Haemost. 2013 Apr;11(4):581-92. doi: 10.1111/jth.12160.


Weibel-Palade bodies (WPBs) are the storage organelles for von Willebrand factor (VWF) in endothelial cells. VWF forms multimers that assemble into tubular structures in WPBs. 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 Willebrand disease. This review reports the recent findings on the effects of VWF mutations on the biosynthetic pathway of VWF and its storage in WPBs. These new findings have deepened our understanding of VWF synthesis, storage, secretion, and function.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Humans
  • Mutation
  • Weibel-Palade Bodies / pathology*
  • von Willebrand Diseases / pathology*
  • von Willebrand Factor / biosynthesis
  • von Willebrand Factor / genetics


  • von Willebrand Factor