von Willebrand factor: the old, the new and the unknown
Article first published online: 12 DEC 2012
© 2012 International Society on Thrombosis and Haemostasis
Journal of Thrombosis and Haemostasis
Volume 10, Issue 12, pages 2428–2437, December 2012
How to Cite
LENTING, P. J., CASARI, C., CHRISTOPHE, O. D. and DENIS, C. V. (2012), von Willebrand factor: the old, the new and the unknown. Journal of Thrombosis and Haemostasis, 10: 2428–2437. doi: 10.1111/jth.12008
- Issue published online: 12 DEC 2012
- Article first published online: 12 DEC 2012
- Accepted manuscript online: 30 SEP 2012 03:45AM EST
- von Willebrand factor, inflammation, cancer, apoptosis, thrombosis.
Summary. von Willebrand factor (VWF) is a protein best known from its critical role in hemostasis. Indeed, any dysfunction of VWF is associated with a severe bleeding tendency known as von Willebrand disease (VWD). Since the first description of the disease by Erich von Willebrand in 1926, remarkable progress has been made with regard to our understanding of the pathogenesis of this disease. The cloning of the gene encoding VWF has allowed numerous breakthroughs, and our knowledge of the epidemiology, genetics and molecular basis of VWD has been rapidly expanding since then. These studies have taught us that VWF is rather unique in terms of its multimeric structure and the unusual mechanisms regulating its participation in the hemostatic process. Moreover, it has become increasingly clear that VWF is a more all-round protein than originally thought, given its involvement in several pathologic processes beyond hemostasis. These include angiogenesis, cell proliferation, inflammation, and tumor cell survival. In the present article, an overview of advances concerning the various structural and functional aspects of VWF will be provided.