VON WILLEBRAND FACTOR ABNORMALITIES STUDIED IN THE MOUSE MODEL: WHAT WE LEARNED ABOUT VWF FUNCTIONS

Caterina Casari, Cecile V Denis
  • Caterina Casari
    Affiliation not present
  • Cecile V Denis
    INSERM U770, 80 rue du Général Leclerc, 94276 Le Kremlin-Bicêtre Cedex, France Tel: 33-1-49-59-56-05 cecile.denis@inserm.fr, | cecile.denis@inserm.fr

Abstract

Up until recently, von Willebrand Factor (VWF) structure-function relationships have only been studied through in vitro approaches. A powerful technique known as hydrodynamic gene transfer, which allows transient expression of a transgene by mouse hepatocytes, has led to an important shift in VWF research. Indeed this approach has now enabled us to transiently express a number of VWF mutants in VWF-deficient mice in order to test the relative importance of specific residues in different aspects of VWF biology and functions in an in vivo setting. As a result, mice reproducing various types of von Willebrand disease have been generated, models that will be useful to test new therapies. This approach also allowed a more precise identification of the importance of VWF interaction with subendothelial collagens and with platelets receptors in hemostasis and thrombosis. The recent advances gathered from these studies as well as the pros and cons of the technique will be reviewed here.

Full Text:

PDF
HTML
Submitted: 2014-06-13 09:55:03
Published: 2013-07-10 00:00:00
Search for citations in Google Scholar
Related articles: Google Scholar
Abstract views:
908

Views:
PDF
321
HTML
8963

Article Metrics

Metrics Loading ...

Metrics powered by PLOS ALM


Copyright (c) 2016 Mediterranean Journal of Hematology and Infectious Diseases

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
 
© PAGEPress 2008-2018     -     PAGEPress is a registered trademark property of PAGEPress srl, Italy.     -     VAT: IT02125780185     •     Privacy