Mediterranean Journal of Hematology and Infectious Diseases

نویسندگان

  • Caterina Casari
  • Peter J. Lenting
  • Olivier D. Christophe
  • Cécile V. Denis
چکیده

Up until recently, von Willebrand Factor (VWF) structure only been studied through in vitro transfer, which allows transient expression of a transgene by mouse hepatocy important shift in VWF research. Indeed this approach has now enabled us to transiently express a number of VWF mutants in VWF residues in different aspects of VWF biolo 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. Although von Willebrand disease (VWD) was first recognized as early as 1926 by Erik von Willebrand, is only in the 1970’s that the protein responsible for this disease, i.e. von Willebrand factor was formally recognized as an independent entity not to be confused with coagulation factor VIII (FVIII). Other articles in this special issue have extensively developed all aspects related to VWF functions in hemostasis/ thrombosis as well as beyond these fields and also the pathological aspects with regard to VWD. Some of these progresses were made possible thanks to the availability of animal models of VWD. Indeed, VWD has been described as naturally occurring in a number www.mjhid.org ISSN 2035-3006 Studied in the Mouse 1,2 and Cécile V. Denis

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تاریخ انتشار 2013