Abstract.
von Willebrand disease is a genetic bleeding disorder that arises from abnormalities in von Willebrand factor, an adhesive glycoprotein involved in both primary hemostasis and coagulation. It is the most common inherited bleeding disorder in humans, and over the years several animal species have also been described as suffering from this disease whether through a spontaneous mutation (pigs, dogs) or a genetically engineered one (mouse). These different animal models are extremely useful in exploring the characteristics of von Willebrand disease and in testing new treatments. This review provides an update of the various von Willebrand disease models and the contribution that these models can make to a better understanding of human von Willebrand disease.
Keywords: Key Words:von Willebrand disease; von Willebrand factor; animal models; hemostasis; thrombosis; atherosclerosis.
Footnotes
Received 5 July 1999; received after revision 13 August 1999; accepted 10 September 1999