Insights from von Willebrand disease animal models

• Published in: Cellular and molecular life sciences : CMLS Vol. 56; no. 11-12; pp. 977 - 990
• Main Authors: Denis, C V, Wagner, D D
• Format: Journal Article
• Language: English
• Published: Switzerland Springer Nature B.V 01-12-1999; Birkhäuser Verlag
• Subjects: Animal diseases; Animal models; Animal species; Animals; Antigens - metabolism; Arteriosclerosis - metabolism; Bleeding Time; Blood Coagulation; Bone Marrow Transplantation; Disease Models, Animal; Dogs; Drug therapy; Genetic disorders; Genetic engineering; Glycoproteins; Health risk assessment; Hemorrhage; Humans; Mice; Mice, Knockout; Platelet Aggregation; Shwartzman Phenomenon; Swine; Thrombosis - metabolism; von Willebrand Diseases - diagnosis; von Willebrand Diseases - genetics; von Willebrand Diseases - metabolism; von Willebrand Diseases - therapy; von Willebrand Factor - chemistry; von Willebrand Factor - genetics; von Willebrand Factor - metabolism
• ISSN: 1420-682X; 1420-9071
• DOI: 10.1007/s000180050487

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.