Insights from von Willebrand disease animal models

Citation
Cv. Denis et Dd. Wagner, Insights from von Willebrand disease animal models, CELL MOL L, 56(11-12), 1999, pp. 977-990
Citations number
137
Categorie Soggetti
Cell & Developmental Biology
Journal title
CELLULAR AND MOLECULAR LIFE SCIENCES
ISSN journal
1420682X → ACNP
Volume
56
Issue
11-12
Year of publication
1999
Pages
977 - 990
Database
ISI
SICI code
1420-682X(199912)56:11-12<977:IFVWDA>2.0.ZU;2-M
Abstract
von Willebrand disease is a genetic bleeding disorder that arises from abno rmalities in von Willebrand factor, an adhesive glycoprotein involved in bo th primary hemostasis and coagulation. It is the most common inherited blee ding disorder in humans, and over the years several animal species have als o been described as suffering from this disease whether through a spontaneo us mutation (pigs, dogs) or a genetically engineered one (mouse). These dif ferent animal models are extremely useful in exploring the characteristics of von Willebrand disease and in testing new treatments. This review provid es an update of the various von Willebrand disease models and the contribut ion that these models can make to st better understanding of human von Will ebrand disease.