Table of Contents
Thrombosis
Volume 2010 (2010), Article ID 461238, 11 pages
http://dx.doi.org/10.1155/2010/461238
Review Article

Porcine and Canine von Willebrand Factor and von Willebrand Disease: Hemostasis, Thrombosis, and Atherosclerosis Studies

1Department of Medicine, Francis Owen Blood Research Laboratory, University of North Carolina, Chapel Hill, NC 27516, USA
2Pathology and Laboratory Medicine, Francis Owen Blood Research Laboratory, University of North Carolina, Chapel Hill, NC 27516, USA
3Division of Laboratory Animal Medicine, Francis Owen Blood Research Laboratory, University of North Carolina, Chapel Hill, NC 27516, USA
4Medicine/Hematology/Oncology, University of Pittsburgh Medical Center, Pittsburgh, PA 15261, USA
5Hemophilia Center of Western PA, Pittsburgh, PA 15213, USA

Received 27 October 2010; Accepted 29 December 2010

Academic Editor: David H. Farrell

Copyright © 2010 Timothy C. Nichols et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Use of animal models of inherited and induced von Willebrand factor (VWF) deficiency continues to advance the knowledge of VWF-related diseases: von Willebrand disease (VWD), thrombotic thrombocytopenic purpura (TTP), and coronary artery thrombosis. First, in humans, pigs, and dogs, VWF is essential for normal hemostasis; without VWF bleeding events are severe and can be fatal. Second, the ADAMTS13 cleavage site is preserved in all three species suggesting all use this mechanism for normal VWF multimer processing and that all are susceptible to TTP when ADAMTS13 function is reduced. Third, while the role of VWF in atherogenesis is debated, arterial thrombosis complicating atherosclerosis appears to be VWF-dependent. The differences in the VWF gene and protein between humans, pigs, and dogs are relatively few but important to consider in the design of VWF-focused experiments. These homologies and differences are reviewed in detail and their implications for research projects are discussed. The current status of porcine and canine VWD are also reviewed as well as their potential role in future studies of VWF-related disorders of hemostasis and thrombosis.