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Advances in Hematology
Volume 2012 (2012), Article ID 214209, 9 pages
http://dx.doi.org/10.1155/2012/214209
Research Article

Characterization of Zebrafish von Willebrand Factor Reveals Conservation of Domain Structure, Multimerization, and Intracellular Storage

1Life Sciences Institute, University of Michigan, Ann Arbor, MI 48109, USA
2Department of Pediatrics, University of Michigan, Room 8301 Medical Science Research Building III, 1150 W. Medical Center Drive, Ann Arbor, MI 48109-5646, USA
3Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI 53226, USA

Received 20 April 2012; Revised 18 June 2012; Accepted 26 July 2012

Academic Editor: Elspeth Payne

Copyright © 2012 Arunima Ghosh 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

von Willebrand disease (VWD) is the most common inherited human bleeding disorder and is caused by quantitative or qualitative defects in von Willebrand factor (VWF). VWF is a secreted glycoprotein that circulates as large multimers. While reduced VWF is associated with bleeding, elevations in overall level or multimer size are implicated in thrombosis. The zebrafish is a powerful genetic model in which the hemostatic system is well conserved with mammals. The ability of this organism to generate thousands of offspring and its optical transparency make it unique and complementary to mammalian models of hemostasis. Previously, partial clones of zebrafish vwf have been identified, and some functional conservation has been demonstrated. In this paper we clone the complete zebrafish vwf cDNA and show that there is conservation of domain structure. Recombinant zebrafish Vwf forms large multimers and pseudo-Weibel-Palade bodies (WPBs) in cell culture. Larval expression is in the pharyngeal arches, yolk sac, and intestinal epithelium. These results provide a foundation for continued study of zebrafish Vwf that may further our understanding of the mechanisms of VWD.