characterization of zebrafish von willebrand factor reveals conservation of domain structure, multimerization, and intracellular storage对斑马鱼血管性血友病因子显示域结构,保护multimerization,细胞内储存.pdfVIP

Hindawi Publishing Corporation Advances in Hematology Volume 2012, Article ID 214209, 9 pages doi:10.1155/2012/214209 Research Article Characterization of Zebrafish von Willebrand Factor Reveals Conservation of Domain Structure, Multimerization, and Intracellular Storage Arunima Ghosh,1 Andy Vo,2 Beverly K. Twiss,2 Colin A. Kretz,1 Mary A. Jozwiak,3 Robert R. Montgomery,3 and Jordan A. Shavit2 1 Life Sciences Institute, University of Michigan, Ann Arbor, MI 48109, USA 2 Department of Pediatrics, University of Michigan, Room 8301 Medical Science Research Building III, 1150 W. Medical Center Drive, Ann Arbor, MI 48109-5646, USA 3 Blood Research Institute, Medical College of Wisconsin, Milwaukee, WI 53226, USA Correspondence should be addressed to Jordan A. Shavit, jshavit@ 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. 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,