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Blood, 1 January 2005, Vol. 105, No. 1, pp. 145-152. Prepublished online as a Blood First Edition Paper on August 26, 2004; DOI 10.1182/blood-2004-02-0464. This Article Full Text (PDF) All Versions of this Article: 2004-02-0464v1 105/1/145    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Haberichter, S. L Articles by Montgomery, R. R Search for Related Content PubMed PubMed Citation Articles by Haberichter, S. L Articles by Montgomery, R. R Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted February 5, 2004 Accepted August 12, 2004 Re-establishment of VWF-dependent Weibel-Palade bodies in VWD endothelial cells Sandra L Haberichter*, Elizabeth P Merricks, Scot A Fahs, Pamela A Christopherson, Timothy C Nichols, and Robert R Montgomery Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA; Children's Research Institute, Children's Hospital of Wisconsin, Milwaukee, WI, USA Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Chapel Hill, NC, USA Blood Research Institute, Blood Center of Southeastern Wisconsin, Milwaukee, WI, USA Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA; Blood Research Institute, Blood Center of Southeastern Wisconsin, Milwaukee, WI, USA; Children's Research Institute, Children's Hospital of Wisconsin, Milwaukee, WI, USA * Corresponding author; email: shaberic{at}mcw.edu. Type 3 von Willebrand disease (VWD) is a severe hemorrhagic defect in humans. We now identify the homozygous mutation in the Chapel Hill strain of canine type 3 VWD that results in premature termination of von Willebrand factor (VWF) protein synthesis. We cultured endothelium from VWD and normal dogs to study intracellular VWF trafficking and Weibel-Palade body formation. Weibel-Palade bodies could not be identified in the canine VWD aortic endothelial cells (VWD-AEC) by P-selectin, VWFpp, or VWF immunostaining and confocal microscopy. We demonstrate the re-establishment of Weibel-Palade bodies that recruit endogenous P-selectin by expressing wild-type VWF in VWD-AEC. Expression of mutant VWF proteins confirmed that VWF multimerization is not necessary for Weibel-Palade body creation. Although the VWF propeptide is required for the formation of Weibel-Palade bodies, it cannot independently induce the formation of the granule. These VWF-null endothelial cells provide a unique opportunity to examine the biogenesis of Weibel-Palade bodies in endothelium from a canine model of type 3 VWD. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: J. Hol, A. M. Kuchler, F.-E. Johansen, B. Dalhus, G. Haraldsen, and I. Oynebraten Molecular Requirements for Sorting of the Chemokine Interleukin-8/CXCL8 to Endothelial Weibel-Palade Bodies J. Biol. Chem., August 28, 2009; 284(35): 23532 - 23539. [Abstract] [Full Text] [PDF] M. G. Rondaij, R. Bierings, E. L. van Agtmaal, K. A. Gijzen, E. Sellink, A. Kragt, S. S. G. Ferguson, K. Mertens, M. J. Hannah, J. A. van Mourik, et al. Guanine exchange factor RalGDS mediates exocytosis of Weibel-Palade bodies from endothelial cells Blood, July 1, 2008; 112(1): 56 - 63. [Abstract] [Full Text] [PDF] R.-H. Huang, Y. Wang, R. Roth, X. Yu, A. R. Purvis, J. E. Heuser, E. H. Egelman, and J. E. Sadler Assembly of Weibel Palade body-like tubules from N-terminal domains of von Willebrand factor PNAS, January 15, 2008; 105(2): 482 - 487. [Abstract] [Full Text] [PDF] D. J. Metcalf, T. D. Nightingale, H. L. Zenner, W. W. Lui-Roberts, and D. F. Cutler Formation and function of Weibel-Palade bodies J. Cell Sci., January 1, 2008; 121(1): 19 - 27. [Abstract] [Full Text] [PDF] R. R. Montgomery A package for VWD endothelial cells Blood, June 15, 2006; 107(12): 4580 - 4581. [Full Text] [PDF] S. F. De Meyer, K. Vanhoorelbeke, M. K. Chuah, I. Pareyn, V. Gillijns, R. P. Hebbel, D. Collen, H. Deckmyn, and T. VandenDriessche Phenotypic correction of von Willebrand disease type 3 blood-derived endothelial cells with lentiviral vectors expressing von Willebrand factor Blood, June 15, 2006; 107(12): 4728 - 4736. [Abstract] [Full Text] [PDF] M. G. Rondaij, R. Bierings, A. Kragt, J. A. van Mourik, and J. Voorberg Dynamics and Plasticity of Weibel-Palade Bodies in Endothelial Cells Arterioscler Thromb Vasc Biol, May 1, 2006; 26(5): 1002 - 1007. [Abstract] [Full Text] [PDF]

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