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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.
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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY
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
From the Department of Pediatrics, Medical College of Wisconsin, Milwaukee; the Blood Research Institute, Blood Center of Southeastern Wisconsin, Milwaukee; the Children's Research Institute; Children's Hospital of Wisconsin, Milwaukee; and the Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Chapel Hill.
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-AECs) by P-selectin, VWFpp, or VWF immunostaining and confocal microscopy. We demonstrate the reestablishment of Weibel-Palade bodies that recruit endogenous P-selectin by expressing wild-type VWF in VWD-AECs. 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.
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