Analysis of the storage and secretion of von Willebrand factor in blood outgrowth endothelial cells derived from patients with von Willebrand disease-Reference-Cited by-同舟云学术

Analysis of the storage and secretion of von Willebrand factor in blood outgrowth endothelial cells derived from patients with von Willebrand disease

Published:2013-04-04 Issue:14 Volume:121 Page:2762-2772
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Wang Jiong-Wei¹²,Bouwens Eveline A. M.³⁴,Pintao Maria Carolina¹,Voorberg Jan³⁵,Safdar Huma¹,Valentijn Karine M.⁶,de Boer Hetty C.⁷,Mertens Koen³⁴,Reitsma Pieter H.¹,Eikenboom Jeroen¹

Affiliation:

1. Einthoven Laboratory for Experimental Vascular Medicine, Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands;

2. Laboratory of Experimental Cardiology, University Medical Centre Utrecht, Utrecht, The Netherlands;

3. Department of Plasma Proteins, Sanquin Research, Amsterdam, The Netherlands;

4. Department of Pharmaceutics, Utrecht Institute for Pharmaceutical Sciences, Utrecht University, Utrecht, The Netherlands;

5. Sanquin-Academic Medical Center Landsteiner Laboratory, Amsterdam, The Netherlands; and

6. Department of Molecular Cell Biology, and

7. Department of Nephrology, Leiden University Medical Center, Leiden, The Netherlands

Abstract

Key Points Isolation of BOECs from multiple patients with VWD is feasible, and the study of BOECs helps explain the pathogenic complexity of VWD. Abnormalities in WPB biogenesis and exocytosis and defects in VWF string formation correlate with the phenotypic features of patients with VWD.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/121/14/2762/1365537/2762.pdf

Reference43 articles.

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4. Cell biology of von Willebrand factor.;Wagner;Annu Rev Cell Biol,1990

5. Basal secretion of von Willebrand factor from human endothelial cells.;Giblin;Blood,2008

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