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Blood, 21 May 2009, Vol. 113, No. 21, pp. 5049-5057. Prepublished online as a Blood First Edition Paper on March 24, 2009; DOI 10.1182/blood-2008-10-165621. This Article Full Text (PDF) All Versions of this Article: blood-2008-10-165621v1 113/21/5049    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 De Meyer, S. F. Articles by Vanhoorelbeke, K. Search for Related Content PubMed PubMed Citation Articles by De Meyer, S. F. Articles by Vanhoorelbeke, K. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted October 31, 2008 Accepted February 26, 2009 Von Willebrand factor to the rescue Simon F. De Meyer*, Hans Deckmyn, and Karen Vanhoorelbeke Laboratory for Thrombosis Research, IRC, KULeuven Campus Kortrijk, Kortrijk, Belgium * Corresponding author; email: simon.demeyer{at}kuleuven-kortrijk.be. Von Willebrand factor (VWF) is a large multimeric adhesive glycoprotein with complex roles in thrombosis and hemostasis. Abnormalities in VWF give rise to a variety of bleeding complications, known as von Willebrand disease (VWD), the most common inherited bleeding disorder in man. Current treatment of VWD is based on the replacement of the deficient or dysfunctional protein either by endogenous release from endothelial Weibel-Palade bodies or by administration of plasma-derived VWF concentrates. During the last years, several efforts have been made to optimize existing therapies for VWD, but also to devise new approaches, such as inducing endogenous expression with interleukin-11, administering exogenous recombinant VWF, or introducing the protein via gene delivery. Clearly, the efficacy of any strategy will depend on several factors, including, for example, the quantity, activity and stability of the delivered VWF. The inherent complexity of VWF biosynthesis, which involves extensive post-translational processing, may be limiting in terms of producing active VWF outside of its native cellular sources. This review summarizes recent progress in the development of different treatment strategies for VWD, including those that are established, and those that are at the experimental stage. Potential pitfalls and benefits of each strategy are discussed. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: F. Rodeghiero, G. Castaman, and A. Tosetto How I treat von Willebrand disease Blood, August 6, 2009; 114(6): 1158 - 1165. [Abstract] [Full Text] [PDF]

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