| VWF propeptide and ratios between VWF, VWF propeptide and FVIII in the characterization of type 1 von Willebrand disease. | |
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MedLine Citation:
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PMID: 23349392 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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During post-translational modifications of von Willebrand factor (VWF) the VWF propeptide (VWFpp) is cleaved. The ratio between VWFpp and VWF antigen (VWF:Ag) and the ratio between factor VIII (FVIII:C) and VWF:Ag may be used to assess synthesis and clearance of VWF. We analyzed the contribution of VWFpp and ratios of VWFpp/VWF:Ag and FVIII:C/VWF:Ag in the pathophysiological characterization of type 1 VWD in the MCMDM-1VWD study. The VWFpp/VWF:Ag and FVIII:C/VWF:Ag ratios were increased among patients compared with unaffected family members and healthy controls. The VWFpp/VWF:Ag ratio was higher in individuals heterozygous for missense mutations than in those heterozygous for null alleles. In contrast, the FVIII:C/VWF:Ag ratio was highest among heterozygotes for VWF null alleles. The ratios of VWFpp/VWF:Ag and FVIII:C/VWF:Ag indicate that the pathophysiological mechanisms of type 1 VWD include reduced production and accelerated clearance of VWF, but that often a combination of both mechanisms is implicated. |
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Authors:
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Jeroen Eikenboom; Augusto B Federici; Richard J Dirven; Giancarlo Castaman; Francesco Rodeghiero; Ulrich Budde; Reinhard Schneppenheim; Javier Batlle; Maria Teresa Canciani; Jenny Goudemand; Ian Peake; Anne Goodeve |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2013-1-24 |
Journal Detail:
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Title: Blood Volume: - ISSN: 1528-0020 ISO Abbreviation: Blood Publication Date: 2013 Jan |
Date Detail:
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Created Date: 2013-1-25 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7603509 Medline TA: Blood Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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Einthoven Laboratory for Experimental Vascular Medicine, Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, Netherlands; |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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