Storage of Factor VIII Variants with Impaired von Willebrand Factor Binding in Weibel-Palade Bodies in Endothelial Cells

BACKGROUND: Point mutations resulting in reduced factor VIII (FVIII) binding to von Willebrand factor (VWF) are an important cause of mild/moderate hemophilia A. Treatment includes desmopressin infusion, which concomitantly increases VWF and FVIII plasma levels, apparently from storage pools contain...

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Autori principali: van den Biggelaar, Maartje, Bouwens, Eveline A. M., Voorberg, Jan, Mertens, Koen
Natura: Artigo
Lingua:Inglês
Pubblicazione: Public Library of Science 2011
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Research Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3166073/
https://ncbi.nlm.nih.gov/pubmed/21909383
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0024163
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