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Biogenesis of Weibel-Palade bodies in von Willebrand’s disease variants with impaired von Willebrand factor intrachain or interchain disulfide bond formation

Background Mutations of cysteine residues in von Willebrand factor are known to reduce the storage and secretion of this factor, thus leading to reduced antigen levels. However, one cysteine mutation, p.Cys2773Ser, has been found in patients with type 2A(IID) von Willebrand’s disease who have normal...

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Auteurs principaux: Jiong-Wei Wang, Dafna J. Groeneveld, Guy Cosemans, Richard J. Dirven, Karine M. Valentijn, Jan Voorberg, Pieter H. Reitsma, Jeroen Eikenboom
Format: Artigo
Langue:Inglês
Publié: Ferrata Storti Foundation 2012-06-01
Collection:Haematologica
Accès en ligne:https://haematologica.org/article/view/6324
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