Article
Biogenesis of Weibel-Palade bodies in von Willebrand's disease variants with impaired von Willebrand factor intrachain or interchain disulfide bond formation.
Haematologica - 1 Jun 2012
Wang Jiong-Wei, Groeneveld Dafna J, Cosemans Guy, Dirven Richard J, Valentijn Karine M, Voorberg Jan, Reitsma Pieter H, Eikenboom Jeroen
Abstract excerpt
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 no...
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