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...
| Autores principales: | , , , , , , , |
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| Formato: | Artículo |
| Lenguaje: | English |
| Publicado: |
Ferrata Storti Foundation
2012-06-01
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| Colección: | Haematologica |
| Acceso en línea: | https://haematologica.org/article/view/6324 |