Cargando…
Structures of VWF tubules before and after concatemerization reveal a mechanism of disulfide bond exchange
von Willebrand factor (VWF) is an adhesive glycoprotein that circulates in the blood as disulfide-linked concatemers and functions in primary hemostasis. The loss of long VWF concatemers is associated with the excessive bleeding of type 2A von Willebrand disease (VWD). Formation of the disulfide bon...
Autores principales: | Anderson, Jacob R., Li, Jing, Springer, Timothy A., Brown, Alan |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Society of Hematology
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9507011/ https://www.ncbi.nlm.nih.gov/pubmed/35776905 http://dx.doi.org/10.1182/blood.2022016467 |
Ejemplares similares
-
Novel cysteine substitution p.(Cys1084Tyr) causes variable expressivity of qualitative and quantitative VWF defects
por: Rawley, Orla, et al.
Publicado: (2022) -
SIPA in 10 milliseconds: VWF tentacles agglomerate and capture platelets under high shear
por: Liu, Zixiang Leonardo, et al.
Publicado: (2022) -
Multifaceted pathomolecular mechanism of a VWF large deletion involved in the pathogenesis of severe VWD
por: Yadegari, Hamideh, et al.
Publicado: (2022) -
von Willebrand factor antigen levels are associated with burden of rare nonsynonymous variants in the VWF gene
por: Sadler, Brooke, et al.
Publicado: (2021) -
Impaired exercise capacity in post–COVID-19 syndrome: the role of VWF-ADAMTS13 axis
por: Prasannan, Nithya, et al.
Publicado: (2022)