Article
The type 2B p.R1306W natural mutation of von Willebrand factor dramatically enhances the multimer sensitivity to shear stress.
Journal of thrombosis and haemostasis : JTH - 1 Sept 2013
Scaglione G L, Lancellotti S, Papi M, De Spirito M, Maiorana A, Baronciani L, Pagliari M T, Arcovito A, Di Stasio E, Peyvandi F, De Cristofaro R
Abstract excerpt
BACKGROUND: Shear stress triggers conformational stretching of von Willebrand factor (VWF), which is responsible for its self-association and binding to the platelet receptor glycoprotein (GP)Ibα. This phenomenon supports primary hemostasis under flow. Type 2B VWF natural mutants are considered to have increased affinity for platelet GPIbα. OBJECTIVES: To assess the mechanism responsible for the enhanced...
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