Life in the shadow of a dominant partner: the FVIII-VWF association and its clinical implications for hemophilia A.

@article{Pipe2016LifeIT,
  title={Life in the shadow of a dominant partner: the FVIII-VWF association and its clinical implications for hemophilia A.},
  author={Steven W Pipe and Robert R. Montgomery and Kathleen P Pratt and Peter J Lenting and David P. Lillicrap},
  journal={Blood},
  year={2016},
  volume={128 16},
  pages={
          2007-2016
        }
}
A normal hemostatic response to vascular injury requires both factor VIII (FVIII) and von Willebrand factor (VWF). In plasma, VWF and FVIII normally circulate as a noncovalent complex, and each has a critical function in the maintenance of hemostasis. Furthermore, the interaction between VWF and FVIII plays a crucial role in FVIII function, immunogenicity, and clearance, with VWF essentially serving as a chaperone for FVIII. Several novel recombinant FVIII (rFVIII) therapies for hemophilia A… CONTINUE READING
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