Use of a mouse model to elucidate the phenotypic effects of the von Willebrand factor cleavage mutants, Y1605A/M1606A and R1597W.

@article{Pruss2012UseOA,
  title={Use of a mouse model to elucidate the phenotypic effects of the von Willebrand factor cleavage mutants, Y1605A/M1606A and R1597W.},
  author={Cynthia M. Pruss and Mia Golder and Andrea R Bryant and Carol Hegadorn and Sandra L. Haberichter and David Lillicrap},
  journal={Journal of thrombosis and haemostasis : JTH},
  year={2012},
  volume={10 5},
  pages={940-50}
}
BACKGROUND von Willebrand Factor (VWF) is tightly regulated by the metalloproteinase ADAMTS13, which cleaves VWF to reduce VWF multimer size and binding affinity for collagen and platelets. OBJECTIVE This study examines two VWF mutations, R1597W (enhanced cleavage) and Y1605A-M1606A (decreased cleavage), to determine their impact on VWF, in addition to ADAMTS13-mediated cleavage. METHODS In vitro mouse ADAMTS13 digestions were performed on recombinant proteins. VWF knockout mice received… CONTINUE READING