Characterization of recombinant von Willebrand factor corresponding to mutations in type IIA and type IIB von Willebrand disease.

@article{Ribba1992CharacterizationOR,
  title={Characterization of recombinant von Willebrand factor corresponding to mutations in type IIA and type IIB von Willebrand disease.},
  author={A S Ribba and Jan Voorberg and Dominique Meyer and H. Pannekoek and Genevi{\`e}ve Pi{\'e}tu},
  journal={The Journal of biological chemistry},
  year={1992},
  volume={267 32},
  pages={23209-15}
}
Type IIA and IIB von Willebrand disease (vWD) result from defects in von Willebrand factor (vWF). Although both type IIA and IIB vWD are characterized by the absence of high molecular weight multimers in plasma, vWF from patients with type IIA vWD demonstrates a decreased affinity for the platelet receptor glycoprotein Ib (GPIb), whereas vWF from patients with type IIB vWD show an increased affinity for GPIb. To investigate how structural alterations in vWF affect its interaction with GPIb, we… CONTINUE READING

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