Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura.

@article{Furlan1997DeficientAO,
  title={Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura.},
  author={Miha Furlan and Rodolfo Robles and Max Solenthaler and Marcus Wassmer and Patrick Sandoz and Bernhard L{\"a}mmle},
  journal={Blood},
  year={1997},
  volume={89 9},
  pages={3097-103}
}
In patients with thrombotic thrombocytopenic purpura (TTP), excessive intravascular platelet aggregation has been associated with appearance in plasma of unusually large von Willebrand factor (vWF) multimers. These extremely adhesive vWF multimers may arise due to deficiency of a "depolymerase" cleaving vWF to smaller molecular forms, either by reducing the interdimeric disulfide bridges or by proteolytic degradation. We studied the activity of a recently described vWF-cleaving protease in four… CONTINUE READING

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