Interplay between ADAMTS13 and von Willebrand factor in inherited and acquired thrombotic microangiopathies.

@article{Soejima2005InterplayBA,
  title={Interplay between ADAMTS13 and von Willebrand factor in inherited and acquired thrombotic microangiopathies.},
  author={Kenji Soejima and Tomohiro Nakagaki},
  journal={Seminars in hematology},
  year={2005},
  volume={42 1},
  pages={56-62}
}
The presence of unusually large multimers of von Willebrand factor (VWF) is thought to be a major pathogenic factor for thrombotic thrombocytopenic purpura (TTP). ADAMTS13 is a protease that regulates the multimeric size and function of VWF by cleaving VWF. Hence, congenital or acquired deficiency of ADAMTS13 causes life-threatening illness of TTP. Mutations in the ADAMTS13 gene cause inherited TTP, and the development of autoantibodies that inhibit ADAMTS13 activity frequently are associated… CONTINUE READING
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