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Potential for Recombinant ADAMTS13 as an Effective Therapy for Acquired Thrombotic Thrombocytopenic Purpura
Author(s) -
Claudia Tersteeg,
Alexandra Schiviz,
Simon F. De Meyer,
Barbara Plaimauer,
Friedrich Scheiflinger,
Hanspeter Rottensteiner,
Karen Vanhoorelbeke
Publication year - 2015
Publication title -
arteriosclerosis thrombosis and vascular biology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.007
H-Index - 270
eISSN - 1524-4636
pISSN - 1079-5642
DOI - 10.1161/atvbaha.115.306014
Subject(s) - adamts13 , thrombotic thrombocytopenic purpura , medicine , recombinant dna , immunology , platelet , biology , biochemistry , gene
The metalloprotease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) regulates the size of von Willebrand factor multimers. A deficiency in ADAMTS13 activity is associated with the life-threatening disease thrombotic thrombocytopenic purpura (TTP). The vast majority of patients have acquired TTP, where circulating anti-ADAMTS13 autoantibodies are causative for the decreased ADAMTS13 activity. Current treatment consists of plasma exchange, but improved therapies are highly warranted.

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