Title: Plasmin Cleavage of von Willebrand Factor as an Emergency Bypass for ADAMTS13 Deficiency in Thrombotic Microangiopathy
Authors: Tersteeg, Claudia ×
de Maat, Steven
De Meyer, Simon
Smeets, Michel W J
Barendrecht, Arjan D
Roest, Mark
Pasterkamp, Gerard
Fijnheer, Rob
Vanhoorelbeke, Karen
de Groot, Philip G
Maas, Coen #
Issue Date: Mar-2014
Publisher: Lippincott Williams & Wilkins
Series Title: Circulation vol:129 issue:12 pages:1320-1331
Abstract: Von Willebrand factor (VWF) multimer size is controlled through continuous proteolysis by ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type I motif, member 13). This prevents spontaneous platelet agglutination and microvascular obstructions. ADAMTS13 deficiency is associated with thrombotic thrombocytopenic purpura (TTP), where life-threatening episodes of microangiopathy damage kidneys, heart and brain. Enigmatically, a complete ADAMTS13 deficiency does not lead to continuous microangiopathy. We hypothesized that plasmin, the key enzyme of the fibrinolytic system, serves as a physiological backup enzyme for ADAMTS13 in the degradation of pathological platelet-VWF complexes.
ISSN: 0009-7322
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Non-KU Leuven Association publications
× corresponding author
# (joint) last author

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