ADAMTSbd13

(redirected from VWFCP)

ADAMTSbd13

An ADAMTSs family enzyme encoded by ADAMTS13 on chromosome 9q34, which is identical to vWFCP (von Willebrand factor-cleaving protease, the name by which it is more commonly known in haematology). ADAMTS13/vWFCP degrades large vWF multimers by cleaving monomeric subunits, the absence of which (due to mutations) causes thrombotic thrombocytopenic purpura.
ADAMTS13 mRNA is expressed in liver, placenta, ovary, various other tissues, and in platelets.
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The ADAMTS-13 protein is an enzyme, Willebrand factor-cleaving protease (vWFCP).
TTP is associated with an acquired or an inherited deficiency of the von Willebrand factor - cleaving protease ADAMTS 13.8 Decreased von Willebrand factor cleaving protease activity (VWFCP, ADAMTS13) leads to persistence of unusually large multimers of von Willebrand factor (vWF) that bind to platelets, causing platelet aggregates, and multiple platelet thrombi in many organs.
(9) Furlan called this activity VWF-cleaving protease (VWFcp).