ADAMTSbd13

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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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References in periodicals archive ?
The incidence of thrombotic thrombocytopenic purpurahemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency.
A study reported that ADAMTS-13 has the capacity to facilitate angiogenesis when it is in its full-length form and promotes tube formation, proliferation, and migration of human umbilical vein endothelial cells (28).
The ADAMTS-13 protein is an enzyme, Willebrand factor-cleaving protease (vWFCP).
Structure-function and regulation of ADAMTS-13 protease.
TTP results from immunoglobulin G (IgG) and autoantibodies to von Willebrand factor-cleaving protease (ADAMTS-13).
Nolasco et al., "ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions," Blood, vol.
Cinotti et al., "ADAMTS-13 activity in the presence of elevated von Willebrand factor levels as a novel mechanism of residual platelet reactivity in high risk coronary patients on antiplatelet treatment," Thrombosis Research, vol.
A presumptive diagnosis of TTP was made, and plasma exchange was initiated after an ADAMTS-13 level was drawn.
There is no sharp boundary between the markers and biomarkers of endothelial cells, since some of them are not only expressed at the surface, but cut off the cells (shedding) to the circulation (ACE, VWF, ADAMTS-13, soluble adhesion molecules, etc.).