The incidence of thrombotic thrombocytopenic purpurahemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13
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).
protein is an enzyme, Willebrand factor-cleaving protease (vWFCP).
Structure-function and regulation of ADAMTS-13
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.).
Due to deficiency of ADAMTS-13
, ULMM can't be spliced to useful sized vWF-multimers.
Cleavage of ultra-large von Willebrand factor by ADAMTS-13
under flow conditions.