The role of ADAMT-13 in platelet adhesion in flow: methods for diagnosis of thrombotic thrombocytopenic purpura.
Von willebrand factor (VWF) is synthesized and released from endothelial cells and megakaryocytes. VWF multimers that are immobilized on the activated endothelial cells and unfolded by high shear stress, undergo proteolysis by specific VWF-cleaving protease, ADAMTS-13. Severe deficiency of ADAMTS-13 is followed by accumulation of ultralarge VWF multimers and development of thrombotic thrombocytopenic purpura (TTP). Several methods for detection of ADAMTS-13 activity are proposed. However, all these assays are lacking the condition of shear stress, in which rapid cleavage of VWF multimeric strings by the ADAMTS-13 occurs. Impact- R method testing the effect of plasma on normal platelet adhesion under shear stress allows rapid screening of acute TTP and discrimination from other thrombotic microangiopathies.
Shenkman
Boris
B
Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel-Hashomer, Israel. Borshenk@sheba.health.gov.il
D051722:ADAM Proteins / Q000032:analysis* / Q000502:physiology*; D000071120:ADAMTS13 Protein; D019281:Dimerization; D018056:Hemorheology; D006801:Humans; D010973:Platelet Adhesiveness; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis*; D013314:Stress, Mechanical; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human