The role of ADAMT-13 in platelet adhesion in flow: methods for diagnosis of thrombotic thrombocytopenic purpura

Pathophysiol Haemost Thromb. 2006;35(1-2):98-102. doi: 10.1159/000093550.

Abstract

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.

Publication types

  • Review

MeSH terms

  • ADAM Proteins / analysis*
  • ADAM Proteins / physiology*
  • ADAMTS13 Protein
  • Dimerization
  • Hemorheology
  • Humans
  • Platelet Adhesiveness*
  • Purpura, Thrombotic Thrombocytopenic / diagnosis*
  • Stress, Mechanical
  • von Willebrand Factor / metabolism

Substances

  • von Willebrand Factor
  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human