Platelet activation and the formation of the platelet plug: deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura

Arterioscler Thromb Vasc Biol. 2003 Mar 1;23(3):388-96. doi: 10.1161/01.ATV.0000058401.34021.D4. Epub 2003 Jan 30.

Abstract

In the circulation, a plasma metalloprotease, ADAMTS13, cleaves von Willebrand factor (vWF) in a shear-dependent manner. This article reviews the role of this cleavage in regulating vWF-platelet interaction and proposes a scheme for understanding how a deficiency of ADAMTS13 results in the development of microthrombi in patients with thrombotic thrombocytopenic purpura.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • ADAM Proteins
  • ADAMTS13 Protein
  • Child
  • Endoplasmic Reticulum / metabolism
  • Hemolytic-Uremic Syndrome / physiopathology
  • Humans
  • Metalloendopeptidases / deficiency*
  • Metalloendopeptidases / genetics
  • Mutation
  • Platelet Activation*
  • Purpura, Thrombotic Thrombocytopenic / diagnosis
  • Purpura, Thrombotic Thrombocytopenic / etiology*
  • Purpura, Thrombotic Thrombocytopenic / metabolism*
  • Stress, Mechanical
  • Thrombosis / metabolism
  • von Willebrand Factor / metabolism

Substances

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