Role for platelet glycoprotein Ib-IX and effects of its inhibition in endotoxemia-induced thrombosis, thrombocytopenia, and mortality

Arterioscler Thromb Vasc Biol. 2013 Nov;33(11):2529-37. doi: 10.1161/ATVBAHA.113.302339. Epub 2013 Sep 19.

Abstract

Objective: Poor prognosis of sepsis is associated with bacterial lipopolysaccharide (LPS)-induced intravascular inflammation, microvascular thrombosis, thrombocytopenia, and disseminated intravascular coagulation. Platelets are critical for thrombosis, and there has been increasing evidence of the importance of platelets in endotoxemia. The platelet adhesion receptor, the glycoprotein Ib-IX complex (GPIb-IX), mediates platelet adhesion to inflammatory vascular endothelium and exposed subendothelium. Thus, we have investigated the role of GPIb-IX in LPS-induced platelet adhesion, thrombosis, and thrombocytopenia.

Approach and results: LPS-induced mortality is significantly decreased in mice expressing a functionally deficient mutant of GPIbα. Furthermore, we have developed a micellar peptide inhibitor, MPαC (C13H27CONH-SIRYSGHpSL), which selectively inhibits the von Willebrand factor -binding function of GPIb-IX and GPIb-IX-mediated platelet adhesion under flow without affecting GPIb-IX-independent platelet activation. MPαC inhibits platelet adhesion to LPS-stimulated endothelial cells in vitro and alleviates LPS-induced thrombosis in glomeruli in mice. Importantly, MPαC reduces mortality in LPS-challenged mice, suggesting a protective effect of this inhibitor during endotoxemia. Interestingly, MPαC, but not the integrin antagonist, Integrilin, alleviated LPS-induced thrombocytopenia.

Conclusions: These data indicate an important role for the platelet adhesion receptor GPIb-IX in LPS-induced thrombosis and thrombocytopenia, and suggest the potential of targeting GPIb as an antiplatelet strategy in managing endotoxemia.

Keywords: GPIb-IX; endotoxemia; lipopolysaccharide; platelet; platelet inhibitor; thrombocytopenia; thrombosis.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Endothelium, Vascular / metabolism
  • Endotoxemia / drug therapy
  • Endotoxemia / metabolism*
  • Endotoxemia / mortality
  • Humans
  • Lipopolysaccharides / pharmacology
  • Membrane Glycoproteins / antagonists & inhibitors
  • Membrane Glycoproteins / genetics
  • Membrane Glycoproteins / metabolism*
  • Mice
  • Mice, Transgenic
  • Peptides / pharmacology
  • Platelet Adhesiveness / drug effects
  • Platelet Adhesiveness / physiology
  • Platelet Aggregation Inhibitors / pharmacology
  • Platelet Glycoprotein GPIb-IX Complex / antagonists & inhibitors
  • Platelet Glycoprotein GPIb-IX Complex / genetics
  • Platelet Glycoprotein GPIb-IX Complex / metabolism*
  • Recombinant Fusion Proteins / genetics
  • Recombinant Fusion Proteins / metabolism
  • Thrombocytopenia / drug therapy
  • Thrombocytopenia / metabolism*
  • Thrombocytopenia / mortality
  • Thrombosis / drug therapy
  • Thrombosis / metabolism*
  • Thrombosis / mortality
  • von Willebrand Factor / metabolism

Substances

  • Lipopolysaccharides
  • Membrane Glycoproteins
  • Peptides
  • Platelet Aggregation Inhibitors
  • Platelet Glycoprotein GPIb-IX Complex
  • Recombinant Fusion Proteins
  • adhesion receptor
  • von Willebrand Factor