Genetic deletion of platelet glycoprotein Ib alpha but not its extracellular domain protects from atherosclerosis

Thromb Haemost. 2014 Dec;112(6):1252-63. doi: 10.1160/TH14-02-0130. Epub 2014 Aug 7.

Abstract

The pathogenesis of atherosclerosis involves the interplay of haematopoietic, stromal and endothelial cells. Platelet interactions with endothelium and leukocytes are pivotal for atherosclerosis promotion. Glycoprotein (GP) Ibα is the ligand-binding subunit of the platelet GPIb-IX-V receptor complex; its deficiency causes the Bernard-Soulier syndrome (BSS), characterised by absent platelet GPIb-IX-V, macrothrombocytopenia and bleeding. We designed this study to determine the role of platelet GPIbα in the pathogenesis of atherosclerosis using two unique knockout models. Ldlr-/- mice were reconstituted with wild-type (wt), GPIbα-/- (lacks GPIbα) or chimeric IL-4R/GPIbα-Tg (lacks GPIbα extracellular domain) bone marrow and assayed for atherosclerosis development after feeding with pro-atherogenic "western diet". Here, we report that Ldlr-/-mice reconstituted with GPIbα-/- bone marrow developed less atherosclerosis compared to wt controls; accompanied by augmented accumulation of pro-inflammatory CD11b+ and CD11c+ myeloid cells, reduced oxLDL uptake and decreased TNF and IL 12p35 gene expression in the aortas. Flow cytometry and live cell imaging in whole blood-perfused microfluidic chambers revealed reduced platelet-monocyte aggregates in GPIbα-/- mice, which resulted in decreased monocyte activation. Interestingly, Ldlr-/-mice reconstituted with IL-4R/GPIbα-Tg bone marrow, producing less abnormal platelets, showed atherosclerotic lesions similar to wt mice. Platelet interaction with blood monocytes and accumulation of myeloid cells in the aortas were also essentially unaltered. Moreover, only complete GPIbα ablation altered platelet microparticles and CCL5 chemokine production. Thus, atherosclerosis reduction in mice lacking GPIbα may not result from the defective GPIbα-ligand binding, but more likely is a consequence of functional defects of GPIbα-/- platelets and reduced blood platelet counts.

Keywords: Aorta; atherosclerosis; inflammation; myeloid cells; platelets.

Publication types

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

MeSH terms

  • Animals
  • Aortic Diseases / blood
  • Aortic Diseases / genetics
  • Aortic Diseases / pathology
  • Aortic Diseases / prevention & control*
  • Atherosclerosis / blood
  • Atherosclerosis / genetics
  • Atherosclerosis / pathology
  • Atherosclerosis / prevention & control*
  • Bernard-Soulier Syndrome / genetics
  • Bernard-Soulier Syndrome / metabolism*
  • Blood Platelets / metabolism
  • Bone Marrow Transplantation
  • CD11b Antigen / metabolism
  • CD11c Antigen / metabolism
  • Chemokine CCL5 / metabolism
  • Diet, Western
  • Disease Models, Animal
  • Female
  • Inflammation Mediators / metabolism
  • Interleukin-12 Subunit p35 / metabolism
  • Lipoproteins, LDL / metabolism
  • Male
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Monocytes / metabolism
  • Platelet Adhesiveness
  • Platelet Glycoprotein GPIb-IX Complex / genetics
  • Platelet Glycoprotein GPIb-IX Complex / metabolism*
  • Protein Structure, Tertiary
  • Receptors, Interleukin-4 / metabolism
  • Receptors, LDL / deficiency
  • Receptors, LDL / genetics
  • Tumor Necrosis Factor-alpha / metabolism

Substances

  • CD11b Antigen
  • CD11c Antigen
  • Ccl5 protein, mouse
  • Chemokine CCL5
  • Il12a protein, mouse
  • Inflammation Mediators
  • Interleukin-12 Subunit p35
  • Lipoproteins, LDL
  • Platelet Glycoprotein GPIb-IX Complex
  • Receptors, Interleukin-4
  • Receptors, LDL
  • Tumor Necrosis Factor-alpha
  • adhesion receptor
  • oxidized low density lipoprotein