Molecular basis of sickle cell-endothelial cell interactions

Academic Article


  • Adherence of sickle erythrocytes to microvascular endothelium is posited to initiate or contribute to sickle cell vaso-occlusive pain episodes. Adherence and occlusion in vivo may depend on hemodynamics interacting with plasma, erythrocyte, and endothelial cell factors. Four receptor-mediated adherence pathways have been described to date: adherence mediated by high molecular weight von Willebrand factor multimers bridging glycoprotein Ib- like and integrin receptors on sickle cells and similar receptors on endothelial cells; thrombospondin bridging CD36 on sickle reticulocytes and the α(v)β3 integrin on large-vessel endothelial cells or α(v)β3 and CD36 on microvascular endothelium; binding of sickle reticulocyte α4β1 receptors to vascular cell adhesion molecule 1 expressed on endothelial cells stimulated by cytokine or double-stranded RNA viruses; and binding of sickle cells to endothelial cell-associated fibronectin via sickle reticulocyte α4β1 activated by phorbol ester or interleukin-8. The significance of these adherence pathways in sickle cell vaso-occlusion is discussed.
  • Published In

    Digital Object Identifier (doi)

    Author List

  • Wick TM; Eckman JR
  • Start Page

  • 118
  • End Page

  • 124
  • Volume

  • 3
  • Issue

  • 2