Plasmodium falciparum erythrocyte membrane protein 1 variants induce cell swelling and disrupt the blood-brain barrier in cerebral malaria

J Exp Med. 2021 Mar 1;218(3):e20201266. doi: 10.1084/jem.20201266.

Abstract

Cerebral malaria (CM) is caused by the binding of Plasmodium falciparum-infected erythrocytes (IEs) to the brain microvasculature, leading to inflammation, vessel occlusion, and cerebral swelling. We have previously linked dual intercellular adhesion molecule-1 (ICAM-1)- and endothelial protein C receptor (EPCR)-binding P. falciparum parasites to these symptoms, but the mechanism driving the pathogenesis has not been identified. Here, we used a 3D spheroid model of the blood-brain barrier (BBB) to determine unexpected new features of IEs expressing the dual-receptor binding PfEMP1 parasite proteins. Analysis of multiple parasite lines shows that IEs are taken up by brain endothelial cells in an ICAM-1-dependent manner, resulting in breakdown of the BBB and swelling of the endothelial cells. Via ex vivo analysis of postmortem tissue samples from CM patients, we confirmed the presence of parasites within brain endothelial cells. Importantly, this discovery points to parasite ingress into the brain endothelium as a contributing factor to the pathology of human CM.

Publication types

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

MeSH terms

  • Adult
  • Animals
  • Blood-Brain Barrier / pathology*
  • Endocytosis
  • Endothelial Cells / metabolism
  • Endothelial Cells / ultrastructure
  • Endothelial Protein C Receptor / metabolism
  • Erythrocytes / parasitology
  • Erythrocytes / pathology
  • Humans
  • Intercellular Adhesion Molecule-1 / metabolism
  • Malaria, Cerebral / parasitology*
  • Malaria, Cerebral / pathology*
  • Microvilli / metabolism
  • Models, Biological
  • Molecular Docking Simulation
  • Parasites / metabolism
  • Plasmodium falciparum / isolation & purification
  • Plasmodium falciparum / ultrastructure
  • Protein Binding
  • Protein Isoforms / metabolism
  • Protozoan Proteins / genetics*
  • Rats
  • Spheroids, Cellular / metabolism
  • Spheroids, Cellular / pathology

Substances

  • Endothelial Protein C Receptor
  • Protein Isoforms
  • Protozoan Proteins
  • erythrocyte membrane protein 1, Plasmodium falciparum
  • Intercellular Adhesion Molecule-1