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Modeling complement activation on human glomerular microvascular endothelial cells.

Kes H Stevens1, Laura M Baas1, Thea J A M van der Velden1

  • 1Department of Pediatric Nephrology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, Netherlands.

Frontiers in Immunology
|November 13, 2023
PubMed
Summary

A new ex vivo model effectively measures complement deposition in atypical hemolytic uremic syndrome (aHUS). This tool aids in understanding aHUS pathophysiology and endothelial complement activation in kidney disease.

Keywords:
C5b-9alternative pathwayatypical hemolytic uremic syndromecomplement systemeculizumabglomerular endothelium

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Area of Science:

  • Nephrology
  • Immunology
  • Cell Biology

Background:

  • Atypical hemolytic uremic syndrome (aHUS) is a rare kidney disease.
  • It stems from complement alternative pathway dysregulation, damaging glomerular endothelium.
  • Understanding aHUS pathophysiology requires effective models for endothelial complement deposition.

Purpose of the Study:

  • To validate an ex vivo model for measuring complement deposition.
  • To assess complement deposition on human glomerular microvascular endothelial cells (GMVECs) from controls and aHUS patients.
  • To investigate the utility of this model in studying aHUS.

Main Methods:

  • Human test sera were incubated with GMVECs.
  • Complement deposition (C5b-9) was visualized and quantified using immunofluorescence microscopy.
  • The model was validated using zymosan-activated serum, NHS, and the C5 inhibitor eculizumab.

Main Results:

  • The model showed increased C5b-9 deposition in zymosan-activated serum compared to NHS.
  • Atypical hemolytic uremic syndrome patient sera exhibited elevated C5b-9 deposition during acute phases.
  • Remission samples showed normalized deposition, and the model responded to eculizumab treatment.

Conclusions:

  • A robust ex vivo model was developed to measure C5b-9 complement deposition on GMVECs.
  • This model accurately reflects complement deposition in aHUS.
  • The model serves as a valuable tool for studying aHUS and other diseases involving endothelial complement activation.