In vitro interactions of c-ANCA (antibodies to proteinase 3) with human endothelial cells

Adv Exp Med Biol. 1993;336:109-13. doi: 10.1007/978-1-4757-9182-2_18.


Several concepts concerning the pathogenicity of antineutrophil cytoplasmic antibodies (ANCA) exist, but till now only sparse data about ANCA-endothelial interactions are available. In this study we have investigated the expression of proteinase 3 (PR-3) in human umbilical endothelial cells (HEC) using purified anti-PR-3 antibodies (C-ANCA) of patients with Wegener's granulomatosis (WG) and monoclonal antibodies to PR-3 (human and murine) as probes. Performing cyto-ELISAs, laser scanning microscopy and Western blot we were able to show that treatment of HEC with IL-1-alpha led to an increased PR-3 expression in the cytoplasm and to a transient translocation into the EC-membrane. Representing an important missing link of ANCA-endothelial interactions, our data give a hint at a possible direct pathogenicity of anti-PR-3 antibodies in WG and other vasculitides.

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Monoclonal / immunology
  • Autoantibodies / immunology*
  • Endothelium, Vascular / enzymology
  • Endothelium, Vascular / immunology*
  • Enzyme-Linked Immunosorbent Assay
  • Fluorescent Antibody Technique
  • Granulomatosis with Polyangiitis / immunology*
  • Humans
  • Immunoglobulin G / immunology*
  • Interleukin-1 / pharmacology
  • Myeloblastin
  • Serine Endopeptidases / analysis
  • Serine Endopeptidases / immunology*
  • Umbilical Cord


  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Monoclonal
  • Autoantibodies
  • Immunoglobulin G
  • Interleukin-1
  • Serine Endopeptidases
  • Myeloblastin