Anti-neutrophil cytoplasm antibodies can recognize vascular endothelial cell-bound anti-neutrophil cytoplasm antibody-associated autoantigens

Exp Nephrol. 1993 May-Jun;1(3):190-5.


Anti-neutrophil cytoplasm antibodies (ANCA) are strongly associated with the development of systemic vasculitis. Myeloperoxidase and proteinase-3 have been identified as targets for P-ANCA and C-ANCA, respectively. Both enzymes are released from neutrophil azurophil granules following neutrophil activation and both are highly cationic. Purified myeloperoxidase is demonstrated to bind non-convalently to endothelial cell membranes, to retain its enzymic function following binding, and to retain its antigenicity for P-ANCA. Endothelial cell-bound myeloperoxidase enhances complement-dependent cytotoxicity of some P-ANCA sere that also contain anti-endothelial cell antibodies. Studies using purified proteinase-3 show that it also can bind to endothelial cells and be recognized by C-ANCA. The interactions of myeloperoxidase and proteinase-3 with endothelial cells and ANCA may thus contribute to the development of vascular injury in patients with systemic vasculitis.

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies / immunology*
  • Autoantigens / immunology*
  • Cells, Cultured
  • Complement System Proteins / immunology
  • Cytoplasm / immunology
  • Cytotoxicity, Immunologic
  • Endothelium, Vascular / drug effects
  • Endothelium, Vascular / immunology*
  • Fluorescent Antibody Technique
  • Granulomatosis with Polyangiitis / etiology
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Hydrogen Peroxide / pharmacology
  • In Vitro Techniques
  • Myeloblastin
  • Neutrophils / enzymology
  • Neutrophils / immunology*
  • Peroxidase / immunology
  • Peroxidase / pharmacology
  • Polyarteritis Nodosa / etiology
  • Polyarteritis Nodosa / immunology
  • Serine Endopeptidases / immunology


  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Autoantigens
  • Complement System Proteins
  • Hydrogen Peroxide
  • Peroxidase
  • Serine Endopeptidases
  • Myeloblastin