Extensive complement activation in hereditary porcine membranoproliferative glomerulonephritis type II (porcine dense deposit disease)

Am J Pathol. 1993 Nov;143(5):1356-65.


Massive glomerular deposits of C3 and the terminal C5b-9 complement complex (TCC), but no immune complex deposits were detected by immunofluorescence in porcine membranoproliferative glomerulonephritis type II. TCC deposits were always observed with concomitant deposits of vitronectin (S-protein) in membranoproliferative glomerulonephritis, in contrast to a piglet with mesangial glomerulopathy where TCC was present without vitronectin co-deposition. Enzyme immunoassays revealed extensive systemic complement activation in 1-week-old affected piglets, observed by low plasma C3 (about 5% of normal) and high plasma TCC (about 10 x normal). Affected piglets revealed some plasma complement activation already at birth, 3 to 4 weeks before recognizable clinical disease. It is concluded that porcine membranoproliferative glomerulonephritis represents a nonimmune complex-mediated glomerulonephritis caused by unrestricted systemic complement activation with C3 consumption, TCC formation, and glomerular trapping of complement activation products. A pathogenetic mechanism of a defective or missing complement regulation protein is suggested.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Complement C3 / analysis*
  • Complement C3 / immunology
  • Complement C3 / urine
  • Complement Membrane Attack Complex / analysis*
  • Complement Membrane Attack Complex / immunology
  • Complement Membrane Attack Complex / urine
  • Cross Reactions / immunology*
  • Disease Models, Animal
  • Fluorescent Antibody Technique
  • Glomerulonephritis, Membranoproliferative / genetics
  • Glomerulonephritis, Membranoproliferative / immunology*
  • Humans
  • Kidney Glomerulus / chemistry*
  • Swine


  • Complement C3
  • Complement Membrane Attack Complex