Absence of complement component 3 does not prevent classical pathway-mediated hemolysis

Blood Adv. 2019 Jun 25;3(12):1808-1814. doi: 10.1182/bloodadvances.2019031591.

Abstract

Complement component 3 (C3) is emerging as a potential therapeutic target. We studied complement-mediated hemolysis using normal and C3-depleted human sera, wild-type (WT) and C3-deficient rat sera, and WT and C3 knockout rat models. In all of the in vitro and in vivo experiments, we found that the loss of C3 did not prevent classical pathway-mediated hemolysis, but it did almost abolish alternative pathway-mediated hemolysis. Experiments using preassembled classical pathway C3 convertases confirmed that C4b2a directly activated complement component 5 (C5), leading to membrane attack complex formation and hemolysis. Our results suggest that targeting C3 should effectively inhibit hemolysis and tissue damage mediated by the alternative pathway of complement activation, but this approach might have limited efficacy in treating classical pathway-mediated pathological conditions.

Publication types

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

MeSH terms

  • Animals
  • Complement C3 / antagonists & inhibitors
  • Complement C3 / metabolism*
  • Complement C3 / therapeutic use
  • Complement C3-C5 Convertases / metabolism*
  • Complement C5 / drug effects
  • Complement C5 / metabolism*
  • Female
  • Hemolysis / drug effects
  • Hemolysis / immunology*
  • Humans
  • Male
  • Models, Animal
  • Rats

Substances

  • Complement C3
  • Complement C5
  • Complement C3-C5 Convertases