Crry deficiency in complement sufficient mice: C3 consumption occurs without associated renal injury

Mol Immunol. 2009 Feb;46(5):803-11. doi: 10.1016/j.molimm.2008.09.003. Epub 2008 Oct 22.

Abstract

The rodent-specific complement regulator complement receptor 1-related gene/protein-y (Crry) is critical for complement homeostasis. Gene deletion is 100% embryonically lethal; Crry-deficient (Crry(-/-)) mice were rescued by back-crossing onto C3 deficiency, confirming that embryo loss was complement mediated. In order to rescue viable Crry(-/-) mice without deleting C3, we have tested inhibition of C5 during gestation. Crry(+/-) females were given neutralizing anti-C5 mAb immediately prior to mating with Crry(+/-) males and C5 inhibition maintained through pregnancy. A single, healthy Crry(-/-) female was obtained and mating with Crry(+/-) males yielded healthy litters containing equal numbers of Crry(+/-) and Crry(-/-) pups. Inter-crossing Crry(-/-) mice yielded healthy litters of expected size. Although the mice were not anemic, exposure of Crry(-/-) erythrocytes to normal mouse serum caused C3 deposition and lysis, while transfusion into normal or C6(-/-) mice resulted in rapid clearance. Complement activity and C3 levels in Crry(-/-) mice were markedly reduced. Comparison with factor H deficient (CfH(-/-)) mice revealed similar levels of residual C3; however, unlike the CfH(-/-) mice, Crry(-/-) mice showed no evidence of renal injury, demonstrating distinct roles for these regulators in protecting the kidney.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antibodies, Monoclonal / pharmacology
  • Complement C3 / genetics
  • Complement C3 / immunology*
  • Complement C5 / antagonists & inhibitors
  • Complement C5 / genetics
  • Complement C5 / immunology
  • Complement C6 / genetics
  • Complement C6 / immunology
  • Complement Factor H / genetics
  • Complement Factor H / immunology
  • Crosses, Genetic
  • Embryo Loss / genetics
  • Embryo Loss / immunology
  • Female
  • Homeostasis / genetics
  • Homeostasis / immunology*
  • Kidney / immunology*
  • Kidney / injuries
  • Male
  • Mice
  • Mice, Knockout
  • Pregnancy
  • Receptors, Complement / genetics
  • Receptors, Complement / immunology*
  • Receptors, Complement 3b

Substances

  • Antibodies, Monoclonal
  • Complement C3
  • Complement C5
  • Complement C6
  • Cr1l protein, mouse
  • Receptors, Complement
  • Receptors, Complement 3b
  • Complement Factor H