Deletion of Crry and DAF on murine platelets stimulates thrombopoiesis and increases factor H-dependent resistance of peripheral platelets to complement attack

J Immunol. 2013 Mar 15;190(6):2886-95. doi: 10.4049/jimmunol.1202536. Epub 2013 Feb 6.

Abstract

Complement receptor 1-related gene/protein y (Crry) and decay-accelerating factor (DAF) are two murine membrane C3 complement regulators with overlapping functions. Crry deletion is embryonically lethal whereas DAF-deficient mice are generally healthy. Crry(-/-)DAF(-/-) mice were viable on a C3(-/-) background, but platelets from such mice were rapidly destroyed when transfused into C3-sufficient mice. In this study, we used the cre-lox system to delete platelet Crry in DAF(-/-) mice and studied Crry/DAF-deficient platelet development in vivo. Rather than displaying thrombocytopenia, Pf4-Cre(+)-Crry(flox/flox) mice had normal platelet counts and their peripheral platelets were resistant to complement attack. However, chimera mice generated with Pf4-Cre(+)-Crry(flox/flox) bone marrows showed platelets from C3(-/-) but not C3(+/+) recipients to be sensitive to complement activation, suggesting that circulating platelets in Pf4-Cre(+)-Crry(flox/flox) mice were naturally selected in a complement-sufficient environment. Notably, Pf4-Cre(+)-Crry(flox/flox) mouse platelets became complement susceptible when factor H function was blocked. Examination of Pf4-Cre(+)-Crry(flox/flox) mouse bone marrows revealed exceedingly active thrombopoiesis. Thus, under in vivo conditions, Crry/DAF deficiency on platelets led to abnormal platelet turnover, but peripheral platelet count was compensated for by increased thrombopoiesis. Selective survival of Crry/DAF-deficient platelets aided by factor H protection and compensatory thrombopoiesis demonstrates the cooperation between membrane and fluid phase complement inhibitors and the body's ability to adaptively respond to complement regulator deficiencies.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Blood Platelets / cytology
  • Blood Platelets / immunology*
  • Blood Platelets / metabolism
  • CD55 Antigens / blood
  • CD55 Antigens / genetics*
  • Cell Survival / genetics
  • Cell Survival / immunology
  • Complement C3 / biosynthesis
  • Complement C3 / deficiency
  • Complement Factor H / deficiency
  • Complement Factor H / genetics
  • Complement Factor H / physiology*
  • Complement Pathway, Alternative / genetics
  • Complement Pathway, Alternative / immunology*
  • Down-Regulation / genetics
  • Down-Regulation / immunology*
  • Humans
  • Megakaryocytes / immunology
  • Megakaryocytes / metabolism
  • Mice
  • Mice, 129 Strain
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Mice, Transgenic
  • Models, Animal
  • Protein Binding / genetics
  • Protein Binding / immunology
  • Random Allocation
  • Receptors, Complement / blood
  • Receptors, Complement / deficiency*
  • Receptors, Complement / genetics
  • Receptors, Complement 3b
  • Thrombopoiesis / genetics
  • Thrombopoiesis / immunology*
  • Up-Regulation / genetics
  • Up-Regulation / immunology*

Substances

  • CD55 Antigens
  • CR1L protein, human
  • Complement C3
  • Receptors, Complement
  • Receptors, Complement 3b
  • Complement Factor H