Loss of c-REL but not NF-κB2 prevents autoimmune disease driven by FasL mutation

Cell Death Differ. 2015 May;22(5):767-78. doi: 10.1038/cdd.2014.168. Epub 2014 Oct 31.

Abstract

FASL/FAS signaling imposes a critical barrier against autoimmune disease and lymphadenopathy. Mutant mice unable to produce membrane-bound FASL (FasL(Δm/Δm)), a prerequisite for FAS-induced apoptosis, develop lymphadenopathy and systemic autoimmune disease with immune complex-mediated glomerulonephritis. Prior to disease onset, FasL(Δm/Δm) mice contain abnormally high numbers of leukocytes displaying activated and elevated NF-κB-regulated cytokine levels, indicating that NF-κB-dependent inflammation may be a key pathological driver in this multifaceted autoimmune disease. We tested this hypothesis by genetically impairing canonical or non-canonical NF-κB signaling in FasL(Δm/Δm) mice by deleting the c-Rel or NF-κB2 genes, respectively. Although the loss of NF-κB2 reduced the levels of inflammatory cytokines and autoantibodies, the impact on animal survival was minor due to substantially accelerated and exacerbated lymphoproliferative disease. In contrast, a marked increase in lifespan resulting from the loss of c-REL coincided with a striking reduction in classical parameters of autoimmune pathology, including the levels of cytokines and antinuclear autoantibodies. Notably, the decrease in regulatory T-cell numbers associated with loss of c-REL did not exacerbate autoimmunity in FasL(Δm/Δm)c-rel(-/-) mice. These findings indicate that selective inhibition of c-REL may be an attractive strategy for the treatment of autoimmune pathologies driven by defects in FASL/FAS signaling that would be expected to circumvent many of the complications caused by pan-NF-κB inhibition.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Autoimmune Diseases / genetics
  • Autoimmune Diseases / metabolism*
  • Autoimmune Diseases / pathology
  • Autoimmune Diseases / prevention & control
  • Fas Ligand Protein / genetics
  • Fas Ligand Protein / metabolism*
  • Mice
  • Mice, Knockout
  • Mutation*
  • NF-kappa B p52 Subunit / genetics
  • NF-kappa B p52 Subunit / metabolism*
  • Proto-Oncogene Proteins c-rel / genetics
  • Proto-Oncogene Proteins c-rel / metabolism*
  • Signal Transduction / genetics
  • fas Receptor / genetics
  • fas Receptor / metabolism*

Substances

  • Fas Ligand Protein
  • Fas protein, mouse
  • Fasl protein, mouse
  • NF-kappa B p52 Subunit
  • Nfkb2 protein, mouse
  • Proto-Oncogene Proteins c-rel
  • fas Receptor