c-FLIP Expression in Foxp3-Expressing Cells Is Essential for Survival of Regulatory T Cells and Prevention of Autoimmunity

Cell Rep. 2017 Jan 3;18(1):12-22. doi: 10.1016/j.celrep.2016.12.022.

Abstract

Regulatory T (Treg) cells are critical for the shutdown of immune responses and have emerged as valuable targets of immunotherapies. Treg cells can rapidly proliferate; however, the homeostatic processes that limit excessive Treg cell numbers are poorly understood. Here, we show that, compared to conventional T cells, Treg cells have a high apoptosis rate ex vivo correlating with low c-FLIP expression. Treg-specific deletion of c-FLIP in mice resulted in fatal autoimmune disease of a scurfy-like phenotype characterized by absent peripheral Treg cells, activation of effector cells, multi-organ immune cell infiltration, and premature death. Surprisingly, blocking CD95L did not rescue Treg survival in vivo, suggesting additional survival functions of c-FLIP in Treg cells in addition to its classical role in the inhibition of death receptor signaling. Thus, our data reveal a central role for c-FLIP in Treg cell homeostasis and prevention of autoimmunity.

Keywords: Cflar; apoptosis; c-FLIP; regulatory T cell.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antibodies, Blocking / pharmacology
  • Autoimmunity* / drug effects
  • CASP8 and FADD-Like Apoptosis Regulating Protein / metabolism*
  • Caspase Inhibitors / pharmacology
  • Cell Death / drug effects
  • Cell Survival / drug effects
  • Fas Ligand Protein / metabolism
  • Forkhead Transcription Factors / metabolism*
  • Gene Deletion
  • Lymph Nodes / cytology
  • Mice
  • Phenotype
  • Spleen / cytology
  • T-Lymphocytes, Regulatory / drug effects
  • T-Lymphocytes, Regulatory / immunology*
  • fas Receptor / metabolism

Substances

  • Antibodies, Blocking
  • CASP8 and FADD-Like Apoptosis Regulating Protein
  • Caspase Inhibitors
  • Cflar protein, mouse
  • Fas Ligand Protein
  • Forkhead Transcription Factors
  • Foxp3 protein, mouse
  • fas Receptor