c-FLIP maintains tissue homeostasis by preventing apoptosis and programmed necrosis

Sci Signal. 2012 Dec 18;5(255):ra93. doi: 10.1126/scisignal.2003558.

Abstract

As a catalytically inactive homolog of caspase-8, a proapoptotic initiator caspase, c-FLIP blocks apoptosis by binding to and inhibiting caspase-8. The transcription factor nuclear factor κB (NF-κB) plays a pivotal role in maintaining the homeostasis of the intestine and the liver by preventing death receptor-induced apoptosis, and c-FLIP plays a role in the NF-κB-dependent protection of cells from death receptor signaling. Because c-Flip-deficient mice die in utero, we generated conditional c-Flip-deficient mice to investigate the contribution of c-FLIP to homeostasis of the intestine and the liver at developmental and postnatal stages. Intestinal epithelial cell (IEC)- or hepatocyte-specific deletion of c-Flip resulted in perinatal lethality as a result of the enhanced apoptosis and programmed necrosis of the IECs and the hepatocytes. Deficiency in the gene encoding tumor necrosis factor-α (TNF-α) receptor 1 (Tnfr1) partially rescued perinatal lethality and the development of colitis in IEC-specific c-Flip-deficient mice but did not rescue perinatal lethality in hepatocyte-specific c-Flip-deficient mice. Moreover, adult mice with interferon (IFN)-inducible deficiency in c-Flip died from hepatitis soon after depletion of c-FLIP. Pretreatment of IFN-inducible c-Flip-deficient mice with a mixture of neutralizing antibodies against TNF-α, Fas ligand (FasL), and TNF-related apoptosis-inducing ligand (TRAIL) prevented hepatitis. Together, these results suggest that c-FLIP controls the homeostasis of IECs and hepatocytes by preventing cell death induced by TNF-α, FasL, and TRAIL.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antibodies, Neutralizing / pharmacology
  • Apoptosis*
  • CASP8 and FADD-Like Apoptosis Regulating Protein / genetics
  • CASP8 and FADD-Like Apoptosis Regulating Protein / metabolism*
  • Caspase 8
  • Colitis / genetics
  • Colitis / metabolism
  • Colitis / pathology
  • Fas Ligand Protein / antagonists & inhibitors
  • Fas Ligand Protein / genetics
  • Fas Ligand Protein / metabolism
  • Hepatitis / genetics
  • Hepatitis / metabolism
  • Hepatitis / pathology
  • Hepatocytes / metabolism*
  • Hepatocytes / pathology
  • Homeostasis*
  • Intestinal Mucosa / metabolism*
  • Intestines / pathology
  • Liver / metabolism*
  • Liver / pathology
  • Mice
  • Mice, Mutant Strains
  • NF-kappa B / genetics
  • NF-kappa B / metabolism
  • Necrosis / genetics
  • Necrosis / metabolism
  • Necrosis / pathology
  • Organ Specificity / drug effects
  • Organ Specificity / genetics
  • Protein Binding / drug effects
  • Protein Binding / genetics
  • Receptors, Tumor Necrosis Factor, Type I / genetics
  • Receptors, Tumor Necrosis Factor, Type I / metabolism
  • TNF-Related Apoptosis-Inducing Ligand / genetics
  • TNF-Related Apoptosis-Inducing Ligand / metabolism
  • Tumor Necrosis Factor-alpha / antagonists & inhibitors
  • Tumor Necrosis Factor-alpha / genetics
  • Tumor Necrosis Factor-alpha / metabolism

Substances

  • Antibodies, Neutralizing
  • CASP8 and FADD-Like Apoptosis Regulating Protein
  • Cflar protein, mouse
  • Fas Ligand Protein
  • Fasl protein, mouse
  • NF-kappa B
  • Receptors, Tumor Necrosis Factor, Type I
  • TNF-Related Apoptosis-Inducing Ligand
  • Tnfrsf1a protein, mouse
  • Tnfsf10 protein, mouse
  • Tumor Necrosis Factor-alpha
  • Casp8 protein, mouse
  • Caspase 8