RIPK3 promotes cell death and NLRP3 inflammasome activation in the absence of MLKL

Nat Commun. 2015 Feb 18:6:6282. doi: 10.1038/ncomms7282.

Abstract

RIPK3 and its substrate MLKL are essential for necroptosis, a lytic cell death proposed to cause inflammation via the release of intracellular molecules. Whether and how RIPK3 might drive inflammation in a manner independent of MLKL and cell lysis remains unclear. Here we show that following LPS treatment, or LPS-induced necroptosis, the TLR adaptor protein TRIF and inhibitor of apoptosis proteins (IAPs: X-linked IAP, cellular IAP1 and IAP2) regulate RIPK3 and MLKL ubiquitylation. Hence, when IAPs are absent, LPS triggers RIPK3 to activate caspase-8, promoting apoptosis and NLRP3-caspase-1 activation, independent of RIPK3 kinase activity and MLKL. In contrast, in the absence of both IAPs and caspase-8, RIPK3 kinase activity and MLKL are essential for TLR-induced NLRP3 activation. Consistent with in vitro experiments, interleukin-1 (IL-1)-dependent autoantibody-mediated arthritis is exacerbated in mice lacking IAPs, and is reduced by deletion of RIPK3, but not MLKL. Therefore RIPK3 can promote NLRP3 inflammasome and IL-1β inflammatory responses independent of MLKL and necroptotic cell death.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Apoptosis
  • Autoantibodies / chemistry
  • Bone Marrow Cells / cytology*
  • Carrier Proteins / metabolism*
  • Caspase 8 / metabolism
  • Enzyme Activation
  • Female
  • Inflammasomes / metabolism*
  • Inflammation
  • Inhibitor of Apoptosis Proteins / metabolism
  • Interleukin-1beta / metabolism
  • Lipopolysaccharides / chemistry
  • Liver / embryology
  • Male
  • Mice
  • Mice, Inbred C57BL
  • NLR Family, Pyrin Domain-Containing 3 Protein
  • Necrosis
  • Protein Kinases / metabolism*
  • Receptor-Interacting Protein Serine-Threonine Kinases / metabolism*
  • Tumor Necrosis Factor-alpha / metabolism
  • X-Linked Inhibitor of Apoptosis Protein / metabolism

Substances

  • Autoantibodies
  • Birc4 protein, mouse
  • Carrier Proteins
  • Inflammasomes
  • Inhibitor of Apoptosis Proteins
  • Interleukin-1beta
  • Lipopolysaccharides
  • NLR Family, Pyrin Domain-Containing 3 Protein
  • Nlrp3 protein, mouse
  • Tumor Necrosis Factor-alpha
  • X-Linked Inhibitor of Apoptosis Protein
  • MLKL protein, mouse
  • Protein Kinases
  • Receptor-Interacting Protein Serine-Threonine Kinases
  • Ripk3 protein, mouse
  • Casp8 protein, mouse
  • Caspase 8