NLRC5 senses NAD+ depletion, forming a PANoptosome and driving PANoptosis and inflammation

Cell. 2024 Jul 25;187(15):4061-4077.e17. doi: 10.1016/j.cell.2024.05.034. Epub 2024 Jun 14.

Abstract

NLRs constitute a large, highly conserved family of cytosolic pattern recognition receptors that are central to health and disease, making them key therapeutic targets. NLRC5 is an enigmatic NLR with mutations associated with inflammatory and infectious diseases, but little is known about its function as an innate immune sensor and cell death regulator. Therefore, we screened for NLRC5's role in response to infections, PAMPs, DAMPs, and cytokines. We identified that NLRC5 acts as an innate immune sensor to drive inflammatory cell death, PANoptosis, in response to specific ligands, including PAMP/heme and heme/cytokine combinations. NLRC5 interacted with NLRP12 and PANoptosome components to form a cell death complex, suggesting an NLR network forms similar to those in plants. Mechanistically, TLR signaling and NAD+ levels regulated NLRC5 expression and ROS production to control cell death. Furthermore, NLRC5-deficient mice were protected in hemolytic and inflammatory models, suggesting that NLRC5 could be a potential therapeutic target.

Keywords: ASC; DAMP; NLRC5; NLRP12; NLRP3; PAMP; PANoptosis; RIPK3; ROS; TLRs; TNF; apoptosis; caspase; colitis; heme; hemophagocytic lymphohistiocytosis; inflammasome; inflammatory cell death; necroptosis; pyroptosis.

MeSH terms

  • Animals
  • Apoptosis Regulatory Proteins / genetics
  • Apoptosis Regulatory Proteins / metabolism
  • Calcium-Binding Proteins
  • Cytokines / metabolism
  • HEK293 Cells
  • Humans
  • Immunity, Innate
  • Inflammasomes / metabolism
  • Inflammation* / metabolism
  • Intracellular Signaling Peptides and Proteins* / genetics
  • Intracellular Signaling Peptides and Proteins* / metabolism
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • NAD* / metabolism
  • Reactive Oxygen Species / metabolism
  • Signal Transduction
  • Toll-Like Receptors / metabolism

Substances

  • Intracellular Signaling Peptides and Proteins
  • NAD
  • NLRC5 protein, mouse
  • Reactive Oxygen Species
  • NLRC5 protein, human
  • Inflammasomes
  • Apoptosis Regulatory Proteins
  • Ipaf protein, mouse
  • Toll-Like Receptors
  • Cytokines
  • Calcium-Binding Proteins