Review
NLR activation takes a direct route

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For the first time there is now clear biochemical and biophysical evidence indicating that members of the nucleotide-binding domain and leucine-rich repeat containing (NLR) family can be activated as a result of direct interaction between the receptor and ligand. NLRX1 leucine-rich repeats bind to RNA; murine NAIP (NLR family, apoptosis inhibitory protein) 5 binds flagellin directly; and NOD (nucleotide-binding oligomerization domain containing) 1 and NOD2 may interact directly with fragments of peptidoglycan. It remains to be seen if NLRP3 has a specific ligand, but progress has been made in addressing its mechanism of activation, with cellular imbalances and mitochondrial dysfunction being important. This review updates our understanding of NLR activation in light of these recent advances and their impact on the NLR research.

Section snippets

NLR activation and signalling in innate immunity

The basics of the innate immune response are conserved throughout evolution. Germ-line-encoded proteins known as pattern recognition receptors (PRRs) function by recognising ‘danger signals’ and then initiating a protective innate immune response. Danger signals can be derived from both exogenous and endogenous sources and include: pathogen-derived proteins, lipids, nucleic acids, and polysaccharides; crystalline materials; high levels of ATP; specific host proteins and nucleic acid; and

Do NOD1 and NOD2 interact directly with peptidoglycan fragments?

NOD1 and NOD2 are activated by fragments of peptidoglycan (Figure 1) from the bacterial cell wall. Peptidoglycan fragments containing DAP, found in Gram-negative bacteria [19] and a few Gram-positive bacteria such as Listeria spp. and Bacillus spp. 20, 21, activate NOD1. By contrast, NOD2 responds to MDP, which is found in Gram-positive bacteria, Gram-negative bacteria, and mycobacteria [22]. In fact, mycobacterial N-glycolyl MDP is the most potent NOD2 agonist identified to date [23]. Early

The LRR domain of NLRX1 directly binds RNA ligands

Nucleic acid agonists are detected predominantly by members of the TLR, RLR, and ALR receptor families, and there is clear structural evidence for the direct binding of the nucleic acid ligand to these receptors. For example, crystal structures have been resolved for TLR3 bound to its double-stranded (ds)RNA ligand [27], for RIG-I and various RNA ligands 28, 29, 30, 31, 32, and the HIN (hemopoietic interferon-inducible nuclear protein) 200 domains of both AIM2 and IFI (interferon gamma

Activation of the NLRC4/NAIP inflammasome requires direct interaction with bacterial proteins

NLRC4 and NAIP form an inflammasome (Figure 1) in response to flagellin and components of bacterial type III secretion systems (T3SSs), such as the protein PrgJ (pathogenicity 1 island effector protein J) from Salmonella spp. 36, 37, 38. Recognition of T3SS proteins depends on C-terminal peptide sequences similar to regions of flagellin, mutation of which impairs NLRC4/NAIP activation [38]. Originally, NLRC4 was believed to be responsible for ligand detection 36, 37. More recently, the Shao and

Cellular stresses activate NLRP3. Is the activating ligand of exogenous or endogenous origin?

NLRP3 is activated in response to an overwhelming array of stimuli that cause various biochemical responses in the cell and disrupt the homeostatic balance (Table 1) [3]. These include potassium efflux, membrane permeabilisation, lysosomal leakage, mitochondrial dysfunction, and reactive oxygen species (ROS) (Figure 1 and Table 1) [3]. The importance of ROS in NLRP3 activation is currently under debate because many of the pharmacological ROS inhibitors also impair NLRP3 priming [44]. It seems

NLRC3, NLRC5, NLRP6, and NLRP10: NLRs as immune regulators

Not all NLRs described to date are proinflammatory and do not therefore require an activatory ligand. NLRC3 and NLRP6 antagonise inflammatory signalling 56, 57, 58, 59, 60, 61, whereas NLRC5 and NLRP10 appear to regulate transcription of MHC I and development of the adaptive immune response, respectively 62, 63, 64, 65.

NLRC3 inhibits NF-κB activation by TLR signalling pathways through interaction with the downstream signalling adaptor TRAF (tumour necrosis factor receptor-associated factor) 6.

Concluding remarks

Without question our continual desire to understand NLR biology is driving forward innate immune research. It continues to provide a clear picture of the underlying biological events that occur during the detection of, and response to, cellular damage of both an endogenous and exogenous nature. It also continues to enhance our understanding of disease progression, genetic susceptibility to disease, and the interplay between the innate and adaptive arms of the immune response. Despite the rapid

Acknowledgements

The work in the laboratory of T.P.M. is funded by The Wellcome Trust (WT0805090MA).

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