Nlrp11

NLRPs (acronym for "NIGHT, LRR and PYD domains-containing protein) are, together with the NOD1 and NOD2 proteins, members of the NLR (Nod-like Receptor) protein family and play a major role in innate immunity as pathogen recognition receptors (PPRs). Like the NOD proteins, NLRPs are expressed exclusively cytoplasmically. All NLRPs (they all contain a pyrin domain) are encoded by a common gene family in humans. NLRPs are characterised by their ability to activate different inflammatory complexes.

NLRP11 is a cytosolic protein encoded in humans by the NLRP11 gene located on chromosome 19q13.42-q13.43 within a cluster of other NLRP genes.

Inflammasomes are differently composed cytosolic protein complexes, whereby the different NLRPs are of great importance for their functionality. Inflammasomes are predominantly found in immune cells such as dendritic cells and macrophages.

The activation of an inflammasome complex leads to the expression of different caspases, which convert inactive interleukin-1beta and interleukin-18 into their active form.

Most short NLRPs have an N-terminal pyrin domain (PYD), followed by the NIGHT domain, a NIGHT-associated domain (NAD), and a C-terminal leucine-rich region (LRR).

Little is known about the functions and the mode of operation of NLRP11. It is assumed that NLRP11 has a regulatory function in the innate and adaptive immune system.

In juvenile idiopathic polyarthritis, polymorphisms in the NLRP family have been detected, for example in NLRP2, NLRP9 and NLRP11. Apparently, the genes of these pathogen recognition receptors are important for the pathogenesis of this autoimmune disease.

Furthermore, in Crohn's disease there are indications of SNP polymorphisms in the NLRP11 gene

1. Cummings JR et al.(2010)The genetics of NOD-like receptors in Crohn's disease. Tissue antigen 76:48-56.
2. Tadaki H et al (2011) De novo 19q13.42 duplications involving NLRP gene cluster in a patient with systemic-onset juvenile idiopathic arthritis.J Hum gene 56:343-347.