NLRs
NOD-like receptors (NLRs) are pattern-recognition receptors, as Toll-like receptors, that are able through the recognition of pathogen-associated molecular patterns and danger-associated molecular patterns to sense microbe-dependent and microbe-independent danger and thereby initiate innate immune responses.
In some autoinflammatory conditions, abnormalities in NLR signaling pathways are involved in pathogenesis, as exemplified by NOD2 mutations associated with Crohn disease.
Some other NLRs are components of the inflammasome, a caspase-1- and prointerleukin-1beta-activating complex.
Pathology
Some monogenic hereditary inflammatory diseases, such as Muckle-Wells syndrome, are associated with mutations in proteins that modulate the activity of the inflammasome, and on some multifactorial disorders, such as Type 2 diabetes and hypertension.
References
McDermott MF, Tschopp J. From inflammasomes to fevers, crystals and hypertension: how basic research explains inflammatory diseases. Trends Mol Med. 2007 Sep;13(9):381-8. PMID: 17822957