Pyrin is produced in certain white blood cells (neutrophils, eosinophils and monocytes) that play a role in inflammation and in fighting infection.

Pyrin may direct the migration of white blood cells to sites of inflammation and stop or slow the inflammatory response when it is no longer needed.

Among people with familial Mediterranean fever, this particular mutation is also associated with an increased risk of developing amyloidosis, a complication in which abnormal protein deposits can lead to kidney failure.

[6] These kinases phosphorylate two serine residues located in the linker encoded by MEFV exon 2, allowing proteins 14.3.3 to keep pyrin inflammasome in an inactive state.

[8] Furthermore, while the trigger of FMF flares remain unknown, steroid hormone catabolites (pregnanolone and etiocholanaolone) have been shown to activate the pyrin inflammasome, in vitro, by interacting with the B30.2 domain (coded by exon 10).