The pyrin inflammasome has a critical role in mediating inflammation in autoimmune diseases linked to dysregulation in the actin polymerization pathway. Thus, assembly of the pyrin inflammasome is initiated in response to fluctuations in cytoplasmic homeostasis and perturbations in cytoskeletal dynamics.
Pyrin, a candidate Pattern Recognition Receptors (PRRs), can bind to the inflammasome adaptor ASC to form a caspase 1-activating complex.
Mutations in the Pyrin-encoding gene, MEFV, cause a human autoinflammatory disease known as familial Familial Mediterranean fever
Pyrin mediates caspase 1 inflammasome activation in response to Rho-glucosylation activity of cytotoxin TcdB, a major virulence factor of Clostridium difficile, which causes most cases of nosocomial diarrhea.