The Pyrin Family of Fever Genes

Periodic Fever Syndromes

“…28,38 Treatment options for the periodic fever syndromes often overlap, with successful therapies, including NSAIDs, corticosteroids, anti-TNF agents, and anti-IL-1 agents. Because of the commonality of pyrin gene family (including cryopyrin) involvement in most of these disorders, 39 similar treatments antagonizing infl ammation, cytokine processing, and apoptotic pathways have proven effective across syndromes.…”

Liver aspirate from a Shar Pei dog

“…[2][3][4][7][8][9] FMF is the result of a mutation in the gene MEFV which codes for pyrin, a protein that helps regulate molecular signaling pathways involved in inflammation, cytokine and chemokine processing, and apoptosis. 10 To our knowledge, the pyrin genes of Shar Pei dogs have not been investigated.…”

Missense Mutations in the MEFV Gene Are Associated with Fibromyalgia Syndrome and Correlate with Elevated IL-1β Plasma Levels

“…The NLR (nucleotide binding domain and leucine rich, or NOD-like receptor, where NOD is an abbreviation for nucleotide oligomerization domain) family of genes also contain the PYD domain [27], [28], [29], [30]. While the pyrin protein is thought to function in apoptotic and inflammatory signaling pathways [31], [32], its exact function has been debated. In a better-studied system, cryopyrin or NLRP3, which also has the PYD domain, is activated to assemble the inflammasome [33], a multi-protein structure that ultimately results in activation of pro-caspase-1 that, in turn, processes pro-IL-1β to IL-1β.…”