cryopyrin

NLRP3

A gene located on chromosome 1q44 that encodes a pyrin-like protein that interacts with the apoptosis-associated speck-like protein PYCARD/ASC. NLRP3 has a caspase recruitment domain, and belongs to the NALP3 inflammasome complex; it plays a role in regulating inflammation and apoptosis.
 
Molecular pathology
NLRP3 mutations are associated with chronic infantile neurological cutaneous and articular (CINCA) syndrome, familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome and neonatal-onset multisystem inflammatory disease (NOMID).

cryopyrin

A protein coded for by the cold-induced auto-inflammatory syndrome 1 gene. Cryopyrin gene mutations cause three rare hereditary periodic-fever disorders. But the protein has more recently (2006) been found to be importantly involved in the production of INFLAMMATION in innate immunity. See also INFLAMMASOME.
References in periodicals archive ?
About CAPS and NOMID Cryopyrin associated periodic syndromes (CAPS) are a group of rare inherited autoinflammatory diseases caused by autosomal dominant mutations in a gene called NLRP3.
Researchers have found mutations in the cryopyrin gene in this group of diseases, and what is especially exciting is that trials with the inter leukin-1 receptor antagonist anakinra have shown dramatic clinical benefit," he said (N.
Researchers have found that alterations in the cryopyrin protein lead to over-production of IL-1, resulting in an inflammatory response and the symptoms of CAPS.
The revised agreement builds on the previous agreement that gave Sobi rights for Kineret within the field of Rheumatoid arthritis (RA) and four orphan drug indications, including Cryopyrin Associated Periodic Syndrome (CAPS).
Cryopyrin, the protein encoded by NLRP3, controls the inflammasome, a crucial molecular platform that regulates activation of caspase-1 and processing of interleukin (IL)-1[beta], two key mediators of inflammation (Martinon and Tschopp, 2006; Mariathasan and Monack, 2007; Petrilli et al.
Genetic variation in proteins of the cryopyrin inflammasome influences susceptibility and severity of rheumatoid arthritis (the Swedish TIRA project).
Sobi has filed an application for an EU Marketing Authorization with the European Medicines Agency (EMA) for KineretA (anakinra) for the indication of cryopyrin associated periodic syndromes (CAPS).
Ilaris is already approved for treating children and adults suffering from cryopyrin associated periodic syndrome (CAPS) in the EU, US, Switzerland and Japan.
Efficacy and safety of canakinumab (ACZ885), a fully human anti-interleukin-1beta antibody, in cryopyrin associated periodic fever syndrome: Results of a multicenter, randomized, double-blind, phase III study.