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 ?
b) positive results in Phase 2 or 3 trial, (c) proof of concept obtained, (d) no positive results in Phase 2 or 3, RA: rheumatoid arthritis, JIA: juvenile idiopathic arthritis, CD: Crohn's disease, UC: ulcerative colitis, AS: ankylosing spondylitis, SpA: spondyloathritis, PS: psoriasis, PsA: psoriatic arthritis, CAPS: cryopyrin associated periodic syndrome, FMF: familial medetrian fever, SLE: systemic lupus erythematosus, pSS: primary Sjogren's syndrome, SSc: systemic sclerosis, CCA: giant cell arthritis, TA: Takayasu arteritis, AAV: ANCA associated vasculitis, AOSD: adult-onset Still's disease
In pre-clinical studies, ATI-450 was active in blocking multiple manifestations of the Neonatal-onset Multisystem Inflammatory Disease, or NOMID, form of Cryopyrin associated periodic syndromes, or CAPS, using an inducible transgenic murine model of the disease.
The diseases described in this scope include FMF, tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency/hyperimmunoglobulin D syndrome (MKD-HIDS), NLRP12- related syndrome (NLRP12AD), Blau syndrome, pyogenic arthritis, pyoderma gangrenosum and acne syndrome (PAPAs), early-onset sarcoidosis (EOS), Majeed syndrome (MS), interleukin-1 receptor antagonist deficiency (DIRA), cryopyrin associated periodic syndromes (CAPS), IL-36 receptor antagonist deficiency (DITRA), CARD14-mediated pustular psoriasis (CAMPS) and chronic atypical neutrophilic dermatosis, lypodistrophy, and elevated temperature (CANDLE).
Pannexin-1-mediated recognition of bacterial molecules activates the cryopyrin inflammasome independent of Toll-like receptor signaling.
Cryopyrin activates the inflammasome in response to toxins and ATP.
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.
The clinical continuum of cryopyrinopathies: novel CIAS1 mutations in North American patients and a new cryopyrin model.
positive results in Phase 2 or 3 trial, $ proof of concept obtained, x no positive results in Phase 2 or 3, RA: rheumatoid arthritis, JIA: juvenile idiopathic arthritis, CD: Crohn's disease, UC: ulcerative colitis, AS: ankylosing spondylitis, SpA: spondyloathritis, PS: psoriasis, PsA: psoriatic arthritis, CAPS: cryopyrin associated periodic syndrome, FMF: familial medetrian fever, SLE: systemic lupus erythematosus, pSS: primary Sjogren's syndrome, SSc: systemic sclerosis, CCA: giant cell arthritis, TA: Takayasu arteritis, AAV: ANCA associated vasculitis, AOSD: adult-onset Still's disease Fig.
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.
CIAS1 encodes cryopyrin, which belongs to a group of interacting proteins involved in regulating inflammation and programmed cell death; this plays a crucial role in ridding the body of cells that are no longer needed.
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).