2020
DOI: 10.1002/jlb.3mr0720-513r
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NLRP3 inflammasome priming: A riddle wrapped in a mystery inside an enigma

Abstract: The NLRP3 (NOD‐, LRR‐, and pyrin domain‐containing protein 3) inflammasome is an immunological sensor that detects a wide range of microbial‐ and host‐derived signals. Inflammasome activation results in the release of the potent pro‐inflammatory cytokines IL‐1β and IL‐18 and triggers a form of inflammatory cell death known as pyroptosis. Excessive NLRP3 activity is associated with the pathogenesis of a wide range of inflammatory diseases, thus NLRP3 activation mechanisms are an area of intensive research. NLRP… Show more

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Cited by 149 publications
(150 citation statements)
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References 149 publications
(250 reference statements)
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“…However, excessive or altered regulation of NLRP3 inflammasome activity is related to the pathogenesis of a wide variety of inflammatory, autoimmune, and degenerative diseases. 11,12 The pleiotropic roles of the NLRP3 inflammasome have been reviewed extensively elsewhere in terms of physiological responses and in the context of a variety of human diseases. [13][14][15][16] In addition, the mechanisms of noncanonical and one-step NLRP3 inflammasome activation are beyond the scope of this review.…”
Section: Introductionmentioning
confidence: 99%
See 1 more Smart Citation
“…However, excessive or altered regulation of NLRP3 inflammasome activity is related to the pathogenesis of a wide variety of inflammatory, autoimmune, and degenerative diseases. 11,12 The pleiotropic roles of the NLRP3 inflammasome have been reviewed extensively elsewhere in terms of physiological responses and in the context of a variety of human diseases. [13][14][15][16] In addition, the mechanisms of noncanonical and one-step NLRP3 inflammasome activation are beyond the scope of this review.…”
Section: Introductionmentioning
confidence: 99%
“…Due to its prion-like properties, ASC forms large fibrillar aggregates known as "specks". 11,12,[17][18][19][20] NLRP3 inflammasome activation generally requires two steps, i.e., priming (Signal 1) and protein complex assembly (Signal 2) (Fig. 1).…”
Section: Introductionmentioning
confidence: 99%
“…A European initiative SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) providing the treatment recommendations for the management of CAPS patients has highlighted the dramatically improved outcomes in CAPS patients on the IL-1 blockade ( 64 ). However, as the inflammation in CAPS patients is also driven by the mutations in NLRP3, novel molecules inhibiting NLRP3 inflammasome, such as tranilast and inzomelid, are currently also evaluated in phase I and II clinical trials ( 23 , 25 ). Therapeutic potential of targeting NLRP3 priming and posttranslational modifications is yet to be uncovered ( 25 ).…”
Section: Cryopyrin-associated Periodic Syndromementioning
confidence: 99%
“…However, as the inflammation in CAPS patients is also driven by the mutations in NLRP3, novel molecules inhibiting NLRP3 inflammasome, such as tranilast and inzomelid, are currently also evaluated in phase I and II clinical trials ( 23 , 25 ). Therapeutic potential of targeting NLRP3 priming and posttranslational modifications is yet to be uncovered ( 25 ).…”
Section: Cryopyrin-associated Periodic Syndromementioning
confidence: 99%
See 1 more Smart Citation