Confounding role of tumor necrosis factor in cryopyrin-associated periodic syndromes.
J Clin Invest
; 127(12): 4235-4237, 2017 12 01.
Article
in En
| MEDLINE
| ID: mdl-29130931
ABSTRACT
The NLRP3 inflammasome is a critical component of the innate immune system and can be activated in response to microbial and endogenous danger signals. Activation of the NLRP3 inflammasome results in caspase-1-dependent secretion of the proinflammatory cytokines IL-1ß and IL-18. Gain-of-function missense mutations in NLRP3 result in a group of autoinflammatory diseases collectively known as the cryopyrin-associated periodic syndromes (CAPS). CAPS patients have traditionally been successfully treated with therapeutics targeting the IL-1 pathway; however, there are a number of identified CAPS patients who show only a partial response to IL-1 blockade. In this issue of the JCI, McGeough et al. demonstrated that TNF-α, in addition to IL-1ß, plays an important role in promoting NLRP3 inflammasomopathies.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Cryopyrin-Associated Periodic Syndromes
Type of study:
Prognostic_studies
/
Risk_factors_studies
Limits:
Humans
Language:
En
Journal:
J Clin Invest
Year:
2017
Document type:
Article