What is NLRC4 Inflammasome?
What is NLRC4 Inflammasome?
NLRC4 EFFECTOR MECHANISMS. Activation of the NLRC4 inflammasome appears to activate several different host signaling events. NLRC4 was first identified as potential nucleotide binding protein that was capable of activating CASP1, the primary protease responsible for converting pro-IL-1β to active, secreted IL-1β2, 28.
What does NLRC4 stand for?
NLRC4 (NLR Family CARD Domain Containing 4) is a Protein Coding gene. Diseases associated with NLRC4 include Familial Cold Autoinflammatory Syndrome 4 and Autoinflammation With Infantile Enterocolitis.
What does NLRP3 bind to?
Immunity 20, 319–325 (2004). This is the first report showing that NLRP3 assembles an inflammasome complex that mediates cleavage of IL-1β by caspase 1. Duncan, J. A. et al. Cryopyrin/NALP3 binds ATP/dATP, is an ATPase, and requires ATP binding to mediate inflammatory signaling.
What are the trigger molecules that activate Inflammasome?
The NLRC4 inflammasome is generally activated by PAMPs including bacterial flagellin and type III secretion system (T3SS) components. Unlike other NLRPs, NLRC4 partners with another NLR family member, NAIP, which directly binds to bacterial ligands.
What is NLRC4 mutation?
When mutations cause the NLRC4 protein to always be active, it results in widespread activation of the immune system even when bacteria are not present. This uncontrolled activation causes damage to the patient’s healthy cells resulting in the symptoms of AIFEC.
What is NLRC4 GOF?
NLRC4 gain-of-function (GOF) mutations have been associated with early-onset recurrent fever, recurrent macrophagic activation syndrome and enterocolitis.
How are inflammasomes activated?
Inflammasome formation is triggered by a range of substances that emerge during infections, tissue damage or metabolic imbalances. Once the protein complexes have formed, the inflammasomes activate caspase 1, which proteolytically activates the pro-inflammatory cytokines interleukin-1β (IL-1β)3 and IL-18.
Is NLRP3 a PRR?
NLRP3 is a component of the innate immune system that functions as a pattern recognition receptor (PRR) that recognizes pathogen-associated molecular patterns (PAMPs).
