| First Author | Eeckhout E | Year | 2023 |
| Journal | Front Immunol | Volume | 14 |
| Pages | 1272639 | PubMed ID | 38090573 |
| Mgi Jnum | J:343204 | Mgi Id | MGI:7564500 |
| Doi | 10.3389/fimmu.2023.1272639 | Citation | Eeckhout E, et al. (2023) The autoinflammation associated NLRC4(V341A) mutation increases microbiota-independent IL-18 production but does not recapitulate human autoinflammatory symptoms in mice. Front Immunol 14 |
| abstractText | Background: Autoinflammation with infantile enterocolitis (AIFEC) is an often fatal disease caused by gain-of-function mutations in the NLRC4 inflammasome. This inflammasomopathy is characterized by macrophage activation syndrome (MAS)-like episodes as well as neonatal-onset enterocolitis. Although elevated IL-18 levels were suggested to take part in driving AIFEC pathology, the triggers for IL-18 production and its ensuing pathogenic effects in these patients are incompletely understood. |
