Pyogenic bacterial infections in humans with IRAK-4 deficiency.
Science
; 299(5615): 2076-9, 2003 Mar 28.
Article
em En
| MEDLINE
| ID: mdl-12637671
ABSTRACT
Members of the Toll-like receptor (TLR) and interleukin-1 receptor (IL-1R) superfamily share an intracytoplasmic Toll-IL-1 receptor (TIR) domain, which mediates recruitment of the interleukin-1 receptor-associated kinase (IRAK) complex via TIR-containing adapter molecules. We describe three unrelated children with inherited IRAK-4 deficiency. Their blood and fibroblast cells did not activate nuclear factor kappaB and mitogen-activated protein kinase (MAPK) and failed to induce downstream cytokines in response to any of the known ligands of TIR-bearing receptors. The otherwise healthy children developed infections caused by pyogenic bacteria. These findings suggest that, in humans, the TIR-IRAK signaling pathway is crucial for protective immunity against specific bacteria but is redundant against most other microorganisms.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Infecções Pneumocócicas
/
Infecções Estafilocócicas
/
Fosfotransferases (Aceptor do Grupo Álcool)
/
Proteínas de Drosophila
Idioma:
En
Revista:
Science
Ano de publicação:
2003
Tipo de documento:
Article
País de afiliação:
França