Transient impairment of bacterial immunity in human IRAK-4 deficiency

C Picard, A Puel, C Lung Ku, J Bustamante, M Bonnet, C Soudais, S Dupuis, C Elbim, David Lammas, G Davies, S Al Hajjar, S Al-Jumaah, I Al-Mohsen, A Aderem, A Al-Ghonaium, H Tufenkeji, M-A Gougerot-Pocidalo, G Courtois, A Ozinsky, J-L Casanova

Research output: Contribution to journalArticle

735 Citations (Scopus)


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 mitogein-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.
Original languageEnglish
Pages (from-to)2076-9
Number of pages4
Publication statusPublished - 1 Jan 2003


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