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Pyogenic bacterial infections in humans with IRAK-4 deficiency.

Picard, Capucine, Puel, Anne, Bonnet, Marion ORCID:, Ku, Cheng-Lung, Bustamante, Jacinta, Yang, Kun, Soudais, Claire and Casanova, Jean-Laurent 2003. Pyogenic bacterial infections in humans with IRAK-4 deficiency. Science 299 (5615) , pp. 2076-2079. 10.1126/science.1081902

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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 κB 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.

Item Type: Article
Date Type: Published Online
Status: Published
Schools: Medicine
Publisher: American Association for the Advancement of Science
ISSN: 0036-8075
Date of Acceptance: 27 February 2003
Last Modified: 09 Nov 2022 09:38

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