Pyogenic bacterial infections in humans with IRAK-4 deficiency.

doi:10.1126/science.1081902

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Article

Pyogenic bacterial infections in humans with IRAK-4 deficiency.

Science (New York, N.Y.) - 28 Mar 2003

Picard Capucine, Puel Anne, Bonnet Marion, Ku Cheng-Lung, Bustamante Jacinta, Yang Kun, Soudais Claire, Dupuis Stéphanie, Feinberg Jacqueline, Fieschi Claire, Elbim Carole, Hitchcock Remi, Lammas David, Davies Graham, Al-Ghonaium Abdulaziz, Al-Rayes Hassan, Al-Jumaah Sulaiman, Al-Hajjar Sami, Al-Mohsen Ibrahim Zaid, Frayha Husn H, Rucker Rajivi, Hawn Thomas R, Aderem Alan, Tufenkeji Haysam, Haraguchi Soichi, Day Noorbibi K, Good Robert A, Gougerot-Pocidalo Marie-Anne, Ozinsky Adrian, Casanova Jean-Laurent

Abstract excerpt

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...

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PMID: 12637671
DOI: 10.1126/science.1081902

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