Pyogenic bacterial infections in humans with IRAK-4 deficiency

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

doi:10.1126/science.1081902

Pyogenic bacterial infections in humans with IRAK-4 deficiency

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

^*Corresponding author for this work

Research output: Contribution to journal › Journal Article › peer-review

808 Scopus citations

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 KB 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.

Original language	English
Pages (from-to)	2076-2079
Number of pages	4
Journal	Science
Volume	299
Issue number	5615
DOIs	https://doi.org/10.1126/science.1081902
State	Published - 28 03 2003
Externally published	Yes

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Picard, C., Puel, A., Bonnet, M., Ku, C. L., Bustamante, J., Yang, K., Soudais, C., Dupuis, S., Feinberg, J., Fieschi, C., Elbim, C., Hitchcock, R., Lammas, D., Davies, G., Al-Ghonaium, A., Al-Rayes, H., Al-Jumaah, S., Al-Hajjar, S., Al-Mohsen, I. Z., ... Casanova, J. L. (2003). Pyogenic bacterial infections in humans with IRAK-4 deficiency. Science, 299(5615), 2076-2079. https://doi.org/10.1126/science.1081902

@article{c6ea602e52e342c49f919299d45d5a16,

title = "Pyogenic bacterial infections in humans with IRAK-4 deficiency",

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 KB 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.",

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

year = "2003",

month = mar,

day = "28",

doi = "10.1126/science.1081902",

language = "英语",

volume = "299",

pages = "2076--2079",

journal = "Science",

issn = "0036-8075",

publisher = "American Association for the Advancement of Science",

number = "5615",

}

Picard, C, Puel, A, Bonnet, M, Ku, CL, Bustamante, J, Yang, K, Soudais, C, Dupuis, S, Feinberg, J, Fieschi, C, Elbim, C, Hitchcock, R, Lammas, D, Davies, G, Al-Ghonaium, A, Al-Rayes, H, Al-Jumaah, S, Al-Hajjar, S, Al-Mohsen, IZ, Frayha, HH, Rucker, R, Hawn, TR, Aderem, A, Tufenkeji, H, Haraguchi, S, Day, NK, Good, RA, Gougerot-Pocidalo, MA, Ozinsky, A & Casanova, JL 2003, 'Pyogenic bacterial infections in humans with IRAK-4 deficiency', Science, vol. 299, no. 5615, pp. 2076-2079. https://doi.org/10.1126/science.1081902

TY - JOUR

T1 - Pyogenic bacterial infections in humans with IRAK-4 deficiency

AU - Picard, Capucine

AU - Puel, Anne

AU - Bonnet, Marion

AU - Ku, Cheng Lung

AU - Bustamante, Jacinta

AU - Yang, Kun

AU - Soudais, Claire

AU - Dupuis, Stéphanie

AU - Feinberg, Jacqueline

AU - Fieschi, Claire

AU - Elbim, Carole

AU - Hitchcock, Remi

AU - Lammas, David

AU - Davies, Graham

AU - Al-Ghonaium, Abdulaziz

AU - Al-Rayes, Hassan

AU - Al-Jumaah, Sulaiman

AU - Al-Hajjar, Sami

AU - Al-Mohsen, Ibrahim Zaid

AU - Frayha, Husn H.

AU - Rucker, Rajivi

AU - Hawn, Thomas R.

AU - Aderem, Alan

AU - Tufenkeji, Haysam

AU - Haraguchi, Soichi

AU - Day, Noorbibi K.

AU - Good, Robert A.

AU - Gougerot-Pocidalo, Marie Anne

AU - Ozinsky, Adrian

AU - Casanova, Jean Laurent

PY - 2003/3/28

Y1 - 2003/3/28

N2 - 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 KB 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.

AB - 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 KB 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.

UR - http://www.scopus.com/inward/record.url?scp=0037471003&partnerID=8YFLogxK

U2 - 10.1126/science.1081902

DO - 10.1126/science.1081902

M3 - 文章

C2 - 12637671

AN - SCOPUS:0037471003

SN - 0036-8075

VL - 299

SP - 2076

EP - 2079

JO - Science

JF - Science

IS - 5615

ER -

Pyogenic bacterial infections in humans with IRAK-4 deficiency

Abstract

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