Pyogenic bacterial infections in humans with MyD88 deficiency

Horst Von Bernuth, Capucine Picard, Zhongbo Jin, Rungnapa Pankla, Hui Xiao, Cheng Lung Ku, Maya Chrabieh, Imen Ben Mustapha, Pegah Ghandil, Yildiz Camcioglu, Júlia Vasconcelos, Nicolas Sirvent, Margarida Guedes, Artur Bonito Vitor, María José Herrero-Mata, Juan Ignacio Aróstegui, Carlos Rodrigo, Laia Alsina, Estibaliz Ruiz-Ortiz, Manel JuanClaudia Fortuny, Jordi Yagüe, Jordi Antón, Mariona Pascal, Huey Hsuan Chang, Lucile Janniere, Yoann Rose, Ben Zion Garty, Helen Chapel, Andrew Issekutz, L. Máródi, Carlos Rodriguez-Gallego, Jacques Banchereau, Laurent Abel, Xiaoxia Li, Damien Chaussabel, Anne Puel, Jean Laurent Casanova

Research output: Contribution to journalArticle

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Abstract

MyD88 is a key downstream adapter for most Toll-like receptors (TLRs) and interleukin-1 receptors (IL-1Rs). MyD88 deficiency in mice leads to susceptibility to a broad range of pathogens in experimental settings of infection. We describe a distinct situation in a natural setting of human infection. Nine children with autosomal recessive MyD88 deficiency suffered from life-threatening, often recurrent pyogenic bacterial infections, including invasive pneumococcal disease. However, these patients were otherwise healthy, with normal resistance to other microbes. Their clinical status improved with age, but not due to any cellular leakiness in MyD88 deficiency. The MyD88-dependent TLRs and IL-1Rs are therefore essential for protective immunity to a small number of pyogenic bacteria, but redundant for host defense to most natural infections.

Original languageEnglish
Pages (from-to)691-696
Number of pages6
JournalScience
Volume321
Issue number5889
DOIs
Publication statusPublished - Aug 1 2008

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Toll-Like Receptor 1
Bacterial Infections
Interleukin-1 Receptors
Infection
Immunity
Bacteria

ASJC Scopus subject areas

  • General

Cite this

Von Bernuth, H., Picard, C., Jin, Z., Pankla, R., Xiao, H., Ku, C. L., ... Casanova, J. L. (2008). Pyogenic bacterial infections in humans with MyD88 deficiency. Science, 321(5889), 691-696. https://doi.org/10.1126/science.1158298

Pyogenic bacterial infections in humans with MyD88 deficiency. / Von Bernuth, Horst; Picard, Capucine; Jin, Zhongbo; Pankla, Rungnapa; Xiao, Hui; Ku, Cheng Lung; Chrabieh, Maya; Mustapha, Imen Ben; Ghandil, Pegah; Camcioglu, Yildiz; Vasconcelos, Júlia; Sirvent, Nicolas; Guedes, Margarida; Vitor, Artur Bonito; Herrero-Mata, María José; Aróstegui, Juan Ignacio; Rodrigo, Carlos; Alsina, Laia; Ruiz-Ortiz, Estibaliz; Juan, Manel; Fortuny, Claudia; Yagüe, Jordi; Antón, Jordi; Pascal, Mariona; Chang, Huey Hsuan; Janniere, Lucile; Rose, Yoann; Garty, Ben Zion; Chapel, Helen; Issekutz, Andrew; Máródi, L.; Rodriguez-Gallego, Carlos; Banchereau, Jacques; Abel, Laurent; Li, Xiaoxia; Chaussabel, Damien; Puel, Anne; Casanova, Jean Laurent.

In: Science, Vol. 321, No. 5889, 01.08.2008, p. 691-696.

Research output: Contribution to journalArticle

Von Bernuth, H, Picard, C, Jin, Z, Pankla, R, Xiao, H, Ku, CL, Chrabieh, M, Mustapha, IB, Ghandil, P, Camcioglu, Y, Vasconcelos, J, Sirvent, N, Guedes, M, Vitor, AB, Herrero-Mata, MJ, Aróstegui, JI, Rodrigo, C, Alsina, L, Ruiz-Ortiz, E, Juan, M, Fortuny, C, Yagüe, J, Antón, J, Pascal, M, Chang, HH, Janniere, L, Rose, Y, Garty, BZ, Chapel, H, Issekutz, A, Máródi, L, Rodriguez-Gallego, C, Banchereau, J, Abel, L, Li, X, Chaussabel, D, Puel, A & Casanova, JL 2008, 'Pyogenic bacterial infections in humans with MyD88 deficiency', Science, vol. 321, no. 5889, pp. 691-696. https://doi.org/10.1126/science.1158298
Von Bernuth H, Picard C, Jin Z, Pankla R, Xiao H, Ku CL et al. Pyogenic bacterial infections in humans with MyD88 deficiency. Science. 2008 Aug 1;321(5889):691-696. https://doi.org/10.1126/science.1158298
Von Bernuth, Horst ; Picard, Capucine ; Jin, Zhongbo ; Pankla, Rungnapa ; Xiao, Hui ; Ku, Cheng Lung ; Chrabieh, Maya ; Mustapha, Imen Ben ; Ghandil, Pegah ; Camcioglu, Yildiz ; Vasconcelos, Júlia ; Sirvent, Nicolas ; Guedes, Margarida ; Vitor, Artur Bonito ; Herrero-Mata, María José ; Aróstegui, Juan Ignacio ; Rodrigo, Carlos ; Alsina, Laia ; Ruiz-Ortiz, Estibaliz ; Juan, Manel ; Fortuny, Claudia ; Yagüe, Jordi ; Antón, Jordi ; Pascal, Mariona ; Chang, Huey Hsuan ; Janniere, Lucile ; Rose, Yoann ; Garty, Ben Zion ; Chapel, Helen ; Issekutz, Andrew ; Máródi, L. ; Rodriguez-Gallego, Carlos ; Banchereau, Jacques ; Abel, Laurent ; Li, Xiaoxia ; Chaussabel, Damien ; Puel, Anne ; Casanova, Jean Laurent. / Pyogenic bacterial infections in humans with MyD88 deficiency. In: Science. 2008 ; Vol. 321, No. 5889. pp. 691-696.
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AU - Pascal, Mariona

AU - Chang, Huey Hsuan

AU - Janniere, Lucile

AU - Rose, Yoann

AU - Garty, Ben Zion

AU - Chapel, Helen

AU - Issekutz, Andrew

AU - Máródi, L.

AU - Rodriguez-Gallego, Carlos

AU - Banchereau, Jacques

AU - Abel, Laurent

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AU - Chaussabel, Damien

AU - Puel, Anne

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