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Cited by 1,287

The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.

Pyogenic Bacterial Infections in Humans with MyD88 Deficiency

Open access
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, Maródi, László, Rodriguez-Gallego, Carlos, Banchereau, Jacques, Abel, Laurent, Li, Xiaoxia, Chaussabel, Damien, Puel, Anne, Casanova, Jean-Laurent·2008·Science

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.

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Metadata

Authors
von Bernuth, H., Picard, C., Jin, Z., Pankla, R., Xiao, H., Ku, C.-L., Chrabieh, M., Mustapha, I. B., Ghandil, P., Camcioglu, Y., Vasconcelos, J., Sirvent, N., Guedes, M., Vitor, A. B., Herrero-Mata, M. J., Aróstegui, J. I., Rodrigo, C., Alsina, L., Ruiz-Ortiz, E., Juan, M., Fortuny, C., Yagüe, J., Antón, J., Pascal, M., Chang, H.-H., Janniere, L., Rose, Y., Garty, B.-Z., Chapel, H., Issekutz, A., Maródi, L., Rodriguez-Gallego, C., Banchereau, J., Abel, L., Li, X., Chaussabel, D., Puel, A., & Casanova, J.-L.
Venue
Science
Published
2008-08
Volume
321
Issue
5889
Pages
691-696
ISSN
  • 0036-8075 (ISSN-L)
  • 1095-9203
OpenAlex
W2141040025
Cited by
1,287
References
1
FWCI
40.94
Type
journal article
Language
en
Open access
green

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CoChat. (2026). Citation check: Pyogenic Bacterial Infections in Humans with MyD88 Deficiency. https://app.cochat.ai/papers/10.1126/science.1158298
The paper
von Bernuth, H., Picard, C., Jin, Z., Pankla, R., Xiao, H., Ku, C.-L., Chrabieh, M., Mustapha, I. B., Ghandil, P., Camcioglu, Y., Vasconcelos, J., Sirvent, N., Guedes, M., Vitor, A. B., Herrero-Mata, M. J., Aróstegui, J. I., Rodrigo, C., Alsina, L., Ruiz-Ortiz, E., . . . Casanova, J.-L. (2008). Pyogenic Bacterial Infections in Humans with MyD88 Deficiency. Science. https://doi.org/10.1126/science.1158298

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

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