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Pyogenic Bacterial Infections in Humans with MyD88 Deficiency
Open accessAbstract
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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Janeway, C. A., & Medzhitov, R. (2002) · Annual Review of Immunology · doi:10.1146/annurev.immunol.20.083001.084359 · Cited by 8,394
Cited by
Akira, S., & Takeda, K. (2004) · Nature reviews. Immunology · doi:10.1038/nri1391 · Cited by 8,312
Toll-like receptors and their crosstalk with other innate receptors in infection and immunity
Kawai, T., & Akira, S. (2011) · Immunity · doi:10.1016/j.immuni.2011.05.006 · Cited by 3,658
LPS/TLR4 signal transduction pathway
Lu, Y.-C., Yeh, W.-C., & Ohashi, P. S. (2008) · Cytokine · doi:10.1016/j.cyto.2008.01.006 · Cited by 3,301
Overview of the immune response
Chaplin, D. D. (2010) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2009.12.980 · Cited by 2,235
Toll-like Receptors and the Control of Immunity
Fitzgerald, K. A., & Kagan, J. C. (2020) · Cell · doi:10.1016/j.cell.2020.02.041 · Cited by 2,168
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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- 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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