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Pyogenic bacterial infections in humans with MyD88 deficiency.

Horst von Bernuth 1, 2 Capucine Picard 1, 3, 2 Zhongbo Jin 4, 5 Rungnapa Pankla 6, 5 Hui Xiao 7 L. Ku 1, 2 Maya Chrabieh 1, 2 Imen Ben Mustapha 1, 2, 8 Pegah Ghandil 1, 2 Yildiz Camcioglu 9 Júlia Vasconcelos 10 Nicolas Sirvent 11, 12 Margarida Guedes 10 Artur Bonito Vitor 13 J. Herrero-Mata 13 J. Arostegui 14 Carlos Rodrigo 15 Laia Alsina 16 Estibaliz Ruiz-Ortiz 17 Manel Juan 14 Claudia Fortuny J. Yague 14 J. Anton Mariona Pascal 18 Huey-Hsuan Chang Lucile Janniere 1, 2 Yoann Rose 1, 2 Ben-Zion Garty Helen Chapel 19 Andrew Issekutz 20 L. Marodi 21 Carlos Rodriguez-Gallego 22 Jacques Banchereau 5 Laurent Abel 1, 2 Xiaoxia Li 8 Damien Chaussabel 5 Anne Puel 1, 2 Jean-Laurent Casanova 23, 1, 2, * 
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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Submitted on : Monday, May 8, 2017 - 2:44:21 PM
Last modification on : Thursday, October 20, 2022 - 3:10:07 PM

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Horst von Bernuth, Capucine Picard, Zhongbo Jin, Rungnapa Pankla, Hui Xiao, et al.. Pyogenic bacterial infections in humans with MyD88 deficiency.. Science, 2008, 321 (5889), pp.691-6. ⟨10.1126/science.1158298⟩. ⟨pasteur-01375224⟩



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