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, László Maródi, Carlos Rodriguez-Gallego, Jacques Banchereau, Laurent Abel, Xiaoxia Li, Damien Chaussabel, Anne Puel, Jean Laurent Casanova

Research output: Contribution to journalArticleResearchpeer-review

560 Citations (Scopus)

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
JournalScience
Volume321
DOIs
Publication statusPublished - 1 Aug 2008

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