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Title: Pyogenic bacterial infections in humans with MyD88 deficiency
Authors: 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
UNESCO Clasification: 32 Ciencias médicas
3205 Medicina interna
Keywords: MyD88 Deficiency
Pyogenic bacterial infections
Issue Date: 2008
Journal: 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.
ISSN: 0036-8075
DOI: 10.1126/science.1158298
Source: Science[ISSN 0036-8075],v. 321, p. 691-696
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