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http://hdl.handle.net/10553/48632
Título: | Pyogenic bacterial infections in humans with MyD88 deficiency | Autores/as: | 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 |
Clasificación UNESCO: | 32 Ciencias médicas 3205 Medicina interna |
Palabras clave: | MyD88 Deficiency Pyogenic bacterial infections |
Fecha de publicación: | 2008 | Publicación seriada: | Science | Resumen: | 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. | URI: | http://hdl.handle.net/10553/48632 | ISSN: | 0036-8075 | DOI: | 10.1126/science.1158298 | Fuente: | Science[ISSN 0036-8075],v. 321, p. 691-696 |
Colección: | Artículos |
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