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001-es BibID:	BIBFORM005166
Első szerző:	Bernuth, Horst von
Cím:	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 Juan, Claudia 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
Dátum:	2008
Megjegyzések:	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.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:	Science. - 321 : 5889 (2008), p. 691-696. -
További szerzők:	Picard, Capucine Jin, Zhongbo Pankla, Rungnapa Xiao, Hui Ku, Cheng-Lung Chrabieh, Maya Mustapha, Imen Ben Ghandil, Pegah Camcioglu, Yildiz Vasconcelos, Julia Sirvent, Nicolas Guedes, Margarida Bonito Vitor, Artur 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 C. Maródi László (1949-) (gyermekgyógyász infektológus, immunológus) Rodriguez-Gallego, Carlos Banchereau, Jacques Abel, Laurent Li, Xiaoxia Chaussabel, Damien Puel, Anne Casanova, Jean-Laurent
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