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Pyogenic bacterial infections in humans with MyD88 deficiency.

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.

SUBMITTER: von Bernuth H 

PROVIDER: S-EPMC2688396 | biostudies-literature | 2008 Aug

REPOSITORIES: biostudies-literature

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Pyogenic bacterial infections in humans with MyD88 deficiency.

von Bernuth Horst H   Picard Capucine C   Jin Zhongbo Z   Pankla Rungnapa R   Xiao Hui H   Ku Cheng-Lung CL   Chrabieh Maya M   Mustapha Imen Ben IB   Ghandil Pegah P   Camcioglu Yildiz Y   Vasconcelos Júlia J   Sirvent Nicolas N   Guedes Margarida M   Vitor Artur Bonito AB   Herrero-Mata María José MJ   Aróstegui Juan Ignacio JI   Rodrigo Carlos C   Alsina Laia L   Ruiz-Ortiz Estibaliz E   Juan Manel M   Fortuny Claudia C   Yagüe Jordi J   Antón Jordi J   Pascal Mariona M   Chang Huey-Hsuan HH   Janniere Lucile L   Rose Yoann Y   Garty Ben-Zion BZ   Chapel Helen H   Issekutz Andrew A   Maródi László L   Rodriguez-Gallego Carlos C   Banchereau Jacques J   Abel Laurent L   Li Xiaoxia X   Chaussabel Damien D   Puel Anne A   Casanova Jean-Laurent JL  

Science (New York, N.Y.) 20080801 5889

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  ...[more]

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