Pyogenic bacterial infections in humans with MyD88 deficiency.
نویسندگان
چکیده
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.
منابع مشابه
Primary and Secondary Immunodeficiency Pyogenic Bacterial Infections in Humans With MyD88 Deficiency
PURPOSE OF THE STUDY. Myeloid differentiation protein 88 (MyD88) is a key downstream adapter for receptors of the innate immune system, including most Toll-like receptors (TLRs) and interleukin 1 receptors (IL-1Rs). MyD88 deficiency in mice leads to susceptibility to a broad range of pathogens, and the goal of this study was to determine whether there are children with recurrent infections who ...
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عنوان ژورنال:
- Science
دوره 321 5889 شماره
صفحات -
تاریخ انتشار 2008