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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.

Original publication

DOI

10.1126/science.1158298

Type

Journal article

Journal

Science

Publication Date

01/08/2008

Volume

321

Pages

691 - 696

Keywords

Adolescent, Animals, Bacterial Infections, Cell Line, Transformed, Child, Child, Preschool, Cytokines, Disease Susceptibility, Female, Gene Deletion, Humans, Immunity, Innate, Male, Mice, Mutation, Missense, Myeloid Differentiation Factor 88, Pneumococcal Infections, Pseudomonas Infections, Receptors, Interleukin-1, Signal Transduction, Staphylococcal Infections, Toll-Like Receptors, Transfection