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Pyogenic bacterial infections in humans with MyD88 deficiency

Horst von Bernuth1, Capucine Picard, Zhongbo Jin

  • 1Human Genetics of Infectious Diseases, INSERM U550, Paris, France.

Science (New York, N.Y.)
|August 2, 2008
PubMed

Insights

Myeloid differentiation factor 88 (MyD88) deficiency in children causes severe pyogenic bacterial infections. However, these children show normal resistance to other microbes, indicating MyD88 is crucial for specific bacterial defenses.

Area of Science:

  • Immunology
  • Infectious Diseases
  • Genetics

Background:

  • Myeloid differentiation factor 88 (MyD88) is a critical adapter protein for Toll-like receptors (TLRs) and IL-1 receptors.
  • MyD88 deficiency in mice increases susceptibility to various pathogens in experimental infections.

Purpose of the Study:

  • To investigate the role of MyD88 in human natural infections.
  • To characterize the clinical presentation and immune response in individuals with MyD88 deficiency.

Main Methods:

  • Clinical case study of nine children with autosomal recessive MyD88 deficiency.
  • Assessment of resistance to a broad range of microbial pathogens.
  • Evaluation of clinical status and immune function over time.

Main Results:

  • Children with MyD88 deficiency experienced life-threatening, recurrent pyogenic bacterial infections, including invasive pneumococcal disease.
  • These patients exhibited normal resistance to other microbial infections.
  • Clinical improvement was observed with age, unrelated to cellular leakiness.

Conclusions:

  • MyD88-dependent TLRs and IL-1Rs are essential for immunity against specific pyogenic bacteria.
  • MyD88 is redundant for host defense against most natural infections.
  • This highlights a specific role for MyD88 in combating certain bacterial pathogens in humans.

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