Pyogenic Bacterial Infections in Humans with MyD88 Deficiency
Délégation Paris 5 · Inserm · +9 more institutions
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…
Citation impact
- FWCI
- 39.91
- Percentile
- 100%
- References
- 54
Authors
38- HVHorst von Bernuth
Délégation Paris 5, Inserm, Baylor University, Université Paris Cité, Assistance Publique – Hôpitaux de Paris, Human Genetic of Infectious Diseases
- CPCapucine Pïcard
Délégation Paris 5, Inserm, Baylor University, Université Paris Cité, Assistance Publique – Hôpitaux de Paris, Human Genetic of Infectious Diseases
- ZJZhongbo Jin
Délégation Paris 5, Inserm, Baylor University, Université Paris Cité, Assistance Publique – Hôpitaux de Paris, Human Genetic of Infectious Diseases
- RPRungnapa Pankla
Délégation Paris 5, Inserm, Baylor University, Université Paris Cité, Assistance Publique – Hôpitaux de Paris, Human Genetic of Infectious Diseases
- HXHui Xiao
Délégation Paris 5, Inserm, Baylor University, Université Paris Cité, Assistance Publique – Hôpitaux de Paris, Human Genetic of Infectious Diseases
Topics & keywords
- Immunology
- Immunity
- Receptor
- Innate immune system
- Disease
- Biology
- Medicine
- Microbiology