Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.
MyD88 is a key downstream adapter for most Toll-like receptors(TLRs) and interleukin-1 receptors (IL-1Rs). MyD88 deficiencyin mice leads to susceptibility to a broad range of pathogensin experimental settings of infection. We describe a distinctsituation in a natural setting of human infection. Nine childrenwith autosomal recessive MyD88 deficiency suffered from life-threatening,often recurrent pyogenic bacterial infections, including invasivepneumococcal disease. However, these patients were otherwisehealthy, with normal resistance to other microbes. Their clinicalstatus improved with age, but not due to any cellular leakinessin MyD88 deficiency. The MyD88-dependent TLRs and IL-1Rs aretherefore essential for protective immunity to a small numberof pyogenic bacteria, but redundant for host defense to mostnatural infections.
1 Human Genetics of Infectious Diseases, INSERM U550, Paris, France. 2 Paris Descartes University, France. 3 Study Center of Primary Immunodeficiencies, Assistance Publique Hôpitaux de Paris, Necker Hospital, Paris, France. 4 Baylor Institute for Immunology Research, Dallas, TX 75204, USA. 5 Baylor University, Waco, TX 76798, USA. 6 Khon Kaen University, Thailand. 7 Cleveland Clinic Foundation, OH 44195, USA. 8 Pasteur Institute of Tunis, Tunisia. 9 Cerrahpasa Medical School, Istanbul University, Turkey. 10 General Hospital of Santo António, Porto, Portugal. 11 University Hospital Archet 2, Nice, France. 12 Hospital S.João, Porto, Portugal. 13 LIRAD–Banco de Sangre y Tejidos, Instituto de Investigación Germans Trias i Pujol, Badalona, Barcelona, Spain. 14 Immunology Department, Hospital Clinic, IDIBAPS, Barcelona, Spain. 15 Germans Trias i Pujol Hospital, Barcelona Autonomous University, Spain. 16 Sant Joan de Déu Hospital, Barcelona University, Spain. 17 Dendritic Cell Immunobiology, Institut Pasteur and INSERM U818, Paris, France. 18 Schneider Children's Medical Center, Petah Tiqva, Israel. 19 University of Oxford and Oxford Radcliffe Hospital, Oxford, UK. 20 Dalhousie University, Halifax, Nova Scotia, Canada. 21 Debrecen University, Hungary. 22 Gran Canaria Dr Negrin Hospital, Las Palmas de Gran Canaria, Spain. 23 Pediatric Hematology-Immunology Unit, Necker Hospital, Paris, France.
* To whom correspondence should be addressed. E-mail: jean-laurent.casanova{at}inserm.fr
