Pyogenic bacterial infections in humans with MyD88 deficiency

Horst von Bernuth; Capucine Picard; Zhongbo Jin; Rungnapa Pankla; Hui Xiao; Cheng-Lung Ku; Maya Chrabieh; Imen Ben Mustapha; Pegah Ghandil; Yildiz Camcioglu; Julia Vasconcelos; Nicolas Sirvent; Margarida Guedes; Artur Bonito Vitor; Maria Jose Herrero-Mata; Juan Ignacio Arostegui; Carlos Rodrigo; Laia Alsina; Estibaliz Ruiz-Ortiz; Manel Juan; Claudia Fortuny; Jordi Yague; Jordi Anton; Mariona Pascal; Huey-Hsuan Chang; Lucile Janniere; Yoann Rose; Ben-Zion Garty; Helen Chapel; Andrew Issekutz; Laszlo Marodi; Carlos Rodriguez-Gallego; Jacques Banchereau; Laurent Abel; Xiaoxia Li; Damien Chaussabel; Anne Puel; Jean-Laurent Casanova

doi:10.1126/science.1158298

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Pyogenic bacterial infections in humans with MyD88 deficiency

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Pyogenic bacterial infections in humans with MyD88 deficiency

Horst von Bernuth, Capucine Picard, Zhongbo Jin, Rungnapa Pankla, Hui Xiao, Cheng-Lung Ku, Maya Chrabieh, Imen Ben Mustapha, Pegah Ghandil, Yildiz Camcioglu, …

Science (American Association for the Advancement of Science), Vol.321(5889), pp.691-696

01/08/2008

DOI: https://doi.org/10.1126/science.1158298

PMID: 18669862

Résumé

Multidisciplinary Sciences

Science & Technology

Science & Technology - Other Topics

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.

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Titre: Pyogenic bacterial infections in humans with MyD88 deficiency
Créateurs - sans rôle: Horst von Bernuth - INSERM, U550, Paris, France
Capucine Picard - INSERM, U550, Paris, France
Zhongbo Jin - Baylor Inst Immunol Res, Dallas, TX 75204 USA
Rungnapa Pankla - Baylor Inst Immunol Res, Dallas, TX 75204 USA
Hui Xiao - Cleveland Clin Fdn, Cleveland, OH 44195 USA
Cheng-Lung Ku - INSERM, U550, Paris, France
Maya Chrabieh - INSERM, U550, Paris, France
Imen Ben Mustapha - INSERM, U550, Paris, France
Pegah Ghandil - INSERM, U550, Paris, France
Yildiz Camcioglu - Istanbul Univ, Cerrahpasa Med Sch, Istanbul, Turkey
Julia Vasconcelos - Gen Hosp Santo Antonio, Oporto, Portugal
Nicolas Sirvent - Univ Hosp Archet 2, Nice, France
Margarida Guedes - Gen Hosp Santo Antonio, Oporto, Portugal
Artur Bonito Vitor - Hosp Sao Joao, Oporto, Portugal
Maria Jose Herrero-Mata - Inst Invest Germans Trias & Pujol, LIRAD Banco Sangre & Tejidos, Barcelona, Spain
Juan Ignacio Arostegui - IDIBAPS, Dept Immunol, Hosp Clin, Barcelona, Spain
Carlos Rodrigo - Autonomous Univ Barcelona, Hosp Germans Trias & Pujol, Barcelona, Spain
Laia Alsina - Univ Barcelona, Sant Joan Deu Hosp, Barcelona, Spain
Estibaliz Ruiz-Ortiz - Inst Invest Germans Trias & Pujol, LIRAD Banco Sangre & Tejidos, Barcelona, Spain
Manel Juan - IDIBAPS, Dept Immunol, Hosp Clin, Barcelona, Spain
Claudia Fortuny - Univ Barcelona, Sant Joan Deu Hosp, Barcelona, Spain
Jordi Yague - IDIBAPS, Dept Immunol, Hosp Clin, Barcelona, Spain
Jordi Anton - Univ Barcelona, Sant Joan Deu Hosp, Barcelona, Spain
Mariona Pascal - IDIBAPS, Dept Immunol, Hosp Clin, Barcelona, Spain
Huey-Hsuan Chang - Inst Pasteur, Paris, France
Lucile Janniere - INSERM, U550, Paris, France
Yoann Rose - INSERM, U550, Paris, France
Ben-Zion Garty - Schneider Childrens Med Ctr, Petah Tiqwa, Israel
Helen Chapel - Univ Oxford, Oxford, England
Andrew Issekutz - Dalhousie Univ, Halifax, NS, Canada
Laszlo Marodi - Univ Debrecen, H-4012 Debrecen, Hungary
Carlos Rodriguez-Gallego - Gran Canaria Dr Negrin Hosp, Las Palmas Gran Canaria, Spain
Jacques Banchereau - Baylor Inst Immunol Res, Dallas, TX 75204 USA
Laurent Abel - Inserm
Xiaoxia Li - Cleveland Clin Fdn, Cleveland, OH 44195 USA
Damien Chaussabel - Baylor Inst Immunol Res, Dallas, TX 75204 USA
Anne Puel - INSERM, U550, Paris, France
Jean-Laurent Casanova - INSERM, U550, Paris, France
Détails de publication: Science (American Association for the Advancement of Science), Vol.321(5889), pp.691-696
Éditeur: Amer Assoc Advancement Science
Nombre de pages: 6
Note de subvention: U19 AIO57234-02 / PHS HHS; United States Department of Health & Human Services; United States Public Health Service U19 AI057234; U19 AI057234-02 / NIAID NIH HHS; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute of Allergy & Infectious Diseases (NIAID) U19AI057234 / NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute of Allergy & Infectious Diseases (NIAID) Howard Hughes Medical Institute
Identifiants: 99148757709311
Unité académique: Recherche hors université
Langue: English
Type de ressource: Journal article