Pyogenic bacterial infections in humans with MyD88 deficiency

被引：593

作者：

von Bernuth, Horst ^{[1
,2
]}

Picard, Capucine ^{[1
,2
,3
]}

Jin, Zhongbo ^{[4
,5
]}

Pankla, Rungnapa ^{[4
,6
]}

Xiao, Hui ^{[7
]}

Ku, Cheng-Lung ^{[1
,2
]}

Chrabieh, Maya ^{[1
,2
]}

Ben Mustapha, Imen ^{[1
,2
,8
]}

Ghandil, Pegah ^{[1
,2
]}

Camcioglu, Yildiz ^{[9
]}

Vasconcelos, Julia ^{[10
]}

Sirvent, Nicolas ^{[11
]}

Guedes, Margarida ^{[10
]}

Vitor, Artur Bonito ^{[12
]}

Herrero-Mata, Maria Jose ^{[13
]}

Arostegui, Juan Ignacio ^{[14
]}

Rodrigo, Carlos ^{[15
]}

Alsina, Laia ^{[16
]}

Ruiz-Ortiz, Estibaliz ^{[13
]}

Juan, Manel ^{[14
]}

Fortuny, Claudia ^{[16
]}

Yague, Jordi ^{[14
]}

Anton, Jordi ^{[16
]}

Pascal, Mariona ^{[14
]}

Chang, Huey-Hsuan ^{[17
,18
]}

Janniere, Lucile ^{[1
,2
]}

Rose, Yoann ^{[1
,2
]}

Garty, Ben-Zion ^{[19
]}

Chapel, Helen ^{[20
,21
]}

Issekutz, Andrew ^{[22
]}

Marodi, Laszlo ^{[23
]}

Rodriguez-Gallego, Carlos ^{[24
]}

Banchereau, Jacques ^{[4
]}

Abel, Laurent ^{[1
,2
]}

Li, Xiaoxia ^{[7
]}

Chaussabel, Damien ^{[4
]}

Puel, Anne ^{[1
,2
]}

Casanova, Jean-Laurent ^{[1
,2
,25
]}

机构：

[1] INSERM, U550, Paris, France

[2] Univ Paris 05, F-75270 Paris 06, France

[3] Hop Necker Enfants Malad, Study Ctr Primary Immunodeficiencies, Assistance Publ Hop Paris, Paris, France

[4] Baylor Inst Immunol Res, Dallas, TX 75204 USA

[5] Baylor Univ, Waco, TX 76798 USA

[6] Khon Kaen Univ, Khon Kaen, Thailand

[7] Cleveland Clin Fdn, Cleveland, OH 44195 USA

[8] Pasteur Inst Tunis, Tunis, Tunisia

[9] Istanbul Univ, Cerrahpasa Med Sch, Istanbul, Turkey

[10] Gen Hosp Santo Antonio, Oporto, Portugal

[11] Univ Hosp Archet 2, Nice, France

[12] Hosp Sao Joao, Oporto, Portugal

[13] Inst Invest Germans Trias & Pujol, LIRAD Banco Sangre & Tejidos, Barcelona, Spain

[14] IDIBAPS, Dept Immunol, Hosp Clin, Barcelona, Spain

[15] Autonomous Univ Barcelona, Hosp Germans Trias & Pujol, Barcelona, Spain

[16] Univ Barcelona, Sant Joan Deu Hosp, Barcelona, Spain

[17] Inst Pasteur, Paris, France

[18] INSERM, U818, Paris, France

[19] Schneider Childrens Med Ctr, Petah Tiqwa, Israel

[20] Univ Oxford, Oxford, England

[21] Oxford Radcliffe Hosp, Oxford, England

[22] Dalhousie Univ, Halifax, NS, Canada

[23] Univ Debrecen, H-4012 Debrecen, Hungary

[24] Gran Canaria Dr Negrin Hosp, Las Palmas Gran Canaria, Spain

[25] Hop Necker Enfants Malad, Pediat Hematol Immunol Unit, Paris, France

来源：

SCIENCE | 2008年 / 321卷 / 5889期

关键词：

D O I：

10.1126/science.1158298

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

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.

引用

页码：691 / 696

页数：6

共 29 条

[1] Targeted disruption of the MyD88 gene results in loss of IL-1- and IL-18-mediated function
Adachi, O
Kawai, T
Takeda, K
Matsumoto, M
Tsutsui, H
Sakagami, M
Nakanishi, K
Akira, S
[J]. IMMUNITY, 1998, 9 (01) : 143 - 150
[2] Myeloid differentiation factor 88-dependent signalling controls bacterial growth during colonization and systemic pneumococcal disease in mice
Albiger, B
Sandgren, A
Katsuragi, H
Meyer-Hoffert, U
Beiter, K
Wartha, F
Hornef, M
Normark, S
Normark, BH
[J]. CELLULAR MICROBIOLOGY, 2005, 7 (11) : 1603 - 1615
[3] Genetic analysis of host resistance: Toll-like receptor signaling and immunity at large
Beutler, Bruce
Jiang, Zhengfan
Georgel, Philippe
Crozat, Karine
Croker, Ben
Rutschmann, Sophie
Du, Xin
Hoebe, Kasper
[J]. ANNUAL REVIEW OF IMMUNOLOGY, 2006, 24 : 353 - 389
[4] Perspective - Primary immunodeficiencies: A field in its infancy
Casanova, Jean-Laurent
Abel, Laurent
[J]. SCIENCE, 2007, 317 (5838) : 617 - 619
[5] Herpes simplex virus encephalitis in human UNC-93B deficiency
Casrouge, Armanda
Zhang, Shen-Ying
Eidenschenk, Celine
Jouanguy, Emmanuelle
Puel, Anne
Yang, Kun
Alcais, Alexandre
Picard, Capucine
Mahfoufi, Nora
Nicolas, Nathalie
Lorenzo, Lazaro
Plancoulaine, Sabine
Senechal, Brigitte
Geissmann, Frederic
Tabeta, Koichi
Hoebe, Kasper
Du, Xin
Miller, Richard L.
Heron, Benedicte
Mignot, Cyril
de Villemeur, Thierry Billette
Lebon, Pierre
Dulac, Olivier
Rozenberg, Flore
Beutler, Bruce
Tardieu, Marc
Abel, Laurent
Casanova, Jean-Laurent
[J]. SCIENCE, 2006, 314 (5797) : 308 - 312
[6] Human complete Stat-1 deficiency is associated with defective type I and IIIFN responses in vitro but immunity to some low virulence viruses in vivo
Chapgier, Ariane
Wynn, Robert F.
Jouanguy, Emmanuelle
Filipe-Santos, Orchidee
Zhang, Shenying
Feinberg, Jacqueline
Hawkins, Kay
Casanova, Jean-Laurent
Arkwright, Peter D.
[J]. JOURNAL OF IMMUNOLOGY, 2006, 176 (08) : 5078 - 5083
[7] Adjuvant-enhanced antibody responses in the absence of toll-like receptor signaling
Gavin, Amanda L.
Hoebe, Kasper
Duong, Bao
Ota, Takayuki
Martin, Christopher
Beutler, Bruce
Nemazee, David
[J]. SCIENCE, 2006, 314 (5807) : 1936 - 1938
[8] HIRSCH MS, 1970, J IMMUNOL, V104, P1160
[9] Identification of Lps2 as a key transducer of MyD88-independent TIR signalling
Hoebe, K
Du, X
Georgel, P
Janssen, E
Tabeta, K
Kim, SO
Goode, J
Lin, P
Mann, N
Mudd, S
Crozat, K
Sovath, S
Han, J
Beutler, B
[J]. NATURE, 2003, 424 (6950) : 743 - 748
[10] Innate immune recognition
Janeway, CA
Medzhitov, R
[J]. ANNUAL REVIEW OF IMMUNOLOGY, 2002, 20 : 197 - 216

← 1 2 3 →