Impaired T-cell receptor activation in IL-1 receptor-associated kinase-4-deficient patients

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

卷:126

Article

McDonald, Douglas R.^1,2  Goldman, Frederick^3,4  Gomez-Duarte, Oscar D.⁵  Issekutz, Andrew C.⁶  Kumararatne, Dinakantha S.⁷  Doffinger, Rainer⁷  Geha, Raif S.^1,2 

[1] Childrens Hosp, Div Immunol, Boston, MA 02115 USA

[2] Harvard Univ, Sch Med, Dept Pediat, Boston, MA 02115 USA

[3] Childrens Hosp Alabama, Div Hematol & Oncol, Birmingham, AL USA

[4] Childrens Hosp Alabama, Dept Pediat, Birmingham, AL USA

[5] Univ Iowa, Childrens Hosp, Dept Pediat, Iowa City, IA USA

[6] Dalhousie Univ, Dept Pediat, Halifax, NS, Canada

[7] Addenbrookes Hosp, Dept Clin Biochem & Immunol, Cambridge, England

关键词: IL-1 receptor-associated kinase 4;    T cell;    T-cell receptor;    cytokines;    TNF-alpha;    IL-2;    IL-6;    IFN-gamma;    CD25;    CD69;   

DOI  :  10.1016/j.jaci.2010.05.013

来源: Elsevier

【 摘 要 】

Background: IL-1 receptor-associated kinase 4 (IRAK-4) is an effector of the Toll-like receptor and IL-1 receptor pathways that plays a critical role in innate immune responses. The role of IRAK-4 in adaptive immune functions in human subjects is incompletely understood. Objective: We sought to evaluate T-cell function in IRAK-4 deficient patients. Methods: We compared upregulation of CD25 and CD69 on T cells and production of IL-2, IL-6, and IFN-gamma after stimulation of PBMCs from 4 IRAK-4-deficient patients and healthy control subjects with anti-CD3 and anti-CD28. Results: Upregulation of CD25 and CD69 on T cells and production of IL-6 and IFN-gamma, but not IL-2, was significantly reduced in IRAK-4-deficient patients. Conclusions: IRAK-4-deficient patients have defects in T-cell activation. (J Allergy Clin Immunol 2010;126:332-7.)

【 授权许可】

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