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

Abstract

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.

Keywords: CD25; CD69; IFNγ; IL-2; IL-6; IRAK-4; T cell; T cell receptor; TNFα; cytokines.