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Review
. 2017 Oct:98:15-26.
doi: 10.1016/j.cyto.2017.02.006. Epub 2017 Mar 7.

Interferon regulatory factor signaling in autoimmune disease

Bharati Matta  1 , Su Song  1 , Dan Li  1 , Betsy J Barnes  2
Affiliations
Review

Interferon regulatory factor signaling in autoimmune disease

Bharati Matta et al. Cytokine. 2017 Oct.

Abstract

Interferon regulatory factors (IRFs) play critical roles in pathogen-induced innate immune responses and the subsequent induction of adaptive immune response. Dysregulation of IRF signaling is therefore thought to contribute to autoimmune disease pathogenesis. Indeed, numerous murine in vivo studies have documented protection from or enhanced susceptibility to particular autoimmune diseases in Irf-deficient mice. What has been lacking, however, is replication of these in vivo observations in primary immune cells from patients with autoimmune disease. These types of studies are essential as the majority of in vivo data support a protective role for IRFs in Irf-deficient mice, yet IRFs are often found to be overexpressed in patient immune cells. A significant body of work is beginning to emerge from both of these areas of study - mouse and human.

Keywords: Autoimmune disease; Cell signaling; Interferon regulatory factors; Pathogenesis.

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Figures

Figure 1.
Figure 1.. Implications of IRF signaling in human autoimmune diseases.
Schematic summarizing the effect(s) of IRF signaling in specific cell types, in response to specific stimuli, and the specific autoimmune disease it has been implicated in. Gene expression affected by individual IRFs is shown by matched colored font for each IRF. For example, the IRF1 molecule is shown in blue and gene expression affected by IRF1 is shown in blue. Overlapping functions for the IRFs are written in black font. Diseases affected by the IRFs are shown at the bottom surrounded by green. MS, multiple sclerosis; SS, Sjögren’s syndrome; RA, rheumatoid arthritis; IBD, inflammatory bowel disease.
Figure 2.
Figure 2.. Cell type-specific IRF signaling in human SLE.
Schematic summarizing the role of individual IRFs in SLE pathogenesis. The primary downstream effect of all indicated IRFs is on type I IFN expression.
Figure 3.
Figure 3.. Cell-type specific roles for IRFs in murine lupus.
A schematic showing which cell types IRF functions have been implicated in from Irf-deficient models of murine lupus.
See this image and copyright information in PMC

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