RelB-deficient autoinflammatory pathology presents as interferonopathy, but in mice is interferon-independent.

ABSTRACT:

Background

Autoimmune diseases are leading causes of ill health and morbidity and have diverse etiology. Two signaling pathways are key drivers of autoimmune pathology, interferon and nuclear factor-κB (NF-κB)/RelA, defining the 2 broad labels of interferonopathies and relopathies. Prior work has established that genetic loss of function of the NF-κB subunit RelB leads to autoimmune and inflammatory pathology in mice and humans.

Objective

We sought to characterize RelB-deficient autoimmunity by unbiased profiling of the responses of immune sentinel cells to stimulus and to determine the functional role of dysregulated gene programs in the RelB-deficient pathology.

Methods

Transcriptomic profiling was performed on fibroblasts and dendritic cells derived from patients w

SUBMITTER: Navarro HI 

PROVIDER: S-EPMC10858800 | biostudies-literature | 2023 Nov

REPOSITORIES: biostudies-literature