Reactive oxygen species deficiency induces autoimmunity with type 1 interferon signature.

ABSTRACT:

Aims

Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by mutations in the phagocyte reactive oxygen species (ROS)-producing NOX2 enzyme complex and characterized by recurrent infections associated with hyperinflammatory and autoimmune manifestations. A translational, comparative analysis of CGD patients and the corresponding ROS-deficient Ncf1(m1J) mutated mouse model was performed to reveal the molecular pathways operating in NOX2 complex deficient inflammation.

Results

A prominent type I interferon (IFN) response signature that was accompanied by elevated autoantibody levels was identified in both mice and humans lacking functional NOX2 complex. To further underline the systemic lupus erythematosus (SLE)-related autoimmune process, we show that naïv

SUBMITTER: Kelkka T 

PROVIDER: S-EPMC4224049 | biostudies-literature | 2014 Dec

REPOSITORIES: biostudies-literature