Interferon gamma rebalances immunopathological signatures in chronic granulomatous disease through metabolic rewiring.

ABSTRACT:

Abstract

Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by recurrent life-threatening infections and hyperinflammatory complications. It is caused by mutations in the reduced nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex and the consequent loss of reactive oxygen species (ROS) production. Recombinant human interferon gamma (rIFN-γ) prophylaxis reduces the risk of severe infections, but the mechanisms behind its efficacy in CGD are still an open question, as it does not restore NADPH oxidase-dependent ROS production. Here, we demonstrate that the innate immune cells of patients with CGD are transcriptionally and functionally reprogrammed to a hyperactive inflammatory status, displaying an impaired in vitro induction of trained i

SUBMITTER: Bruno M 

PROVIDER: S-EPMC12556223 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature