Caspase-11 drives macrophage hyperinflammation in models of Polg-related mitochondrial disease.

ABSTRACT: Mitochondrial diseases (MtD) represent a significant public health challenge due to their heterogenous clinical presentation, often severe and progressive symptoms, and lack of effective therapies. Environmental exposures, such bacterial and viral infection, can further compromise mitochondrial function and exacerbate the progression of MtD. Infections in MtD patients more frequently progress to sepsis, pneumonia, and other detrimental inflammatory endpoints. However, the underlying immune alterations that enhance immunopathology in MtD remain unclear, constituting a key gap in knowledge that complicates treatment and increases mortality in this vulnerable population. Here we employ in vitro and in vivo approaches to clarify the molecular and cellular basis for innate immune hyperactivity

SUBMITTER: VanPortfliet JJ 

PROVIDER: S-EPMC11118447 | biostudies-literature | 2025 Jan

REPOSITORIES: biostudies-literature