Transcriptomic analysis of WT and Polg D257A/D257A lungs six hours after intratrachael instillation of Pseudomonas aeruginosa

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. This dataset reports transcriptomic changes in lungs from wild-type mice and a mouse model of polymerase gamma (Polg)-related MtD (Polg D257A/D257A) six hours post intratrachael instillation of Pseudomonas aeruginosa strain PAO1. These data reveal a hyperinflammatory innate immune status in Polg D257A lungs characterized by elevated expression of genes involved in interferon and inflammatory cell death pathways. This work sheds new light on innate immune dysregulation in a model of mitochondrial dysfunction and reveals potential targets for limiting infection- and inflammation-related complications in Polg-related MtD.