Primary mitochondrial diseases: The intertwined pathophysiology of bioenergetic dysregulation, oxidative stress and neuroinflammation.
Kevin AguilarPatrycja JakubekAntonio ZorzanoMariusz Roman WieckowskiPublished in: European journal of clinical investigation (2024)
In summary, this review provides a comprehensive overview of bioenergetic alterations and redox imbalance in cellular models of PMDs while underscoring the significance of neuroinflammation as a potential driver in disease progression.