![]() GSH has two characteristic structural properties: a γ-glutamyl bond and a sulfhydryl group, which give GSH its distinctive qualities-stability and reductive power. ![]() Furthermore, the involvement of mitochondrial ROS in the signaling of new prescribed drugs and in other pathologies (or in other unmet medical needs, such as gender differences or coronavirus disease of 2019 (COVID-19) treatment) is still being revealed guaranteeing that research on mGSH will be an interesting topic for years to come. In this review, we will highlight the participation of mGSH as a contributor to disease progression in pathologies as diverse as Alzheimer’s disease, alcoholic and non-alcoholic steatohepatitis, or diabetic nephropathy. Since mitochondrial redox disorders play a central part in many diseases, harboring optimal levels of mGSH is vitally important. mGSH’s ability to act directly or as a co-factor in reactions catalyzed by other mitochondrial enzymes makes its presence essential to avoid or to repair oxidative modifications that can lead to mitochondrial dysfunction and subsequently to cell death. Among the numerous enzymatic and non-enzymatic antioxidant systems present in mitochondria, mitochondrial glutathione (mGSH) emerges as the main line of defense for maintaining the appropriate mitochondrial redox environment. Mitochondria are the main source of reactive oxygen species (ROS), most of them deriving from the mitochondrial respiratory chain.
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