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Pre-marketing studies revealed an incidence of less than 1% of hemolysis and methemoglobinemia among 703 patients

We further speculated that loss of the extracellular GSH pool in gsh2 mutants could change the external environment and consequently affect extracellular redox homeostasis

This conversion can be inhibited by targeting mitochondrial ROS with antioxidants or selenium, further highlighting the importance of ROS in fibroblast-to-myofibroblast conversion [81, 82]

Jafari, N., Broer, L., van Duijn, C

In addition, metabolomics analysis showed an increased levels of glutamate, glutamine, glycine, aspartate and DMG in G93A-SOD1 compared to control cells (Fig