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Depletion of mitochondrial glutathione has been associated with increased neuronal injury and has been implicated in the pathogenesis of several neurodegenerative diseases, including Alzheimers, Parkinsons, and multiple sclerosis

The second mechanism operating here is thought to result from these molecules reducing the amount of (GSH-H) -1 and (GSH-2H) -2

The below is not intended as a comprehensive review of ALS pathophysiology, but instead to highlight mechanisms targeted by drugs currently in phase 1, 2, and 3 studies

This pilot study was designed to demonstrate that (in)GSH results in an increase in the GSH signal in brain, and not to generate a comprehensive pharmacokinetic profile of the increased brain GSH signal