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The ability of NSI-189 to reverse established neuropathy is encouraging, as intervention is the most likely scenario for translation to clinical use, particularly as large fiber conduction slowing and pain are currently accepted readouts for therapeutic efficacy in clinical trials [29]
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The partial compensation for GSH deficiency afforded by NAC in Gclc(h/h) hepatocytes is likely mediated by: (i) GSH synthesis by the salvage pathway, in which GGT utilizes cystine to produce χ-glutamylcystine thereby bypassing the action of GCL (Njalsson and Norgren 2005)
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