Variations in the intracellular redox state can, therefore, transiently modify the activity of Nrf2 and its activation has been shown to counteract a number of pathological mechanisms associated with several neurodegenerative diseases, such as Alzheimer’s disease, Parkinson’s disease (PD), Amyotrophic Lateral Sclerosis, Huntington’s disease and Multiple Sclerosis [9]. The gene discussed is NFE2L2; the disease is juvenile Huntington disease.