Thus, St-Pierre et al. showed that the decrease in transcription and activity of peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1 α) in an HD mice model and the brain tissues from HD patients led to the downregulation of genes encoding SOD1, SOD2, and glutathione peroxidase, resulting an increased oxidative damage and neuronal death [55]. This evidence concerns the gene SOD1 and Huntington disease.