Direct administration of lentiviral vector expressing PGC-1α into the striatum of R6/2 transgenic HD mice completely prevented neuronal atrophy, complementing in vitro data showing PGC-1α overexpression reversed mitochondrial dysfunction in mutant STHdhQ111 cells, and abrogated the toxicity of mHtt in transfected primary striatal neurons, potentially through inducing the expression of genes encoding reactive oxygen species defence enzymes including Cu/Zn superoxide dismutase (SOD1), manganese SOD (SOD2), catalase, and glutathione peroxidase [105, 106]. This evidence concerns the gene SOD1 and Huntington disease.