For example, activation of mitochondria-related genes, such as Sirtuin 3 (SIRT3), Peroxisome proliferator-activated receptor gamma co-activator-1 alpha (PGC1alpha), Uncoupling protein (UCP), has shown to alleviate HD symptoms in different models [23, 24, 28], highly suggesting improvement of mitochondrial functions is an important therapeutical strategy for HD (Fig. 1). This evidence concerns the gene SIRT3 and Huntington disease.