At the molecular level, PGC-1α stimulates TFEB, a master regulator of the autophagy-lysosome pathway, thereby promoting HTT protein turnover and elimination.474,475 At the organelle level, several publications advocated the role of PGC-1α in HD-related mitochondrial impairment and its potential as a therapeutic target to treat HD.470,476–478 PGC-1α upregulation increases mitochondrial mass and rebalances mitochondrial dynamics as well as promoting the mitochondrial fusion.477 In BAT from HD mice, a decrement in the numbers of functional mitochondria and ATP/ADP ratio are found. This evidence concerns the gene PPARGC1A and Huntington disease.