PPARGC1A and juvenile Huntington disease: Peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1α) is a transcriptional coactivator that regulates a wide range of genes involved in mitochondrial biogenesis, fatty acid oxidation, and oxidative metabolism (Wu et al., 1999; Liang and Ward, 2006), which has been implicated in Huntington’s disease, Parkinson’s diseases, ALS, and other neurodegenerative diseases (Róna-Vörös and Weydt, 2010).