PPARGC1A and juvenile Huntington disease: Pgc-1α null mice develop spongiform neurodegeneration in the striatum and deep layers of the cortex (Ma et al., 2010), and this phenotype has been implicated in PD and Huntington disease (Cui et al., 2006; St-Pierre et al., 2006; Tsunemi and La Spada, 2012), strongly suggesting that PGC-1α plays a significant role in maintaining neuronal function.