Recent studies have demonstrated that the SIRT1/PGC-1α signaling pathway exerts potential neuroprotective properties in neurodegenerative diseases [38,39] such as Alzheimer’s disease [40], Parkinson’s disease 41], Huntington’s disease [39], acute stroke [41,42], chronic epilepsy, and status epilepticus [43,44,45,46]. The gene discussed is PPARGC1A; the disease is juvenile Huntington disease.