To determine to what extent our findings in animal models of neurodegenerative diseases are representative to the human conditions, we investigated the mRNA expression of SIRT3, canonical PPARGC1A and CNS-specific PPARGC1A in spinal cord and cortex of ALS patients and in the striatum and cerebellum of HD patients. This evidence concerns the gene PPARGC1A and amyotrophic lateral sclerosis.