Further, Prp-SIRT1 mice are resistant to proteotoxic stress by an ALS-linked mutant of superoxide dismutase 1 (SOD1), due partly to SIRT1-mediated deacetylation of the heat shock factor 1 (HSF1) and the resulting upregulation of HSP70i11. This evidence concerns the gene SIRT1 and amyotrophic lateral sclerosis.