Because chaperones and HSPs bind to exposed surface hydrophobic domains to assist protein re-folding or degradation, mutant H46R/H48Q SOD1 mice were crossed with the HSF1+/0 transgenic mice (H46R/H48QxHSF1) in order to determine the effects of HSF1 upregulation on disease progression of ALS. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.