Human superoxide dismutase 1 (hSOD1) is an incredibly stable metalloenzyme and has become a paradigm for investigating the destabilizing effect on protein folding and misfolding linked to the fatal neurodegenerative disease, amyotrophic lateral sclerosis (ALS) (Valentine et al., 2005; Rakhit and Chakrabartty, 2006). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.