It is interesting to note that expression of mutant TAR DNA binding protein 43 (TDP-43) and Fused in Sarcoma (FUS), pathogenic proteins also known to be found in ALS patients (Arai et al., 2006; Neumann et al., 2006; Kwiatkowski et al., 2009; Vance et al., 2009), can increase the immunoreactivity for misfolded SOD1 using a disease-specific antibody (3H1), both in patients and cultured human cells (SH-SY5Y; Pokrishevsky et al., 2012). Here, TARDBP is linked to amyotrophic lateral sclerosis.