TARDBP and amyotrophic lateral sclerosis: Thus, potentiated Hsp104 variants suppressed the toxicity of TDP-43WT, which forms cytoplasmic aggregates in the vast majority (~95%) of ALS cases, as well as the toxicity of rare ALS-linked TDP-43 variants that are found in sporadic and familial forms of ALS (Lagier-Tourenne et al., 2010; Robberecht and Philips, 2013; Sreedharan et al., 2008).