TARDBP and amyotrophic lateral sclerosis: As such, previous studies have determined that inducing the HSR by either overexpressing HSR components or utilizing pharmacological agonists reduces the number and toxicity of protein aggregates in both SOD1 and TDP-43 ALS cell culture and mouse models (Kieran et al., 2004; Kalmar et al., 2008; Crippa et al., 2010, 2016; Lin et al., 2013, 2016; Chen et al., 2016; Ganassi et al., 2016).