Fibroblasts from ALS patients with TARDBP and C9ORF72 mutations have shown aberrant formation of SG and/or phospho-TDP-43 aggregates in response to chronic oxidative stress [116] and ALS-causing FUS mutations have been shown to result in the aberrant incorporation of FUS into SGs [47,125,126,127]. Here, C9orf72 is linked to amyotrophic lateral sclerosis.