Conversely, mutations in TIA-1 [28] or ATXN2 [25], which are crucial components of stress granules [86,87], have also been identified as either causing ALS or increasing its risk, and ATXN2 altered the subcellular distribution and toxicity of TARDBP with enhanced mutant FUS toxicity [16,25,88]. The gene discussed is ATXN2; the disease is amyotrophic lateral sclerosis.