ALS-linked mutants of nuclear RNA-binding proteins TDP43, FUS, hnRNPA1, hnRNPA2B1, ATXN2, and matrin 3 (MATR3) form mislocalized cytoplasmic aggregates because of the irreversible interactions with their prion-like low-complexity domains [162]. This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.