ALS models are commonly based on mutant superoxide dismutase 1 (SOD1), TAR DNA-binding protein 43 (TDP-43), fused in sarcoma or translocated in liposarcoma (FUS), the chromosome 9 open reading frame 72 (C9orf72) gene, ubiquilin 2 (UBQLN2), matrin 3 (MATR3), and senataxin (SETX) [275]. Here, SETX is linked to amyotrophic lateral sclerosis.