Although sporadic in most cases, familial forms of ALS have been associated with mutations in specific genes, including SOD1, TDP-43, fused in sarcoma/translocated in liposarcoma (FUS/TLS, which we will refer to hereafter as FUS), VCP, optineurin, tank-binding kinase 1 (TBK1), dynactin subunit 1, angiogenin, C9orf72, ubiquilin 2, sequestosome 1 (p62), profiling-1, hnRNP A1, matrin-3, tubulin α-4A chain, and coiled-coil-helix-coiled-coil-helix domain-containing protein 10 (reviewed by Taylor et al., 2016). Here, FUS is linked to amyotrophic lateral sclerosis.