Mutations in C9ORF72, TARDBP, FUS, VCP, UBQLN2, SQSTM1, and CHMP2B have all been associated with both ALS and FTD [46, 84], and mutations in hgRNPA2B1 and can cause multisystem proteinopathy and ALS [80]. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.