Mutations in several familial ALS genes such as TARDBP, SOD1, SQSTM1, VCP, and CHCHD10 have been reported in relation to FOSMN syndrome, and TDP-43-positive intraneuronal inclusions are identified in the brain, spinal cord, and dorsal root ganglia, suggesting that FOSMN is most likely to be a TDP-43 proteinopathy within the ALS-FTLD spectrum [191,192,193,194]. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.