Mutations in other genes can cause ALS at lower frequencies and include FUS [7], TDP43/TARDBP [8,9], optineurin/OPTN [10], Profilin/PFN1 [11], SQSTM1 [12,13] and ubiquilin2/UBQLN2 [14]. The gene discussed is UBQLN2; the disease is amyotrophic lateral sclerosis.