Whilst 90% of ALS cases are sporadic, 10% of cases are familial, caused by mutations in genes encoding ubiquitously expressed proteins, including transactive response DNA binding protein (TDP-43), fused in sarcoma (FUS), optineurin, superoxide dismutase 1 (SOD1), and Chromosome 9 open reading frame 72 (C9orf72) (Renton et al., 2014) (Table 1). The gene discussed is OPTN; the disease is amyotrophic lateral sclerosis.