The notion that autophagy dysfunction contributes to ALS-FTD pathogenesis is strongly supported by the identification of numerous ALS-FTD genes involved in autophagy regulation, including SQSTM1 (Fecto et al., 2011), OPTN (Maruyama et al., 2010; Wong and Holzbaur, 2014), TBK1 (Cirulli et al., 2015; Freischmidt et al., 2015; Pottier et al., 2015), and VCP (Johnson et al., 2010). The gene discussed is OPTN; the disease is amyotrophic lateral sclerosis.