In familial ALS patients, causal or risk-increasing roles in the pathogenesis of ALS have been reported for mutations in autophagy- and mitophagy-related genes, including ALS2/Alsin, charged multivesicular protein 2B (CHMP2B), C9orf72, dynactin 1 (DCTN1), Optineurin, p62/SQSTM1 (sequestosome 1), Ubiquilin 2, and Valosin-containing protein (VCP) (Yang et al., 2001; Munch et al., 2004; Parkinson et al., 2006; Johnson et al., 2010; Maruyama et al., 2010; DeJesus-Hernandez et al., 2011; Deng et al., 2011; Fecto et al., 2011; Rubino et al., 2012; Williams et al., 2012; Farg et al., 2014). The gene discussed is ALS2; the disease is amyotrophic lateral sclerosis.