In recent years, genetic analyses revealed Sig-1R gene mutations involved in a juvenile form of ALS (Al-Saif et al., 2011; Watanabe et al., 2016) and in forms of motor neuropathies (Almendra et al., 2018; Ververis et al., 2019). The gene discussed is SIGMAR1; the disease is amyotrophic lateral sclerosis.