SIGMAR1 and amyotrophic lateral sclerosis: Functional studies to determine molecular mechanism showed that ALS associated Sigmar1 mutations (p.E102Q and p.L95 fs) (Al-Saif et al., 2011; Watanabe et al., 2016) are uniformly unstable and non−functional when expressed in Neuro2a (N2a) cells, suggesting a role of Sigmar1’s loss of function in ALS (Al-Saif et al., 2011; Watanabe et al., 2016).