Particularly, the Sig-1R is broadly expressed in cells of the CNS and is involved in the pathogenesis of CNS diseases, including Alzheimer’s disease (AD; Lahmy et al., 2013), Huntington’s disease (HD; Ryskamp D. et al., 2017), Parkinson’s disease (PD; Francardo et al., 2014) and amyotrophic lateral sclerosis (ALS; Ono et al., 2014). Here, SIGMAR1 is linked to amyotrophic lateral sclerosis.