SIGMAR1 and amyotrophic lateral sclerosis: In addition, the administration of Sig-1R ligands was shown to exert neuroprotection in various experimental models of MN degeneration, including in vitro excitotoxicity [11,12] and in vivo models of ALS [12,13,14,15], spinal root injury [16,17], and the wobbler mouse, a model of spontaneous MN degeneration [18].