SIGMAR1 and amyotrophic lateral sclerosis: Also, in a murine model of ALS, Sig-1R agonists enhanced preservation of spinal MNs and extended the lifespan of SOD1G93A mice, by acting on different pathways: PRE-084 treatment via modulation of NMDA calcium influx [25], pridopidine and SA4503 through activation of the ERK pathway [23,26].