SIGMAR1 and amyotrophic lateral sclerosis: Whereas previous studies have reported that PRE-084 (Mancuso et al., 2012) and SA4503 (Ono et al., 2014) treatment ameliorated the progression and survival of spinal MNs, we report for the first time that a Sig-1R antagonist, such as BD1063, has also neuroprotective effects in preclinical models of ALS.