SOD1 and amyotrophic lateral sclerosis: In this connection, it was reported that high levels of ER stress markers were observed in motor neurons derived from patients with amyotrophic lateral sclerosis, a severe motor neuron disease, carrying the A4V mutation in superoxide dismutase 1 (Kiskinis et al., 2014; Wainger et al., 2014); and that oral administration of sodium phenylbutyrate and taurursodiol, chemical chaperones that mitigate ER stress, significantly slowed functional decline in patients with amyotrophic lateral sclerosis (Paganoni et al., 2021; Paganoni et al., 2020).