Dreser et al. evaluated the known SigmaR1 E102Q mutation associated with Amyotrophic Lateral Sclerosis (ALS) and SigmaR1-mediated neurodegeneration to study the effects of SigmaR1 on protein homeostasis, using immunohistochemistry, immunocytochemistry, and mitochondrial toxicity assays [28]. Here, SIGMAR1 is linked to amyotrophic lateral sclerosis.