Although the exact mechanisms by which diverse SOD1 mutations all cause ALS remains unclear, many toxic effects caused by SOD1 mutants have been reported, including excitotoxicity, oxidative stress, endoplasmic reticulum (ER) stress, mitochondrial dysfunction, axonal transport disruption, prion-like propagation, and non-cell autonomous toxicity of neuroglia (Hayashi et al., 2016). Here, SOD1 is linked to amyotrophic lateral sclerosis.