Misfolded SOD1 has been implicated in the induction of endoplasmic reticulum (ER) stress [12, 18], defective axonal transport [7], alteration of motor neuron excitability [19], and mitochondrial dysfunction [11, 13, 14, 20] in SOD1-mediated ALS disease models. Here, SOD1 is linked to amyotrophic lateral sclerosis.