Transgenic mice with human SOD1G93A (B6SJL-TgN[SOD1-G93A]1Gur) are one of the most widely used models to study ALS as they recapitulate the pathophysiology in humans, such as motor neuron (MN) loss, degradation of neuromuscular junctions, axonal degeneration and limb paralysis [3, 8–11]. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.