The neurodegenerative changes observed in Gde2−/− animals show pronounced overlap with degenerative hallmarks observed in the spinal cords of mouse models of ALS such as the SOD1G93A animal, which harbors a transgene of the human SOD1 gene carrying the G93A mutation associated with fALS [27–29]. Here, SOD1 is linked to amyotrophic lateral sclerosis.