In addition, SOD1 toxicity has been shown to be linked to changes in the immune system, which has also been shown to contribute to the motor neuron pathology seen in ALS; however, the exact nature of the pathogenic mechanisms provoking motor neuron degeneration in mutant SOD1-linked ALS remains elusive (Ince et al., 2011). Here, SOD1 is linked to amyotrophic lateral sclerosis.