Intriguingly, SMN reduction in the G93A-SOD1 mouse model of ALS is effective in modifying the phenotypic severity in these ALS mice26, and the overexpression of SMN in SOD1 mice, as well as in mice transgenic for an ALS –associated mutant TDP-43, attenuates motor neuron degeneration27,32. Here, SMN1 is linked to amyotrophic lateral sclerosis.