While the SOD1 model may only represent a small fraction of the heterogeneous disease population and posits a note of caution when interpreting mSOD1 mouse phenotypes at large across idiopathic forms of ALS, recent findings supporting the view of OS and mitochondrial damage to play a role in non-SOD1 ALS also come from cell and invertebrate models (Carri et al., 2015). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.