Although the recent genetic discoveries in the field of ALS particularly relating to alterations in TARDBP (Kabashi et al., 2008), FUS/TLS (Kwiatkowski et al., 2009) and C9ORF72 (DeJesus-Hernandez et al., 2011; Renton et al., 2011) do identify previously unknown disease mechanisms requiring investigation, clearly SOD1 remains of key importance for understanding motor neuron degeneration and there is a need for better models to complement the repertoire of models currently available. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.