Transgenic mouse models developed to harbour mutations found in fALS patients mimic the progressive nature of the disease, aspects of motor neuron death, and histopathological hallmarks associated with clinical ALS [5,6].These animal models have been crucial in demonstrating that it is not a reduction of dismutase activity per se that predisposes the system to an ALS phenotype, but rather the acquisition of novel toxic properties in the SOD1 protein product [7]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.