Mice overexpressing the ALS-linked mutant superoxide dismutase (SOD) 1 gene [mutant SOD1 mice (Gurney et al., 1994; Wong et al., 1995; Bruijn et al., 1997)] are a well-characterized rodent model for ALS, allowing a time-course pathological analysis of the spinal cord where motor neuron loss and subsequent neuroinflammation are observed. Here, SOD1 is linked to amyotrophic lateral sclerosis.