These data are the first to be presented in the context of human ALS patients with SOD1 mutations, though are in broad agreement with previous studies of mutant Sod1 rodent models of ALS that implicate a reduction in GluA2 expression in spinal motor neurons 11, 13, 23, 24, 25, 26, 27. This evidence concerns the gene GRIA2 and amyotrophic lateral sclerosis.