Remarkably, while transgenic mice ubiquitously expressing fALS-associated SOD1 mutants (e.g., G93A, G37R, and G85R) develop a rapidly progressive disease, with lower MN degeneration, reminiscent of human ALS (Gurney et al., 1994; Dal Canto and Gurney, 1995; Wong et al., 1995; Mourelatos et al., 1996; Bruijn et al., 1997), selective expression of the same mutations in MNs did not cause significant cell death. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.