SOD1 and amyotrophic lateral sclerosis: A potentiation is shown here in hSOD1G85R Tg mice, and similar effects have previously been reported for ALS-linked hSOD1 mutants, including G93A [23, 26, 27], G37R [24, 28], L126Z [23, 24], A4V [23], and G85R [25] as well as for experimental SOD1 mutants such as V103Z [28] and T116X [29].