Classically used as a model for amyotrophic lateral sclerosis (ALS), transgenic rats expressing the mutated form of the human superoxide dismutase 1 (hSOD1G93A [18] showing a toxic gain of function [19]) are characterized by excitotoxic insults and increased nitroxidative stress [20,21]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.