Despite a number of former studies highlighted a significant deregulation of other PKC- isozymes (α, β, ζ and δ) in the motor neurons of ALS patients and in SOD1-G93A murine models [17,18,19,35,36], no previous studies have investigated the role of the ε-isozyme in ALS pathophysiology. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.