From Charcot to SOD1 Mechanisms of Selective Motor Neuron Death in ALS

(Reaume et al., 1996). Further, some mutants such as SOD1 retain full specific activity (Borchelt et al., The sixth anniversary has just passed since the land1994), while neither the age of onset nor rapidity of mark discovery (Rosen et al., 1993) that mutation in the progression of human disease correlate with dismutase enzyme superoxide dismutase 1 (SOD1) is a primary activity levels (Cudkowicz et al., 1997). The inescapable cause of a proportion of a dominantly inherited form conclusion from this abundance of evidence is that the of amyotrophic lateral sclerosis (ALS), more familiarly mutants have acquired one or more toxic properties, known in the United States as Lou Gehrig’s disease. irrespective of the amount of SOD1 activity each retains. First described by Charcot 124 years before that, the