To investigate the pathophysiology of ALS and the role of mutated SOD1 in disease development and progression of ALS, a transgenic mouse model was created (tg(SOD1*G93A)1Gur), which expresses mutant SOD1 (SOD1*G93A) and develops adult-onset neurodegeneration of neurons in the lumbar spinal cord and motor cortex and progressive motor deficits, which leads to paralysis [9,10,11,12]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.