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Amyotrophic Lateral sclerosis (ALS) is an adult-onset neurodegenerative disease affecting motor neurons and leading to paralysis and death of the patients. Missense mutations in the gene for the ubiquitously expressed Cu/Zn superoxide dismutase (SOD1) are the best known cause for familial ALS leading to motor neuron death through an acquired toxic function still unidentified. Mice expressing ubiqu ...