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Amyotrophic Lateral Sclerosis ALS is a motor neuron disease, a progressive and ultimately fatal neurodegenerative disorder characterized by motor neuron degeneration and subsequent muscle atrophy

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On the other hand, mechanistic experiments demonstrated that compound 67 promoted (i) an alteration in the energetic metabolism with a significant loss of ATP that is associated with the depolarization of mitochondrial potential ( m ), (ii) a decrease of intracellular ATP levels with an alteration of plasma membrane permeability, and (iii) a significant ROS production, which is the typical mode of action found for 4-aminoquinolines

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