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ALS is characterized by the degeneration of both upper motor neurons, which project from the cortex to the brainstem and the spinal cord, and lower motor neurons, which project from the brainstem or spinal cord to muscle

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Also, poly (GR) 80 interacts with mitochondrial ribosomal proteins, inducing mitochondrial dysfunction, in motor neurons differentiated from C9orf72 patient iPSCs, compared to controls (Lopez-Gonzalez et al., 2016)