Demyelination and axonal degeneration in the spinal cord of optn−/− ALS mice were obstructed in both RIPK1- and RIPK3-deficient mice or were prevented by Nec-1s-mediated inhibition of RIPK1, thus showing improvements in motor performance, survival, and axonal pathology. This evidence concerns the gene RIPK3 and amyotrophic lateral sclerosis.