Optineurin-deficient (optn−/−) mice developed neuropathology resembling ALS, due to a higher level of RIPK1, RIPK3, and pMLKL and necroptosis in the spinal cord of optn−/− mice, which was rescued by inactivating RIPK1-kinase activity. This evidence concerns the gene OPTN and amyotrophic lateral sclerosis.