OPTN and amyotrophic lateral sclerosis: We found that these intracytoplasmic inclusions, in the spinal cord of ALS patients with the heterozygous OPTN-E478G or homozygous Q398X mutation, stained positive for linear ubiquitin and activated NF-κB (P-p65) in partial co-localization with P-TDP-43 (Fig. 6 and Supplementary Fig. 13).