In this study, we predicted a relationship between NEAT1 deficiency and TDP-43 pathology from ALS postmortem spinal cords and analyzed the role of TDP-43 on NEAT1 using cultured cells, primary neurons, and TDP-43-cKO mice, indicating that TDP-43 positively regulates NEAT1 expression. Here, TARDBP is linked to amyotrophic lateral sclerosis.