Degeneration of NOS neurons in the ENS and a high transgene expression of human mutant TDP-43 in the nuclei of neurons of the myenteric plexus. Mice had an extremely rigid abdomen (intestinal obstruction), a thinned colon, enlarged cecum and distension of the small intestines. For the degeneration of NOS neurons, the number of neurons was decreased and the neurons that remained were enlarged. Nitric oxide synthase expressing neurons were severely affected in terminal ileum and colon. The gene discussed is TARDBP; the disease is intestinal obstruction.