Previous studies have demonstrated intestinal blockage and/or megacolon as a major phenotype in prion-promoter driven TDP-43 mouse overexpression models [40, 41, 44] however, the TDP-43WTxQ331K mice displayed normal bowel movements throughout their lives, and physical examination of the intestines immediately following euthanasia did not detect any evidence of intestinal blockage or megacolon. The gene discussed is TARDBP; the disease is Aganglionic megacolon.