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. 2020 Dec 16;12(1):1–24. doi: 10.1016/j.jcmgh.2020.12.010

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© 2020 The Authors

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

PMC Copyright notice

mNCCs are the embryonic origin of segmental ENS in SSHD. (A) Schematic model of current ENS development. vNCCs invade the foregut and migrate rostrocaudally to colonize the gut. (B) Model of modified ENS development. mNCCs migrate along the gut mesentery and undergo mesentery–gut migration to make a complementary contribution to the eNCC pool. (C) Pathogenic model of HSCR. Defective migration of both eNCCs and mNCCs jointly results in aganglionosis in the distal bowel. (D) Pathogenic model of SSHD. mNCCs preferentially migrate via the gut mesentery to form segmental ENS within the aganglionic gut.