The authors have no conflict of interest.
Building a brain in the gut: development of the enteric nervous system
Article first published online: 27 NOV 2012
© 2012 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd
Volume 83, Issue 4, pages 307–316, April 2013
How to Cite
Building a brain in the gut: development of the enteric nervous system., , .
- Issue published online: 12 MAR 2013
- Article first published online: 27 NOV 2012
- Accepted manuscript online: 20 NOV 2012 07:51AM EST
- Manuscript Revised: 1 NOV 2012
- Manuscript Accepted: 1 NOV 2012
- Manuscript Received: 2 OCT 2012
- NIH. Grant Number: R01DK080914
- enteric nervous system;
- Hirschsprung disease;
- enteric neuropathies;
- neural crest cells;
The enteric nervous system (ENS), the intrinsic innervation of the gastrointestinal tract, is an essential component of the gut neuromusculature and controls many aspects of gut function, including coordinated muscular peristalsis. The ENS is entirely derived from neural crest cells (NCC) which undergo a number of key processes, including extensive migration into and along the gut, proliferation, and differentiation into enteric neurons and glia, during embryogenesis and fetal life. These mechanisms are under the molecular control of numerous signaling pathways, transcription factors, neurotrophic factors and extracellular matrix components. Failure in these processes and consequent abnormal ENS development can result in so-called enteric neuropathies, arguably the best characterized of which is the congenital disorder Hirschsprung disease (HSCR), or aganglionic megacolon. This review focuses on the molecular and genetic factors regulating ENS development from NCC, the clinical genetics of HSCR and its associated syndromes, and recent advances aimed at improving our understanding and treatment of enteric neuropathies.