Development and developmental disorders of the enteric nervous system

Nat Rev Gastroenterol Hepatol. 2013 Jan;10(1):43-57. doi: 10.1038/nrgastro.2012.234. Epub 2012 Dec 11.


The enteric nervous system (ENS) arises from neural crest-derived cells that migrate into and along the gut, leading to the formation of a complex network of neurons and glial cells that regulates motility, secretion and blood flow. This Review summarizes the progress made in the past 5 years in our understanding of ENS development, including the migratory pathways of neural crest-derived cells as they colonize the gut. The importance of interactions between neural crest-derived cells, between signalling pathways and between developmental processes (such as proliferation and migration) in ensuring the correct development of the ENS is also presented. The signalling pathways involved in ENS development that were determined using animal models are also described, as is the evidence for the involvement of the genes encoding these molecules in Hirschsprung disease-the best characterized paediatric enteric neuropathy. Finally, the aetiology and treatment of Hirschsprung disease in the clinic and the potential involvement of defects in ENS development in other paediatric motility disorders are outlined.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cell Movement
  • Cell Proliferation
  • Enteric Nervous System / cytology
  • Enteric Nervous System / embryology*
  • Enteric Nervous System / physiology
  • Gastrointestinal Diseases / embryology*
  • Gastrointestinal Diseases / pathology
  • Gastrointestinal Diseases / physiopathology
  • Hirschsprung Disease / embryology*
  • Hirschsprung Disease / pathology
  • Hirschsprung Disease / physiopathology
  • Humans
  • Mice
  • Models, Animal
  • Neural Crest / cytology
  • Neural Crest / embryology*
  • Neural Crest / physiology
  • Signal Transduction / physiology*