Enteric nervous system development and Hirschsprung's disease: advances in genetic and stem cell studies

Nat Rev Neurosci. 2007 Jun;8(6):466-79. doi: 10.1038/nrn2137.


The enteric nervous system (ENS) has been explored by developmental neurobiologists and medical researchers for decades. Whereas developmental biologists have been unravelling the molecular mechanisms underlying the migration, proliferation and differentiation of the neural crest derivatives that give rise to the ENS, human geneticists have been uncovering the genetic basis for diseases of the ENS, notably Hirschsprung's disease. Here we discuss the exciting recent advances, including novel transgenic and genetic tools, a broadening range of model organisms, and the pursuit of ENS stem cells as a therapeutic tool, that are bringing these fields closer together.

Publication types

  • Review

MeSH terms

  • Animals
  • Cell Differentiation / genetics
  • Cell Movement / genetics
  • Enteric Nervous System / embryology*
  • Enteric Nervous System / metabolism
  • Enteric Nervous System / physiopathology
  • Gene Expression Regulation, Developmental / genetics*
  • Hirschsprung Disease / genetics
  • Hirschsprung Disease / metabolism
  • Hirschsprung Disease / physiopathology*
  • Humans
  • Neural Crest / embryology
  • Neural Crest / metabolism
  • Neural Crest / physiopathology
  • Neurons* / cytology
  • Neurons* / physiology
  • Receptors, Nerve Growth Factor / genetics
  • Signal Transduction / genetics
  • Stem Cells / cytology
  • Stem Cells / physiology*


  • Receptors, Nerve Growth Factor