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The <jats:styled-content style=\"fixed-case\">ENS<\/jats:styled-content> is entirely derived from neural crest cells (<jats:styled-content style=\"fixed-case\">NCC<\/jats:styled-content>) 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 <jats:styled-content style=\"fixed-case\">ENS<\/jats:styled-content> development can result in so\u2010called enteric neuropathies, arguably the best characterized of which is the congenital disorder Hirschsprung disease (<jats:styled-content style=\"fixed-case\">HSCR<\/jats:styled-content>), or aganglionic megacolon. 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