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Building a brain in the gut: development of the enteric nervous system
Author(s) -
Goldstein AM,
Hofstra RMW,
Burns AJ
Publication year - 2013
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1111/cge.12054
Subject(s) - enteric nervous system , neural crest , biology , hirschsprung's disease , neuroscience , gastrointestinal tract , interstitial cell of cajal , gut–brain axis , neurotrophic factors , disease , microbiology and biotechnology , bioinformatics , pathology , gut flora , immunology , medicine , genetics , embryo , immunohistochemistry , biochemistry , receptor
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.