Chapter 11. Development of the Enteric Nervous System in Relation to Hirschsprung's Disease

  1. Patrizia Ferretti3,
  2. Andrew Copp3,
  3. Cheryll Tickle4 and
  4. Gudrun Moore5
  1. Heather M. Young1,
  2. Donald F. Newgreen2 and
  3. Alan J. Burns3

Published Online: 25 JAN 2010

DOI: 10.1002/9780470090121.ch11

Embryos, Genes and Birth Defects, Second Edition

Embryos, Genes and Birth Defects, Second Edition

How to Cite

Young, H. M., Newgreen, D. F. and Burns, A. J. (2008) Development of the Enteric Nervous System in Relation to Hirschsprung's Disease, in Embryos, Genes and Birth Defects, Second Edition (eds P. Ferretti, A. Copp, C. Tickle and G. Moore), John Wiley & Sons Ltd, Oxford, UK. doi: 10.1002/9780470090121.ch11

Editor Information

  1. 3

    Neural Development Unit/Developmental Biology Unit, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK

  2. 4

    Division of Cell and Developmental Biology, School of Life Sciences, MSI/WTB Complex, University of Dundee, Dow Street, Dundee, UK

  3. 5

    Clinical and Molecular Genetics, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK

Author Information

  1. 1

    Department of Anatomy and Cell Biology, University of Melbourne, 3010 Victoria, Australia

  2. 2

    The Murdoch Children's Research Institute, Royal Children's Hospital, Parkville, 3052 Victoria, Australia

  3. 3

    Neural Development Unit/Developmental Biology Unit, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK

Publication History

  1. Published Online: 25 JAN 2010
  2. Published Print: 21 NOV 2008

ISBN Information

Print ISBN: 9780470090107

Online ISBN: 9780470090121

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Keywords:

  • development of enteric nervous system and Hirschsprung's disease;
  • enteric nervous system (ENS);
  • RET - receptor tyrosine kinase, activated by GDNF;
  • GDNF family - GDNF, neurturin (NRTN), artemin, persephin;
  • endothelins (Et-1, Et-2 and Et-3) - peptides acting via G-protein-coupled receptors;
  • protein kinase A (PKA) - interactions between Ret- or Ednrb-mediating signalling;
  • hedgehog signalling system;
  • bone morphogenetic proteins (BMPs);
  • netrin/DCC signalling and submucosal ganglia formation;
  • neuregulins - signalling proteins binding to erbB3 or erbB4 receptor tyrosine kinases

Summary

This chapter contains sections titled:

  • Introduction

  • Anatomy and function of the ENS

  • The best-characterized developmental defect of the ENS – Hirschsprung's disease

  • Cell biology of ENS development

  • Molecular biology of ENS development and Hirschsprung-like dysplasias

  • HSCR: current and future treatments

  • Conclusions

  • References