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Animal Models of Aganglionosis

  • Chapter
Hirschsprung's Disease and Allied Disorders

Abstract

Humans are not the only mammals to suffer from aganglionosis. Aganglionosis has also been described in mice, rats, horses, cats and dogs. Rodent animal models have contributed significantly to our understanding of Hirschsprung’s disease (HSCR). Over the last decade, the understanding of the genetics and cell biology of the development of the enteric nervous system (ENS) has made great progress. Rodent animal models have shown many points of correlation with humans in regard to ENS development, both normal and abnormal. Nevertheless, the link between the genotype and the phenotype is often indirect, and so many questions have yet to be answered. This chapter deals with the characteristics of aganglionosis in rodents with emphasis on how knowledge of the animal models has contributed to our understanding of the genetics and pathogenesis of HSCR and allied disorders.

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Authors and Affiliations

  1. The Children’s Hospital at Westmead, Sydney, Australia

    A.M. Alzahem

  2. The New Children’s Hospital, Department of Surgical Research, Royal Alexandra Hospital for Children, Sydney, Australia

    D.T. Cass

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  1. A.M. Alzahem
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  2. D.T. Cass
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Editors and Affiliations

  1. Immenzaun 6a, 51429, Bergisch Gladbach, Germany

    Alexander Matthias Holschneider

  2. The Children’s Hospital of Cologne, Amsterdamerstr. 59, 50735 , Cologne, Germany

    Alexander Matthias Holschneider

  3. Our Lady’s Children’s Hospital, Children’s Research Centre, University College of Dublin, Crumlin, Dublin 12, Ireland

    Prem Puri

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© 2008 Springer-Verlag Berlin Heidelberg

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Alzahem, A., Cass, D. (2008). Animal Models of Aganglionosis. In: Holschneider, A., Puri, P. (eds) Hirschsprung's Disease and Allied Disorders. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-33935-9_4

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  • DOI: https://doi.org/10.1007/978-3-540-33935-9_4

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-33934-2

  • Online ISBN: 978-3-540-33935-9

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