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Animal Models of Aganglionosis

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Hirschsprung's Disease and Allied Disorders

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Abstract

Animal models of aganglionosis have contributed greatly to our understanding of the genetic basis of Hirschsprung’s disease (HSCR) and its complexity. Knockout mouse models are the most commonly used animal models, but also other animals such as rats, rabbits or Zebrafish have been successfully used for studies of aganglionosis. Mainly four main types of animal models are used for investigation: natural occurring models, transgenic animals, teratogen-induced models, and surgically created models. Natural occurring models need no or little interference prior to the study. Transgenic animals not only mimic the natural condition but also help to identify specific genes involved in the regulation of the disease. Teratogen-induced models have the drawback of exposure to a generalized noxa, which could lead to widespread detriments rather than simply targeting a specific organ system. Surgical created models can closely simulate manlike conditions and surgical procedures. Animal models of aganglionosis, especially since advances in knockout and transgenic technology, have been essential in the discovery of genes, molecules, and pathways related to HSCR and are also crucial for stem cell transplantation studies, a promising approach to drug treatment for aganglionosis in the future. In further time course, animal models of aganglionosis will continue to play a key role in the anatomic, physiologic, and pharmacologic understanding of HSCR.

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  1. Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany

    Julia Brendel

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    Prem Puri

  3. School of Medicine and Medical Science and Conway Institute of Biomedical Research, University College Dublin, Dublin, Ireland

    Prem Puri

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Brendel, J., Puri, P. (2019). Animal Models of Aganglionosis. In: Puri, P. (eds) Hirschsprung's Disease and Allied Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-15647-3_5

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