Animal Models of Hirschsprung's Disease: State of the Art in Translating Experimental Research to the Bedside
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In brief
In brief
Comprehensive review of animal models used to study Hirschsprung's disease, including teratogen-induced, surgical, naturally occurring, and knockout models. Examines how these models advance understanding of enteric nervous system development, gene mutations affecting neural crest cell migration, and translation of findings to clinical practice.
Written by the GCMD Library team from the article.
Hirschsprung's disease (HSCR) is caused by incomplete colonization of enteric neural crest-derived cell (ENCC) in the bowel, the failure of ENCCs to proliferate, differentiate, and migrate leads to an absence of enteric neurons in the distal colon, resulting in colonic motility dysfunction. Various animal models of HSCR have been important in the understanding of the anatomy and pathophysiology of the disease and in the discovery of genes involved in HSCR. Four types of HSCR animal models have been developed: teratogen-induced, surgically created, naturally occurring models, and knockout models. Mutations in several genes affect enteric nervous system (ENS) development and can have pleiotropic effects on this system. Furthermore, certain animal models are informative regarding how such molecules control the development and functional differentiation of the ENS. In this article, we summarize recent advances in this field and highlight opportunities for new discoveries.