For many patients with Hirschsprung disease, the underlying pathogenic mechanisms are unknown. A new study using a novel line of transgenic mice and tissue from patients with Hirschsprung disease suggests that overproduction of collagen VI could contribute to pathogenesis and the increased incidence of Hirschsprung disease in patients with Down syndrome.
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Long range synchronization within the enteric nervous system underlies propulsion along the large intestine in mice
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Acknowledgements
H.M.Y. is supported by National Health and Medical Research Council (NHMRC) Senior Research Fellowship APP1002506; S.J.M. is supported by NHMRC project grants APP1047953 and APP1043397.
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Young, H., McKeown, S. Hirschsprung disease — laying down a suitable path. Nat Rev Gastroenterol Hepatol 13, 7–8 (2016). https://doi.org/10.1038/nrgastro.2015.213
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DOI: https://doi.org/10.1038/nrgastro.2015.213
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