All Is Not Normal in “Normoganglionic” Bowel Regions of Hirschsprung Disease Model Mice
نویسنده
چکیده
irschsprung disease is a congenital disorder in Hwhich neurons are missing from the distal bowel. As the enteric nervous system (ENS) is essential for propulsive gut motility, affected infants have intractable constipation. The current treatment for Hirschsprung disease involves surgical removal of the distal aganglionic region and anastomosis of the ganglionic bowel to the anus. Despite this, many patients with Hirschsprung disease
منابع مشابه
Neuron and glia generating progenitors of the mammalian enteric nervous system isolated from foetal and postnatal gut cultures.
Cultures of dissociated foetal and postnatal mouse gut gave rise to neurosphere-like bodies, which contained large numbers of mature neurons and glial cells. In addition to differentiated cells, neurosphere-like bodies included proliferating progenitors which, when cultured at clonal densities, gave rise to colonies containing many of the neuronal subtypes and glial cells present in the mammali...
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BACKGROUND & AIMS In Hirschsprung disease (HSCR), neural crest-derived progenitors (NCPs) fail to completely colonize the intestine so that the enteric nervous system (ENS) is absent from distal bowel. Despite removal of the aganglionic region, many HSCR patients suffer from residual intestinal dysmotility. To test the hypothesis that inappropriate lineage segregation of NCPs in proximal gangli...
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