Analysis Tools
nervous system
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• while neuronal processes from segments of the E13.5 proximal midgut penetrate a collagen matrix in the presence of GDNF, the neuron cell bodies or glial cells do not as in the case of wild-type neurons
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• enteric neural crest cells exhibit an abnormal association with neuronal processes
• beginning at E12.5, enteric neural crest cells exhibit increased aggregation and form abnormal clusters that are depend on calcium mechanisms
• in culture, enteric neural crest cells form aggregates instead of forming scattered neuronal networks as do wild-type cells
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• in mutants a severe alteration of the neuronal network rostral to the migratory front in observed
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• bundles of extrinsic neurons innervate the aganglionic segments of the colon
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digestive/alimentary system
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• majority of newborn mutants have a distended ascending colon and caecum
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embryo
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• in conditional mutants from E11.5, enteric neural crest cells show a delay in colonization of the gut; difference in distance traveled by cells in mutants and controls increases with time
• neural crest cells from mutants fail to invade the hindgut, leading to an aganglionosis of the descending colon after birth
• however, the number of enteric neural crest cells, differentiation and radial distribution of enteric neural crest cells are normal
• when transplanted into wild-type mice, enteric neural crest cells only migrate 66.5% of the distance that wild-type cells migrate
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• enteric neural crest cells exhibit an abnormal association with neuronal processes
• beginning at E12.5, enteric neural crest cells exhibit increased aggregation and form abnormal clusters that are depend on calcium mechanisms
• in culture, enteric neural crest cells form aggregates instead of forming scattered neuronal networks as do wild-type cells
|
cellular
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• in conditional mutants from E11.5, enteric neural crest cells show a delay in colonization of the gut; difference in distance traveled by cells in mutants and controls increases with time
• neural crest cells from mutants fail to invade the hindgut, leading to an aganglionosis of the descending colon after birth
• however, the number of enteric neural crest cells, differentiation and radial distribution of enteric neural crest cells are normal
• when transplanted into wild-type mice, enteric neural crest cells only migrate 66.5% of the distance that wild-type cells migrate
|
|
• while neuronal processes from segments of the E13.5 proximal midgut penetrate a collagen matrix in the presence of GDNF, the neuron cell bodies or glial cells do not as in the case of wild-type neurons
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Hirschsprung's disease | DOID:10487 |
OMIM:600156 OMIM:606874 OMIM:606875 OMIM:608462 OMIM:611644 |
J:108439 | |
