digestive/alimentary system
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• Background Sensitivity: phenotype is more severe when crossed to C57BL/6J females with all offspring completely lacking ganglion cells in the distal large intestine compared to only 1/3 of offspring from crosses to C3HeB/FeJ females
• Background Sensitivity: in crosses to C3HeB/FeJ females the other 2/3 of offspring have reduced numbers of ganglion or no detectable changes in ganglion numbers
• hypoganglionosis without aganglionosis is confined to the distal 1-2 cm of the adult colon
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• hypoganglionosis without aganglionosis is confined to the distal 1-2 cm of the adult colon
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• aganglionosis or hypoganglionosis always involves the distal rectum
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integument
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• almost all have one or more white paws
• more than half have ventral white spots
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cellular
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• delayed colonization of the intestine by vagal neural crest cells between E11.0 to E13.5
• reduced density of neuroblasts in the intestine between E11.0 to E13.5 especially near the migration front
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nervous system
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• almost all display aganglionosis or hypoganglionosis
• Background Sensitivity: phenotype is more severe when crossed to C57BL/6J females with all offspring completely lacking ganglion cells in the distal large intestine compared to only 1/3 of offspring from crosses to C3HeB/FeJ females
• Background Sensitivity: in crosses to C3HeB/FeJ females the other 2/3 of offspring have reduced numbers of ganglion or no detectable changes in ganglion numbers
• aganglionic segements of the large intestine lack both myenteric and submucosal ganglion cells
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embryo
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• delayed colonization of the intestine by vagal neural crest cells between E11.0 to E13.5
• reduced density of neuroblasts in the intestine between E11.0 to E13.5 especially near the migration front
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pigmentation
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• almost all have one or more white paws
• more than half have ventral white spots
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