Analysis Tools
mortality/aging
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• surviving mutants die by 40 days of age, with most dying during the week after weaning
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• many die during the first 5 days of postnatal development
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growth/size/body
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• glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia
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• remain 10-50% smaller throughout life
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• distended abdomen precedes death
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• hypocellularity of the spleen is seen after the development of intestinal obstruction
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digestive/alimentary system
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• distension of the proximal segments of the intestine is seen in mutants with severe intestinal obstruction
• observe dark fecal matter in the intestine and peritoneal cavity and perforation of the intestine
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• most of the Brunner's gland is destroyed and the lumens of many of the remaining ducts are distended
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• severity of damage in the crypts follows a proximal to distal gradient, with mildest changes in the duodenum and most extreme changes in the ileum and colon
• dilation of crypts and formation of concretions and cast-like structures that extend the entire length of the crypts and villi, with crypts and villi almost completely destroyed in some cases
• distended crypts contain increased amounts of mucus and are even present in ileum and colon of mutants without intestinal obstructions
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coiled cecum
(
J:2079
)
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• cecum is coiled and worm-like in appearance and the lumen is narrowed and partially or completely impacted with hard, sticky fecal pellets
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• narrowing of the colon is seen in mutants with severe intestinal obstruction
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• submaxillary glands show varying degrees of disruption of the serous acini, however observe no dilation of ducts or presence of inspissated material in ducts
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• some mutants develop severe intestinal obstruction and meconium ileus consisting of a mixture of mucus and fecal material
• ileum is the common site of obstruction in mice dying just after weaning while the large intestine is in mice dying more than a few days after weaning
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• develop peritonitis
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endocrine/exocrine glands
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• presence of inspissated secretions in various glands
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• most of the Brunner's gland is destroyed and the lumens of many of the remaining ducts are distended
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• severity of damage in the crypts follows a proximal to distal gradient, with mildest changes in the duodenum and most extreme changes in the ileum and colon
• dilation of crypts and formation of concretions and cast-like structures that extend the entire length of the crypts and villi, with crypts and villi almost completely destroyed in some cases
• distended crypts contain increased amounts of mucus and are even present in ileum and colon of mutants without intestinal obstructions
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• submaxillary glands show varying degrees of disruption of the serous acini, however observe no dilation of ducts or presence of inspissated material in ducts
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• glands in the proximal trachea exhibit dilation of the ducts
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• gallbladders are distended or ruptured, however no lesions are observed in the liver
• mutants with intestinal obstructions exhibit almost complete destruction of the gallbladder wall with some polymorphonuclear cells present
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• dramatic alterations are not observed in the pancreas, however it is often smaller and paler and 2 of 5 mutants exhibit one or two lobes that contain some enlarged acini and contain eosinophilic material
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small pancreas
(
J:2079
)
|
• often smaller
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• mutants present with thymic involution after development of intestinal obstruction
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immune system
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• develop peritonitis
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• mutants present with thymic involution after development of intestinal obstruction
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• hypocellularity of the spleen is seen after the development of intestinal obstruction
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liver/biliary system
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• gallbladders are distended or ruptured, however no lesions are observed in the liver
• mutants with intestinal obstructions exhibit almost complete destruction of the gallbladder wall with some polymorphonuclear cells present
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respiratory system
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• glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia
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• increased numbers of goblet cells in the respiratory tract
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• observe squamous metaplasia in the trachea of the oldest surviving mice
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• glands in the proximal trachea exhibit dilation of the ducts
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behavior/neurological
abnormal gait
(
J:2079
)
|
• awkward gait precedes death
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hematopoietic system
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• mutants present with thymic involution after development of intestinal obstruction
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• hypocellularity of the spleen is seen after the development of intestinal obstruction
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reproductive system
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• a successful mating of one female that survived to maturity did not result in pregnancy, possibly indicating infertility, however males exhibit no abnormalities of reproductive organs
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craniofacial
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• glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| cystic fibrosis | DOID:1485 |
OMIM:219700 |
J:2079 | |
