mortality/aging
• at least 60% die within one month after birth due to intestinal obstruction
|
digestive/alimentary system
• exhibit dilatation of the crypts of Lieberkuhn and the presence of eosinophilic concretions in the crypts of the small and large intestine
|
• cAMP stimulated chloride secretion is defective in the colon
|
• many exhibit intestinal disease as indicated by intestinal obstruction
|
growth/size/body
• those that survive the newborn period are smaller and grow more slowly
|
reproductive system
• adult females are almost always sterile, although males are fertile
|
endocrine/exocrine glands
• exhibit dilatation of the crypts of Lieberkuhn and the presence of eosinophilic concretions in the crypts of the small and large intestine
|
• exhibit a variable degree of pancreatic atrophy
|
liver/biliary system
N |
• no abnormal liver/biliary system phenotypes detected
|
respiratory system
N |
• no abnormal respiratory system phenotypes detected
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
cystic fibrosis | DOID:1485 |
OMIM:219700 |
J:29656 |