mortality/aging

premature death ( J:29656 )

• at least 60% die within one month after birth due to intestinal obstruction

digestive/alimentary system

abnormal crypts of Lieberkuhn morphology ( J:29656 )

• exhibit dilatation of the crypts of Lieberkuhn and the presence of eosinophilic concretions in the crypts of the small and large intestine

abnormal digestive system physiology ( J:29656 )

• cAMP stimulated chloride secretion is defective in the colon

intestinal obstruction ( J:29656 )

• many exhibit intestinal disease as indicated by intestinal obstruction

growth/size

decreased body size ( J:29656 )

• those that survive the newborn period are smaller and grow more slowly

reproductive system

female infertility ( J:29656 )

• adult females are almost always sterile, although males are fertile (J:29656)

endocrine/exocrine glands

abnormal crypts of Lieberkuhn morphology ( J:29656 )

• exhibit dilatation of the crypts of Lieberkuhn and the presence of eosinophilic concretions in the crypts of the small and large intestine

abnormal pancreas morphology ( J:29656 )

• exhibit a variable degree of pancreatic atrophy

liver/biliary system

liver/biliary system phenotype ( J:29656 )

N	• no abnormal liver/biliary system phenotypes detected (J:29656)

respiratory system

respiratory system phenotype ( J:29656 )

N	• no abnormal respiratory system phenotypes detected (J:29656)

Mouse Models of Human Disease		OMIM ID	Ref(s)
Cystic Fibrosis; CF		219700	J:29656