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Phenotypes Associated with This Genotype
Genotype
MGI:2177530
Allelic
Composition
Cftrtm1Unc/Cftrtm1Unc
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm1Unc mutation (5 available); any Cftr mutation (13 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• many die during the first 5 days of postnatal development (J:2079)
• many die during the first 5 days of postnatal development (J:2079)
• surviving mutants die by 40 days of age, with most dying during the week after weaning (J:2079)
• surviving mutants die by 40 days of age, with most dying during the week after weaning (J:2079)

growth/size/body
• glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia (J:2079)
• glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia (J:2079)
• remain 10-50% smaller throughout life (J:2079)
• remain 10-50% smaller throughout life (J:2079)
• distended abdomen precedes death (J:2079)
• distended abdomen precedes death (J:2079)

digestive/alimentary system
• distension of the proximal segments of the intestine is seen in mutants with severe intestinal obstruction (J:2079)
• observe dark fecal matter in the intestine and peritoneal cavity and perforation of the intestine (J:2079)
• distension of the proximal segments of the intestine is seen in mutants with severe intestinal obstruction (J:2079)
• observe dark fecal matter in the intestine and peritoneal cavity and perforation of the intestine (J:2079)
• most of the Brunner's gland is destroyed and the lumens of many of the remaining ducts are distended (J:2079)
• most of the Brunner's gland is destroyed and the lumens of many of the remaining ducts are distended (J:2079)
• 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 (J:2079)
• 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 (J:2079)
• distended crypts contain increased amounts of mucus and are even present in ileum and colon of mutants without intestinal obstructions (J:2079)
• 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 (J:2079)
• 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 (J:2079)
• distended crypts contain increased amounts of mucus and are even present in ileum and colon of mutants without intestinal obstructions (J:2079)
• cecum is coiled and worm-like in appearance and the lumen is narrowed and partially or completely impacted with hard, sticky fecal pellets (J:2079)
• cecum is coiled and worm-like in appearance and the lumen is narrowed and partially or completely impacted with hard, sticky fecal pellets (J:2079)
• narrowing of the colon is seen in mutants with severe intestinal obstruction (J:2079)
• narrowing of the colon is seen in mutants with severe intestinal obstruction (J:2079)
• submaxillary glands show varying degrees of disruption of the serous acini, however observe no dilation of ducts or presence of inspissated material in ducts (J:2079)
• submaxillary glands show varying degrees of disruption of the serous acini, however observe no dilation of ducts or presence of inspissated material in ducts (J:2079)
• some mutants develop severe intestinal obstruction and meconium ileus consisting of a mixture of mucus and fecal material (J:2079)
• 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 (J:2079)
• some mutants develop severe intestinal obstruction and meconium ileus consisting of a mixture of mucus and fecal material (J:2079)
• 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 (J:2079)
• develop peritonitis (J:2079)
• develop peritonitis (J:2079)

endocrine/exocrine glands
• presence of inspissated secretions in various glands (J:2079)
• presence of inspissated secretions in various glands (J:2079)
• most of the Brunner's gland is destroyed and the lumens of many of the remaining ducts are distended (J:2079)
• most of the Brunner's gland is destroyed and the lumens of many of the remaining ducts are distended (J:2079)
• 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 (J:2079)
• 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 (J:2079)
• distended crypts contain increased amounts of mucus and are even present in ileum and colon of mutants without intestinal obstructions (J:2079)
• 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 (J:2079)
• 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 (J:2079)
• distended crypts contain increased amounts of mucus and are even present in ileum and colon of mutants without intestinal obstructions (J:2079)
• submaxillary glands show varying degrees of disruption of the serous acini, however observe no dilation of ducts or presence of inspissated material in ducts (J:2079)
• submaxillary glands show varying degrees of disruption of the serous acini, however observe no dilation of ducts or presence of inspissated material in ducts (J:2079)
• gallbladders are distended or ruptured, however no lesions are observed in the liver (J:2079)
• mutants with intestinal obstructions exhibit almost complete destruction of the gallbladder wall with some polymorphonuclear cells presen (J:2079)
• gallbladders are distended or ruptured, however no lesions are observed in the liver (J:2079)
• mutants with intestinal obstructions exhibit almost complete destruction of the gallbladder wall with some polymorphonuclear cells presen (J:2079)
• 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 (J:2079)
• 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 (J:2079)
• often smaller (J:2079)
• often smaller (J:2079)
• mutants present with thymic involution after development of intestinal obstruction (J:2079)
• mutants present with thymic involution after development of intestinal obstruction (J:2079)

immune system
• develop peritonitis (J:2079)
• develop peritonitis (J:2079)
• mutants present with thymic involution after development of intestinal obstruction (J:2079)
• mutants present with thymic involution after development of intestinal obstruction (J:2079)
• hypocellularity of the spleen is seen after the development of intestinal obstruction (J:2079)
• hypocellularity of the spleen is seen after the development of intestinal obstruction (J:2079)

liver/biliary system
• gallbladders are distended or ruptured, however no lesions are observed in the liver (J:2079)
• mutants with intestinal obstructions exhibit almost complete destruction of the gallbladder wall with some polymorphonuclear cells presen (J:2079)
• gallbladders are distended or ruptured, however no lesions are observed in the liver (J:2079)
• mutants with intestinal obstructions exhibit almost complete destruction of the gallbladder wall with some polymorphonuclear cells presen (J:2079)

respiratory system
• glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia (J:2079)
• glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia (J:2079)
• increased numbers of goblet cells in the respiratory tract (J:2079)
• increased numbers of goblet cells in the respiratory tract (J:2079)
• observe squamous metaplasia in the trachea of the oldest surviving mice (J:2079)
• observe squamous metaplasia in the trachea of the oldest surviving mice (J:2079)
• glands in the proximal trachea exhibit dilation of the ducts (J:2079)
• glands in the proximal trachea exhibit dilation of the ducts (J:2079)

behavior/neurological
• awkward gait precedes death (J:2079)
• awkward gait precedes death (J:2079)

hematopoietic system
• mutants present with thymic involution after development of intestinal obstruction (J:2079)
• mutants present with thymic involution after development of intestinal obstruction (J:2079)
• hypocellularity of the spleen is seen after the development of intestinal obstruction (J:2079)
• hypocellularity of the spleen is seen after the development of intestinal obstruction (J:2079)

reproductive system
• 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 (J:2079)
• 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 (J:2079)

craniofacial
• glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia (J:2079)
• glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia (J:2079)

Mouse Models of Human Disease
OMIM ID Ref(s)
Cystic Fibrosis; CF 219700 J:2079


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory