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Phenotypes Associated with This Genotype
Genotype
MGI:2177536
Allelic
Composition
Cftrtm1Kth/Cftrtm1Kth
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm1Kth mutation (1 available); any Cftr mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• about 10% died within 7 days of birth and saw significant mortality around the time of weaning (30 days after birth), with 40% surviving postweaning for at least 8 months

digestive/alimentary system
• mice that died prematurely showed evidence of bowel obstruction and bowel strictures, especially in the cecum
• Brunner's glands were often dilated
• some mice exhibited large casts of mucus material in the jejunum, however lung, pancreas, gall bladder, male reproductive tract, lacrimal gland, and submandibular glands appeared normal
• lacked cAMP-stimulated Cl- secretory current in intestine, indicating defective electrolyte transport
• mice that died prematurely showed evidence of bowel obstruction
• mice that died prematurely showed peritonitis

growth/size/body
• reduced size noticeable as early as 10 days after birth
• by weaning, weight was only 50-60% that of controls and although homozygous mutant mice continued to grow, they never reached the size of controls

homeostasis/metabolism
• defective fluid transport in nasal, intestinal, and pancreatic duct epithelia
• defective electrolyte transport in nasal, intestinal, and pancreatic duct epithelia

immune system
• mice that died prematurely showed peritonitis

endocrine/exocrine glands
• Brunner's glands were often dilated
• reduced absorption of fluid and lower cAMP-stimulated fluid secretion by pancreatic epithelia

Mouse Models of Human Disease
OMIM ID Ref(s)
Cystic Fibrosis; CF 219700 J:29074


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
04/19/2016
MGI 6.03
The Jackson Laboratory