Mouse Genome Informatics
hm
    Cftrtm1Kth/Cftrtm1Kth
involves: 129S7/SvEvBrd * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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
• 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 IDRef(s)
Cystic Fibrosis; CF 219700 J:29074