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Phenotypes Associated with This Genotype
Genotype
MGI:2177533
Allelic
Composition
Cftrtm1Bay/Cftrtm1Bay
Genetic
Background
involves: 129S7/SvEvBrd * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cftrtm1Bay mutation (0 available); any Cftr mutation (13 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 40% died within 1 week after birth, 10% survived the immediate newborn period but died prior to weaning (3 weeks), and the rest survived for long periods (J:15244)
• 40% died within 1 week after birth, 10% survived the immediate newborn period but died prior to weaning (3 weeks), and the rest survived for long periods (J:15244)

digestive/alimentary system
• variable atrophy of acinar cells (J:15244)
• variable atrophy of acinar cells (J:15244)
• dilated crypts (J:15244)
• dilated crypts (J:15244)
• sections of the intestine showed microabscess formation (J:15244)
• sections of the intestine showed microabscess formation (J:15244)
• goblet cell hyperplasia in localized regions of 2-4 day old mice with intestinal obstruction and engorgement of goblet cells in 3-4 week old mice (J:15244)
• goblet cell hyperplasia in localized regions of 2-4 day old mice with intestinal obstruction and engorgement of goblet cells in 3-4 week old mice (J:15244)
• sections of intestine showed ulceration (J:15244)
• sections of intestine showed ulceration (J:15244)
• mucus accumulated in the large intestine (J:15244)
• mucus accumulated in the large intestine (J:15244)
• by 3-4 weeks, colonic glands were dilated and by 6-8 weeks, filamentous amphophilic material (most likely mucus) was present in the glands of the middle to distal colon (J:15244)
• by 3-4 weeks, colonic glands were dilated and by 6-8 weeks, filamentous amphophilic material (most likely mucus) was present in the glands of the middle to distal colon (J:15244)
• cAMP stimulated Cl- secretion was defective in the ileum (J:15244)
• cAMP stimulated Cl- secretion was defective in the ileum (J:15244)
• seen in 40%, consistently just proximal or just distal to the cecum (J:15244)
• seen in 40%, consistently just proximal or just distal to the cecum (J:15244)

growth/size/body
• 40% showed weight loss (J:15244)
• 40% showed weight loss (J:15244)
• some surviving mice were severely cachectic (J:15244)
• some surviving mice were severely cachectic (J:15244)
• surviving mice showed reduced growth rate and averaged less than 70% of the body weight of controls at weaning (J:15244)
• surviving mice showed reduced growth rate and averaged less than 70% of the body weight of controls at weaning (J:15244)
• seen in some 2-4 day old mice (J:15244)
• seen in some 2-4 day old mice (J:15244)

behavior/neurological
• some newborns had not taken milk (J:15244)
• some newborns had not taken milk (J:15244)

endocrine/exocrine glands
• some 3-4 week old mutants showed dilation of acini in the mucosal glands of the pharynx and in the sublingual gland and accumulation of inspissated eosinophilic material in the mucosal glands (J:15244)
• some 3-4 week old mutants showed dilation of acini in the mucosal glands of the pharynx and in the sublingual gland and accumulation of inspissated eosinophilic material in the mucosal glands (J:15244)
• variable atrophy of acinar cells (J:15244)
• variable atrophy of acinar cells (J:15244)
• dilated crypts (J:15244)
• dilated crypts (J:15244)
• sections of the intestine showed microabscess formation (J:15244)
• sections of the intestine showed microabscess formation (J:15244)
• two 3-4 week old mutant mice showed mild focal atrophy in the extra-orbital lacrimal gland (J:15244)
• a 6 week old male displayed retention of intraluminal contents and mild atrophy in the lacrimal gland (J:15244)
• two 3-4 week old mutant mice showed mild focal atrophy in the extra-orbital lacrimal gland (J:15244)
• a 6 week old male displayed retention of intraluminal contents and mild atrophy in the lacrimal gland (J:15244)
• 2/8 of 3-4 week old mutant mice showed gross enlargement of the gall bladder (J:15244)
• however, liver, bile ducts and stomach were normal (J:15244)
• 2/8 of 3-4 week old mutant mice showed gross enlargement of the gall bladder (J:15244)
• however, liver, bile ducts and stomach were normal (J:15244)
• some mutants showed acute inflammation with migration of segmented neurophils into the epithelial lining of the duct, however no inflammation or fibrosis of the pancreatic acinar tissue was observed (J:15244)
• some mutants showed acute inflammation with migration of segmented neurophils into the epithelial lining of the duct, however no inflammation or fibrosis of the pancreatic acinar tissue was observed (J:15244)

homeostasis/metabolism
• cAMP stimulated Cl- secretion was defective in the ileum and cAMP-mediated Cl- conductance was abnormal in tracheas (J:15244)
• cAMP stimulated Cl- secretion was defective in the ileum and cAMP-mediated Cl- conductance was abnormal in tracheas (J:15244)

liver/biliary system
• 2/8 of 3-4 week old mutant mice showed gross enlargement of the gall bladder (J:15244)
• however, liver, bile ducts and stomach were normal (J:15244)
• 2/8 of 3-4 week old mutant mice showed gross enlargement of the gall bladder (J:15244)
• however, liver, bile ducts and stomach were normal (J:15244)

respiratory system
• cAMP-mediated Cl- conductance was abnormal in tracheas (J:15244)
• cAMP-mediated Cl- conductance was abnormal in tracheas (J:15244)

vision/eye
• two 3-4 week old mutant mice showed mild focal atrophy in the extra-orbital lacrimal gland (J:15244)
• a 6 week old male displayed retention of intraluminal contents and mild atrophy in the lacrimal gland (J:15244)
• two 3-4 week old mutant mice showed mild focal atrophy in the extra-orbital lacrimal gland (J:15244)
• a 6 week old male displayed retention of intraluminal contents and mild atrophy in the lacrimal gland (J:15244)

immune system
• some mutants showed acute inflammation with migration of segmented neurophils into the epithelial lining of the duct, however no inflammation or fibrosis of the pancreatic acinar tissue was observed (J:15244)
• some mutants showed acute inflammation with migration of segmented neurophils into the epithelial lining of the duct, however no inflammation or fibrosis of the pancreatic acinar tissue was observed (J:15244)

Mouse Models of Human Disease
OMIM ID Ref(s)
Cystic Fibrosis; CF 219700 J:15244


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
01/26/2016
MGI 6.02
The Jackson Laboratory