Strain Name:

B6.129P2-Cftrtm1Unc/J

Stock Number:

002196

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Availability:

Cryopreserved - Ready for recovery

Mice homozygous for the Cftrtm1Unc mutation die early from intestinal obstruction (ileum or large intestine). Pancreatic involvement has been found in some animals. Mice homozygous for the disrupted gene display many features common to human cystic fibrosis

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Mutant Strain; Targeted Mutation;
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Specieslaboratory mouse
Background Strain C57BL/6J
 
Donating Investigator IMR Colony,   The Jackson Laboratory

Appearance
black
Related Genotype: a/a

Description
Mice homozygous for the Cftrtm1Unc mutation survive approximately 28 days. Death of the homozygotes is usually due to intestinal obstruction (ileum or large intestine). Pancreatic involvement has been found in some animals. A recent report (Kent, et.al.) has found that this mutation on a C57BL/6J genetic background does cause lung disease.

Development
The Cftrtm1Unc mutation was developed in the laboratory of Dr. Oliver Smithies at University of North Carolina by a neo insertion into exon 10 of the Cftr gene. The 129-derived E14TG2a ES cell line was used. The C57BL/6J strain was generated by backcrossing mice carrying the Cftrtm1Unc mutation 10 times to C57BL/6J inbred mice.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Cftrtm1Unc allele
002364   STOCK Cftrtm1Unc Tg(FABPCFTR)1Jaw/J
View Strains carrying   Cftrtm1Unc     (1 strain)

Strains carrying other alleles of Cftr
002515   B6.129S6-Cftrtm1Kth/J
View Strains carrying other alleles of Cftr     (1 strain)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Cystic Fibrosis; CF
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Bronchiectasis with or without Elevated Sweat Chloride 1; BESC1   (CFTR)
Pancreatitis, Hereditary; PCTT   (CFTR)
Vas Deferens, Congenital Bilateral Aplasia Of; CBAVD   (CFTR)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Cftrtm1Unc/Cftrtm1Unc

        B6.129P2-Cftrtm1Unc/J
  • homeostasis/metabolism phenotype
  • *normal* homeostasis/metabolism phenotype
    • although mutant FSH levels are slightly increased relative to wild-type levels, circulating levels of both FSH and LH still remain within normal range at proestrus   (MGI Ref ID J:145380)
    • abnormal phospholipid level
      • homozygotes exhibit a membrane lipid imbalance characterized by an increase in phospholipid-bound arachidonic acid (AA) and a decrease in phospholipid-bound docosahexaenoic acid (DHA), that is most pronounced in the pancreas, lung and ileum, organs affected in cystic fibrosis, and in the heart   (MGI Ref ID J:58571)
      • oral administration of DHA reverses the membrane lipid imbalance   (MGI Ref ID J:58571)
  • digestive/alimentary phenotype
  • abnormal pancreatic acinus morphology
    • massive luminal dilatation   (MGI Ref ID J:58571)
    • oral administration of DHA reverses the pancreatic phenotype   (MGI Ref ID J:58571)
    • pancreatic acinar cell zymogen granule accumulation
      • zymogen granule accumulation at the apical pole of the acinar cells   (MGI Ref ID J:58571)
  • ileum hypertrophy
    • ileal hypertrophy; villus height is increased   (MGI Ref ID J:58571)
    • oral administration of DHA restores villus height to normal   (MGI Ref ID J:58571)
  • intestinal obstruction
    • develop intestinal blockage when fed a normal (solid) diet   (MGI Ref ID J:112450)
  • respiratory system phenotype
  • abnormal respiratory system physiology
    • abnormal nasal potential difference   (MGI Ref ID J:112450)
    • lung inflammation
      • increased inflammatory response to chronic Pseudomonas aeruginosa infection   (MGI Ref ID J:112450)
      • homozygotes exposed to Pseudomonas LPS daily for 3 days exhibit enhanced lung inflammation, as indicated by a significant increase in neutrophil concentration compared to wild-type   (MGI Ref ID J:58571)
      • oral administration of DHA blocks the enhanced neutrophil infiltration in response to Pseudomonas LPS   (MGI Ref ID J:58571)
  • growth/size/body phenotype
  • decreased body weight
    • reduced at 7, 14, and 21 days of age relative to wild-type mice   (MGI Ref ID J:112450)
    • at 6-8 and 14-16 weeks of age, total body weight is reduced by only 15% and 12%, respectively, thus not explaining the larger differences noted in average weight of reproductive organs (~50% and 36%, respectively)   (MGI Ref ID J:145380)
  • immune system phenotype
  • lung inflammation
    • increased inflammatory response to chronic Pseudomonas aeruginosa infection   (MGI Ref ID J:112450)
    • homozygotes exposed to Pseudomonas LPS daily for 3 days exhibit enhanced lung inflammation, as indicated by a significant increase in neutrophil concentration compared to wild-type   (MGI Ref ID J:58571)
    • oral administration of DHA blocks the enhanced neutrophil infiltration in response to Pseudomonas LPS   (MGI Ref ID J:58571)
  • endocrine/exocrine gland phenotype
  • abnormal pancreatic acinus morphology
    • massive luminal dilatation   (MGI Ref ID J:58571)
    • oral administration of DHA reverses the pancreatic phenotype   (MGI Ref ID J:58571)
    • pancreatic acinar cell zymogen granule accumulation
      • zymogen granule accumulation at the apical pole of the acinar cells   (MGI Ref ID J:58571)
  • decreased corpora lutea number
    • female homozygotes show an average of 1.5 +/- 2.0 corpora lutea per ovary vs 9.3 +/- 1.4 in wild-type females, even though other follicle stages are present   (MGI Ref ID J:145380)
  • small ovary
    • at 7 weeks of age, female homozygotes display smaller ovaries than wild-type females   (MGI Ref ID J:145380)
    • decreased ovary weight
      • at 6-8 and 14-16 weeks of age, the average weight of mutant ovaries is reduced by 50% and 36%, respectively, relative to that of wild-type ovaries   (MGI Ref ID J:145380)
      • however, mutant ovarian weight is restored to wild-type values after superovulation   (MGI Ref ID J:145380)
  • reproductive system phenotype
  • abnormal sperm physiology
    • sperm transport within the mutant female reproductive system is significantly impaired: the average number of sperm found in mutant oviducts is only ~10% that of wild-type   (MGI Ref ID J:145380)
    • however, no differences in capacitation of oviductal sperm from mutant and wild-type females are observed   (MGI Ref ID J:145380)
  • abnormal uterine cervix morphology
    • only 1 of 15 female homozygotes showed cervical mucus accumulation with no other physical signs of obstruction in the uterus   (MGI Ref ID J:145380)
  • absent estrous cycle
    • unlike wild-type females, 41.7% of 14-16-wk-old mutant females never enter estrus but are constantly in diestrus   (MGI Ref ID J:145380)
  • decreased corpora lutea number
    • female homozygotes show an average of 1.5 +/- 2.0 corpora lutea per ovary vs 9.3 +/- 1.4 in wild-type females, even though other follicle stages are present   (MGI Ref ID J:145380)
  • decreased litter size
    • female homozygotes show a significant decrease in average number of pups per litter relative to wild-type females (3.55 +/- 1.92 vs 6.56 +/- 2.36, respectively)   (MGI Ref ID J:145380)
  • decreased ovulation rate
    • female homozygotes display reduced oocyte ovulation rates relative to wild-type females   (MGI Ref ID J:145380)
    • however, normal ovulation rates are observed after superovulation with exogenous hormone (PMSG + hCG) injections   (MGI Ref ID J:145380)
  • delayed sexual maturation
    • female homozygotes display a delayed onset of puberty relative to wild-type controls   (MGI Ref ID J:145380)
  • impaired fertilization
    • at 48 hrs after hCG treatment, 100% of mutant oocytes remain unfertilized, whereas the majority of embryos from superovulated wild-type females are at the 2- to 4-cell stages   (MGI Ref ID J:145380)
    • however, no significant differences in in vitro fertilization rates are observed, suggesting that decreased in vivo fertilization is more likely due to inadequate fluid control in the reproductive tract, resulting in decreased sperm number in the oviduct   (MGI Ref ID J:145380)
  • prolonged estrous cycle
    • at 14-16 weeks of age, female homozygotes that display at least one estrous cycle show half as many cycles as wild-type females, resulting in a 2-fold increase in average cycle length   (MGI Ref ID J:145380)
  • reduced female fertility
    • female homozygotes exhibit reduced fertility with significantly fewer numbers of litters and smaller litter sizes relative to wild-type females   (MGI Ref ID J:145380)
    • 20% of female homozygotes are unable to give birth over a 5-month mating period   (MGI Ref ID J:145380)
    • following induction of superovulation, only 1 of 10 mutant females that displayed vaginal plugs gave birth, but that female did give birth to 20 pups   (MGI Ref ID J:145380)
  • small ovary
    • at 7 weeks of age, female homozygotes display smaller ovaries than wild-type females   (MGI Ref ID J:145380)
    • decreased ovary weight
      • at 6-8 and 14-16 weeks of age, the average weight of mutant ovaries is reduced by 50% and 36%, respectively, relative to that of wild-type ovaries   (MGI Ref ID J:145380)
      • however, mutant ovarian weight is restored to wild-type values after superovulation   (MGI Ref ID J:145380)
  • small uterus
    • at 7 weeks of age, female homozygotes display smaller uteri than wild-type females   (MGI Ref ID J:145380)
    • however, no physical signs of obstruction are observed in the uterus   (MGI Ref ID J:145380)
    • decreased uterus weight
      • at 6-8 and 14-16 weeks of age, the average weight of mutant uteri is reduced by 56% and 36%, respectively, relative to that of wild-type uteri   (MGI Ref ID J:145380)
      • however, mutant uterus weight is restored to wild-type values after superovulation   (MGI Ref ID J:145380)
    • thin uterus
      • mutant uteri are thinner than wild-type   (MGI Ref ID J:145380)

Cftrtm1Unc/Cftrtm1Unc

        B6.129P2-Cftrtm1Unc
  • mortality/aging
  • partial postnatal lethality
    • increased relative to homozygotes on a mixed 129P2/OlaHsd and C57BL/6J background   (MGI Ref ID J:44904)
    • at P20 survival is 6.4% of all live births   (MGI Ref ID J:44904)
    • death usually occurs with 3 days of birth mostly from intestinal disease   (MGI Ref ID J:44904)
  • digestive/alimentary phenotype
  • abnormal intestine morphology
    • intestinal disease involving the colon and distal ileum is a common cause of death   (MGI Ref ID J:44904)
  • respiratory system phenotype
  • abnormal bronchus morphology
    • at 6 months of age many bronchiolar surfaces are covered with a thick layer of material that is not seen in wild-type mice or in homozygotes on a mixed 129P2/OlaHsd and C57BL/6J background   (MGI Ref ID J:44904)
    • increase in the amount of acidic mucopolysacharides in material lining the airways compared to wild-type mice and homozygotes on a mixed 129P2/OlaHsd and C57BL/6J background   (MGI Ref ID J:44904)
    • age dependent increase in the relative proportion of non-ciliated cells in the airway epithelium with proliferation of endoplasmic reticulum and increase in the number of secretory granules in these cells; however, this increase is not seen in mice on a mixed 129P2/OlaHsd and C57BL/6J background   (MGI Ref ID J:44904)
  • abnormal pulmonary alveolus morphology
    • as early as P30, patchy lesions consisting of acinar dilation with interstitial thickening and accumulation of inflammatory cells and connective tissue in the alveolar walls are seen consistent with obstructive small airway disease   (MGI Ref ID J:44904)
    • at 6 months of age many alveolar surfaces are covered with a thick layer of material that is not seen in wild-type mice or in homozygotes on a mixed 129P2/OlaHsd and C57BL/6J background   (MGI Ref ID J:44904)
  • abnormal respiratory system physiology
    • elevated negative basal nasal potential difference and absence of response to imposition of a luminally directed Cl- gradient   (MGI Ref ID J:44904)
    • increased lung compliance
      • lung compliance corrected for body weight is increased compared to wild-type mice or homozygous mice on a mixed 129P2/OlaHsd and C57BL/6J background   (MGI Ref ID J:44904)
    • lung inflammation
      • as early as P30, patchy lesions consisting of acinar dilation with interstitial thickening and accumulation of inflammatory cells and connective tissue in the alveolar walls are seen consistent with obstructive small airway disease   (MGI Ref ID J:44904)
      • this pathology becomes more severe with age   (MGI Ref ID J:44904)
      • however, no infection with any pulmonary pathogens is detected   (MGI Ref ID J:44904)
  • pulmonary interstitial fibrosis
    • increased deposition of collagen and other fibrillar material with age   (MGI Ref ID J:44904)
  • growth/size/body phenotype
  • postnatal growth retardation
    • more severe than in homozygous mice on a mixed 129P2/OlaHsd and C57BL/6J background   (MGI Ref ID J:44904)
  • immune system phenotype
  • lung inflammation
    • as early as P30, patchy lesions consisting of acinar dilation with interstitial thickening and accumulation of inflammatory cells and connective tissue in the alveolar walls are seen consistent with obstructive small airway disease   (MGI Ref ID J:44904)
    • this pathology becomes more severe with age   (MGI Ref ID J:44904)
    • however, no infection with any pulmonary pathogens is detected   (MGI Ref ID J:44904)
  • homeostasis/metabolism phenotype
  • pulmonary interstitial fibrosis
    • increased deposition of collagen and other fibrillar material with age   (MGI Ref ID J:44904)

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Cftrtm1Unc/Cftr+

        involves: 129P2/OlaHsd
  • reproductive system phenotype
  • asthenozoospermia
    • male heterozygotes show a significant reduction in sperm motility with compromised forward movement parameters relative to wild-type controls   (MGI Ref ID J:122297)
  • decreased litter size
    • when crossed to wild-type females, male heterozygotes yield a reduced litter size relative to wild-type males (on average 4.20 +/- 1.09 vs 6.22 +/- 0.67, respectively)   (MGI Ref ID J:122297)
  • impaired fertilization
    • in vitro fertilizing capacity of heterozygous mutant sperm is significantly lower than that of wild-type sperm (16% vs 48.5%, respectively)   (MGI Ref ID J:122297)
    • in addition, heterozygous mutant sperm show reduced binding and penetration of zona pellucida-free eggs relative to wild-type sperm   (MGI Ref ID J:122297)
  • impaired sperm capacitation
    • after 2-hr incubation in capacitation-inducing medium, the % of capacitated sperm (B pattern) in male heterozygotes is significantly lower than that of wild-type controls, as shown by CTC staining   (MGI Ref ID J:122297)
    • reduced sperm capacitation is associated with defective HCO3_ transport, including a reduction in the magnitude of HCO3_ -induced membrane hyperpolarization and a decrease in HCO3_ -induced cAMP production relative to wild-type controls   (MGI Ref ID J:122297)
  • reduced male fertility
    • only 5 of 9 male heterozygotes mated with wild-type females produce offspring   (MGI Ref ID J:122297)

Cftrtm1Unc/Cftrtm1Unc

        involves: 129P2/OlaHsd
  • mortality/aging
  • partial postnatal lethality
    • many die during the first 5 days of postnatal development   (MGI Ref ID J:2079)
  • premature death
    • surviving mutants die by 40 days of age, with most dying during the week after weaning   (MGI Ref ID J:2079)
  • growth/size/body phenotype
  • abnormal nasal mucosa morphology
    • glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia   (MGI Ref ID J:2079)
  • abnormal paranasal sinus morphology   (MGI Ref ID J:2079)
  • decreased body size
    • remain 10-50% smaller throughout life   (MGI Ref ID J:2079)
  • distended abdomen
    • distended abdomen precedes death   (MGI Ref ID J:2079)
  • digestive/alimentary phenotype
  • abnormal intestine morphology
    • distension of the proximal segments of the intestine is seen in mutants with severe intestinal obstruction   (MGI Ref ID J:2079)
    • observe dark fecal matter in the intestine and peritoneal cavity and perforation of the intestine   (MGI Ref ID J:2079)
    • abnormal Brunner's gland morphology
      • most of the Brunner's gland is destroyed and the lumens of many of the remaining ducts are distended   (MGI Ref ID J:2079)
    • abnormal colon morphology
      • narrowing of the colon is seen in mutants with severe intestinal obstruction   (MGI Ref ID J:2079)
    • abnormal crypts of Lieberkuhn morphology
      • 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   (MGI Ref ID 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   (MGI Ref ID J:2079)
      • distended crypts contain increased amounts of mucus and are even present in ileum and colon of mutants without intestinal obstructions   (MGI Ref ID J:2079)
    • coiled cecum
      • cecum is coiled and worm-like in appearance and the lumen is narrowed and partially or completely impacted with hard, sticky fecal pellets   (MGI Ref ID J:2079)
  • abnormal submandibular gland morphology
    • submaxillary glands show varying degrees of disruption of the serous acini, however observe no dilation of ducts or presence of inspissated material in ducts   (MGI Ref ID J:2079)
  • intestinal obstruction
    • some mutants develop severe intestinal obstruction and meconium ileus consisting of a mixture of mucus and fecal material   (MGI Ref ID 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   (MGI Ref ID J:2079)
  • peritoneal inflammation
    • develop peritonitis   (MGI Ref ID J:2079)
  • endocrine/exocrine gland phenotype
  • abnormal gland morphology
    • presence of inspissated secretions in various glands   (MGI Ref ID J:2079)
    • abnormal Brunner's gland morphology
      • most of the Brunner's gland is destroyed and the lumens of many of the remaining ducts are distended   (MGI Ref ID J:2079)
    • abnormal crypts of Lieberkuhn morphology
      • 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   (MGI Ref ID 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   (MGI Ref ID J:2079)
      • distended crypts contain increased amounts of mucus and are even present in ileum and colon of mutants without intestinal obstructions   (MGI Ref ID J:2079)
    • abnormal gallbladder morphology
      • gallbladders are distended or ruptured, however no lesions are observed in the liver   (MGI Ref ID J:2079)
      • mutants with intestinal obstructions exhibit almost complete destruction of the gallbladder wall with some polymorphonuclear cells presen   (MGI Ref ID J:2079)
      • dilated gallbladder   (MGI Ref ID J:2079)
    • abnormal pancreas morphology
      • 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   (MGI Ref ID J:2079)
      • small pancreas
        • often smaller   (MGI Ref ID J:2079)
    • abnormal submandibular gland morphology
      • submaxillary glands show varying degrees of disruption of the serous acini, however observe no dilation of ducts or presence of inspissated material in ducts   (MGI Ref ID J:2079)
  • abnormal thymus involution
    • mutants present with thymic involution after development of intestinal obstruction   (MGI Ref ID J:2079)
  • immune system phenotype
  • abnormal thymus involution
    • mutants present with thymic involution after development of intestinal obstruction   (MGI Ref ID J:2079)
  • peritoneal inflammation
    • develop peritonitis   (MGI Ref ID J:2079)
  • spleen hyperplasia
    • hypocellularity of the spleen is seen after the development of intestinal obstruction   (MGI Ref ID J:2079)
  • liver/biliary system phenotype
  • abnormal gallbladder morphology
    • gallbladders are distended or ruptured, however no lesions are observed in the liver   (MGI Ref ID J:2079)
    • mutants with intestinal obstructions exhibit almost complete destruction of the gallbladder wall with some polymorphonuclear cells presen   (MGI Ref ID J:2079)
    • dilated gallbladder   (MGI Ref ID J:2079)
  • respiratory system phenotype
  • abnormal nasal mucosa morphology
    • glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia   (MGI Ref ID J:2079)
  • abnormal paranasal sinus morphology   (MGI Ref ID J:2079)
  • abnormal trachea morphology
    • observe squamous metaplasia in the trachea of the oldest surviving mice   (MGI Ref ID J:2079)
    • glands in the proximal trachea exhibit dilation of the ducts   (MGI Ref ID J:2079)
  • increased respiratory mucosa goblet cell number
    • increased numbers of goblet cells in the respiratory tract   (MGI Ref ID J:2079)
  • behavior/neurological phenotype
  • abnormal gait
    • awkward gait precedes death   (MGI Ref ID J:2079)
  • hematopoietic system phenotype
  • abnormal thymus involution
    • mutants present with thymic involution after development of intestinal obstruction   (MGI Ref ID J:2079)
  • spleen hyperplasia
    • hypocellularity of the spleen is seen after the development of intestinal obstruction   (MGI Ref ID J:2079)
  • reproductive system phenotype
  • female infertility
    • 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   (MGI Ref ID J:2079)
  • craniofacial phenotype
  • abnormal nasal mucosa morphology
    • glands in the nasal mucosa exhibit dilation of ducts but no acinar hyperplasia   (MGI Ref ID J:2079)
  • abnormal paranasal sinus morphology   (MGI Ref ID J:2079)

Cftrtm1Unc/Cftrtm1Unc

        involves: 129P2/OlaHsd * C57BL/6J
  • mortality/aging
  • partial postnatal lethality
    • reduced relative to homozygotes on a congenic C57BL/6J background   (MGI Ref ID J:44904)
    • at P20 survival is 15.5% of all live births   (MGI Ref ID J:44904)
  • respiratory system phenotype
  • *normal* respiratory system phenotype
    • at 6 months of age bronchiolar and alveolar surfaces appear similar to wild-type as does the amount of acidic mucopolysacharides in material lining the airways and the proportion of non-ciliated cells in the broncheolar epithlium, unlike in homozygotes on a congenic C57BL/6J background   (MGI Ref ID J:44904)
    • lung compliance corrected for body weight is similar to wild type lung compliance corrected for body weight is similar to wild-type   (MGI Ref ID J:44904)
    • abnormal respiratory system physiology
      • elevated negative basal nasal potential difference and absence of response to imposition of a luminally directed Cl- gradient   (MGI Ref ID J:44904)
      • abnormal vital capacity
        • forced vital capacity per kg body weight is reduced compared to wild-type mice   (MGI Ref ID J:44904)
  • growth/size/body phenotype
  • postnatal growth retardation
    • less severe than in homozygous mice on a congenic C57BL/6J background   (MGI Ref ID J:44904)

Cftrtm1Unc/Cftrtm1Unc

        involves: 129P2/OlaHsd * C57BL/6 * FVB/N
  • mortality/aging
  • partial postnatal lethality
    • only 5% of mice survive until weaning due to complications from intestinal obstruction   (MGI Ref ID J:21934)
  • digestive/alimentary phenotype
  • abnormal crypts of Lieberkuhn morphology
    • crypts are dilated with mucus   (MGI Ref ID J:21934)
  • abnormal intestinal goblet cell morphology
    • goblet cell hyperplasia occurs throughout the intestinal tract from ileum to the colon   (MGI Ref ID J:21934)
  • coiled cecum
    • the cecum has a coiled "worm-like" structure   (MGI Ref ID J:21934)
  • endocrine/exocrine gland phenotype
  • abnormal crypts of Lieberkuhn morphology
    • crypts are dilated with mucus   (MGI Ref ID J:21934)
  • homeostasis/metabolism phenotype
  • abnormal ion homeostasis
    • cyclic-AMP stimulated transport of Cl- ions does not occur in the intestinal epithelium   (MGI Ref ID J:21934)
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Research Applications
This mouse can be used to support research in many areas including:

Immunology, Inflammation and Autoimmunity Research
Cystic Fibrosis

Cftrtm1Unc related

Metabolism Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Cftrtm1Unc
Allele Name targeted mutation 1, University of North Carolina
Allele Type Targeted (Null/Knockout)
Common Name(s) CFTR S489X-; Cftr-; Cftr-.ko; CftrUNC; S489X; UNC; cftrm1UNC; mCFTR-;
Mutation Made ByDr. Oliver Smithies,   University of North Carolina
Strain of Origin129P2/OlaHsd
ES Cell Line NameE14TG2a
ES Cell Line Strain129P2/OlaHsd
Gene Symbol and Name Cftr, cystic fibrosis transmembrane conductance regulator
Chromosome 6
Gene Common Name(s) ABC35; ABCC7; AW495489; CF; CFTR/MRP; MRP7; RGD1561193; TNR-CFTR; dJ760C5.1; expressed sequence AW495489;
General Note Epithelial tissue from the gastrointestinal tract and airways exhibit abnormal cyclic AMP-mediated chloride ion transport similar to that observed in CF patients (J:23817).
Molecular Note A neomycin selection cassette was inserted into exon 10 at sequences corresponding to codon 489 of the encoded protein. The authors predict that a truncated protein with amino acid 488 changed from isoleucine to alanine and a stop codon at position 489 are produced from this allele. [MGI Ref ID J:2079]

Genotyping

Genotyping Information

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Cftrtm1Unc, Standard PCR


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Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Cftrtm1Unc related

Allard JB; Poynter ME; Marr KA; Cohn L; Rincon M; Whittaker LA. 2006. Aspergillus fumigatus generates an enhanced Th2-biased immune response in mice with defective cystic fibrosis transmembrane conductance regulator. J Immunol 177(8):5186-94. [PubMed: 17015704]  [MGI Ref ID J:139448]

Alper SL; Stewart AK; Vandorpe DH; Clark JS; Zachary Horack R; Simpson JE; Walker NM; Clarke LL. 2011. Native and recombinant Slc26a3 (downregulated in adenoma, Dra) do not exhibit properties of 2Cl-/1HCOFormula exchange. Am J Physiol Cell Physiol 300(2):C276-86. [PubMed: 21068358]  [MGI Ref ID J:168506]

Arquitt CK; Boyd C; Wright JT. 2002. Cystic fibrosis transmembrane regulator gene (CFTR) is associated with abnormal enamel formation. J Dent Res 81(7):492-6. [PubMed: 12161463]  [MGI Ref ID J:105940]

Barker PM; Brigman KK; Paradiso AM; Boucher RC; Gatzy JT. 1995. Cl- secretion by trachea of CFTR (+/-) and (-/-) fetal mouse. Am J Respir Cell Mol Biol 13(3):307-13. [PubMed: 7544595]  [MGI Ref ID J:113069]

Bazett M; Paun A; Haston CK. 2011. MicroRNA profiling of cystic fibrosis intestinal disease in mice. Mol Genet Metab 103(1):38-43. [PubMed: 21333573]  [MGI Ref ID J:172095]

Bazett M; Stefanov AN; Paun A; Paradis J; Haston CK. 2012. Strain-dependent airway hyperresponsiveness and a chromosome 7 locus of elevated lymphocyte numbers in cystic fibrosis transmembrane conductance regulator-deficient mice. J Immunol 188(5):2297-304. [PubMed: 22287709]  [MGI Ref ID J:181250]

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MacDonald KD; McKenzie KR; Henderson MJ; Hawkins CE; Vij N; Zeitlin PL. 2008. Lubiprostone activates non-CFTR-dependent respiratory epithelial chloride secretion in cystic fibrosis mice. Am J Physiol Lung Cell Mol Physiol 295(5):L933-40. [PubMed: 18805957]  [MGI Ref ID J:142850]

Magenheimer BS; St John PL; Isom KS; Abrahamson DR; De Lisle RC; Wallace DP; Maser RL; Grantham JJ; Calvet JP. 2006. Early embryonic renal tubules of wild-type and polycystic kidney disease kidneys respond to cAMP stimulation with cystic fibrosis transmembrane conductance regulator/Na(+),K(+),2Cl(-) Co-transporter-dependent cystic dilation. J Am Soc Nephrol 17(12):3424-37. [PubMed: 17108316]  [MGI Ref ID J:135949]

Mailleau C; Paul A; Colin M; Xing PX; Guernier C; Bernaudin JF; Capeau J; Brahimi-Horn MC. 2001. Glycoconjugate metabolism in a cystic fibrosis knockout mouse model. Mol Genet Metab 72(2):122-31. [PubMed: 11161838]  [MGI Ref ID J:101665]

Mall MA; Button B; Johannesson B; Zhou Z; Livraghi A; Caldwell RA; Schubert SC; Schultz C; O'Neal WK; Pradervand S; Hummler E; Rossier BC; Grubb BR; Boucher RC. 2010. Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice. J Biol Chem 285(35):26945-55. [PubMed: 20566636]  [MGI Ref ID J:166185]

Manson ME; Corey DA; White NM; Kelley TJ. 2008. cAMP-mediated regulation of cholesterol accumulation in cystic fibrosis and Niemann-Pick type C cells. Am J Physiol Lung Cell Mol Physiol 295(5):L809-19. [PubMed: 18790990]  [MGI Ref ID J:142605]

Marcos V; Zhou Z; Yildirim AO; Bohla A; Hector A; Vitkov L; Wiedenbauer EM; Krautgartner WD; Stoiber W; Belohradsky BH; Rieber N; Kormann M; Koller B; Roscher A; Roos D; Griese M; Eickelberg O; Doring G; Mall MA; Hartl D. 2010. CXCR2 mediates NADPH oxidase-independent neutrophil extracellular trap formation in cystic fibrosis airway inflammation. Nat Med 16(9):1018-23. [PubMed: 20818377]  [MGI Ref ID J:164429]

Martin CR; Zaman MM; Ketwaroo GA; Bhutta AQ; Coronel E; Popov Y; Schuppan D; Freedman SD. 2012. CFTR dysfunction predisposes to fibrotic liver disease in a murine model. Am J Physiol Gastrointest Liver Physiol 303(4):G474-81. [PubMed: 22679000]  [MGI Ref ID J:191345]

Martino AT; Mueller C; Braag S; Cruz PE; Campbell-Thompson M; Jin S; Flotte TR. 2011. N-glycosylation augmentation of the cystic fibrosis epithelium improves Pseudomonas aeruginosa clearance. Am J Respir Cell Mol Biol 44(6):824-30. [PubMed: 20693405]  [MGI Ref ID J:185027]

Marvao P; De Jesus Ferreira MC; Bailly C; Paulais M; Bens M; Guinamard R; Moreau R; Vandewalle A; Teulon J. 1998. Cl- absorption across the thick ascending limb is not altered in cystic fibrosis mice. A role for a pseudo-CFTR Cl- channel. J Clin Invest 102(11):1986-93. [PubMed: 9835624]  [MGI Ref ID J:51326]

McDaniel N; Pace AJ; Spiegel S; Engelhardt R; Koller BH; Seidler U; Lytle C. 2005. Role of Na-K-2Cl cotransporter-1 in gastric secretion of nonacidic fluid and pepsinogen. Am J Physiol Gastrointest Liver Physiol 289(3):G550-60. [PubMed: 16093421]  [MGI Ref ID J:101250]

Meissner A; Yang J; Kroetsch JT; Sauve M; Dax H; Momen A; Noyan-Ashraf MH; Heximer S; Husain M; Lidington D; Bolz SS. 2012. Tumor necrosis factor-alpha-mediated downregulation of the cystic fibrosis transmembrane conductance regulator drives pathological sphingosine-1-phosphate signaling in a mouse model of heart failure. Circulation 125(22):2739-50. [PubMed: 22534621]  [MGI Ref ID J:198613]

Mueller C; Braag SA; Keeler A; Hodges C; Drumm M; Flotte TR. 2011. Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses. Am J Respir Cell Mol Biol 44(6):922-9. [PubMed: 20724552]  [MGI Ref ID J:185025]

Nadeau JH. 2003. Modifier genes and protective alleles in humans and mice. Curr Opin Genet Dev 13(3):290-5. [PubMed: 12787792]  [MGI Ref ID J:88012]

Norkina O; De Lisle RC. 2005. Potential genetic modifiers of the cystic fibrosis intestinal inflammatory phenotype on mouse chromosomes 1, 9, and 10. BMC Genet 6(1):29. [PubMed: 15921521]  [MGI Ref ID J:101842]

Norkina O; Kaur S; Ziemer D; De Lisle RC. 2004. Inflammation of the cystic fibrosis mouse small intestine. Am J Physiol Gastrointest Liver Physiol 286(6):G1032-41. [PubMed: 14739145]  [MGI Ref ID J:95681]

Ollero M; Laposata M; Zaman MM; Blanco PG; Andersson C; Zeind J; Urman Y; Kent G; Alvarez JG; Freedman SD. 2006. Evidence of increased flux to n-6 docosapentaenoic acid in phospholipids of pancreas from cftr-/- knockout mice. Metabolism 55(9):1192-200. [PubMed: 16919538]  [MGI Ref ID J:115980]

Ostedgaard LS; Meyerholz DK; Vermeer DW; Karp PH; Schneider L; Sigmund CD; Welsh MJ. 2011. Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice. Proc Natl Acad Sci U S A 108(7):2921-6. [PubMed: 21285372]  [MGI Ref ID J:169220]

Ostedgaard LS; Rogers CS; Dong Q; Randak CO; Vermeer DW; Rokhlina T; Karp PH; Welsh MJ. 2007. Processing and function of CFTR-DeltaF508 are species-dependent. Proc Natl Acad Sci U S A 104(39):15370-5. [PubMed: 17873061]  [MGI Ref ID J:125319]

Pan J; Luk C; Kent G; Cutz E; Yeger H. 2006. Pulmonary neuroendocrine cells, airway innervation, and smooth muscle are altered in Cftr null mice. Am J Respir Cell Mol Biol 35(3):320-6. [PubMed: 16614351]  [MGI Ref ID J:125253]

Parmley RR; Gendler SJ. 1998. Cystic fibrosis mice lacking Muc1 have reduced amounts of intestinal mucus. J Clin Invest 102(10):1798-806. [PubMed: 9819365]  [MGI Ref ID J:51108]

Praetorius J; Friis UG; Ainsworth MA; Schaffalitzky de Muckadell OB; Johansen T. 2002. The cystic fibrosis transmembrane conductance regulator is not a base transporter in isolated duodenal epithelial cells. Acta Physiol Scand 174(4):327-36. [PubMed: 11942920]  [MGI Ref ID J:127847]

Rock JR; O'Neal WK; Gabriel SE; Randell SH; Harfe BD; Boucher RC; Grubb BR. 2009. Transmembrane protein 16A (TMEM16A) is a Ca2+-regulated Cl- secretory channel in mouse airways. J Biol Chem 284(22):14875-80. [PubMed: 19363029]  [MGI Ref ID J:150497]

Romanenko VG; Nakamoto T; Catalan MA; Gonzalez-Begne M; Schwartz GJ; Jaramillo Y; Sepulveda FV; Figueroa CD; Melvin JE. 2008. Clcn2 encodes the hyperpolarization-activated chloride channel in the ducts of mouse salivary glands. Am J Physiol Gastrointest Liver Physiol 295(5):G1058-67. [PubMed: 18801913]  [MGI Ref ID J:142744]

Saadane A; Masters S; DiDonato J; Li J; Berger M. 2007. Parthenolide inhibits IkappaB kinase, NF-kappaB activation, and inflammatory response in cystic fibrosis cells and mice. Am J Respir Cell Mol Biol 36(6):728-36. [PubMed: 17272824]  [MGI Ref ID J:136609]

Saadane A; Soltys J; Berger M. 2005. Role of IL-10 deficiency in excessive nuclear factor-kappaB activation and lung inflammation in cystic fibrosis transmembrane conductance regulator knockout mice. J Allergy Clin Immunol 115(2):405-11. [PubMed: 15696103]  [MGI Ref ID J:105450]

Saussereau EL; Roussel D; Diallo S; Debarbieux L; Edelman A; Sermet-Gaudelus I. 2013. Characterization of nasal potential difference in cftr knockout and F508del-CFTR mice. PLoS One 8(3):e57317. [PubMed: 23505426]  [MGI Ref ID J:199541]

Schiffhauer ES; Vij N; Kovbasnjuk O; Kang PW; Walker D; Lee S; Zeitlin PL. 2013. Dual activation of CFTR and CLCN2 by lubiprostone in murine nasal epithelia. Am J Physiol Lung Cell Mol Physiol 304(5):L324-31. [PubMed: 23316067]  [MGI Ref ID J:195069]

Schroeder TH; Reiniger N; Meluleni G; Grout M; Coleman FT; Pier GB. 2001. Transgenic cystic fibrosis mice exhibit reduced early clearance of Pseudomonas aeruginosa from the respiratory tract. J Immunol 166(12):7410-8. [PubMed: 11390493]  [MGI Ref ID J:128660]

Snouwaert JN; Brigman KK; Latour AM; Iraj E; Schwab U; Gilmour MI; Koller BH. 1995. A murine model of cystic fibrosis. Am J Respir Crit Care Med 151(3 Pt 2):S59-64. [PubMed: 7533607]  [MGI Ref ID J:25032]

Snouwaert JN; Brigman KK; Latour AM; Malouf NN; Boucher RC; Smithies O; Koller BH. 1992. An animal model for cystic fibrosis made by gene targeting. Science 257(5073):1083-8. [PubMed: 1380723]  [MGI Ref ID J:2079]

Soltys J; Bonfield T; Chmiel J; Berger M. 2002. Functional IL-10 deficiency in the lung of cystic fibrosis (cftr(-/-)) and IL-10 knockout mice causes increased expression and function of B7 costimulatory molecules on alveolar macrophages. J Immunol 168(4):1903-10. [PubMed: 11823525]  [MGI Ref ID J:74476]

Stalvey MS; Clines KL; Havasi V; McKibbin CR; Dunn LK; Chung WJ; Clines GA. 2013. Osteoblast CFTR inactivation reduces differentiation and osteoprotegerin expression in a mouse model of cystic fibrosis-related bone disease. PLoS One 8(11):e80098. [PubMed: 24236172]  [MGI Ref ID J:209209]

Stalvey MS; Muller C; Schatz DA; Wasserfall CH; Campbell-Thompson ML; Theriaque DW; Flotte TR; Atkinson MA. 2006. Cystic fibrosis transmembrane conductance regulator deficiency exacerbates islet cell dysfunction after beta-cell injury. Diabetes 55(7):1939-45. [PubMed: 16804061]  [MGI Ref ID J:111876]

Steagall WK; Elmer HL; Brady KG; Kelley TJ. 2000. Cystic fibrosis transmembrane conductance regulator-dependent regulation of epithelial inducible nitric oxide synthase expression Am J Respir Cell Mol Biol 22(1):45-50. [PubMed: 10615064]  [MGI Ref ID J:59774]

Steinberg BE; Huynh KK; Brodovitch A; Jabs S; Stauber T; Jentsch TJ; Grinstein S. 2010. A cation counterflux supports lysosomal acidification. J Cell Biol 189(7):1171-86. [PubMed: 20566682]  [MGI Ref ID J:162688]

Tang S; Beharry S; Kent G; Durie PR. 1999. Synergistic effects of cAMP- and calcium-mediated amylase secretion in isolated pancreatic acini from cystic fibrosis mice. Pediatr Res 45(4 Pt 1):482-8. [PubMed: 10203138]  [MGI Ref ID J:54427]

Teichgraber V; Ulrich M; Endlich N; Riethmuller J; Wilker B; De Oliveira-Munding CC; van Heeckeren AM; Barr ML; von Kurthy G; Schmid KW; Weller M; Tummler B; Lang F; Grassme H; Doring G; Gulbins E. 2008. Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis. Nat Med 14(4):382-91. [PubMed: 18376404]  [MGI Ref ID J:133678]

Thomsson KA; Hinojosa-Kurtzberg M; Axelsson KA; Domino SE; Lowe JB; Gendler SJ; Hansson GC. 2002. Intestinal mucins from cystic fibrosis mice show increased fucosylation due to an induced Fucalpha1-2 glycosyltransferase. Biochem J 367(Pt 3):609-16. [PubMed: 12164788]  [MGI Ref ID J:79985]

Tiesset H; Bernard H; Bartke N; Beermann C; Flachaire E; Desseyn JL; Gottrand F; Husson MO. 2011. (n-3) Long-Chain PUFA Differentially Affect Resistance to Pseudomonas aeruginosa Infection of Male and Female cftr-/- Mice. J Nutr 141(6):1101-7. [PubMed: 21525256]  [MGI Ref ID J:172792]

Trudel S; Kelly M; Fritsch J; Nguyen-Khoa T; Therond P; Couturier M; Dadlez M; Debski J; Touqui L; Vallee B; Ollero M; Edelman A; Brouillard F. 2009. Peroxiredoxin 6 fails to limit phospholipid peroxidation in lung from Cftr-knockout mice subjected to oxidative challenge. PLoS One 4(6):e6075. [PubMed: 19562038]  [MGI Ref ID J:150184]

Tuo B; Wen G; Zhang Y; Liu X; Wang X; Liu X; Dong H. 2009. Involvement of phosphatidylinositol 3-kinase in cAMP- and cGMP-induced duodenal epithelial CFTR activation in mice. Am J Physiol Cell Physiol 297(3):C503-15. [PubMed: 19535511]  [MGI Ref ID J:152105]

Vandivier RW; Richens TR; Horstmann SA; deCathelineau AM; Ghosh M; Reynolds SD; Xiao YQ; Riches DW; Plumb J; Vachon E; Downey GP; Henson PM. 2009. Dysfunctional cystic fibrosis transmembrane conductance regulator inhibits phagocytosis of apoptotic cells with proinflammatory consequences. Am J Physiol Lung Cell Mol Physiol 297(4):L677-86. [PubMed: 19633071]  [MGI Ref ID J:154224]

Velsor LW; Kariya C; Kachadourian R; Day BJ. 2006. Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice. Am J Respir Cell Mol Biol 35(5):579-86. [PubMed: 16763223]  [MGI Ref ID J:126889]

Velsor LW; van Heeckeren A; Day BJ. 2001. Antioxidant imbalance in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice. Am J Physiol Lung Cell Mol Physiol 281(1):L31-8. [PubMed: 11404242]  [MGI Ref ID J:107842]

Walker NM; Simpson JE; Brazill JM; Gill RK; Dudeja PK; Schweinfest CW; Clarke LL. 2009. Role of down-regulated in adenoma anion exchanger in HCO3- secretion across murine duodenum. Gastroenterology 136(3):893-901. [PubMed: 19121635]  [MGI Ref ID J:146913]

White NM; Jiang D; Burgess JD; Bederman IR; Previs SF; Kelley TJ. 2007. Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 292(2):L476-86. [PubMed: 17085523]  [MGI Ref ID J:121216]

Wright JT; Kiefer CL; Hall KI; Grubb BR. 1996. Abnormal enamel development in a cystic fibrosis transgenic mouse model. J Dent Res 75(4):966-73. [PubMed: 8708137]  [MGI Ref ID J:35202]

Wu J; Marmorstein AD; Peachey NS. 2006. Functional abnormalities in the retinal pigment epithelium of CFTR mutant mice. Exp Eye Res 83(2):424-8. [PubMed: 16626699]  [MGI Ref ID J:116327]

Xie W; Fisher JT; Lynch TJ; Luo M; Evans TI; Neff TL; Zhou W; Zhang Y; Ou Y; Bunnett NW; Russo AF; Goodheart MJ; Parekh KR; Liu X; Engelhardt JF. 2011. CGRP induction in cystic fibrosis airways alters the submucosal gland progenitor cell niche in mice. J Clin Invest 121(8):3144-58. [PubMed: 21765217]  [MGI Ref ID J:175894]

Xu WM; Chen J; Chen H; Diao RY; Fok KL; Dong JD; Sun TT; Chen WY; Yu MK; Zhang XH; Tsang LL; Lau A; Shi QX; Shi QH; Huang PB; Chan HC. 2011. Defective CFTR-Dependent CREB Activation Results in Impaired Spermatogenesis and Azoospermia. PLoS One 6(5):e19120. [PubMed: 21625623]  [MGI Ref ID J:172718]

Xu WM; Shi QX; Chen WY; Zhou CX; Ni Y; Rowlands DK; Yi Liu G; Zhu H; Ma ZG; Wang XF; Chen ZH; Zhou SC; Dong HS; Zhang XH; Chung YW; Yuan YY; Yang WX; Chan HC. 2007. Cystic fibrosis transmembrane conductance regulator is vital to sperm fertilizing capacity and male fertility. Proc Natl Acad Sci U S A 104(23):9816-21. [PubMed: 17519339]  [MGI Ref ID J:122297]

Xu Y; Clark JC; Aronow BJ; Dey CR; Liu C; Wooldridge JL; Whitsett JA. 2003. Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency. J Biol Chem 278(9):7674-82. [PubMed: 12482874]  [MGI Ref ID J:107360]

Xu Y; Krause A; Limberis M; Worgall TS; Worgall S. 2013. Low sphingosine-1-phosphate impairs lung dendritic cells in cystic fibrosis. Am J Respir Cell Mol Biol 48(2):250-7. [PubMed: 23239501]  [MGI Ref ID J:205088]

Xu Y; Liu C; Clark JC; Whitsett JA. 2006. Functional genomic responses to cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR(delta508) in the lung. J Biol Chem 281(16):11279-91. [PubMed: 16455659]  [MGI Ref ID J:112698]

Xu Z; Gupta V; Lei D; Holmes A; Carlson E; Gruenert DC. 1998. In-frame elimination of exon 10 in Cftrtm1Unc CF mice. Gene 211(1):117-23. [PubMed: 9573345]  [MGI Ref ID J:47586]

Yamamoto-Mizuma S; Wang GX; Hume JR. 2004. P2Y purinergic receptor regulation of CFTR chloride channels in mouse cardiac myocytes. J Physiol 556(Pt 3):727-37. [PubMed: 14978203]  [MGI Ref ID J:105515]

Young FD; Newbigging S; Choi C; Keet M; Kent G; Rozmahel RF. 2007. Amelioration of cystic fibrosis intestinal mucous disease in mice by restoration of mCLCA3. Gastroenterology 133(6):1928-37. [PubMed: 18054564]  [MGI Ref ID J:135618]

Yu H; Nasr SZ; Deretic V. 2000. Innate lung defenses and compromised Pseudomonas aeruginosa clearance in the malnourished mouse model of respiratory infections in cystic fibrosis. Infect Immun 68(4):2142-7. [PubMed: 10722612]  [MGI Ref ID J:61161]

Zdebik AA; Cuffe JE; Bertog M; Korbmacher C; Jentsch TJ. 2004. Additional disruption of the ClC-2 Cl(-) channel does not exacerbate the cystic fibrosis phenotype of cystic fibrosis transmembrane conductance regulator mouse models. J Biol Chem 279(21):22276-83. [PubMed: 15007059]  [MGI Ref ID J:89831]

Zertal-Zidani S; Busiah K; Edelman A; Polak M; Scharfmann R. 2013. Small-molecule inhibitors of the cystic fibrosis transmembrane conductance regulator increase pancreatic endocrine cell development in rat and mouse. Diabetologia 56(2):330-9. [PubMed: 23178930]  [MGI Ref ID J:194637]

Zhang H; Ameen N; Melvin JE; Vidyasagar S. 2007. Acute inflammation alters bicarbonate transport in mouse ileum. J Physiol 581(Pt 3):1221-33. [PubMed: 17395634]  [MGI Ref ID J:141056]

Zhang PX; Murray TS; Villella VR; Ferrari E; Esposito S; D'Souza A; Raia V; Maiuri L; Krause DS; Egan ME; Bruscia EM. 2013. Reduced caveolin-1 promotes hyperinflammation due to abnormal heme oxygenase-1 localization in lipopolysaccharide-challenged macrophages with dysfunctional cystic fibrosis transmembrane conductance regulator. J Immunol 190(10):5196-206. [PubMed: 23606537]  [MGI Ref ID J:202542]

Zhang Y; Li X; Grassme H; Doring G; Gulbins E. 2010. Alterations in ceramide concentration and pH determine the release of reactive oxygen species by Cftr-deficient macrophages on infection. J Immunol 184(9):5104-11. [PubMed: 20351190]  [MGI Ref ID J:160462]

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van Heeckeren AM; Schluchter M; Xue L; Alvarez J; Freedman S; St George J; Davis PB. 2004. Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice. Infect Immun 72(3):1479-86. [PubMed: 14977953]  [MGI Ref ID J:88586]

van Heeckeren AM; Schluchter MD; Drumm ML; Davis PB. 2004. Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice. Am J Physiol Lung Cell Mol Physiol 287(5):L944-52. [PubMed: 15246977]  [MGI Ref ID J:112450]

Health & husbandry

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

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Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Breeding & HusbandryThis Cftrtm1Unc strain is maintained by mating heterozyous mice to normal wildtype siblings. Heterozygous and normal wildtype mice may be ordered. Expected coat color from breeding:Black

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Cryopreserved

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Price (US dollars $)
Cryorecovery* $2525.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

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Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

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    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3283.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

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Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

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   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

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In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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