Strain Name:

B6.129P2-Cftrtm1Unc/J

Stock Number:

002196

Availability:

Repository-Cryopreserved

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;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6J
GenerationN10
 
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)

Additional Web Information

Congenic Nomenclature

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms
Cystic Fibrosis; CF - Models with phenotypic similarity to human disease where etiologies involve orthologs.1
1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
View Mammalian Phenotype Terms

Mammalian Phenotype Terms
      assigned by genotype

Cftrtm1Unc/Cftrtm1Unc

        B6.129P2-Cftrtm1Unc/J
  • homeostasis/metabolism phenotype
  • abnormal phospholipid level (MGI Ref ID J:58571)
    • 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
    • oral administration of DHA reverses the membrane lipid imbalance
  • digestive/alimentary phenotype
  • abnormal ileum morphology (MGI Ref ID J:58571)
    • ileal hypertrophy; villus height is increased
    • oral administration of DHA restores villus height to normal
  • abnormal pancreatic acinus morphology (MGI Ref ID J:58571)
    • massive luminal dilatation
    • oral administration of DHA reverses the pancreatic phenotype
    • pancreatic acinar cell zymogen granule accumulation (MGI Ref ID J:58571)
      • zymogen granule accumulation at the apical pole of the acinar cells
  • intestinal obstruction (MGI Ref ID J:112450)
    • develop intestinal blockage when fed a normal (solid) diet
  • respiratory system phenotype
  • abnormal respiratory system physiology (MGI Ref ID J:112450)
    • abnormal nasal potential difference
    • lung inflammation (MGI Ref ID J:112450)
      • increased inflammatory response to chronic Pseudomonas aeruginosa infection
      • 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
      • oral administration of DHA blocks the enhanced neutrophil infiltration in response to Pseudomonas LPS
  • growth/size phenotype
  • decreased body weight (MGI Ref ID J:112450)
    • reduced at 7, 14, and 21 days of age relative to wild-type mice
  • immune system phenotype
  • lung inflammation (MGI Ref ID J:112450)
    • increased inflammatory response to chronic Pseudomonas aeruginosa infection
    • 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
    • oral administration of DHA blocks the enhanced neutrophil infiltration in response to Pseudomonas LPS
  • endocrine/exocrine gland phenotype
  • abnormal pancreatic acinus morphology (MGI Ref ID J:58571)
    • massive luminal dilatation
    • oral administration of DHA reverses the pancreatic phenotype
    • pancreatic acinar cell zymogen granule accumulation (MGI Ref ID J:58571)
      • zymogen granule accumulation at the apical pole of the acinar cells

Cftrtm1Unc/Cftrtm1Unc

        B6.129P2-Cftrtm1Unc
  • lethality-postnatal
  • postnatal lethality (MGI Ref ID J:44904)
    • increased relative to homozygotes on a mixed 129P2/OlaHsd and C57BL/6J background at P20 survival is 6.4% of all live births
    • death usually occurs with 3 days of birth mostly from intestinal disease
  • digestive/alimentary phenotype
  • abnormal intestine morphology (MGI Ref ID J:44904)
    • intestinal disease involving the colon and distal ileum is a common cause of death
  • respiratory system phenotype
  • abnormal bronchus morphology (MGI Ref ID J:44904)
    • 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
    • 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
    • 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
  • abnormal respiratory alveoli morphology (MGI Ref ID J:44904)
    • 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
    • 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
  • abnormal respiratory system physiology (MGI Ref ID J:44904)
    • elevated negative basal nasal potential difference and absence of response to imposition of a luminally directed Cl- gradient
    • abnormal lung compliance (MGI Ref ID J:44904)
      • 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
    • lung inflammation (MGI Ref ID J:44904)
      • 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
      • this pathology becomes more severe with age
      • however, no infection with any pulmonary pathogens is detected
  • pulmonary interstitial fibrosis (MGI Ref ID J:44904)
    • increased deposition of collagen and other fibrillar material with age
  • growth/size phenotype
  • postnatal growth retardation (MGI Ref ID J:44904)
    • more severe than in homozygous mice on a mixed 129P2/OlaHsd and C57BL/6J background
  • immune system phenotype
  • lung inflammation (MGI Ref ID J:44904)
    • 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
    • this pathology becomes more severe with age
    • however, no infection with any pulmonary pathogens is detected

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Cftrtm1Unc/Cftrtm1Unc

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

Cftrtm1Unc/Cftrtm1Unc

        involves: 129P2/OlaHsd * C57BL/6J
  • lethality-postnatal
  • postnatal lethality (MGI Ref ID J:44904)
    • reduced relative to homozygotes on a congenic C57BL/6J background
    • at P20 survival is 15.5% of all live births
  • respiratory system phenotype
  • *normal* respiratory system phenotype (MGI Ref ID J:44904)
    • 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
    • lung compliance corrected for body weight is similar to wild type lung compliance corrected for body weight is similar to wild-type
    • abnormal respiratory system physiology (MGI Ref ID J:44904)
      • elevated negative basal nasal potential difference and absence of response to imposition of a luminally directed Cl- gradient
      • abnormal vital capacity (MGI Ref ID J:44904)
        • forced vital capacity per kg body weight is reduced compared to wild-type mice
  • growth/size phenotype
  • postnatal growth retardation (MGI Ref ID J:44904)
    • less severe than in homozygous mice on a congenic C57BL/6J background

Cftrtm1Unc/Cftrtm1Unc

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

Research Applications
This mouse can be used to support research in many areas including:

Immunology and Inflammation Research
Cystic Fibrosis

Cftrtm1Unc related

Metabolism Research

Mouse/Human Gene Homologs
cystic fibrosis

Genes & Alleles

Gene & Allele Information

 
Allele Symbol Cftrtm1Unc
Allele Name targeted mutation 1, University of North Carolina
Allele Type Targeted (knock-out)
Common Name(s) CFTR S489X-; Cftr-; Cftr-.ko; CftrUNC; S489X; UNC; cftrm1UNC; mCFTR-;
Mutation Made By 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 homolog
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).

Heterozygotes secrete half the wild-type amount of intestinal fluid in response to cholera toxin. This is proposed asan advantage for these heterozygotes, providing protection from the dehydration resulting from cholera. A similar effect of the human CFTR mutation might account for the high frequency of mutant genes in the human population (J:20778).

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

Genotyping Protocols

Cftrtm1Unc, STD PCR, vers. 2

Helpful Links

Optimizing PCR Protocols

References

References

Selected Reference(s)

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]

Additional References

Clarke LL; Grubb BR; Gabriel SE; Smithies O; Koller BH; Boucher RC. 1992. Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis. Science 257(5073):1125-8. [PubMed: 1380724]  [MGI Ref ID J:23817]

De Lisle RC; Isom KS; Ziemer D; Cotton CU. 2001. Changes in the exocrine pancreas secondary to altered small intestinal function in the CF mouse. Am J Physiol Gastrointest Liver Physiol 281(4):G899-906. [PubMed: 11557509]  [MGI Ref ID J:72102]

Koller BH; Kim HS; Latour AM; Brigman K; Boucher RC Jr; Scambler P; Wainwright B; Smithies O. 1991. Toward an animal model of cystic fibrosis: targeted interruption of exon 10 of the cystic fibrosis transmembrane regulator gene in embryonic stem cells. Proc Natl Acad Sci U S A 88(23):10730-4. [PubMed: 1720548]  [MGI Ref ID J:2356]

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]

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]

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]

Blanco PG; Zaman MM; Junaidi O; Sheth S; Yantiss RK; Nasser IA; Freedman SD. 2004. Induction of colitis in cftr-/- mice results in bile duct injury. Am J Physiol Gastrointest Liver Physiol 287(2):G491-6. [PubMed: 15064232]  [MGI Ref ID J:95677]

Boncoeur E; Roque T; Bonvin E; Saint-Criq V; Bonora M; Clement A; Tabary O; Henrion-Caude A; Jacquot J. 2008. Cystic fibrosis transmembrane conductance regulator controls lung proteasomal degradation and nuclear factor-kappaB activity in conditions of oxidative stress. Am J Pathol 172(5):1184-94. [PubMed: 18372427]  [MGI Ref ID J:134266]

Bonora M; Bernaudin JF; Guernier C; Brahimi-Horn MC. 2004. Ventilatory responses to hypercapnia and hypoxia in conscious cystic fibrosis knockout mice Cftr-/-. Pediatr Res 55(5):738-46. [PubMed: 14764916]  [MGI Ref ID J:102168]

Bruscia EM; Price JE; Cheng EC; Weiner S; Caputo C; Ferreira EC; Egan ME; Krause DS. 2006. Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation. Proc Natl Acad Sci U S A 103(8):2965-70. [PubMed: 16481627]  [MGI Ref ID J:107307]

Canale-Zambrano JC; Poffenberger MC; Cory SM; Humes DG; Haston CK. 2007. Intestinal phenotype of variable-weight cystic fibrosis knockout mice. Am J Physiol Gastrointest Liver Physiol 293(1):G222-9. [PubMed: 17615178]  [MGI Ref ID J:123741]

Chang CT; Bens M; Hummler E; Boulkroun S; Schild L; Teulon J; Rossier BC; Vandewalle A. 2005. Vasopressin-stimulated CFTR Cl- currents are increased in the renal collecting duct cells of a mouse model of Liddle's syndrome. J Physiol 562(Pt 1):271-84. [PubMed: 15513933]  [MGI Ref ID J:107876]

Chen H; Liu LL; Ye LL; McGuckin C; Tamowski S; Scowen P; Tian H; Murray K; Hatton WJ; Duan D. 2004. Targeted inactivation of cystic fibrosis transmembrane conductance regulator chloride channel gene prevents ischemic preconditioning in isolated mouse heart. Circulation 110(6):700-4. [PubMed: 15289377]  [MGI Ref ID J:102151]

Chroneos ZC; Wert SE; Livingston JL; Hassett DJ; Whitsett JA. 2000. Role of cystic fibrosis transmembrane conductance regulator in pulmonary clearance of Pseudomonas aeruginosa in vivo. J Immunol 165(7):3941-50. [PubMed: 11034402]  [MGI Ref ID J:137901]

Clarke LL; Gawenis LR; Bradford EM; Judd LM; Boyle KT; Simpson JE; Shull GE; Tanabe H; Ouellette AJ; Franklin CL; Walker NM. 2004. Abnormal Paneth cell granule dissolution and compromised resistance to bacterial colonization in the intestine of CF mice. Am J Physiol Gastrointest Liver Physiol 286(6):G1050-8. [PubMed: 14715526]  [MGI Ref ID J:95682]

Clarke LL; Grubb BR; Gabriel SE; Smithies O; Koller BH; Boucher RC. 1992. Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis. Science 257(5073):1125-8. [PubMed: 1380724]  [MGI Ref ID J:23817]

Cohen JC; Lundblad LK; Bates JH; Levitzky M; Larson JE. 2004. The 'Goldilocks effect' in cystic fibrosis: identification of a lung phenotype in the cftr knockout and heterozygous mouse. BMC Genet 5:21. [PubMed: 15279681]  [MGI Ref ID J:101836]

Cohen JC; Morrow SL; Cork RJ; Delcarpio JB; Larson JE. 1998. Molecular pathophysiology of cystic fibrosis based on the rescued knockout mouse model. Mol Genet Metab 64(2):108-18. [PubMed: 9705235]  [MGI Ref ID J:50999]

Cowley EA; Govindaraju K; Guilbault C; Radzioch D; Eidelman DH. 2000. Airway surface liquid composition in mice. Am J Physiol Lung Cell Mol Physiol 278(6):L1213-20. [PubMed: 10835327]  [MGI Ref ID J:66040]

De Lisle RC. 2007. Altered transit and bacterial overgrowth in the cystic fibrosis mouse small intestine. Am J Physiol Gastrointest Liver Physiol 293(1):G104-11. [PubMed: 17363465]  [MGI Ref ID J:123692]

De Lisle RC. 1995. Increased expression of sulfated gp300 and acinar tissue pathology in pancreas of CFTR(-/-) mice. Am J Physiol 268(4 Pt 1):G717-23. [PubMed: 7537458]  [MGI Ref ID J:24488]

De Lisle RC; Isom KS; Ziemer D; Cotton CU. 2001. Changes in the exocrine pancreas secondary to altered small intestinal function in the CF mouse. Am J Physiol Gastrointest Liver Physiol 281(4):G899-906. [PubMed: 11557509]  [MGI Ref ID J:72102]

De Lisle RC; Petitt M; Isom KS; Ziemer D. 1998. Developmental expression of a mucinlike glycoprotein (MUCLIN) in pancreas and small intestine of CF mice. Am J Physiol 275(2 Pt 1):G219-27. [PubMed: 9688648]  [MGI Ref ID J:111694]

De Lisle RC; Roach E; Jansson K. 2007. Effects of laxative and N-acetylcysteine on mucus accumulation, bacterial load, transit, and inflammation in the cystic fibrosis mouse small intestine. Am J Physiol Gastrointest Liver Physiol 293(3):G577-84. [PubMed: 17615175]  [MGI Ref ID J:125234]

Di A; Brown ME; Deriy LV; Li C; Szeto FL; Chen Y; Huang P; Tong J; Naren AP; Bindokas V; Palfrey HC; Nelson DJ. 2006. CFTR regulates phagosome acidification in macrophages and alters bactericidal activity. Nat Cell Biol 8(9):933-44. [PubMed: 16921366]  [MGI Ref ID J:129519]

Dif F; Marty C; Baudoin C; De Vernejoul MC; Levi G. 2004. Severe osteopenia in CFTR-null mice. Bone 35(3):595-603. [PubMed: 15336594]  [MGI Ref ID J:92512]

Dimagno MJ; Lee SH; Hao Y; Zhou SY; McKenna BJ; Owyang C. 2005. A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice. Gastroenterology 129(2):665-81. [PubMed: 16083720]  [MGI Ref ID J:104646]

Durie PR; Kent G; Phillips MJ; Ackerley CA. 2004. Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model. Am J Pathol 164(4):1481-93. [PubMed: 15039235]  [MGI Ref ID J:89164]

Eckman EA; Cotton CU; Kube DM; Davis PB. 1995. Dietary changes improve survival of CFTR S489X homozygous mutant mouse. Am J Physiol 269(5 Pt 1):L625-30. [PubMed: 7491981]  [MGI Ref ID J:29940]

Egan ME; Pearson M; Weiner SA; Rajendran V; Rubin D; Glockner-Pagel J; Canny S; Du K; Lukacs GL; Caplan MJ. 2004. Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects. Science 304(5670):600-2. [PubMed: 15105504]  [MGI Ref ID J:90068]

Fiorotto R; Spirli C; Fabris L; Cadamuro M; Okolicsanyi L; Strazzabosco M. 2007. Ursodeoxycholic acid stimulates cholangiocyte fluid secretion in mice via CFTR-dependent ATP secretion. Gastroenterology 133(5):1603-13. [PubMed: 17983806]  [MGI Ref ID J:130257]

Freedman SD; Katz MH; Parker EM; Laposata M; Urman MY; Alvarez JG. 1999. A membrane lipid imbalance plays a role in the phenotypic expression of cystic fibrosis in cftr(-/-) mice. Proc Natl Acad Sci U S A 96(24):13995-4000. [PubMed: 10570187]  [MGI Ref ID J:58571]

Freedman SD; Kern HF; Scheele GA. 2001. Pancreatic Acinar Cell Dysfunction in CFTR(-/-) Mice Is Associated With Impairments in Luminal pH and Endocytosis. Gastroenterology 121(4):950-7. [PubMed: 11606508]  [MGI Ref ID J:72031]

Gabriel SE; Brigman KN; Koller BH; Boucher RC; Stutts MJ. 1994. Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model [see comments] Science 266(5182):107-9. [PubMed: 7524148]  [MGI Ref ID J:20778]

Gallagher AM; Gottlieb RA. 2001. Proliferation, not apoptosis, alters epithelial cell migration in small intestine of CFTR null mice. Am J Physiol Gastrointest Liver Physiol 281(3):G681-7. [PubMed: 11518680]  [MGI Ref ID J:80886]

Gawenis LR; Hut H; Bot AG; Shull GE; de Jonge HR; Stien X; Miller ML; Clarke LL. 2004. Electroneutral sodium absorption and electrogenic anion secretion across murine small intestine are regulated in parallel. Am J Physiol Gastrointest Liver Physiol 287(6):G1140-9. [PubMed: 15284023]  [MGI Ref ID J:96161]

Gosselin D; Stevenson MM; Cowley EA; Griesenbach U; Eidelman DH; Boule M; Tam MF; Kent G; Skamene E; Tsui LC; Radzioch D. 1998. Impaired ability of Cftr knockout mice to control lung infection with Pseudomonas aeruginosa. Am J Respir Crit Care Med 157(4 Pt 1):1253-62. [PubMed: 9563748]  [MGI Ref ID J:47487]

Grassme H; Jendrossek V; Riehle A; von Kurthy G; Berger J; Schwarz H; Weller M; Kolesnick R; Gulbins E. 2003. Host defense against Pseudomonas aeruginosa requires ceramide-rich membrane rafts. Nat Med 9(3):322-30. [PubMed: 12563314]  [MGI Ref ID J:107219]

Grubb BR; Boucher RC. 1997. Enhanced colonic Na+ absorption in cystic fibrosis mice versus normal mice. Am J Physiol 272(2 Pt 1):G393-400. [PubMed: 9124365]  [MGI Ref ID J:38820]

Grubb BR; Gabriel SE. 1997. Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis. Am J Physiol 273(2 Pt 1):G258-66. [PubMed: 9277402]  [MGI Ref ID J:42490]

Grubb BR; Paradiso AM; Boucher RC. 1994. Anomalies in ion transport in CF mouse tracheal epithelium. Am J Physiol 267(1 Pt 1):C293-300. [PubMed: 8048488]  [MGI Ref ID J:19846]

Grubb BR; Rogers TD; Kulaga HM; Burns KA; Wonsetler RL; Reed RR; Ostrowski LE. 2007. Olfactory epithelia exhibit progressive functional and morphological defects in CF mice. Am J Physiol Cell Physiol 293(2):C574-83. [PubMed: 17428842]  [MGI Ref ID J:125846]

Grubb BR; Vick RN; Boucher RC. 1994. Hyperabsorption of Na+ and raised Ca(2+)-mediated Cl- secretion in nasal epithelia of CF mice. Am J Physiol 266(5 Pt 1):C1478-83. [PubMed: 7515571]  [MGI Ref ID J:18444]

Guilbault C; Novak JP; Martin P; Boghdady ML; Saeed Z; Guiot MC; Hudson TJ; Radzioch D. 2006. Distinct pattern of lung gene expression in the Cftr-KO mice developing spontaneous lung disease compared with their littermate controls. Physiol Genomics 25(2):179-93. [PubMed: 16418321]  [MGI Ref ID J:108346]

Guilbault C; Saeed Z; Downey GP; Radzioch D. 2007. Cystic fibrosis mouse models. Am J Respir Cell Mol Biol 36(1):1-7. [PubMed: 16888286]  [MGI Ref ID J:130524]

Haston CK; Corey M; Tsui LC. 2002. Mapping of genetic factors influencing the weight of cystic fibrosis knockout mice. Mamm Genome 13(11):614-8. [PubMed: 12461646]  [MGI Ref ID J:80597]

Haston CK; Humes DG; Lafleur M. 2007. X chromosome transmission ratio distortion in Cftr +/- intercross-derived mice. BMC Genet 8:23. [PubMed: 17506901]  [MGI Ref ID J:125558]

Haston CK; McKerlie C; Newbigging S; Corey M; Rozmahel R; Tsui LC. 2002. Detection of modifier loci influencing the lung phenotype of cystic fibrosis knockout mice. Mamm Genome 13(11):605-13. [PubMed: 12461645]  [MGI Ref ID J:80598]

Haston CK; Tsui LC. 2003. Loci of intestinal distress in cystic fibrosis knockout mice. Physiol Genomics 12(2):79-84. [PubMed: 12441405]  [MGI Ref ID J:82609]

Hinojosa-Kurtzberg AM; Johansson ME; Madsen CS; Hansson GC; Gendler SJ. 2003. Novel MUC1 splice variants contribute to mucin overexpression in CFTR-deficient mice. Am J Physiol Gastrointest Liver Physiol 284(5):G853-62. [PubMed: 12529261]  [MGI Ref ID J:83392]

Hoffmann N; Rasmussen TB; Jensen PO; Stub C; Hentzer M; Molin S; Ciofu O; Givskov M; Johansen HK; Hoiby N. 2005. Novel mouse model of chronic Pseudomonas aeruginosa lung infection mimicking cystic fibrosis. Infect Immun 73(4):2504-14. [PubMed: 15784597]  [MGI Ref ID J:97174]

Hogan DL; Crombie DL; Isenberg JI; Svendsen P; Schaffalitzky de Muckadell OB ; Ainsworth MA. 1997. CFTR mediates cAMP- and Ca2+-activated duodenal epithelial HCO3- secretion. Am J Physiol 272(4 Pt 1):G872-8. [PubMed: 9142920]  [MGI Ref ID J:40258]

Ianowski JP; Choi JY; Wine JJ; Hanrahan JW. 2007. Mucus secretion by single tracheal submucosal glands from normal and cystic fibrosis transmembrane conductance regulator knockout mice. J Physiol 580(Pt 1):301-14. [PubMed: 17204498]  [MGI Ref ID J:140842]

Jiang C; Akita GY; Colledge WH; Ratcliff RA; Evans MJ; Hehir KM ; St George JA ; Wadsworth SC ; Cheng SH. 1997. Increased contact time improves adenovirus-mediated CFTR gene transfer to nasal epithelium of CF mice. Hum Gene Ther 8(6):671-80. [PubMed: 9113507]  [MGI Ref ID J:40583]

Joo NS; Clarke LL; Han BH; Forte LR; Kim HD. 1999. Cloning of ClC-2 chloride channel from murine duodenum and its presence in CFTR knockout mice. Biochim Biophys Acta 1446(3):431-7. [PubMed: 10524221]  [MGI Ref ID J:57459]

Kaur S; Norkina O; Ziemer D; Samuelson LC; De Lisle RC. 2004. Acidic duodenal pH alters gene expression in the cystic fibrosis mouse pancreas. Am J Physiol Gastrointest Liver Physiol 287(2):G480-90. [PubMed: 15064229]  [MGI Ref ID J:102142]

Kelley TJ; Drumm ML. 1998. Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway epithelial cells. J Clin Invest 102(6):1200-7. [PubMed: 9739054]  [MGI Ref ID J:115209]

Kelley TJ; Elmer HL. 2000. In vivo alterations of IFN regulatory factor-1 and PIAS1 protein levels in cystic fibrosis epithelium. J Clin Invest 106(3):403-10. [PubMed: 10930443]  [MGI Ref ID J:111647]

Kelley TJ; Elmer HL; Corey DA. 2001. Reduced Smad3 protein expression and altered transforming growth factor-beta1-mediated signaling in cystic fibrosis epithelial cells. Am J Respir Cell Mol Biol 25(6):732-8. [PubMed: 11726399]  [MGI Ref ID J:114419]

Kent G; Iles R; Bear CE; Huan LJ; Griesenbach U; McKerlie C; Frndova H; Ackerley C; Gosselin D; Radzioch D; O'Brodovich H; Tsui LC; Buchwald M; Tanswell AK. 1997. Lung disease in mice with cystic fibrosis. J Clin Invest 100(12):3060-9. [PubMed: 9399953]  [MGI Ref ID J:44904]

Kent G; Oliver M; Foskett JK; Frndova H; Durie P; Forstner J; Forstner GG; Riordan JR; Percy D; Buchwald M. 1996. Phenotypic abnormalities in long-term surviving cystic fibrosis mice. Pediatr Res 40(2):233-41. [PubMed: 8827771]  [MGI Ref ID J:35002]

Koller BH; Kim HS; Latour AM; Brigman K; Boucher RC Jr; Scambler P; Wainwright B; Smithies O. 1991. Toward an animal model of cystic fibrosis: targeted interruption of exon 10 of the cystic fibrosis transmembrane regulator gene in embryonic stem cells. Proc Natl Acad Sci U S A 88(23):10730-4. [PubMed: 1720548]  [MGI Ref ID J:2356]

Kuver R; Wong T; Klinkspoor JH; Lee SP. 2006. Absence of CFTR is associated with pleiotropic effects on mucins in mouse gallbladder epithelial cells. Am J Physiol Gastrointest Liver Physiol 291(6):G1148-54. [PubMed: 16825704]  [MGI Ref ID J:116875]

Larson JE; Delcarpio JB; Farberman MM; Morrow SL; Cohen JC. 2000. CFTR modulates lung secretory cell proliferation and differentiation. Am J Physiol Lung Cell Mol Physiol 279(2):L333-41. [PubMed: 10926557]  [MGI Ref ID J:63983]

Lindert J; Perlman CE; Parthasarathi K; Bhattacharya J. 2007. Chloride-dependent secretion of alveolar wall liquid determined by optical-sectioning microscopy. Am J Respir Cell Mol Biol 36(6):688-96. [PubMed: 17290033]  [MGI Ref ID J:136608]

Lu M; Leng Q; Egan ME; Caplan MJ; Boulpaep EL; Giebisch GH; Hebert SC. 2006. CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney. J Clin Invest 116(3):797-807. [PubMed: 16470247]  [MGI Ref ID J:106483]

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]

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]

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]

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; 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]

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]

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]

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; 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]

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]

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]

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]

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; 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]

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]

Zhou L; Dey CR; Wert SE; DuVall MD; Frizzell RA; Whitsett JA. 1994. Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR. Science 266(5191):1705-8. [PubMed: 7527588]  [MGI Ref ID J:21934]

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.

Health & Colony Maintenance Information

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
Diet Information LabDiet® 5K52/5K67

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Pricing for USA, Canada and Mexico shipping destinations View International pricing
Price (US dollars $)
Cryorecovery Fee $1900.00
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.

Additional Supply Details

Pricing for International shipping destinations View USA Canada and Mexico pricing
Price (US dollars $)
Cryorecovery Fee $2470.00
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.

Additional Supply Details

Supply Details

Standard SupplyCryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
Supply Notes
  • Cryorecovery - Standard.
    At least two mice that carry the mutation (if it is a mutant strain) will be provided. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotypes and genders are needed. IMPORTANT NOTE: The genotypes of the 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 for possible genotypes for this specific strain. Animals typically ship within 13 to 16 weeks from your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will typically ship within 25 weeks.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 or 1-207-288-5845.

  • This strain is included in the Induced Mutant Resource Colony collection.
  • Genomic DNA is available for this strain from the Mouse DNA Resource.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
  International - 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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