Strain Name:

B6.129S6-Cftrtm1Kth/J

Stock Number:

002515

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Availability:

Cryopreserved - Ready for recovery

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/6
Donor Strain 129S6 via EK.CC1.2 ES cell line
 
Donating InvestigatorDr. Kirk R. Thomas,   Univ of Utah

Description
The Cftrtm1Kth targeted mutation corresponds to the delta 508 mutation in humans. Homozygous mutant mice show an increased mortality within the first month after birth, with ~60% mortality by post-weaning. Those that survive are fertile, but females are poor breeders. Mutant mice are also reduced in size compared to normal wildtype mice. Those that do not survive to adulthood show bowel obstructions, bowel strictures and peritonitis. Lungs, pancreas, gall bladder, male reproductive tract, lacrimal gland, and submandibular glands from homozygous mice appear normal regardless of the survival of the animal. Homozygous mice also show a tissue-specific loss of CFTR transcripts in the intestine.

Development
The Cftrtm1Kth targeted mutation was made in the laboratory of Dr. Kirk R. Thomas at the University of Utah. The mutated allele contains a 3-bp (CTT) deletion between bases 1656 and 1660 resulting in the loss of a phenylalanine residue in exon 10 at a position which corresponds to human position 508. The 129S6/SvEv-derived CC1.2 ES cell line was used.

Control Information

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

Related Strains

Strains carrying other alleles of Cftr
002196   B6.129P2-Cftrtm1Unc/J
002364   STOCK Cftrtm1Unc Tg(FABPCFTR)1Jaw/J
View Strains carrying other alleles of Cftr     (2 strains)

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

Cftrtm1Kth/Cftrtm1Kth

        B6.129S7-Cftrtm1Kth
  • 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, identical to that in Cftrtm1Unc homozygotes   (MGI Ref ID J:112450)
  • 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)
  • digestive/alimentary phenotype
  • intestinal obstruction
    • develop intestinal blockage when fed a normal (solid) diet   (MGI Ref ID J:112450)
  • immune system phenotype
  • lung inflammation
    • increased inflammatory response to chronic Pseudomonas aeruginosa infection, identical to that in Cftrtm1Unc homozygotes   (MGI Ref ID J:112450)
  • 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, 22.2% 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 (3.81 +/- 1.43 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 induction of 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, 98.4% of mutant oocytes remain unfertilized, whereas the majority of embryos from superovulated wild-type females are found 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)
    • 35.7% 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)
    • 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)
  • endocrine/exocrine gland phenotype
  • 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)
  • 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)

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

Cftrtm1Kth/Cftrtm1Kth

        involves: 129S7/SvEvBrd * C57BL/6J
  • mortality/aging
  • partial postnatal lethality
    • about 10% died within 7 days of birth and saw significant mortality around the time of weaning (30 days after birth), with 40% surviving postweaning for at least 8 months   (MGI Ref ID J:29074)
  • digestive/alimentary phenotype
  • abnormal digestive system physiology
    • lacked cAMP-stimulated Cl- secretory current in intestine, indicating defective electrolyte transport   (MGI Ref ID J:29074)
    • intestinal obstruction
      • mice that died prematurely showed evidence of bowel obstruction   (MGI Ref ID J:29074)
    • peritoneal inflammation
      • mice that died prematurely showed peritonitis   (MGI Ref ID J:29074)
  • abnormal intestine morphology
    • mice that died prematurely showed evidence of bowel obstruction and bowel strictures, especially in the cecum   (MGI Ref ID J:29074)
    • abnormal jejunum morphology
      • some mice exhibited large casts of mucus material in the jejunum, however lung, pancreas, gall bladder, male reproductive tract, lacrimal gland, and submandibular glands appeared normal   (MGI Ref ID J:29074)
    • dilated Brunner's glands
      • Brunner's glands were often dilated   (MGI Ref ID J:29074)
  • growth/size/body phenotype
  • decreased body size
    • reduced size noticeable as early as 10 days after birth   (MGI Ref ID J:29074)
    • decreased body weight
      • by weaning, weight was only 50-60% that of controls and although homozygous mutant mice continued to grow, they never reached the size of controls   (MGI Ref ID J:29074)
  • homeostasis/metabolism phenotype
  • abnormal fluid regulation
    • defective fluid transport in nasal, intestinal, and pancreatic duct epithelia   (MGI Ref ID J:29074)
  • abnormal ion homeostasis
    • defective electrolyte transport in nasal, intestinal, and pancreatic duct epithelia   (MGI Ref ID J:29074)
  • immune system phenotype
  • peritoneal inflammation
    • mice that died prematurely showed peritonitis   (MGI Ref ID J:29074)
  • endocrine/exocrine gland phenotype
  • abnormal pancreas physiology
    • reduced absorption of fluid and lower cAMP-stimulated fluid secretion by pancreatic epithelia   (MGI Ref ID J:29074)
  • dilated Brunner's glands
    • Brunner's glands were often dilated   (MGI Ref ID J:29074)
View Research Applications

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

Immunology, Inflammation and Autoimmunity Research
Cystic Fibrosis

Cftrtm1Kth related

Immunology, Inflammation and Autoimmunity Research
Inflammation
      Inflammatory bowel disease

Metabolism Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Cftrtm1Kth
Allele Name targeted mutation 1, Kirk R Thomas
Allele Type Targeted
Common Name(s) CFTRdeltaF508; deltaF; deltaF508 Cftr;
Mutation Made ByDr. Kirk Thomas,   Univ of Utah
Strain of Origin129S7/SvEvBrd
ES Cell Line NameCC1.2
ES Cell Line Strain129S7/SvEvBrd
Promoter Cftr, cystic fibrosis transmembrane conductance regulator, mouse, laboratory
General Note This (J:29074) and other (J:27734, J:28979) targeted mutations reproduce the common human mutation, eliminating the same phenylalanine from the protein sequence. In at least one of these models, the mutant is temperature sensitive, and can be expressed on the apical membrane when cultured at low temperatures, which is also true of the human mutant lacking the same phenylalanine residue (J:35364).
Molecular Note The allele contains a 3 bp deletion in exon 10 of nucleotides between 1656 to 1660, resulting in the loss of a codon corresponding to a phenylalanine. A neomycin selection cassette was also inserted in intron 10 in reverse transcriptional orientation to the gene. [MGI Ref ID J:29074]

Genotyping

Genotyping Information

Genotyping Protocols

Cftrtm1Kth, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Zeiher BG; Eichwald E; Zabner J; Smith JJ; Puga AP; McCray PB Jr; Capecchi MR; Welsh MJ; Thomas KR. 1995. A mouse model for the delta F508 allele of cystic fibrosis. J Clin Invest 96(4):2051-64. [PubMed: 7560099]  [MGI Ref ID J:29074]

Additional References

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]

Cftrtm1Kth related

Bederman I; Perez A; Henderson L; Freedman JA; Poleman J; Guentert D; Ruhrkraut N; Drumm ML. 2012. Altered de novo lipogenesis contributes to low adipose stores in cystic fibrosis mice. Am J Physiol Gastrointest Liver Physiol 303(4):G507-18. [PubMed: 22679004]  [MGI Ref ID J:191611]

Bodas M; Min T; Mazur S; Vij N. 2011. Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema. J Immunol 186(1):602-13. [PubMed: 21135173]  [MGI Ref ID J:168002]

Bragonzi A; Paroni M; Nonis A; Cramer N; Montanari S; Rejman J; Di Serio C; Doring G; Tummler B. 2009. Pseudomonas aeruginosa microevolution during cystic fibrosis lung infection establishes clones with adapted virulence. Am J Respir Crit Care Med 180(2):138-45. [PubMed: 19423715]  [MGI Ref ID J:164966]

Catalan MA; Nakamoto T; Gonzalez-Begne M; Camden JM; Wall SM; Clarke LL; Melvin JE. 2010. Cftr and ENaC ion channels mediate NaCl absorption in the mouse submandibular gland. J Physiol 588(Pt 4):713-24. [PubMed: 20026617]  [MGI Ref ID J:176780]

Clayburgh DR; Barrett TA; Tang Y; Meddings JB; Van Eldik LJ; Watterson DM; Clarke LL; Mrsny RJ; Turner JR. 2005. Epithelial myosin light chain kinase-dependent barrier dysfunction mediates T cell activation-induced diarrhea in vivo. J Clin Invest 115(10):2702-15. [PubMed: 16184195]  [MGI Ref ID J:101529]

Coleman FT; Mueschenborn S; Meluleni G; Ray C; Carey VJ; Vargas SO; Cannon CL; Ausubel FM; Pier GB. 2003. Hypersusceptibility of cystic fibrosis mice to chronic Pseudomonas aeruginosa oropharyngeal colonization and lung infection. Proc Natl Acad Sci U S A 100(4):1949-54. [PubMed: 12578988]  [MGI Ref ID J:107417]

Colledge WH; Abella BS; Southern KW; Ratcliff R; Jiang C; Cheng SH; MacVinish LJ; Anderson JR; Cuthbert AW; Evans MJ. 1995. Generation and characterization of a delta F508 cystic fibrosis mouse model. Nat Genet 10(4):445-52. [PubMed: 7545494]  [MGI Ref ID J:27734]

Deriy LV; Gomez EA; Zhang G; Beacham DW; Hopson JA; Gallan AJ; Shevchenko PD; Bindokas VP; Nelson DJ. 2009. Disease-causing mutations in the cystic fibrosis transmembrane conductance regulator determine the functional responses of alveolar macrophages. J Biol Chem 284(51):35926-38. [PubMed: 19837664]  [MGI Ref ID J:158205]

DiMagno MJ; Lee SH; Owyang C; Zhou SY. 2010. Inhibition of acinar apoptosis occurs during acute pancreatitis in the human homologue DeltaF508 cystic fibrosis mouse. Am J Physiol Gastrointest Liver Physiol 299(2):G400-12. [PubMed: 20522641]  [MGI Ref ID J:163346]

Falany JL; Greer H; Kovacs T; Sorscher EJ; Falany CN. 2002. Elevation of hepatic sulphotransferase activities in mice with resistance to cystic fibrosis. Biochem J 364(Pt 1):115-20. [PubMed: 11988083]  [MGI Ref ID J:113524]

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]

French PJ; van Doorninck JH; Peters RH; Verbeek E; Ameen NA; Marino CR; de Jonge HR; Bijman J; Scholte BJ. 1996. A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo. J Clin Invest 98(6):1304-12. [PubMed: 8823295]  [MGI Ref ID J:35364]

Freudenberg F; Leonard MR; Liu SA; Glickman JN; Carey MC. 2010. Pathophysiological preconditions promoting mixed 'black' pigment plus cholesterol gallstones in a DeltaF508 mouse model of cystic fibrosis. Am J Physiol Gastrointest Liver Physiol 299(1):G205-14. [PubMed: 20430874]  [MGI Ref ID J:162509]

Garcia MA; Yang N; Quinton PM. 2009. Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion. J Clin Invest 119(9):2613-22. [PubMed: 19726884]  [MGI Ref ID J:152692]

Gee HY; Noh SH; Tang BL; Kim KH; Lee MG. 2011. Rescue of DeltaF508-CFTR Trafficking via a GRASP-Dependent Unconventional Secretion Pathway. Cell 146(5):746-60. [PubMed: 21884936]  [MGI Ref ID J:176213]

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]

Hashimoto Y; Shuto T; Mizunoe S; Tomita A; Koga T; Sato T; Takeya M; Suico MA; Niibori A; Sugahara T; Shimasaki S; Sugiyama T; Scholte B; Kai H. 2011. CFTR-deficiency renders mice highly susceptible to cutaneous symptoms during mite infestation. Lab Invest 91(4):509-18. [PubMed: 21135815]  [MGI Ref ID J:170421]

Henderson LB; Doshi VK; Blackman SM; Naughton KM; Pace RG; Moskovitz J; Knowles MR; Durie PR; Drumm ML; Cutting GR. 2012. Variation in MSRA modifies risk of neonatal intestinal obstruction in cystic fibrosis. PLoS Genet 8(3):e1002580. [PubMed: 22438829]  [MGI Ref ID J:183474]

Hirokawa M; Takeuchi T; Chu S; Akiba Y; Wu V; Guth PH; Engel E; Montrose MH; Kaunitz JD. 2004. Cystic fibrosis gene mutation reduces epithelial cell acidification and injury in acid-perfused mouse duodenum. Gastroenterology 127(4):1162-73. [PubMed: 15480994]  [MGI Ref ID J:93426]

Hodges CA; Palmert MR; Drumm ML. 2008. Infertility in females with cystic fibrosis is multifactorial: evidence from mouse models. Endocrinology 149(6):2790-7. [PubMed: 18325992]  [MGI Ref ID J:145380]

Jin R; Hodges CA; Drumm ML; Palmert MR. 2006. The cystic fibrosis transmembrane conductance regulator (Cftr) modulates the timing of puberty in mice. J Med Genet 43(6):e29. [PubMed: 16740913]  [MGI Ref ID J:200888]

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]

Kibble JD; Neal AM; Colledge WH; Green R; Taylor CJ. 2000. Evidence for cystic fibrosis transmembrane conductance regulator-dependent sodium reabsorption in kidney, using Cftr(tm2cam) mice. J Physiol 526 Pt 1:27-34. [PubMed: 10878096]  [MGI Ref ID J:134183]

Lazrak A; Jurkuvenaite A; Chen L; Keeling KM; Collawn JF; Bedwell DM; Matalon S. 2011. Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane conductance regulator expression in mouse lung. Am J Physiol Lung Cell Mol Physiol 301(4):L557-67. [PubMed: 21743028]  [MGI Ref ID J:176276]

Liu X; Yan Z; Luo M; Engelhardt JF. 2006. Species-specific differences in mouse and human airway epithelial biology of recombinant adeno-associated virus transduction. Am J Respir Cell Mol Biol 34(1):56-64. [PubMed: 16195538]  [MGI Ref ID J:120193]

Livraghi-Butrico A; Kelly EJ; Wilkinson KJ; Rogers TD; Gilmore RC; Harkema JR; Randell SH; Boucher RC; O'Neal WK; Grubb BR. 2013. Loss of Cftr function exacerbates the phenotype of Na+ hyperabsorption in murine airways. Am J Physiol Lung Cell Mol Physiol 304(7):L469-80. [PubMed: 23377346]  [MGI Ref ID J:195159]

Lu M; Dong K; Egan ME; Giebisch GH; Boulpaep EL; Hebert SC. 2010. Mouse cystic fibrosis transmembrane conductance regulator forms cAMP-PKA-regulated apical chloride channels in cortical collecting duct. Proc Natl Acad Sci U S A 107(13):6082-7. [PubMed: 20231442]  [MGI Ref ID J:158652]

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]

Muchekehu RW; Quinton PM. 2010. A new role for bicarbonate secretion in cervico-uterine mucus release. J Physiol 588(Pt 13):2329-42. [PubMed: 20478977]  [MGI Ref ID J:176765]

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]

Okiyoneda T; Niibori A; Harada K; Kohno T; Michalak M; Duszyk M; Wada I; Ikawa M; Shuto T; Suico MA; Kai H. 2008. Role of calnexin in the ER quality control and productive folding of CFTR; differential effect of calnexin knockout on wild-type and DeltaF508 CFTR. Biochim Biophys Acta 1783(9):1585-94. [PubMed: 18457676]  [MGI Ref ID J:136971]

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]

Reynaert I; Van Der Schueren B; Degeest G; Manin M; Cuppens H; Scholte B; Cassiman JJ. 2000. Morphological changes in the vas deferens and expression of the cystic fibrosis transmembrane conductance regulator (CFTR) in control, deltaF508 and knock-out CFTR mice during postnatal life. Mol Reprod Dev 55(2):125-35. [PubMed: 10618651]  [MGI Ref ID J:119599]

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]

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]

Sellers ZM; De Arcangelis V; Xiang Y; Best PM. 2010. Cardiomyocytes with disrupted CFTR function require CaMKII and Ca(2+)-activated Cl(-) channel activity to maintain contraction rate. J Physiol 588(Pt 13):2417-29. [PubMed: 20442264]  [MGI Ref ID J:176767]

Sidani SM; Kirchhoff P; Socrates T; Stelter L; Ferreira E; Caputo C; Roberts KE; Bell RL; Egan ME; Geibel JP. 2007. DeltaF508 mutation results in impaired gastric acid secretion. J Biol Chem 282(9):6068-74. [PubMed: 17178714]  [MGI Ref ID J:120932]

Song Y; Yamamoto A; Steward MC; Ko SB; Stewart AK; Soleimani M; Liu BC; Kondo T; Jin CX; Ishiguro H. 2012. Deletion of Slc26a6 alters the stoichiometry of apical Cl-/HCO-3 exchange in mouse pancreatic duct. Am J Physiol Cell Physiol 303(8):C815-24. [PubMed: 22895259]  [MGI Ref ID J:192779]

Steagall WK; Drumm ML. 1999. Stimulation of cystic fibrosis transmembrane conductance regulator-dependent short-circuit currents across DeltaF508 murine intestines. Gastroenterology 116(6):1379-88. [PubMed: 10348821]  [MGI Ref ID J:55783]

Xiao F; Li J; Singh AK; Riederer B; Wang J; Sultan A; Park H; Lee MG; Lamprecht G; Scholte BJ; De Jonge HR; Seidler U. 2012. Rescue of epithelial HCO3- secretion in murine intestine by apical membrane expression of the cystic fibrosis transmembrane conductance regulator mutant F508del. J Physiol 590(Pt 21):5317-34. [PubMed: 22802588]  [MGI Ref ID J:202084]

Yang N; Garcia MA; Quinton PM. 2013. Normal mucus formation requires cAMP-dependent HCO3- secretion and Ca2+-mediated mucin exocytosis. J Physiol 591(Pt 18):4581-93. [PubMed: 23818690]  [MGI Ref ID J:214187]

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]

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

Animal Health Reports

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

Colony Maintenance

Breeding & HusbandryThis strain is currently being maintained by mating heterozygous siblings; this is the most effective breeding strategy. Homozygous males will breed while homozygous females are very poor breeders. Expected coat color from breeding:Black

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

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.

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).

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).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

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

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


The Jackson Laboratory's Genotype Promise

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.
Ordering Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"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.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

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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