JAX
Mouse Strain Datasheet - 002515
B6.129S6-Cftrtm1Kth/J
Also Known As: ΔF
These Cftr knock-out mice exhibit neonatal lethality with abnormal bowel development. They may be useful in applications related to the study of cystic fibrosis.
Donating Investigator
Dr. Kirk R. Thomas, Univ of Utah
Genetic overview
| Genetic Background | Generation |
|---|---|
|
C57BL/6 |
|
Cftrtm1Kth
| Allele Type | Gene Symbol | Gene Name |
|---|---|---|
| Targeted | Cftr | cystic fibrosis transmembrane conductance regulator |
Research Applications
- Immunology, Inflammation and Autoimmunity Research
- Metabolism Research
Detailed 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 Suggestions
- Wild-type from the colony
- 000664 C57BL/6J
Additional Information
Selected References
- 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: 7560099MGI: J:29074
Cftrtm1Kth
Allele Symbol: Cftrtm1Kth
| Allele Name | targeted mutation 1, Kirk R Thomas |
|---|---|
| Allele Type | Targeted |
| Allele Synonym(s) | CFTRdeltaF508; c.1656_1660del; deltaF; deltaF508 Cftr; p.F508del |
| Gene Symbol and Name | Cftr, cystic fibrosis transmembrane conductance regulator |
| Gene Synonym(s) | ABC35; ABCC7; AW495489; Abcc7; CF; CFTR/MRP; MRP7; RGD1561193; TNR-CFTR; dJ760C5.1; expressed sequence AW495489 |
| Promoter | Cftr, cystic fibrosis transmembrane conductance regulator, mouse, laboratory |
| Strain of Origin | 129S7/SvEvBrd |
| Chromosome | 6 |
| 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. |
| Mutations Made By | Dr. Kirk Thomas, Univ of Utah |
Disease Terms
Research Areas By Genotype
This mouse can be used to support research in many areas including:
- Immunology, Inflammation and Autoimmunity Research
- Cystic Fibrosis
Genotype: Cftrtm1Kth related
- Immunology, Inflammation and Autoimmunity Research
- Inflammation
- Inflammatory bowel disease
- Inflammation
- Metabolism Research
Mammalian Phenotype Terms by Genotype
Genotype: Cftrtm1Kth/Cftrtm1Kth
B6.129S7-Cftrtm1Kth
respiratory system phenotype
- decreased respiratory epithelial chloride transmembrane transport
- after treatment with a chloride-depleted solution with amiloride and forskolin, the nasal potential difference (PD) was significantly different between control mice and homozygous mice (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)
mortality/aging
- increased susceptibility to bacterial infection induced morbidity/mortality
- reduced ability to respond to lung infection elicited with Pseudomonas aeruginosa- laden agarose beads (MGI Ref ID J:112450)
growth/size/body region phenotype
- postnatal growth retardation
- 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)
- weighed significantly less (P 0.05) than homozygote wild-type controls at 7, 14, and 21 days of age (MGI Ref ID J:112450)
digestive/alimentary phenotype
- intestinal obstruction
- develop intestinal blockage when fed a normal (solid) diet (MGI Ref ID J:112450)
immune system phenotype
- increased susceptibility to bacterial infection induced morbidity/mortality
- reduced ability to respond to lung infection elicited with Pseudomonas aeruginosa- laden agarose beads (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)
reproductive system phenotype
- abnormal sperm physiology
- however, no differences in capacitation of oviductal sperm from mutant and wild-type females are observed (MGI Ref ID J:145380)
- 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)
- 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 ovary weight
- decreased ovulation rate
- decreased uterus weight
- 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
- 35.7% of female homozygotes are unable to give birth over a 5-month mating period (MGI Ref ID J:145380)
- 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)
- 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)
- small uterus
- at 7 weeks of age, female homozygotes display smaller uteri than wild-type females (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)
- decreased ovary weight
- small ovary
- at 7 weeks of age, female homozygotes display smaller ovaries than wild-type females (MGI Ref ID J:145380)
homeostasis/metabolism phenotype
- 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
Genotype: Cftrtm1Kth/Cftrtm1Kth
involves: 129S7/SvEvBrd * C57BL/6J
mortality/aging
- postnatal lethality, incomplete penetrance
- 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)
- 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)
- 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)
growth/size/body region 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)
References
- 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: 7560099MGI: 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: 15007059MGI: J:89831
Additional - Cftrtm1Kth related
- Aeffner F; Abdulrahman B; Hickman-Davis JM; Janssen PM; Amer A; Bedwell DM; Sorscher EJ; Davis IC. 2013. Heterozygosity for the F508del mutation in the cystic fibrosis transmembrane conductance regulator anion channel attenuates influenza severity. J Infect Dis 208(5):780-9. PubMed: 23749967MGI: J:232005
- 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: 22679004MGI: 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: 21135173MGI: 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: 19423715MGI: J:164966
- Catalan MA; Kondo Y; Pena-Munzenmayer G; Jaramillo Y; Liu F; Choi S; Crandall E; Borok Z; Flodby P; Shull GE; Melvin JE. 2015. A fluid secretion pathway unmasked by acinar-specific Tmem16A gene ablation in the adult mouse salivary gland. Proc Natl Acad Sci U S A 112(7):2263-8. PubMed: 25646474MGI: J:219999
- 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: 20026617MGI: 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: 16184195MGI: 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: 12578988MGI: 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: 7545494MGI: J:27734
- Darrah RJ; Bederman IR; Mitchell AL; Hodges CA; Campanaro CK; Drumm ML; Jacono FJ. 2013. Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice. J Cyst Fibros 12(4):345-51. PubMed: 23290341MGI: J:232011
- 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: 19837664MGI: 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: 20522641MGI: 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: 11988083MGI: 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: 17983806MGI: 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: 8823295MGI: 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: 20430874MGI: 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: 19726884MGI: 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: 21884936MGI: 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: 16888286MGI: 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: 21135815MGI: 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: 22438829MGI: 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: 15480994MGI: 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: 18325992MGI: 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: 16740913MGI: 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: 9739054MGI: 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: 10878096MGI: 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: 21743028MGI: J:176276
- Lazrak A; Jurkuvenaite A; Ness EC; Zhang S; Woodworth BA; Muhlebach MS; Stober VP; Lim YP; Garantziotis S; Matalon S. 2014. Inter-alpha-inhibitor blocks epithelial sodium channel activation and decreases nasal potential differences in DeltaF508 mice. Am J Respir Cell Mol Biol 50(5):953-62. PubMed: 24303840MGI: J:231992
- 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: 16195538MGI: 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: 23377346MGI: 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: 20231442MGI: 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: 16470247MGI: 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: 20478977MGI: 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: 20724552MGI: 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: 18457676MGI: 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: 17873061MGI: J:125319
- Paradis J; Wilke M; Haston CK. 2010. Osteopenia in Cftr-deltaF508 mice. J Cyst Fibros 9(4):239-45. PubMed: 20570219MGI: J:233126
- Peotta VA; Bhandary P; Ogu U; Volk KA; Roghair RD. 2014. Reduced blood pressure of CFTR-F508del carriers correlates with diminished arterial reactivity rather than circulating blood volume in mice. PLoS One 9(5):e96756. PubMed: 24801204MGI: J:216090
- 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: 10618651MGI: J:119599
- Riazanski V; Gabdoulkhakova AG; Boynton LS; Eguchi RR; Deriy LV; Hogarth DK; Loaec N; Oumata N; Galons H; Brown ME; Shevchenko P; Gallan AJ; Yoo SG; Naren AP; Villereal ML; Beacham DW; Bindokas VP; Birnbaumer L; Meijer L; Nelson DJ. 2015. TRPC6 channel translocation into phagosomal membrane augments phagosomal function. Proc Natl Acad Sci U S A 112(47):E6486-95. PubMed: 26604306MGI: J:228125
- 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: 19363029MGI: J:150497
- Ruan YC; Wang Y; Da Silva N; Kim B; Diao RY; Hill E; Brown D; Chan HC; Breton S. 2014. CFTR interacts with ZO-1 to regulate tight junction assembly and epithelial differentiation through the ZONAB pathway. J Cell Sci 127(Pt 20):4396-408. PubMed: 25107366MGI: J:216308
- 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: 11390493MGI: 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: 20442264MGI: J:176767
- Sellers ZM; Kovacs A; Weinheimer CJ; Best PM. 2013. Left ventricular and aortic dysfunction in cystic fibrosis mice. J Cyst Fibros 12(5):517-24. PubMed: 23269368MGI: J:232013
- 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: 17178714MGI: 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: 22895259MGI: 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: 10348821MGI: J:55783
- Woods PS; Tazi MF; Chesarino NM; Amer AO; Davis IC. 2015. TGF-beta-induced IL-6 prevents development of acute lung injury in influenza A virus-infected F508del CFTR-heterozygous mice. Am J Physiol Lung Cell Mol Physiol 308(11):L1136-44. PubMed: 25840995MGI: J:232267
- 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: 22802588MGI: 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: 23818690MGI: 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: 15007059MGI: 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: 15246977MGI: J:112450