The following strains are either available or coming soon from the CF Mouse Models Core. Availability is dependent on strain.

References
1.            Snouwaert, J.N., et al., An animal model for cystic fibrosis made by gene targeting. Science, 1992. 257(5073): p. 1083-8.
2.            Zeiher, B.G., et al., A mouse model for the delta F508 allele of cystic fibrosis. J Clin Invest, 1995. 96(4): p. 2051-64.
3.            van Heeckeren, A.M., et al., Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice. Am J Physiol Lung Cell Mol Physiol, 2004. 287(5): p. L944-52.
4.            Zhou, L., et al., Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR. Science, 1994. 266(5191): p. 1705-8.
5.            Darrah, R.J., et al., Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice. J Cyst Fibros, 2013. 12(4): p. 345-51.
6.            Hodges, C.A., et al., Generation of a conditional null allele for Cftr in mice. Genesis, 2008. 46(10): p. 546-52.
7.            Hodges, C.A., et al., Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium. Am J Physiol Gastrointest Liver Physiol, 2011. 301(3): p. G528-36.
8.            McHugh, D.R., et al., A G542X cystic fibrosis mouse model for examining nonsense mutation directed therapies. PLoS One, 2018. 13(6): p. e0199573.
9.           Gawenis, L.R., et al., A BAC Transgene Expressing Human CFTR under Control of Its Regulatory Elements Rescues Cftr Knockout Mice. Sci Rep, 2019. 9(1): p. 11828.

Below is a short description of each strain. All mice are on the C57Bl/6J inbred background. ​
  1. Cftrtm1Unc (S489X mutation)- Snouwaert et al., 1992.[1]  These mice have the S489X mutation and a neomycin cassette within exon 11.
  2. Cftrtm1Kth (F508del mutation)- Zehier et al., 1995.[2]  These mice have the F508del mutation and a neomycin cassette insert between exon 11 and 12.
  3. Cftrtm2Mrc (R117H mutation)- van Heeckeren et al., 2004. [3] These mice have the R117H  mutation in exon 4 and a neomycin cassette insert between exon 4 and 5.
  4. Cftrtm1Unc Tg(FABPCFTR)1Jaw /Cwr  (gut corrected mice with S489X mutation)- Zhou et al, 1994.[4]  These mice have the S489X mutation but also express human Cftr from the fatty acid binding protein promoter (FABP).  Since this allows for Cftr expression in the intestines, there is very little intestinal obstruction in these CF mice and have been labeled “gut-corrected”.  However human CFTR may be expressed elsewhere in the mouse.
  5. Cftrtm1KthTg(FABPCFTR)1Jaw /Cwr  (gut corrected mice with F508del mutation)- crossed Zhou mutation with Cftrtm1Kth.[4, 5]  These mice have the F508del mutation but also express human Cftr from the fatty acid binding protein promoter (FABP).  Since this allows for Cftr expression in the intestines, there is very little intestinal obstruction in these CF mice and have been labeled “gut-corrected”.  However human CFTR may be expressed elsewhere in the mouse
  6. Cftrtm1.1Cwr (CftrΔ10 Exon 11 deleted)-Hodges et al., 2008. [6] These mice are missing exon 11.  This exon is also referred to as exon 10 using legacy exon numbering.
  7. Cftrtm1Cwr (Cftrfl10Exon 11 conditional) -Hodges et al., 2008. [6] These mice have loxp sites around exon 11 and are conditional.  Thus if crossed with Cre expressing mice, exon 11 can be deleted in tissues of interest. This exon is also referred to as exon 10 using legacy exon numbering.
  8. Cftrtm2Cwr (Cftrinvfl10 Exon 11 inverted conditional) Hodges et al., 2011. [7]  These mice have loxp sites around an inverted exon 11 and are conditional.  Thus if crossed with Cre expressing mice, exon 11 can be inverted back into the functional form in tissues of interest.  This is a restoration of Cftr model. This exon is also referred to as exon 10 using legacy exon numbering.
  9. Cftrem1Cwr (F508del mutation)- These mice have the ΔF508 mutation created using endonuclease mediated gene editing. This mouse has a milder CF phenotype and similar to Cftrtm1Eur that also contains a F508del. (Manuscript in preparation)
  10. Cftrem2Cwr (T-insertion or null)- These mice have a T base pair insertion in exon 11 creating a null mutation. This mutation was created using endonuclease mediated gene editing. (Manuscript in preparation)
  11. Cftrem3Cwr (G542X)- McHugh et al., 2018 [8]These mice have the G542X mutation created using endonuclease mediated gene editing.
  12. Cftrem4Cwr (G551D)- These mice have the G551D mutation created using endonuclease mediated gene editing. (MIP)
  13. Cftrem5Cwr (W1282X)- These mice have the W1282X mutation created using endonuclease mediated gene editing. (MIP)
  14. B6-Tg(CFTR)1Lcl/Cwr- Gawenis et al., 2019. [9]These mice carry a BAC transgene with the complete human CFTR gene including 40.1 kb upstream and 25kb downstream of CFTR.  This is the wildtype version of this mouse and complements loss of mouse Cftr.
  15. B6-Tg(CFTRF508del)Cwr – These mice have the F508del mutation created using endonuclease mediated gene editing in the mice in Gawenis et al., 2019 [9] that carry a BAC transgene with the complete human CFTR gene including 40.1 kb upstream and 25kb downstream of CFTR.  This is the F508del version of this mouse. (MIP)
  16. B6-Tg(CFTRG551D)Cwr – These mice have the G551D mutation created using endonuclease mediated gene editing in the mice in Gawenis et al., 2019 [9] that carry a BAC transgene with the complete human CFTR gene including 40.1 kb upstream and 25kb downstream of CFTR.  This is the G551D version of this mouse. (MIP)
  17. B6-Tg(CFTRG542X)Cwr – These mice have the G542X mutation created using endonuclease mediated gene editing in the mice in Gawenis et al., 2019 [9] that carry a BAC transgene with the complete human CFTR gene including 40.1 kb upstream and 25kb downstream of CFTR.  This is the G542X version of this mouse. (Still characterizing for publication)
  18. B6-Tg(CFTRW1282X)Cwr – These mice have the W1282X mutation created using endonuclease mediated gene editing in the mice in Gawenis et al., 2019 [9] that carry a BAC transgene with the complete human CFTR gene including 40.1 kb upstream and 25kb downstream of CFTR.  This is the W1282X version of this mouse. (Still characterizing for publication)
  19. B6-Tg(CFTR3849+10kbC>T)Cwr– These mice have the 3849+10kb C>T mutation created using endonuclease mediated gene editing in the mice in Gawenis et al., 2019 [9] that carry a BAC transgene with the complete human CFTR gene including 40.1 kb upstream and 25kb downstream of CFTR.  This is the 3849+10kb C>T version of this mouse. (Still characterizing for publication)
  20. Cftrem6Cwr (WT human exon 11 replacement)- These mice have a wildtype human exon 11 replaced for mouse exon 11 created using endonuclease mediated gene editing. In addition, they have ~100bp of human intron 10 and ~100bp of human intron 11 surrounding human exon 11.
  21. Cftrem7Cwr (WT human exon 12 replacement)- These mice have a wildtype human exon 12 replaced for mouse exon 12 created using endonuclease mediated gene editing. In addition, they have ~100bp of human intron 11 and ~100bp of human intron 12 surrounding human exon 12.
  22. Cftrem8Cwr (human exon 12 replacement-G542X)- These mice have a human exon 12 with G542X replaced for mouse exon 12 created using endonuclease mediated gene editing. In addition, they have ~100bp of human intron 11 and ~100bp of human intron 12 surrounding human exon 12.
  23. Cftrem9Cwr (human exon 12 replacement-R553X)- These mice have a human exon 12 with R553X replaced for mouse exon 12 created using endonuclease mediated gene editing. In addition, they have ~100bp of human intron 11 and ~100bp of human intron 12 surrounding human exon 12.
  24. Cftrem10Cwr (human exon 11 replacement-F508del)- These mice have a human exon 11 with F508del  replaced for mouse exon 11 created using endonuclease mediated gene editing. In addition, they have ~100bp of human intron 10 and ~100bp of human intron 11 surrounding human exon 11.