Publications resulting from the use of mice and services provided by the CFMRC

  1. Sun Y, Chatterjee S, Lian X, Traylor Z, Sattiraju SR, Xiao Y, Dilliard SA, Sung YC, Kim M, Lee SM, Moore S, Wang X, Zhang D, Wu S, Basak P, Wang J, Liu J, Mann RJ, LePage DF, Jiang W, Abid S, Hennig M, Martinez A, Wustman BA, Lockhart DJ, Jain R, Conlon RA, Drumm ML, Hodges CA, Siegwart DJ. In vivo editing of lung stem cells for durable gene correction in mice. Science. 2024;384(6701):1196-202. Epub 20240613. doi: 10.1126/science.adk9428. PubMed PMID: 38870301.
  2. Wei T, Sun Y, Cheng Q, Chatterjee S, Traylor Z, Johnson LT, Coquelin ML, Wang J, Torres MJ, Lian X, Wang X, Xiao Y, Hodges CA, Siegwart DJ. Lung SORT LNPs enable precise homology-directed repair mediated CRISPR/Cas genome correction in cystic fibrosis models. Nat Commun. 2023;14(1):7322. Epub 20231111. doi: 10.1038/s41467-023-42948-2. PubMed PMID: 37951948; PMCID: PMC10640563.
  3. Sarthi JB, Trumbull AM, Abazari SM, van Unen V, Chan JE, Joo NS, Jiang Y, Kuo CJ, Sellers ZM. Critical role of down-regulated in adenoma bicarbonate transporter in linaclotide stimulated intestinal bicarbonate secretion. bioRxiv. 2023. Epub 20230507. doi: 10.1101/2023.05.05.539132. PubMed PMID: 37205513; PMCID: PMC10187319.
  4. Rossi A, Bragonzi A, Medede M, De Fino I, Lippi G, Prosdocimi M, Tamanini A, Cabrini G, Dechecchi MC. β-sitosterol ameliorates inflammation and Pseudomonas aeruginosa lung infection in a mouse model. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2023;22(1):156-60. Epub 20220813. doi: 10.1016/j.jcf.2022.08.005. PubMed PMID: 35973900.
  5. Jennings S, Hu Y, Wellems D, Luo M, Scull C, Taylor CM, Nauseef WM, Wang G. Neutrophil Defect and Lung Pathogen Selection in Cystic Fibrosis. J Leukoc Biol. 2023. Epub 20230320. doi: 10.1093/jleuko/qiad033. PubMed PMID: 36976023.
  6. De M, Hisert KB, Liles WC, Manicone AM, Hemann EA, Long ME. MEK1/2 inhibition decreases pro-inflammatory responses in macrophages from people with cystic fibrosis and mitigates severity of illness in experimental murine methicillin-resistant Staphylococcus aureus infection. bioRxiv. 2023. Epub 20230122. doi: 10.1101/2023.01.22.525092. PubMed PMID: 36712028; PMCID: PMC9882267.
  7. Cigana C, Giannella R, Colavolpe A, Alcalá-Franco B, Mancini G, Colombi F, Bigogno C, Bastrup U, Bertoni G, Bragonzi A. Mutual Effects of Single and Combined CFTR Modulators and Bacterial Infection in Cystic Fibrosis. Microbiol Spectr. 2023;11(1):e0408322. Epub 20230110. doi: 10.1128/spectrum.04083-22. PubMed PMID: 36625583; PMCID: PMC9927584.
  8. Choi KM, Cho SH, Kim JH, Kim AL, Kong X, Yoon JC. CFTR regulates brown adipocyte thermogenesis via the cAMP/PKA signaling pathway. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2023;22(1):132-9. Epub 20220908. doi: 10.1016/j.jcf.2022.08.012. PubMed PMID: 36088207.
  9. Scull CE, Luo M, Jennings S, Taylor CM, Wang G. Cftr deletion in mouse epithelial and immune cells differentially influence the intestinal microbiota. Commun Biol. 2022;5(1):1130. Epub 20221026. doi: 10.1038/s42003-022-04101-5. PubMed PMID: 36289287; PMCID: PMC9605958.
  10. Rigauts C, Aizawa J, Taylor SL, Rogers GB, Govaerts M, Cos P, Ostyn L, Sims S, Vandeplassche E, Sze M, Dondelinger Y, Vereecke L, Van Acker H, Simpson JL, Burr L, Willems A, Tunney MM, Cigana C, Bragonzi A, Coenye T, Crabbé A. R othia mucilaginosa is an anti-inflammatory bacterium in the respiratory tract of patients with chronic lung disease. Eur Respir J. 2022;59(5). Epub 20220505. doi: 10.1183/13993003.01293-2021. PubMed PMID: 34588194; PMCID: PMC9068977.
  11. Piotrowski-Daspit AS, Barone C, Lin CY, Deng Y, Wu D, Binns TC, Xu E, Ricciardi AS, Putman R, Garrison A, Nguyen R, Gupta A, Fan R, Glazer PM, Saltzman WM, Egan ME. In vivo correction of cystic fibrosis mediated by PNA nanoparticles. Sci Adv. 2022;8(40):eabo0522. Epub 20221005. doi: 10.1126/sciadv.abo0522. PubMed PMID: 36197984; PMCID: PMC9534507.
  12. Öz HH, Cheng EC, Di Pietro C, Tebaldi T, Biancon G, Zeiss C, Zhang PX, Huang PH, Esquibies SS, Britto CJ, Schupp JC, Murray TS, Halene S, Krause DS, Egan ME, Bruscia EM. Recruited monocytes/macrophages drive pulmonary neutrophilic inflammation and irreversible lung tissue remodeling in cystic fibrosis. Cell Rep. 2022;41(11):111797. doi: 10.1016/j.celrep.2022.111797. PubMed PMID: 36516754; PMCID: PMC9833830.
  13. Kim J, Jozic A, Lin Y, Eygeris Y, Bloom E, Tan X, Acosta C, MacDonald KD, Welsher KD, Sahay G. Engineering Lipid Nanoparticles for Enhanced Intracellular Delivery of mRNA through Inhalation. ACS Nano. 2022;16(9):14792-806. Epub 20220829. doi: 10.1021/acsnano.2c05647. PubMed PMID: 36038136; PMCID: PMC9939008.
  14. Fan Z, Pitmon E, Wen L, Miller J, Ehinger E, Herro R, Liu W, Chen J, Mikulski Z, Conrad DJ, Marki A, Orecchioni M, Kumari P, Zhu YP, Marcovecchio PM, Hedrick CC, Hodges CA, Rathinam VA, Wang K, Ley K. Bone Marrow Transplantation Rescues Monocyte Recruitment Defect and Improves Cystic Fibrosis in Mice. Journal of immunology (Baltimore, Md : 1950). 2022;208(3):745-52. Epub 20220114. doi: 10.4049/jimmunol.1901171. PubMed PMID: 35031577; PMCID: PMC8855460.
  15. Di Pietro C, Öz HH, Zhang PX, Cheng EC, Martis V, Bonfield TL, Kelley TJ, Jubin R, Abuchowski A, Krause DS, Egan ME, Murray TS, Bruscia EM. Recruitment of monocytes primed to express heme oxygenase-1 ameliorates pathological lung inflammation in cystic fibrosis. Exp Mol Med. 2022;54(5):639-52. Epub 20220517. doi: 10.1038/s12276-022-00770-8. PubMed PMID: 35581352; PMCID: PMC9166813.
  16. Brindisi M, Barone S, Rossi A, Cassese E, Del Gaudio N, Feliz Morel Á J, Filocamo G, Alberico A, De Fino I, Gugliandolo D, Babaei M, Bove G, Croce M, Montesano C, Altucci L, Bragonzi A, Summa V. Efficacy of selective histone deacetylase 6 inhibition in mouse models of Pseudomonas aeruginosa infection: A new glimpse for reducing inflammation and infection in cystic fibrosis. Eur J Pharmacol. 2022;936:175349. Epub 20221026. doi: 10.1016/j.ejphar.2022.175349. PubMed PMID: 36309047.
  17. Boyne K, Corey DA, Zhao P, Lu B, Boron WF, Moss FJ, Kelley TJ. Carbonic anhydrase and soluble adenylate cyclase regulation of cystic fibrosis cellular phenotypes. American journal of physiology Lung cellular and molecular physiology. 2022;322(3):L333-l47. Epub 20220105. doi: 10.1152/ajplung.00022.2021. PubMed PMID: 34986321; PMCID: PMC8858677.
  18. Barbato E, Darrah R, Kelley TJ. The circadian system in cystic fibrosis mice is regulated by histone deacetylase 6. Am J Physiol Cell Physiol. 2022;323(4):C1112-C20. Epub 20220905. doi: 10.1152/ajpcell.00248.2022. PubMed PMID: 36062879; PMCID: PMC9555305.
  19. Badr A, Eltobgy M, Krause K, Hamilton K, Estfanous S, Daily KP, Abu Khweek A, Hegazi A, Anne MNK, Carafice C, Robledo-Avila F, Saqr Y, Zhang X, Bonfield TL, Gavrilin MA, Partida-Sanchez S, Seveau S, Cormet-Boyaka E, Amer AO. CFTR Modulators Restore Acidification of Autophago-Lysosomes and Bacterial Clearance in Cystic Fibrosis Macrophages. Front Cell Infect Microbiol. 2022;12:819554. Epub 20220216. doi: 10.3389/fcimb.2022.819554. PubMed PMID: 35252032; PMCID: PMC8890004.
  20. van Heeckeren AM, Sutton MT, Fletcher DR, Hodges CA, Caplan AI, Bonfield TL. Enhancing Cystic Fibrosis Immune Regulation. Front Pharmacol. 2021;12:573065. Epub 20210513. doi: 10.3389/fphar.2021.573065. PubMed PMID: 34054509; PMCID: PMC8155373.
  21. Talbi K, Cabrita I, Kraus A, Hofmann S, Skoczynski K, Kunzelmann K, Buchholz B, Schreiber R. The chloride channel CFTR is not required for cyst growth in an ADPKD mouse model. FASEB J. 2021;35(10):e21897. doi: 10.1096/fj.202100843R. PubMed PMID: 34473378.
  22. Semaniakou A, Chappe F, Anini Y, Chappe V. VIP reduction in the pancreas of F508del homozygous CF mice and early signs of Cystic Fibrosis Related Diabetes (CFRD). Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2021;20(5):881-90. Epub 20210524. doi: 10.1016/j.jcf.2021.05.006. PubMed PMID: 34034984.
  23. Semaniakou A, Brothers S, Gould G, Zahiremani M, Paton J, Chappe F, Li A, Anini Y, Croll RP, Chappe V. Disrupted local innervation results in less VIP expression in CF mice tissues. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2021;20(1):154-64. Epub 20200627. doi: 10.1016/j.jcf.2020.06.013. PubMed PMID: 32600901.
  24. Raffay TM, Bonilla-Fernandez K, Jafri A, Sopi RB, Smith LA, Cui F, O'Reilly M, Zhang R, Hodges CA, MacFarlane PM, Deutsch G, Martin RJ, Gaston B. Bronchopulmonary Dysplasia and Pulmonary Hypertension. The Role of Smooth Muscle adh5. American journal of respiratory cell and molecular biology. 2021;65(1):70-80. doi: 10.1165/rcmb.2020-0289OC. PubMed PMID: 33780653; PMCID: PMC8320118.
  25. Pophal M, Grimmett ZW, Chu C, Margevicius S, Raffay T, Ross K, Jafri A, Giddings O, Stamler JS, Gaston B, Reynolds JD. Airway Thiol-NO Adducts as Determinants of Exhaled NO. Antioxidants (Basel). 2021;10(10). Epub 20210926. doi: 10.3390/antiox10101527. PubMed PMID: 34679661; PMCID: PMC8532745.
  26. Loeven NA, Perault AI, Cotter PA, Hodges CA, Schwartzman JD, Hampton TH, Bliska JB. The Burkholderia cenocepacia Type VI Secretion System Effector TecA Is a Virulence Factor in Mouse Models of Lung Infection. mBio. 2021;12(5):e0209821. Epub 20210928. doi: 10.1128/mBio.02098-21. PubMed PMID: 34579569; PMCID: PMC8546862.
  27. Litman PM, Day A, Kelley TJ, Darrah RJ. Serum inflammatory profiles in cystic fibrosis mice with and without Bordetella pseudohinzii infection. Sci Rep. 2021;11(1):17535. Epub 20210902. doi: 10.1038/s41598-021-97033-9. PubMed PMID: 34475490; PMCID: PMC8413329.
  28. Hamilton K, Krause K, Badr A, Daily K, Estfanous S, Eltobgy M, Khweek AA, Anne MNK, Carafice C, Baetzhold D, Tonniges JR, Zhang X, Gavrilin MA, Parinandi NL, Amer AO. Defective immunometabolism pathways in cystic fibrosis macrophages. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2021;20(4):664-72. Epub 20201115. doi: 10.1016/j.jcf.2020.10.006. PubMed PMID: 33208300; PMCID: PMC8121894.
  29. Bacci G, Rossi A, Armanini F, Cangioli L, De Fino I, Segata N, Mengoni A, Bragonzi A, Bevivino A. Lung and Gut Microbiota Changes Associated with Pseudomonas aeruginosa Infection in Mouse Models of Cystic Fibrosis. Int J Mol Sci. 2021;22(22). Epub 20211110. doi: 10.3390/ijms222212169. PubMed PMID: 34830048; PMCID: PMC8625166.
  30. Ortiz-Munoz G, Yu MA, Lefrancais E, Mallavia B, Valet C, Tian JJ, Ranucci S, Wang KM, Liu Z, Kwaan N, Dawson D, Kleinhenz ME, Khasawneh FT, Haggie PM, Verkman AS, Looney MR. Cystic fibrosis transmembrane conductance regulator dysfunction in platelets drives lung hyperinflammation. J Clin Invest. 2020;130(4):2041-53. doi: 10.1172/JCI129635. PubMed PMID: 31961827; PMCID: PMC7108932.
  31. Ng HP, Jennings S, Wellems D, Sun F, Xu J, Nauseef WM, Wang G. Myeloid CFTR loss-of-function causes persistent neutrophilic inflammation in cystic fibrosis. J Leukoc Biol. 2020;108(6):1777-85. Epub 20200612. doi: 10.1002/jlb.3a0520-193rr. PubMed PMID: 32531843; PMCID: PMC7686288.
  32. Meeker SM, Mears KS, Sangwan N, Brittnacher MJ, Weiss EJ, Treuting PM, Tolley N, Pope CE, Hager KR, Vo AT, Paik J, Frevert CW, Hayden HS, Hoffman LR, Miller SI, Hajjar AM. CFTR dysregulation drives active selection of the gut microbiome. PLoS Pathog. 2020;16(1):e1008251. Epub 20200121. doi: 10.1371/journal.ppat.1008251. PubMed PMID: 31961914; PMCID: PMC6994172.
  33. McHugh DR, Cotton CU, Hodges CA. Synergy between Readthrough and Nonsense Mediated Decay Inhibition in a Murine Model of Cystic Fibrosis Nonsense Mutations. Int J Mol Sci. 2020;22(1). Epub 20201231. doi: 10.3390/ijms22010344. PubMed PMID: 33396210; PMCID: PMC7794695.
  34. Lorè NI, Sipione B, He G, Strug LJ, Atamni HJ, Dorman A, Mott R, Iraqi FA, Bragonzi A. Collaborative Cross Mice Yield Genetic Modifiers for Pseudomonas aeruginosa Infection in Human Lung Disease. mBio. 2020;11(2). Epub 20200303. doi: 10.1128/mBio.00097-20. PubMed PMID: 32127447; PMCID: PMC7064750.
  35. Kramer EL, Madala SK, Hudock KM, Davidson C, Clancy JP. Subacute TGFβ Exposure Drives Airway Hyperresponsiveness in Cystic Fibrosis Mice through the PI3K Pathway. American journal of respiratory cell and molecular biology. 2020;62(5):657-67. doi: 10.1165/rcmb.2019-0158OC. PubMed PMID: 31922900; PMCID: PMC7193791.
  36. Kaji I, Roland JT, Watanabe M, Engevik AC, Goldstein AE, Hodges CA, Goldenring JR. Lysophosphatidic Acid Increases Maturation of Brush Borders and SGLT1 Activity in MYO5B-deficient Mice, a Model of Microvillus Inclusion Disease. Gastroenterology. 2020;159(4):1390-405.e20. Epub 20200612. doi: 10.1053/j.gastro.2020.06.008. PubMed PMID: 32534933; PMCID: PMC8240502.
  37. Isopi E, Mattoscio D, Codagnone M, Mari VC, Lamolinara A, Patruno S, D'Aurora M, Cianci E, Nespoli A, Franchi S, Gatta V, Dubourdeau M, Moretti P, Di Sabatino M, Iezzi M, Romano M, Recchiuti A. Resolvin D1 Reduces Lung Infection and Inflammation Activating Resolution in Cystic Fibrosis. Front Immunol. 2020;11:581. Epub 20200428. doi: 10.3389/fimmu.2020.00581. PubMed PMID: 32528461; PMCID: PMC7247852.
  38. Hao S, Roesch EA, Perez A, Weiner RL, Henderson LC, Cummings L, Consiglio P, Pajka J, Eisenberg A, Yeh L, Cotton CU, Drumm ML. Inactivation of CFTR by CRISPR/Cas9 alters transcriptional regulation of inflammatory pathways and other networks. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2020;19(1):34-9. Epub 20190522. doi: 10.1016/j.jcf.2019.05.003. PubMed PMID: 31126900.
  39. Gaston B, Smith L, Bosch J, Seckler J, Kunze D, Kiselar J, Marozkina N, Hodges CA, Wintrobe P, McGee K, Morozkina TS, Burton ST, Lewis T, Strassmaier T, Getsy P, Bates JN, Lewis SJ. Voltage-gated potassium channel proteins and stereoselective S-nitroso-l-cysteine signaling. JCI Insight. 2020;5(18). Epub 20200917. doi: 10.1172/jci.insight.134174. PubMed PMID: 32790645; PMCID: PMC7526540.
  40. Gardner AI, Haq IJ, Simpson AJ, Becker KA, Gallagher J, Saint-Criq V, Verdon B, Mavin E, Trigg A, Gray MA, Koulman A, McDonnell MJ, Fisher AJ, Kramer EL, Clancy JP, Ward C, Schuchman EH, Gulbins E, Brodlie M. Recombinant Acid Ceramidase Reduces Inflammation and Infection in Cystic Fibrosis. Am J Respir Crit Care Med. 2020;202(8):1133-45. doi: 10.1164/rccm.202001-0180OC. PubMed PMID: 32569477; PMCID: PMC7560813.
  41. Corey DA, Rymut SM, Kelley TJ. Alleviation of depression-like behavior in a cystic fibrosis mouse model by Hdac6 depletion. Sci Rep. 2020;10(1):16278. Epub 20201001. doi: 10.1038/s41598-020-73298-4. PubMed PMID: 33004910; PMCID: PMC7530985.
  42. Bonfield TL. Preclinical Modeling for Therapeutic Development in Cystic Fibrosis. Am J Respir Crit Care Med. 2020;201(3):267-8. doi: 10.1164/rccm.201910-2056ED. PubMed PMID: 31697560; PMCID: PMC6999106.
  43. Vega G, Guequén A, Johansson MEV, Arike L, Martínez-Abad B, Nyström EEL, Scudieri P, Pedemonte N, Millar-Büchner P, Philp AR, Galietta LJ, Hansson GC, Flores CA. Normal Calcium-Activated Anion Secretion in a Mouse Selectively Lacking TMEM16A in Intestinal Epithelium. Front Physiol. 2019;10:694. Epub 20190613. doi: 10.3389/fphys.2019.00694. PubMed PMID: 31263421; PMCID: PMC6585864.
  44. Rosenjack J, Hodges CA, Darrah RJ, Kelley TJ. HDAC6 depletion improves cystic fibrosis mouse airway responses to bacterial challenge. Sci Rep. 2019;9(1):10282. Epub 20190716. doi: 10.1038/s41598-019-46555-4. PubMed PMID: 31311988; PMCID: PMC6635416.
  45. Riquelme SA, Lozano C, Moustafa AM, Liimatta K, Tomlinson KL, Britto C, Khanal S, Gill SK, Narechania A, Azcona-Gutiérrez JM, DiMango E, Saénz Y, Planet P, Prince A. CFTR-PTEN-dependent mitochondrial metabolic dysfunction promotes Pseudomonas aeruginosa airway infection. Sci Transl Med. 2019;11(499). doi: 10.1126/scitranslmed.aav4634. PubMed PMID: 31270271; PMCID: PMC6784538.
  46. Polverino F, Lu B, Quintero JR, Vargas SO, Patel AS, Owen CA, Gerard NP, Gerard C, Cernadas M. CFTR regulates B cell activation and lymphoid follicle development. Respir Res. 2019;20(1):133. Epub 20190701. doi: 10.1186/s12931-019-1103-1. PubMed PMID: 31262295; PMCID: PMC6604167.
  47. Lu B, Li L, Schneider M, Hodges CA, Cotton CU, Burgess JD, Kelley TJ. Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2019;18(2):175-81. Epub 20180622. doi: 10.1016/j.jcf.2018.06.005. PubMed PMID: 29941319; PMCID: PMC6309594.
  48. Lai N, Kummitha C, Drumm M, Hoppel C. Alterations of skeletal muscle bioenergetics in a mouse with F508del mutation leading to a cystic fibrosis-like condition. Am J Physiol Endocrinol Metab. 2019;317(2):E327-e36. Epub 20190618. doi: 10.1152/ajpendo.00064.2019. PubMed PMID: 31211618; PMCID: PMC6732463.
  49. Huang Y, Arora K, Mun KS, Yang F, Moon C, Yarlagadda S, Jegga A, Weaver T, Naren AP. Targeting DNAJB9, a novel ER luminal co-chaperone, to rescue ΔF508-CFTR. Sci Rep. 2019;9(1):9808. Epub 20190708. doi: 10.1038/s41598-019-46161-4. PubMed PMID: 31285458; PMCID: PMC6614449.
  50. Hodges CA, Conlon RA. Delivering on the promise of gene editing for cystic fibrosis. Genes Dis. 2019;6(2):97-108. Epub 2019/06/14. doi: 10.1016/j.gendis.2018.11.005. PubMed PMID: 31193992; PMCID: PMC6545485.
  51. Gawenis LR, Hodges CA, McHugh DR, Valerio DM, Miron A, Cotton CU, Liu J, Walker NM, Strubberg AM, Gillen AE, Mutolo MJ, Kotzamanis G, Bosch J, Harris A, Drumm ML, Clarke LL. A BAC Transgene Expressing Human CFTR under Control of Its Regulatory Elements Rescues Cftr Knockout Mice. Sci Rep. 2019;9(1):11828. Epub 20190814. doi: 10.1038/s41598-019-48105-4. PubMed PMID: 31413336; PMCID: PMC6694137.
  52. Einisman HJ, Gaston B, Wijers C, Smith LA, Lewis TH, Lewis SJ, Raffay TM. Tracheomalacia in bronchopulmonary dysplasia: Trachealis hyper-relaxant responses to S-nitrosoglutathione in a hyperoxic murine model. Pediatr Pulmonol. 2019;54(12):1989-96. Epub 20190904. doi: 10.1002/ppul.24513. PubMed PMID: 31486289; PMCID: PMC7329187.
  53. Darrah RJ, Jacono FJ, Joshi N, Mitchell AL, Sattar A, Campanaro CK, Litman P, Frey J, Nethery DE, Barbato ES, Hodges CA, Corvol H, Cutting GR, Knowles MR, Strug LJ, Drumm ML. AGTR2 absence or antagonism prevents cystic fibrosis pulmonary manifestations. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2019;18(1):127-34. Epub 2018/06/26. doi: 10.1016/j.jcf.2018.05.013. PubMed PMID: 29937318.
  54. Cutone A, Lepanto MS, Rosa L, Scotti MJ, Rossi A, Ranucci S, De Fino I, Bragonzi A, Valenti P, Musci G, Berlutti F. Aerosolized Bovine Lactoferrin Counteracts Infection, Inflammation and Iron Dysbalance in A Cystic Fibrosis Mouse Model of Pseudomonas aeruginosa Chronic Lung Infection. Int J Mol Sci. 2019;20(9). Epub 20190430. doi: 10.3390/ijms20092128. PubMed PMID: 31052156; PMCID: PMC6540064.
  55. Caution K, Pan A, Krause K, Badr A, Hamilton K, Vaidya A, Gosu H, Daily K, Estfanous S, Gavrilin MA, Drew ME, Cormet-Boyaka E, Chen X, Frankhouser DE, Bundschuh R, Yan P, Dakhlallah D, Amer AO. Methylomic correlates of autophagy activity in cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2019;18(4):491-500. Epub 20190206. doi: 10.1016/j.jcf.2019.01.011. PubMed PMID: 30737168; PMCID: PMC6591064.
  56. Borcherding DC, Siefert ME, Lin S, Brewington J, Sadek H, Clancy JP, Plafker SM, Ziady AG. Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia. J Clin Invest. 2019;129(8):3448-63. Epub 20190530. doi: 10.1172/jci96273. PubMed PMID: 31145101; PMCID: PMC6668689.
  57. Barbato E, Mianzo H, Litman P, Darrah R. Dysregulation of Circadian Rhythm Gene Expression in Cystic Fibrosis Mice. J Circadian Rhythms. 2019;17:2. Epub 20190418. doi: 10.5334/jcr.175. PubMed PMID: 31065288; PMCID: PMC6484366.
  58. Robledo-Avila FH, Ruiz-Rosado JD, Brockman KL, Kopp BT, Amer AO, McCoy K, Bakaletz LO, Partida-Sanchez S. Dysregulated Calcium Homeostasis in Cystic Fibrosis Neutrophils Leads to Deficient Antimicrobial Responses. Journal of immunology (Baltimore, Md : 1950). 2018;201(7):2016-27. Epub 20180817. doi: 10.4049/jimmunol.1800076. PubMed PMID: 30120123; PMCID: PMC6143431.
  59. Robinson E, MacDonald KD, Slaughter K, McKinney M, Patel S, Sun C, Sahay G. Lipid Nanoparticle-Delivered Chemically Modified mRNA Restores Chloride Secretion in Cystic Fibrosis. Mol Ther. 2018;26(8):2034-46. Epub 20180615. doi: 10.1016/j.ymthe.2018.05.014. PubMed PMID: 29910178; PMCID: PMC6094356.
  60. Philp AR, Riquelme TT, Millar-Buchner P, Gonzalez R, Sepulveda FV, Cid LP, Flores CA. Kcnn4 is a modifier gene of intestinal cystic fibrosis preventing lethality in the Cftr-F508del mouse. Sci Rep. 2018;8(1):9320. Epub 2018/06/20. doi: 10.1038/s41598-018-27465-3. PubMed PMID: 29915289; PMCID: PMC6006244.
  61. McHugh DR, Steele MS, Valerio DM, Miron A, Mann RJ, LePage DF, Conlon RA, Cotton CU, Drumm ML, Hodges CA. A G542X cystic fibrosis mouse model for examining nonsense mutation directed therapies. PLoS One. 2018;13(6):e0199573. Epub 2018/06/21. doi: 10.1371/journal.pone.0199573. PubMed PMID: 29924856; PMCID: PMC6010256.
  62. McHugh DR, Cotton CU, Moss FJ, Vitko M, Valerio DM, Kelley TJ, Hao S, Jafri A, Drumm ML, Boron WF, Stern RC, McBennett K, Hodges CA. Linaclotide improves gastrointestinal transit in cystic fibrosis mice by inhibiting sodium/hydrogen exchanger 3. Am J Physiol Gastrointest Liver Physiol. 2018;315(5):G868-g78. Epub 20180817. doi: 10.1152/ajpgi.00261.2017. PubMed PMID: 30118317; PMCID: PMC9925117.
  63. Loré NI, Cigana C, Sipione B, Bragonzi A. The impact of host genetic background in the Pseudomonas aeruginosa respiratory infections. Mamm Genome. 2018;29(7-8):550-7. Epub 20180612. doi: 10.1007/s00335-018-9753-8. PubMed PMID: 29947963; PMCID: PMC7087806.
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