Publications

Here is a list of some of our publications:

Mikami, Y., B. R. Grubb, T. D. Rogers, H. Dang, T. Asakura, P. Kota, R. C. Gilmore, K. Okuda, L. C. Morton, L. Sun, G. Chen, J. A. Wykoff, C. Ehre, J. Vilar, C. van Heusden, A. Livraghi-Butrico, M. Gentzsch, B. Button, M. J. Stutts, S. H. Randell, W. K. O’Neal and R. C. Boucher (2023). “Chronic airway epithelial hypoxia exacerbates injury in muco-obstructive lung disease through mucus hyperconcentration.” Sci Transl Med 15(699): eabo7728.

Ramalho, A. S., F. Amato and M. Gentzsch (2023). “Patient-derived cell models for personalized medicine approaches in cystic fibrosis.” J Cyst Fibros 22 Suppl 1(Suppl 1): S32-s38.

Cholon, D. M. and M. Gentzsch (2022). “Established and novel human translational models to advance cystic fibrosis research, drug discovery, and optimize CFTR-targeting therapeutics.” Curr Opin Pharmacol 64: 102210.

Ghigo, A., A. Murabito, V. Sala, A. R. Pisano, S. Bertolini, A. Gianotti, E. Caci, A. Montresor, A. Premchandar, F. Pirozzi, K. Ren, A. Della Sala, M. Mergiotti, W. Richter, E. de Poel, M. Matthey, S. Caldrer, R. A. Cardone, F. Civiletti, A. Costamagna, N. L. Quinney, C. Butnarasu, S. Visentin, M. R. Ruggiero, S. Baroni, S. G. Crich, D. Ramel, M. Laffargue, C. G. Tocchetti, R. Levi, M. Conti, X. Y. Lu, P. Melotti, C. Sorio, V. De Rose, F. Facchinetti, V. Fanelli, D. Wenzel, B. K. Fleischmann, M. A. Mall, J. Beekman, C. Laudanna, M. Gentzsch, G. L. Lukacs, N. Pedemonte and E. Hirsch (2022). “A PI3Kγ mimetic peptide triggers CFTR gating, bronchodilation, and reduced inflammation in obstructive airway diseases.” Sci Transl Med 14(638): eabl6328.

Harrison, N. L., G. W. Abbott, M. Gentzsch, A. Aleksandrov, A. Moroni, G. Thiel, S. Grant, C. G. Nichols, H. A. Lester, A. Hartel, K. Shepard, D. C. Garcia and M. Yazawa (2022). “How many SARS-CoV-2 “viroporins” are really ion channels?” Commun Biol 5(1): 859.

Lee, R. E., C. A. Lewis, L. He, E. C. Bulik-Sullivan, S. C. Gallant, T. M. Mascenik, H. Dang, D. M. Cholon, M. Gentzsch, L. C. Morton, J. T. Minges, J. W. Theile, N. A. Castle, M. R. Knowles, A. J. Kimple and S. H. Randell (2022). “Small-molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough.” J Clin Invest 132(18).

Morrison, C. B., K. M. Shaffer, K. C. Araba, M. R. Markovetz, J. A. Wykoff, N. L. Quinney, S. Hao, M. F. Delion, A. L. Flen, L. C. Morton, J. Liao, D. B. Hill, M. L. Drumm, W. K. O’Neal, M. Kesimer, M. Gentzsch and C. Ehre (2022). “Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration.” Eur Respir J 59(2).

Ribeiro, C. M. P. and M. Gentzsch (2022). “Editorial overview – 2022 respiratory issue: Cystic fibrosis pathophysiology, models, and novel therapies.” Curr Opin Pharmacol 67: 102289.

Ruan, J., D. Liang, W. Yan, Y. Zhong, D. C. Talley, G. Rai, D. Tao, C. A. LeClair, A. Simeonov, Y. Zhang, F. Chen, N. L. Quinney, S. E. Boyles, D. M. Cholon, M. Gentzsch, M. J. Henderson, F. Xue and S. Fang (2022). “A small-molecule inhibitor and degrader of the RNF5 ubiquitin ligase.” Mol Biol Cell 33(13): ar120.

Dang, Y., C. van Heusden, V. Nickerson, F. Chung, Y. Wang, N. L. Quinney, M. Gentzsch, S. H. Randell, H. M. Moulton, R. Kole, A. Ni, R. L. Juliano and S. M. Kreda (2021). “Enhanced delivery of peptide-morpholino oligonucleotides with a small molecule to correct splicing defects in the lung.” Nucleic Acids Res 49(11): 6100-6113.

Farinha, C. M. and M. Gentzsch (2021). “Revisiting CFTR Interactions: Old Partners and New Players.” Int J Mol Sci 22(24).

Gentzsch, M., D. M. Cholon, N. L. Quinney, M. E. B. Martino, J. T. Minges, S. E. Boyles, T. N. Guhr Lee, C. R. Esther, Jr. and C. M. P. Ribeiro (2021). “Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.” Front Pharmacol 12: 628722.

He, L., A. S. Kennedy, S. Houck, A. Aleksandrov, N. L. Quinney, A. Cyr-Scully, D. M. Cholon, M. Gentzsch, S. H. Randell, H. Y. Ren and D. M. Cyr (2021). “DNAJB12 and Hsp70 triage arrested intermediates of N1303K-CFTR for endoplasmic reticulum-associated autophagy.” Mol Biol Cell 32(7): 538-553.

Okuda, K., H. Dang, Y. Kobayashi, G. Carraro, S. Nakano, G. Chen, T. Kato, T. Asakura, R. C. Gilmore, L. C. Morton, R. E. Lee, T. Mascenik, W. N. Yin, S. M. Barbosa Cardenas, Y. K. O’Neal, C. E. Minnick, M. Chua, N. L. Quinney, M. Gentzsch, C. W. Anderson, A. Ghio, H. Matsui, T. Nagase, L. E. Ostrowski, B. R. Grubb, J. C. Olsen, S. H. Randell, B. R. Stripp, P. R. Tata, W. K. O’Neal and R. C. Boucher (2021). “Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia.” Am J Respir Crit Care Med 203(10): 1275-1289.

Ribeiro, C. M. P. and M. Gentzsch (2021). “Impact of Airway Inflammation on the Efficacy of CFTR Modulators.” Cells 10(11).

Xu, J., A. Livraghi-Butrico, X. Hou, C. Rajagopalan, J. Zhang, J. Song, H. Jiang, H. G. Wei, H. Wang, M. Bouhamdan, J. Ruan, D. Yang, Y. Qiu, Y. Xie, R. Barrett, S. McClellan, H. Mou, Q. Wu, X. Chen, T. D. Rogers, K. J. Wilkinson, R. C. Gilmore, C. R. Esther, Jr., K. Zaman, X. Liang, M. Sobolic, L. Hazlett, K. Zhang, R. A. Frizzell, M. Gentzsch, W. K. O’Neal, B. R. Grubb, Y. E. Chen, R. C. Boucher and F. Sun (2021). “Phenotypes of CF rabbits generated by CRISPR/Cas9-mediated disruption of the CFTR gene.” JCI Insight 6(1).

Gentzsch, M. and B. C. Rossier (2020). “A Pathophysiological Model for COVID-19: Critical Importance of Transepithelial Sodium Transport upon Airway Infection.” Function (Oxf) 1(2): zqaa024.

Guhr Lee, T. N., D. M. Cholon, N. L. Quinney, M. Gentzsch and C. R. Esther (2020). “Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment.” J Cyst Fibros 19(5): 746-751.

McCravy, M. S., N. L. Quinney, D. M. Cholon, S. E. Boyles, T. J. Jensen, A. A. Aleksandrov, S. H. Donaldson, P. G. Noone and M. Gentzsch (2020). “Personalised medicine for non-classic cystic fibrosis resulting from rare CFTR mutations.” Eur Respir J 56(1).

Armirotti, A., V. Tomati, E. Matthes, G. Veit, D. M. Cholon, P. W. Phuan, C. Braccia, D. Guidone, M. Gentzsch, G. L. Lukacs, A. S. Verkman, L. J. V. Galietta, J. W. Hanrahan and N. Pedemonte (2019). “Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity.” Sci Rep 9(1): 10310.

Clancy, J. P., C. U. Cotton, S. H. Donaldson, G. M. Solomon, D. R. VanDevanter, M. P. Boyle, M. Gentzsch, J. A. Nick, B. Illek, J. C. Wallenburg, E. J. Sorscher, M. D. Amaral, J. M. Beekman, A. P. Naren, R. J. Bridges, P. J. Thomas, G. Cutting, S. Rowe, A. G. Durmowicz, M. Mense, K. D. Boeck, W. Skach, C. Penland, E. Joseloff, H. Bihler, J. Mahoney, D. Borowitz and K. L. Tuggle (2019). “CFTR modulator theratyping: Current status, gaps and future directions.” J Cyst Fibros 18(1): 22-34.

Gentzsch, M., D. M. Cholon, N. L. Quinney, S. E. Boyles, M. E. B. Martino and C. M. P. Ribeiro (2018). “The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model.” Eur Respir J 52(6).

Tomati, V., E. Caci, L. Ferrera, E. Pesce, E. Sondo, D. M. Cholon, N. L. Quinney, S. E. Boyles, A. Armirotti, R. Ravazzolo, L. J. Galietta, M. Gentzsch and N. Pedemonte (2019). “Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.” JCI Insight 4(7).