Selected Bibliography:
- Gentzsch M, Baker B, Cholon DM, Kam CW, McKinzie CJ, Despotes KA, Boyles SE, Quinney NL, Esther CR Jr, Ribeiro CMP. Cystic fibrosis airway inflammation enables elexacaftor/tezacaftor/ivacaftor-mediated rescue of N1303K CFTR mutation. ERJ Open Res. 2024 Jan 15;10(1):00746-2023. doi: 10.1183/23120541.00746-2023. PMID: 38226069; PMCID: PMC10789252.
- Cholon DM, Greenwald MA, Higgs MG, Quinney NL, Boyles SE, Meinig SL, Minges JT, Chaubal A, Tarran R, Ribeiro CMP, Wolfgang MC, Gentzsch M. A Novel Co-Culture Model Reveals Enhanced CFTR Rescue in Primary Cystic Fibrosis Airway Epithelial Cultures with Persistent Pseudomonas aeruginosa Infection. Cells. 2023 Nov 13;12(22):2618. doi: 10.3390/cells12222618. PMID: 37998353; PMCID: PMC10670530.
- Ruan J, Liang D, Yan W, Zhong Y, Talley DC, Rai G, Tao D, LeClair CA, Simeonov A, Zhang Y, Chen F, Quinney NL, Boyles SE, Cholon DM, Gentzsch M, Henderson MJ, Xue F, Fang S. A small-molecule inhibitor and degrader of the RNF5 ubiquitin ligase. Mol Biol Cell. 2022 Nov 1;33(13):ar120. doi: 10.1091/mbc.E22-06-0233. PMID: 36074076. PMCID: PMC9634977.
- Lee RE, Lewis CA, He L, Bulik-Sullivan EC, Gallant SC, Mascenik TM, Dang H, Cholon DM, Gentzsch M, Morton LC, Minges JT, Theile JW, Castle NA, Knowles MR, Kimple AJ, Randell SH. Small-molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough. J Clin Invest. 2022 Sep 15;132(18):e154571. doi: 10.1172/JCI154571. PMID: 35900863; PMCID: PMC9479597.
- Cholon DM, Gentzsch M. Established and novel human translational models to advance cystic fibrosis research, drug discovery, and optimize CFTR-targeting therapeutics. Curr Opin Pharmacol. 2022 Jun;64:102210. doi: 10.1016/j.coph.2022.102210. PMID: 35462105. PMCID: PMC9167699.
- He L, Kennedy AS, Houck S, Aleksandrov A, Quinney NL, Cyr-Scully A, Cholon DM, Gentzsch M, Randell SH, Ren HY, Cyr DM. DNAJB12 and Hsp70 Triage Arrested Intermediates of N1303K-CFTR for ER Associated-Autophagy. Mol Biol Cell. 2021 Apr 1;32(7):538-553. doi: 10.1091/mbc.E20-11-0688. PMID: 33534640. PMCID: PMC8101465.
- Gentzsch M, Cholon DM, Quinney NL, Martino MEB, Minges JT, Boyles SE, Guhr Lee TN, Esther CR Jr, Ribeiro CMP. Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies. Front Pharmacol. 2021 Mar 30;12:628722. doi: 10.3389/fphar.2021.628722. PMID: 33859562; PMCID: PMC8042279.
- Guhr Lee TN, Cholon DM, Quinney NL, Gentzsch M, Esther CR Jr. Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment. J Cyst Fibros. 2020 Sep;19(5):746-751. doi: 10.1016/j.jcf.2020.04.010. PMID: 32536510; PMCID: PMC7492410.
- McCravy MS, Quinney NL, Cholon DM, Boyles SE, Jensen TJ, Aleksandrov AA, Donaldson SH, Noone PG, Gentzsch M. Personalised medicine for non-classic cystic fibrosis resulting from rare CFTR mutations. Eur Respir J. 2020 Jul 30;56(1):2000062. doi: 10.1183/13993003.00062-2020. PMID: 32265312. PMCID: PMC7406131.
- Armirotti A, Tomati V, Matthes E, Veit G, Cholon DM, Phuan PW, Braccia C, Guidone D, Gentzsch M, Lukacs GL, Verkman AS, Galietta LJV, Hanrahan JW, Pedemonte N. Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity. Sci Rep. 2019 Jul 16;9(1):10310. doi: 10.1038/s41598-019-46639-1. PMID: 31311979. PMCID: PMC6635361.
- Gentzsch M, Cholon DM, Quinney NL, Boyles SE, Martino MEB, Ribeiro CMP. The Cystic Fibrosis Airway Milieu Enhances Rescue of F508del in a Pre-Clinical Model. Eur Respir J. 2018 Dec 20;52(6). doi: 10.1183/13993003.01133-2018. PMID: 30287473. PMCID: PMC6482470.
- Tomati V, Caci E, Ferrera L, Pesce E, Sondo E, Cholon DM, Quinney NL, Boyles SE, Armirotti A, Ravazzolo R, Galietta LJ, Gentzsch M, Pedemonte N. Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia. JCI Insight. 2018 Feb 8;3(3). doi: 10.1172/jci.insight.98699. PMID: 29415893. PMCID: PMC5821210.
- Cholon DM, Gentzsch M. Recent progress in translational cystic fibrosis research using precision medicine strategies. J Cyst Fibros. 2018 Mar;17(2S):S52-S60. doi: 10.1016/j.jcf.2017.09.005. PMID: 28986017. PMCID: PMC5828944.
- Cholon DM, Esther CR Jr, Gentzsch M. Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation. Expert Rev Precis Med Drug Dev. 2016;1(3):235-243. doi: 10.1080/23808993.2016.1175299. PMID: 27482545. PMCID: PMC4963025.
- Gentzsch M, Ren HY, Houck SA, Quinney NL, Cholon DM, Sopha P, Chaudhry IG, Das J, Dokholyan NV, Randell SH, Cyr DM. Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770. Am J Physiol Lung Cell Mol Physiol. 2016 Sep 1;311(3):L550-9. PMID: 27402691. PMCID: PMC5142211.
- Cholon DM, Quinney NL, Fulcher ML, Esther CR Jr, Das J, Dokholyan NV, Randell SH, Boucher RC, Gentzsch M. Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis. Sci Transl Med. 2014 Jul 23;6(246):246ra96. doi: 10.1126/scitranslmed.3008680. PMID: 25101886. PMCID: PMC4272825.
- Leduc I, Fusco WG, Choudhary N, Routh PA, Cholon DM, Hobbs MM, Almond GW, Orndorff PE, Elkins C. Passive immunization with a polyclonal antiserum to the hemoglobin receptor of Haemophilus ducreyi confers protection against a homologous challenge in the experimental swine model of chancroid. Infect Immun. 2011 Aug;79(8):3168-77. doi: 10.1128/IAI.00017-11. PMID: 21646451. PMCID: PMC3147553.
- Fusco WG, Afonina G, Nepluev I, Cholon DM, Choudhary N, Routh PA, Almond GW, Orndorff PE, Staats H, Hobbs MM, Leduc I, Elkins C. Immunization with the Haemophilus ducreyi hemoglobin receptor HgbA with adjuvant monophosphoryl lipid A protects swine from a homologous but not a heterologous challenge. Infect Immun. 2010 Sep;78(9):3763-72. doi: 10.1128/IAI.00217-10. PMID: 20584974. PMCID: PMC2937461.
- Cholon DM, O’Neal WK, Randell SH, Riordan JR, Gentzsch M. Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures. Am J Physiol Lung Cell Mol Physiol. 2010 Mar;298(3):L304-14. doi: 10.1152/ajplung.00016.2009. PMID: 20008117. PMCID: PMC2838667.
- Cholon DM, Cutter D, Richardson SK, Sethi S, Murphy TF, Look DC, St Geme JW 3rd. Serial isolates of persistent Haemophilus influenzae in patients with chronic obstructive pulmonary disease express diminishing quantities of the HMW1 and HMW2 adhesins. Infect Immun. 2008 Oct;76(10):4463-8. doi: 10.1128/IAI.00499-08. PMID: 18678658. PMCID: PMC2546813.