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A mouse model for the delta F508 allele of cystic fibrosis.

B G Zeiher, … , M J Welsh, K R Thomas

B G Zeiher, … , M J Welsh, K R Thomas

Published October 1, 1995
Citation Information: J Clin Invest. 1995;96(4):2051-2064. https://doi.org/10.1172/JCI118253.

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Title and authors	Publication	Year
Increased NFAT and NFκB signalling contribute to the hyperinflammatory phenotype in response to Aspergillus fumigatus in a mouse model of cystic fibrosis Bercusson A, Williams TJ, Simmonds NJ, Alton EW, Griesenbach U, Shah A, Warris A, Armstrong-James D	PLOS Pathogens	2025
Autophagy in Disease Onset and Progression. Wang H, Li X, Zhang Q, Fu C, Jiang W, Xue J, Liu S, Meng Q, Ai L, Zhi X, Deng S, Liang W	Aging and disease	2024
Think Beyond the Room: Measuring Relative Humidity in the Home Cage and Its Impact on Reproduction in Laboratory Mice, Mus musculus Barabas AJ, Conlon RA, Hodges CA	Animals : an Open Access Journal from MDPI	2024
Loss-of-function CFTR p.G970D missense mutation might cause congenital bilateral absence of the vas deferens and be associated with impaired spermatogenesis. Hou JW, Li XL, Wang L, Dai CL, Li N, Jiang XH, Tan YQ, Tian EP, Li QT, Xu WM	Asian Journal of Andrology	2023
The Cystic Fibrosis treatment, Trikafta, affects growth, viability, and cell wall of Aspergillus fumigatus biofilms Jane Jones, Kaesi Morelli, Elisa Vesely, Charles Puerner, Chetan Pavuluri, Brandon Ross, Norman Rhijn, Michael Bromley, Robert Cramer	mBio	2023
CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity. Kramer EL, Hudock KM, Davidson CR, Clancy JP	Respiratory Research	2023
CFTR Modulators Restore Acidification of Autophago-Lysosomes and Bacterial Clearance in Cystic Fibrosis Macrophages A Badr, M Eltobgy, K Krause, K Hamilton, S Estfanous, K Daily, A Khweek, A Hegazi, M Anne, C Carafice, F Robledo-Avila, Y Saqr, X Zhang, T Bonfield, M Gavrilin, S Partida-Sanchez, S Seveau, E Cormet-Boyaka, A Amer	Frontiers in Cellular and Infection Microbiology	2022
Bone Marrow Transplantation Rescues Monocyte Recruitment Defect and Improves Cystic Fibrosis in Mice. 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	Journal of immunology (Baltimore, Md. : 1950)	2022
The human ATP-binding cassette (ABC) transporter superfamily. Dean M, Moitra K, Allikmets R	Human Mutation	2022
Investigating the Implications of CFTR Exon Skipping Using a Cftr Exon 9 Deleted Mouse Model. Martinovich KM, Kicic A, Stick SM, Johnsen RD, Fletcher S, Wilton SD	Frontiers in pharmacology	2022
Bicarbonate transport of airway surface epithelia in luminally perfused mice bronchioles. Liu L, Yamamoto A, Yamaguchi M, Taniguchi I, Nomura N, Nakakuki M, Kozawa Y, Fukuyasu T, Higuchi M, Niwa E, Tamada T, Ishiguro H	The journal of physiological sciences : JPS	2022
CFTR regulates brown adipocyte thermogenesis via the cAMP/PKA signaling pathway. Choi KM, Cho SH, Kim JH, Kim AL, Kong X, Yoon JC	Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society	2022
Rescue from Pseudomonas aeruginosa Airway Infection via Stem Cell Transplantation K Brinkert, S Hedtfeld, A Burhop, R Gastmeier, P Gad, D Wedekind, C Kloth, J Rothschuh, N Lachmann, M Hetzel, AC Jirmo, E Lopez-Rodriguez, C Brandenberger, G Hansen, A Schambach, M Ackermann, B Tümmler, A Munder	Molecular Therapy	2021
Overcoming Challenges to Make Bacteriophage Therapy Standard Clinical Treatment Practice for Cystic Fibrosis RN Ng, AS Tai, BJ Chang, SM Stick, A Kicic	Frontiers in microbiology	2021
On the Corner of Models and Cure: Gene Editing in Cystic Fibrosis M Ensinck, A Mottais, C Detry, T Leal, MS Carlon	Frontiers in pharmacology	2021
Gene Editing in Rabbits: Unique Opportunities for Translational Biomedical Research J Xu, J Zhang, D Yang, J Song, B Pallas, C Zhang, J Hu, X Peng, ND Christensen, R Han, YE Chen	Frontiers in Genetics	2021
Therapeutic Uses of Bacterial Subunit Toxins C Lingwood	Toxins	2021
Role for animal models in understanding essential fatty acid deficiency in cystic fibrosis DH Hryciw, CA Jackson, N Shrestha, D Parsons, M Donnelley, AJ McAinch	Cellular and Molecular Life Sciences	2021
Defective Glyoxalase 1 Contributes to Pathogenic Inflammation in Cystic Fibrosis M Pariano, C Costantini, I Santarelli, M Puccetti, S Giovagnoli, VN Talesa, L Romani, C Antognelli	Human vaccines	2021
Anakinra Activates Superoxide Dismutase 2 to Mitigate Inflammasome Activity M Pariano, S Pieroni, AD Luca, RG Iannitti, M Borghi, M Puccetti, S Giovagnoli, G Renga, F DOnofrio, MM Bellet, C Stincardini, MA Della-Fazia, G Servillo, FL van de Veerdonk, C Costantini, L Romani	International journal of molecular sciences	2021
Targeted Drug Delivery Technologies Potentiate the Overall Therapeutic Efficacy of an Indole Derivative in a Mouse Cystic Fibrosis Setting M Puccetti, M Pariano, G Renga, I Santarelli, F DOnofrio, MM Bellet, C Stincardini, A Bartoli, C Costantini, L Romani, M Ricci, S Giovagnoli	Cells	2021
Serum inflammatory profiles in cystic fibrosis mice with and without Bordetella pseudohinzii infection PM Litman, A Day, TJ Kelley, RJ Darrah	Scientific Reports	2021
Small Molecule CCR4 Antagonists Protect Mice from Aspergillus Infection and Allergy S Bozza, RG Iannitti, M Pariano, G Renga, C Costantini, L Romani, J Bayry	Biomolecules	2021
Impaired cholesterol metabolism in the mouse model of cystic fibrosis. A preliminary study F Amato, A Castaldo, G Castaldo, G Cernera, G Corso, E Ferrari, M Gelzo, R Monzani, VR Villella, V Raia, A Motta	PloS one	2021
New generation ENaC inhibitors detach cystic fibrosis airway mucus bundles via sodium/hydrogen exchanger inhibition M Giorgetti, N Klymiuk, A Bähr, M Hemmerling, L Jinton, R Tarran, A Malmgren, A Åstrand, GC Hansson, A Ermund	European Journal of Pharmacology	2021
Quorum sensing inhibitory effect of bergamot oil and aspidosperma extract against Chromobacterium violaceum and Pseudomonas aeruginosa SO Ahmed, HH Zedan, YM Ibrahim	Archives of Microbiology	2021
Methodological tools to study species of the genus Burkholderia VC Scoffone, G Trespidi, G Barbieri, S Irudal, A Israyilova, S Buroni	Applied Microbiology and Biotechnology	2021
Might Routine Vitamin A Monitoring in Cystic Fibrosis Patients Reduce Virus-Mediated Lung Pathology? RE Sealy, SL Surman, P Vogel, JL Hurwitz	Frontiers in immunology	2021
A most formidable arsenal: genetic technologies for building a better mouse JF Clark, CJ Dinsmore, P Soriano	Genes & development	2020
Ion Channel Signature in Healthy Pancreas and Pancreatic Ductal Adenocarcinoma J Schnipper, I Dhennin-Duthille, A Ahidouch, H Ouadid-Ahidouch	Frontiers in pharmacology	2020
IRE1α kinase–mediated unconventional protein secretion rescues misfolded CFTR and pendrin H Park, DH Shin, JR Sim, S Aum, MG Lee	Science Advances	2020
Ebselen attenuates tobramycin-induced ototoxicity in mice R Gu, RJ Longenecker, J Homan, J Kil	Journal of Cystic Fibrosis	2020
Scnn1b -Transgenic BALB/c Mice as a Model of Pseudomonas aeruginosa Infections of the Cystic Fibrosis Lung KJ Brao, BP Wille, J Lieberman, RK Ernst, ME Shirtliff, JM Harro, M Raffatellu	Infection and immunity	2020
Alleviation of depression-like behavior in a cystic fibrosis mouse model by Hdac6 depletion DA Corey, SM Rymut, TJ Kelley	Scientific Reports	2020
Short Chain Fatty Acids Effect on Chloride Channel ClC-2 as a Possible Mechanism for Lubiprostone Intestinal Action MA Catalán, F Julio-Kalajzić, MI Niemeyer, LP Cid, FV Sepúlveda	Cells	2020
Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR F Degrugillier, A Aissat, V Prulière-Escabasse, L Bizard, B Simonneau, X Decrouy, C Jiang, D Rotin, P Fanen, S Simon	International journal of molecular sciences	2020
A TLR2-Activating Fraction From Mycobacterium abscessus Rough Variant Demonstrates Vaccine and Diagnostic Potential VL Moigne, AL Roux, A Jobart-Malfait, L Blanc, K Chaoui, O Burlet-Schiltz, JL Gaillard, S Canaan, J Nigou, JL Herrmann	Frontiers in Cellular and Infection Microbiology	2020
Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia Dana C. Borcherding, Matthew Siefert, Songbai Lin, John J. Brewington, Hesham Sadek, John Clancy, Scott Plafker, Assem Ziady	Journal of Clinical Investigation	2019
HDAC6 depletion improves cystic fibrosis mouse airway responses to bacterial challenge J Rosenjack, CA Hodges, RJ Darrah, TJ Kelley	Scientific Reports	2019
Evaluation of eluforsen, a novel RNA oligonucleotide for restoration of CFTR function in in vitro and murine models of p.Phe508del cystic fibrosis W Beumer, J Swildens, T Leal, S Noel, H Anthonijsz, G van der Horst, H Kuiperij-Boersma, M Potman, C van Putten, P Biasutto, G Platenburg, H de Jonge, N Henig, T Ritsema, M Koval	PloS one	2019
Immunopathology of Airway Surface Liquid Dehydration Disease BW Lewis, S Patial, Y Saini	Journal of Immunology Research	2019
Short-term CFTR inhibition reduces islet area in C57BL/6 mice D Khan, R Kelsey, RR Maheshwari, VM Stone, A Hasib, FN Koivula, A Watson, S Harkin, N Irwin, JA Shaw, NH McClenaghan, V Venglovecz, A Ébert, M Flodström-Tullberg, MG White, C Kelly	Scientific Reports	2019
Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR SJ Bose, MJ Bijvelds, Y Wang, J Liu, Z Cai, AG Bot, HR de Jonge, DN Sheppard	American journal of physiology. Lung cellular and molecular physiology	2019
Animal Models in the Pathophysiology of Cystic Fibrosis A Semaniakou, RP Croll, V Chappe	Frontiers in pharmacology	2019
Characterization of two rat models of cystic fibrosis—KO and F508del CFTR—Generated by Crispr‐Cas9 E Dreano, M Bacchetta, J Simonin, L Galmiche, C Usal, L Slimani, J Sadoine, L Tesson, I Anegon, JP Concordet, A Hatton, L Vignaud, D Tondelier, I SermetGaudelus, M Chanson, CH Cottart		2019
Acetyl-CoA carboxylase inhibition regulates microtubule dynamics and intracellular transport in cystic fibrosis epithelial cells SM Rymut, B Lu, A Perez, DA Corey, K Lamb, CU Cotton, TJ Kelley	American journal of physiology. Lung cellular and molecular physiology	2019
Targeting DNAJB9, a novel ER luminal co-chaperone, to rescue ΔF508-CFTR Y Huang, K Arora, KS Mun, F Yang, CS Moon, S Yarlagadda, A Jegga, T Weaver, AP Naren	Scientific Reports	2019
Dysregulation of Circadian Rhythm Gene Expression in Cystic Fibrosis Mice E Barbato, H Mianzo, P Litman, R Darrah	Journal of circadian rhythms	2019
Alterations of skeletal muscle bioenergetics in a mouse with F508del mutation leading to a cystic fibrosis-like condition N Lai, C Kummitha, M Drumm, C Hoppel	American journal of physiology. Endocrinology and metabolism	2019
CFTR constrains the differentiation from mouse embryonic stem cells to intestine lineage cells P Li, J Singh, Y Sun, X Ma, P Yuan	Biochemical and Biophysical Research Communications	2019
CFTR Therapeutics Normalize Cerebral Perfusion Deficits in Mouse Models of Heart Failure and Subarachnoid Hemorrhage D Lidington, JC Fares, FE Uhl, DD Dinh, JT Kroetsch, M Sauvé, FA Malik, F Matthes, L Vanherle, A Adel, A Momen, H Zhang, R Aschar-Sobbi, WD Foltz, H Wan, M Sumiyoshi, RL Macdonald, M Husain, PH Backx, SP Heximer, A Meissner, SS Bolz	JACC: Basic to Translational Science	2019
AGTR2 absence or antagonism prevents cystic fibrosis pulmonary manifestations RJ Darrah, FJ Jacono, N Joshi, AL Mitchell, A Sattar, CK Campanaro, P Litman, J Frey, DE Nethery, ES Barbato, CA Hodges, H Corvol, GR Cutting, MR Knowles, LJ Strug, ML Drumm	Journal of Cystic Fibrosis	2018
Animal models of biliary injury and altered bile acid metabolism V Mariotti, M Strazzabosco, L Fabris, DF Calvisi	Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease	2018
Airway disease phenotypes in animal models of cystic fibrosis A McCarron, M Donnelley, D Parsons	Respiratory Research	2018
Structure-guided combination therapy to potently improve the function of mutant CFTRs G Veit, H Xu, E Dreano, RG Avramescu, M Bagdany, LK Beitel, A Roldan, MA Hancock, C Lay, W Li, K Morin, S Gao, PA Mak, E Ainscow, AP Orth, P McNamara, A Edelman, S Frenkiel, E Matouk, I Sermet-Gaudelus, WG Barnes, GL Lukacs	Nature Medicine	2018
Animal models for cystic fibrosis liver disease (CFLD) R Fiorotto, M Amenduni, V Mariotti, M Cadamuro, L Fabris, C Spirli, M Strazzabosco	Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease	2018
Variable Responses to CFTR Correctors in vitro: Estimating the Design Effect in Precision Medicine E Matthes, J Goepp, C Martini, J Shan, J Liao, DY Thomas, JW Hanrahan	Frontiers in pharmacology	2018
Src kinase inhibition reduces inflammatory and cytoskeletal changes in ΔF508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy: Fiorotto, Amenduni, et al R Fiorotto, M Amenduni, V Mariotti, L Fabris, C Spirli, M Strazzabosco	Hepatology	2018
Long-term dietary (n-3) polyunsaturated fatty acids show benefits to the lungs of Cftr F508del mice C Portal, V Gouyer, R Léonard, MO Husson, F Gottrand, JL Desseyn, I Puebla	PloS one	2018
A G542X cystic fibrosis mouse model for examining nonsense mutation directed therapies DR McHugh, MS Steele, DM Valerio, A Miron, RJ Mann, DF LePage, RA Conlon, CU Cotton, ML Drumm, CA Hodges, AS Lewin	PloS one	2018
Kcnn4 is a modifier gene of intestinal cystic fibrosis preventing lethality in the Cftr-F508del mouse AR Philp, TT Riquelme, P Millar-Büchner, R González, FV Sepúlveda, LP Cid, CA Flores	Scientific Reports	2018
Consuming Genistein Improves Survival Rates in the Absence of Laxative in ΔF508-CF Female Mice Ryan, N Fairbourn, C Mylavarapu, A Dbeis, T Bowman, A Chandrashekar, T Banayat, C Hodges, L Al-Nakkash	Nutrients	2018
Nasal Potential Difference to Quantify Trans-epithelial Ion Transport in Mice M Beka, T Leal	Journal of visualized experiments : JoVE	2018
A pathogenic role for cystic fibrosis transmembrane conductance regulator in celiac disease VR Villella, A Venerando, G Cozza, S Esposito, E Ferrari, R Monzani, MC Spinella, V Oikonomou, G Renga, A Tosco, F Rossin, S Guido, M Silano, E Garaci, YK Chao, C Grimm, A Luciani, L Romani, M Piacentini, V Raia, G Kroemer, L Maiuri	The EMBO Journal	2018
Use of zebrafish models to investigate rare human disease KI Adamson, E Sheridan, AJ Grierson	Journal of medical genetics	2018
Linaclotide improves gastrointestinal transit in cystic fibrosis mice by inhibiting sodium/hydrogen exchanger 3 DR McHugh, CU Cotton, FJ Moss, M Vitko, DM Valerio, TJ Kelley, S Hao, A Jafri, ML Drumm, WF Boron, RC Stern, K McBennett, CA Hodges	AJP Gastrointestinal and Liver Physiology	2018
Different immunohistochemical localization for TMEM16A and CFTR in acinar and ductal cells of rat major salivary glands and exocrine pancreas T Yokoyama, M Takemoto, M Hirakawa, T Saino	Acta Histochemica	2018
Guanylate cyclase 2C agonism corrects CFTR mutants KAVISHA ARORA, Yunjie Huang, Kyushik Mun, Sunitha Yarlagadda, Nambirajan Sundaram, Marco M. Kessler, Gerhard Hannig, Caroline B. Kurtz, Inmaculada Silos-Santiago, Michael Helmrath, Joseph Palermo, John Clancy, Kris Steinbrecher, Anjaparavanda Naren	JCI Insight	2017
Defective CFTR leads to aberrant β-catenin activation and kidney fibrosis JT Zhang, Y Wang, JJ Chen, XH Zhang, JD Dong, LL Tsang, XR Huang, Z Cai, HY Lan, XH Jiang, HC Chan	Scientific Reports	2017
Genetic Modification of the Lung Directed Toward Treatment of Human Disease D Sondhi, KM Stiles, BP De, RG Crystal	Human Gene Therapy	2017
Thymosin α1 represents a potential potent single-molecule-based therapy for cystic fibrosis L Romani, V Oikonomou, S Moretti, RG Iannitti, MC D'Adamo, VR Villella, M Pariano, L Sforna, M Borghi, MM Bellet, F Fallarino, MT Pallotta, G Servillo, E Ferrari, P Puccetti, G Kroemer, M Pessia, L Maiuri, AL Goldstein, E Garaci	Nature Medicine	2017
Growth deficits in cystic fibrosis mice begin in utero prior to IGF-1 reduction R Darrah, I Bederman, M Vitko, DM Valerio, ML Drumm, CA Hodges, JM Beekman	PloS one	2017
Improved Growth Patterns in Cystic Fibrosis Mice after Loss of Histone Deacetylase 6 SM Rymut, DA Corey, DM Valerio, BO Erokwu, CA Flask, TJ Kelley, CA Hodges	Scientific Reports	2017
Abnormal CFTR Affects Glucagon Production by Islet α Cells in Cystic Fibrosis and Polycystic Ovarian Syndrome WQ Huang, JH Guo, C Yuan, YG Cui, FY Diao, MK Yu, JY Liu, YC Ruan, HC Chan	Frontiers in physiology	2017
Biliary Disease G Hirschfield, D Adams, E Liaskou	Biliary Disease	2017
Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia S Ahmadi, Z Bozoky, MD Paola, S Xia, C Li, AP Wong, L Wellhauser, SV Molinski, W Ip, H Ouyang, J Avolio, JD Forman-Kay, F Ratjen, JA Hirota, J Rommens, J Rossant, T Gonska, TJ Moraes, CE Bear	npj Genomic Medicine	2017
Cysteamine re-establishes the clearance of Pseudomonas aeruginosa by macrophages bearing the cystic fibrosis-relevant F508del-CFTR mutation E Ferrari, R Monzani, VR Villella, S Esposito, F Saluzzo, F Rossin, M D'Eletto, A Tosco, FD Gregorio, V Izzo, MC Maiuri, G Kroemer, V Raia, L Maiuri	Cell Death and Disease	2017
Glucose-Sensitive CFTR Suppresses Glucagon Secretion by Potentiating KATP Channels in Pancreatic Islet α Cells WQ Huang, JH Guo, XH Zhang, MK Yu, YW Chung, YC Ruan, HC Chan	Endocrinology	2017
Cystic Fibrosis Mice Develop Spontaneous Chronic Bordetella Airway Infections R Darrah, T Bonfield, J J LiPuma, P Litman, C A Hodges, F Jacono, M Drumm	Journal of infectious pulmonary diseases	2017
Impact of gene editing on the study of cystic fibrosis PT Harrison, DJ Sanz, JA Hollywood	Human Genetics	2016
New use for an old drug: COX-independent anti-inflammatory effects of sulindac in models of cystic fibrosis: COX-independent effects of sulindac in CF models J Rocca, S Manin, A Hulin, A Aissat, W Verbecq-Morlot, V Prulière-Escabasse, A Wohlhuter-Haddad, R Epaud, P Fanen, A Tarze	British Journal of Pharmacology	2016
The cystic fibrosis transmembrane conductance regulator controls biliary epithelial inflammation and permeability by regulating Src tyrosine kinase activity: Fiorotto et al R Fiorotto, A Villani, A Kourtidis, R Scirpo, M Amenduni, PJ Geibel, M Cadamuro, C Spirli, PZ Anastasiadis, M Strazzabosco	Hepatology	2016
A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR A Tosco, FD Gregorio, S Esposito, DD Stefano, I Sana, E Ferrari, A Sepe, L Salvadori, P Buonpensiero, AD Pasqua, R Grassia, CA Leone, S Guido, GD Rosa, S Lusa, G Bona, G Stoll, MC Maiuri, A Mehta, G Kroemer, L Maiuri, V Raia	Cell Death and Differentiation	2016
Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences GM Lavelle, MM White, N Browne, NG McElvaney, EP Reeves	BioMed Research International	2016
Epithelial Anion Transport as Modulator of Chemokine Signaling A Schnúr, P Hegyi, S Rousseau, GL Lukacs, G Veit	Mediators of Inflammation	2016
A novel guluronate oligomer improves intestinal transit and survival in cystic fibrosis mice M Vitko, DM Valerio, PD Rye, E Onsøyen, AH Myrset, A Dessen, ML Drumm, CA Hodges	Journal of Cystic Fibrosis	2016
Early pulmonary disease manifestations in cystic fibrosis mice RJ Darrah, AL Mitchell, CK Campanaro, ES Barbato, P Litman, A Sattar, CA Hodges, ML Drumm, FJ Jacono	Journal of Cystic Fibrosis	2016
Analysis of nasal potential in murine cystic fibrosis models MF da Cunha, J Simonin, A Sassi, R Freund, A Hatton, CH Cottart, N Elganfoud, R Zoubairi, C Dragu, JP Jais, A Hinzpeter, A Edelman, I Sermet-Gaudelus	The International Journal of Biochemistry & Cell Biology	2016
Early pulmonary disease manifestations in cystic fibrosis mice RJ Darrah, AL Mitchell, CK Campanaro, ES Barbato, P Litman, A Sattar, CA Hodges, ML Drumm, FJ Jacono	Journal of Cystic Fibrosis	2016
Azithromycin Attenuates Pseudomonas-Induced Lung Inflammation by Targeting Bacterial Proteins Secreted in the Cultured Medium T Leal, G Bergamini, F Huaux, N Panin, S Noel, B Dhooghe, JB Haaf, P Mauri, S Motta, DD Silvestre, P Melotti, C Sorio	Frontiers in immunology	2016
Genetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation V Tomati, E Sondo, A Armirotti, E Caci, E Pesce, M Marini, A Gianotti, YJ Jeon, M Cilli, A Pistorio, L Mastracci, R Ravazzolo, B Scholte, Z Ronai, LJ Galietta, N Pedemonte	Scientific Reports	2015
Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity FA Malik, A Meissner, I Semenkov, S Molinski, S Pasyk, S Ahmadi, HH Bui, CE Bear, D Lidington, SS Bolz, MB Butterworth	PloS one	2015
Novel Hits in the Correction of ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein: Synthesis, Pharmacological, and ADME Evaluation of Tetrahydropyrido[4,3- d ]pyrimidines for the Potential Treatment of Cystic Fibrosis E Pesci, L Bettinetti, P Fanti, LJ Galietta, SL Rosa, L Magnoni, N Pedemonte, GL Sardone, L Maccari	Journal of Medicinal Chemistry	2015
Engineering large animal models of human disease: Engineering large animal models of human disease CB Whitelaw, TP Sheets, SG Lillico, BP Telugu	The Journal of Pathology	2015
5’-adenosine monophosphate mediated cooling treatment enhances ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) stability in vivo Y Zhang, WG OBrien, Z Zhao, CC Lee	Journal of Biomedical Science	2015
Eccrine sweat gland development and sweat secretion CY Cui, D Schlessinger	Experimental Dermatology	2015
The ΔF508 Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Is Associated With Progressive Insulin Resistance and Decreased Functional β-Cell Mass in Mice G Fontés, J Ghislain, I Benterki, B Zarrouki, D Trudel, Y Berthiaume, V Poitout	Diabetes	2015
Hyper-osmolarity and calcium chelation: Effects on cystic fibrosis mucus A Ermund, LN Meiss, JK Gustafsson, GC Hansson	European Journal of Pharmacology	2015
A fluid secretion pathway unmasked by acinar-specific Tmem16A gene ablation in the adult mouse salivary gland MA Catalán, Y Kondo, G Peña-Munzenmayer, Y Jaramillo, F Liu, S Choi, E Crandall, Z Borok, P Flodby, GE Shull, JE Melvin	Proceedings of the National Academy of Sciences	2015
Novel personalized therapies for cystic fibrosis: treating the basic defect in all patients MD Amaral	Journal of Internal Medicine	2015
Cystic fibrosis mouse model-dependent intestinal structure and gut microbiome M Bazett, L Honeyman, AN Stefanov, CE Pope, LR Hoffman, CK Haston	Mammalian Genome	2015
Cholic Acid Induces a Cftr Dependent Biliary Secretion and Liver Growth Response in Mice FA Bodewes, MJ Bijvelds, W Vries, JF Baller, AS Gouw, HR de Jonge, HJ Verkade, CM Rodriguez-Ortigosa	PloS one	2015
Localization of cystic fibrosis transmembrane conductance regulator signaling complexes in human salivary gland striated duct cells VZ Zinn, A Khatri, MI Mednieks, AR Hand	European Journal of Oral Sciences	2015
Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epithelium NA McNeer, K Anandalingam, RJ Fields, C Caputo, S Kopic, A Gupta, E Quijano, L Polikoff, Y Kong, R Bahal, JP Geibel, PM Glazer, WM Saltzman, ME Egan	Nature Communications	2015
TGF-β-induced IL-6 prevents development of acute lung injury in influenza A virus-infected F508del CFTR-heterozygous mice PS Woods, MF Tazi, NM Chesarino, AO Amer, IC Davis	American journal of physiology. Lung cellular and molecular physiology	2015
Resveratrol increases F508del-CFTR dependent salivary secretion in cystic fibrosis mice B Dhooghe, C Bouckaert, A Capron, P Wallemacq, T Leal, S Noel	Biology Open	2015
Vaccine strategies against cystic fibrosis pathogens VL Moigne, JL Gaillard, JL Herrmann	Human Vaccines & Immunotherapeutics	2015
CFTR interacts with ZO-1 to regulate tight junction assembly and epithelial differentiation through the ZONAB pathway YC Ruan, Y Wang, ND Silva, B Kim, RY Diao, E Hill, D Brown, HC Chan, S Breton	Journal of cell science	2014
Glucose-induced electrical activities and insulin secretion in pancreatic islet β-cells are modulated by CFTR JH Guo, H Chen, YC Ruan, XL Zhang, XH Zhang, KL Fok, LL Tsang, MK Yu, WQ Huang, X Sun, YW Chung, X Jiang, Y Sohma, HC Chan	Nature Communications	2014
Carbachol-induced colonic mucus formation requires transport via NKCC1, K+ channels and CFTR JK Gustafsson, SK Lindén, AH Alwan, BJ Scholte, GC Hansson, H Sjövall	Pflügers Archiv - European Journal of Physiology	2014
Role of IL-1β in Experimental Cystic Fibrosis upon P. aeruginosa Infection J Palomo, T Marchiol, J Piotet, L Fauconnier, M Robinet, F Reverchon, ML Bert, D Togbe, R Buijs-Offerman, M Stolarczyk, VF Quesniaux, BJ Scholte, B Ryffel, M Wu	PloS one	2014
Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation DD Stefano, VR Villella, S Esposito, A Tosco, A Sepe, FD Gregorio, L Salvadori, R Grassia, CA Leone, GD Rosa, MC Maiuri, M Pettoello-Mantovani, S Guido, A Bossi, A Zolin, A Venerando, LA Pinna, A Mehta, G Bona, G Kroemer, L Maiuri, V Raia	Autophagy	2014
Inter-α-Inhibitor Blocks Epithelial Sodium Channel Activation and Decreases Nasal Potential Differences in ΔF508 Mice A Lazrak, A Jurkuvenaite, EC Ness, S Zhang, BA Woodworth, MS Muhlebach, VP Stober, YP Lim, S Garantziotis, S Matalon	American journal of respiratory cell and molecular biology	2014
Marked repression of CFTR mRNA in the transgenic Cftrtm1kth mouse model S Zhang, NK Ranganath, D Skinner, DM Bedwell, JA Buckley-Lanier, EJ Sorscher, BA Woodworth	Journal of Cystic Fibrosis	2014
Animal Models in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis MJ Pollheimer, P Fickert	Clinical Reviews in Allergy & Immunology	2014
FOXO1 Content Is Reduced in Cystic Fibrosis and Increases with IGF-I Treatment A Smerieri, L Montanini, L Maiuri, S Bernasconi, M Street	International journal of molecular sciences	2014
Hypertonic saline releases the attached small intestinal cystic fibrosis mucus A Ermund, LN Meiss, BJ Scholte, GC Hansson	Clinical and Experimental Pharmacology and Physiology	2014
Microbial-induced meprin β cleavage in MUC2 mucin and a functional CFTR channel are required to release anchored small intestinal mucus André Schütte, Anna Ermund, Christoph Becker-Pauly, Malin E. V. Johansson, Ana M. Rodriguez-Pineiro, Fredrik Bäckhed, Stefan Müller, Daniel Lottaz, Judith S. Bond, Gunnar C. Hansson	Proceedings of the National Academy of Sciences	2014
Reduced Blood Pressure of CFTR-F508del Carriers Correlates with Diminished Arterial Reactivity Rather than Circulating Blood Volume in Mice VA Peotta, P Bhandary, U Ogu, KA Volk, RD Roghair, J Karhausen	PloS one	2014
Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis F Huaux, S Noel, B Dhooghe, N Panin, SL Re, D Lison, P Wallemacq, E Marbaix, BJ Scholte, P Lebecque, T Leal	PloS one	2013
Electrophysiological Evidence for the Presence of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Mouse Sperm FF Dulce, AJ José, M Pablo, J Escoffier, FV Sepúlveda, B Enrique, O Gerardo, V Pablo, D Alberto	Journal of Cellular Physiology	2013
The Cystic Fibrosis of Exocrine Pancreas M Wilschanski, I Novak	Cold Spring Harbor Perspectives in Medicine	2013
Left ventricular and aortic dysfunction in cystic fibrosis mice ZM Sellers, A Kovacs, CJ Weinheimer, PM Best	Journal of Cystic Fibrosis	2013
Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice RJ Darrah, IR Bederman, AL Mitchell, CA Hodges, CK Campanaro, ML Drumm, FJ Jacono	Journal of Cystic Fibrosis	2013
Lubiprostone Decreases Mouse Colonic Inner Mucus Layer Thickness and Alters Intestinal Microbiota MW Musch, Y Wang, EC Claud, EB Chang	Digestive Diseases and Sciences	2013
Heterozygosity for the F508del Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Anion Channel Attenuates Influenza Severity F Aeffner, B Abdulrahman, JM Hickman-Davis, PM Janssen, A Amer, DM Bedwell, EJ Sorscher, IC Davis	The Journal of Infectious Diseases	2013
Lung inflammation in cystic fibrosis: Pathogenesis and novel therapies B Dhooghe, S Noël, F Huaux, T Leal	Clinical Biochemistry	2013
Emery and Rimoin's Principles and Practice of Medical Genetics DC Wallace, MT Lott, V Procaccio	Emery and Rimoin's Principles and Practice of Medical Genetics	2013
Animal Models for the Study of Human Disease HA Lawson	Animal Models for the Study of Human Disease	2013
Loss of Cftr function exacerbates the phenotype of Na(+) hyperabsorption in murine airways A Livraghi-Butrico, EJ Kelly, KJ Wilkinson, TD Rogers, RC Gilmore, JR Harkema, SH Randell, RC Boucher, WK O'Neal, BR Grubb	American journal of physiology. Lung cellular and molecular physiology	2013
Reduced caveolin-1 promotes hyperinflammation due to abnormal heme oxygenase-1 localization in lipopolysaccharide-challenged macrophages with dysfunctional cystic fibrosis transmembrane conductance regulator PX Zhang, TS Murray, VR Villella, E Ferrari, S Esposito, A D'Souza, V Raia, L Maiuri, DS Krause, ME Egan, EM Bruscia	Journal of immunology (Baltimore, Md. : 1950)	2013
Correction of chloride transport and mislocalization of CFTR protein by vardenafil in the gastrointestinal tract of cystic fibrosis mice B Dhooghe, S Noël, C Bouzin, G Behets-Wydemans, T Leal	PloS one	2013
Ion Transport in Human Pancreatic Duct Epithelium, Capan-1 Cells, Is Regulated by Secretin, VIP, Acetylcholine, and Purinergic Receptors: J Wang, I Novak	Pancreas	2013
Ceramide in cystic fibrosis RM Ziobro, BD Henry, AB Lentsch, MJ Edwards, J Riethmüller, E Gulbins	Clinical Lipidology	2013
Correction of Chloride Transport and Mislocalization of CFTR Protein by Vardenafil in the Gastrointestinal Tract of Cystic Fibrosis Mice B Dhooghe, S Noël, C Bouzin, G Behets-Wydemans, T Leal, JL Desseyn	PloS one	2013
Loss of Cftr function exacerbates the phenotype of Na + hyperabsorption in murine airways A Livraghi-Butrico, EJ Kelly, KJ Wilkinson, TD Rogers, RC Gilmore, JR Harkema, SH Randell, RC Boucher, WK O'Neal, BR Grubb	American journal of physiology. Lung cellular and molecular physiology	2013
A Review of Animal Models of Nonneoplastic Pancreatic Diseases JR Foster	Toxicologic pathology	2013
Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype JK Gustafsson, A Ermund, D Ambort, ME Johansson, HE Nilsson, K Thorell, H Hebert, H Sjovall, GC Hansson	Journal of Experimental Medicine	2012
Ulcerative colitis patients in remission have an altered secretory capacity in the proximal colon despite macroscopically normal mucosa: Colonic anion secretion in UC patients JK Gustafsson, GC Hansson, H Sjövall	Neurogastroenterology & Motility	2012
Rescue of murine F508del CFTR activity in native intestine by low temperature and proteasome inhibitors M Wilke, A Bot, H Jorna, BJ Scholte, HR de Jonge	PloS one	2012
Structure-activity relationships of cyanoquinolines with corrector-potentiator activity in ΔF508 cystic fibrosis transmembrane conductance regulator protein JM Knapp, AB Wood, PW Phuan, MW Lodewyk, DJ Tantillo, AS Verkman, MJ Kurth	Journal of Medicinal Chemistry	2012
PDE5 Inhibitors as Potential Tools in the Treatment of Cystic Fibrosis S Noel, B Dhooghe, T Leal	Frontiers in pharmacology	2012
Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator A Luciani, VR Villella, S Esposito, M Gavina, I Russo, M Silano, S Guido, M Pettoello-Mantovani, R Carnuccio, B Scholte, AD Matteis, MC Maiuri, V Raia, A Luini, G Kroemer, L Maiuri	Autophagy	2012
Lentiviral vector gene transfer to porcine airways PL Sinn, AL Cooney, M Oakland, DE Dylla, TJ Wallen, AA Pezzulo, EH Chang, PB McCray	Molecular Therapy — Nucleic Acids	2012
Variation in MSRA Modifies Risk of Neonatal Intestinal Obstruction in Cystic Fibrosis LB Henderson, VK Doshi, SM Blackman, KM Naughton, RG Pace, J Moskovitz, MR Knowles, PR Durie, ML Drumm, GR Cutting	PLoS genetics	2012
The ΔF508-CFTR mutation inhibits wild-type CFTR processing and function when co-expressed in human airway epithelia and in mouse nasal mucosa TA Tucker, JA Fortenberry, A Zsembery, LM Schwiebert, EM Schwiebert	BMC Physiology	2012
Inherent and Benzo[a]pyrene-Induced Differential Aryl Hydrocarbon Receptor Signaling Greatly Affects Life Span, Atherosclerosis, Cardiac Gene Expression, and Body and Heart Growth in Mice JS Kerley-Hamilton, HW Trask, CJ Ridley, E DuFour, C Lesseur, CS Ringelberg, KL Moodie, SL Shipman, M Korc, J Gui, NW Shworak, CR Tomlinson	Toxicological Sciences	2012
Gene Targeting P Tvrdik, MR Capecchi	The Mouse Nervous System	2012
Principles and Practice of Clinical Research J Sun, BD Freeman, C Natanson	Principles and Practice of Clinical Research	2012
Cystic fibrosis: Insight into CFTR pathophysiology and pharmacotherapy B Lubamba, B Dhooghe, S Noel, T Leal	Clinical Biochemistry	2012
Defects in gallbladder emptying and bile Acid homeostasis in mice with cystic fibrosis transmembrane conductance regulator deficiencies D Debray, D Rainteau, V Barbu, M Rouahi, HE Mourabit, S Lerondel, C Rey, L Humbert, D Wendum, CH Cottart, P Dawson, N Chignard, C Housset	Gastroenterology	2012
The CFTR and ENaC debate: how important is ENaC in CF lung disease? JF Collawn, A Lazrak, Z Bebok, S Matalon	American journal of physiology. Lung cellular and molecular physiology	2012
Decreasing Poly(ADP-Ribose) Polymerase Activity Restores ΔF508 CFTR Trafficking SM Anjos, R Robert, D Waller, DL Zhang, H Balghi, HM Sampson, F Ciciriello, P Lesimple, GW Carlile, J Goepp, J Liao, P Ferraro, R Phillipe, F Dantzer, JW Hanrahan, DY Thomas	Frontiers in pharmacology	2012
The CFTR and ENaC debate: how important is ENaC in CF lung disease? JF Collawn, A Lazrak, Z Bebok, S Matalon	American journal of physiology. Lung cellular and molecular physiology	2012
Transgenic hCFTR expression fails to correct β-ENaC mouse lung disease BR Grubb, WK O'Neal, LE Ostrowski, SM Kreda, B Button, RC Boucher	American journal of physiology. Lung cellular and molecular physiology	2012
Altered de novo lipogenesis contributes to low adipose stores in cystic fibrosis mice I Bederman, A Perez, L Henderson, JA Freedman, J Poleman, D Guentert, N Ruhrkraut, ML Drumm	AJP Gastrointestinal and Liver Physiology	2012
A quantitative analysis of electrolyte exchange in the salivary duct K Patterson, MA Catalán, JE Melvin, DI Yule, EJ Crampin, J Sneyd	AJP Gastrointestinal and Liver Physiology	2012
Deletion of Slc26a6 alters the stoichiometry of apical Cl − /HCO 3− exchange in mouse pancreatic duct Y Song, A Yamamoto, MC Steward, SB Ko, AK Stewart, M Soleimani, BC Liu, T Kondo, CX Jin, H Ishiguro	American journal of physiology. Cell physiology	2012
Impaired CFTR-Dependent Amplification of FSH-Stimulated Estrogen Production in Cystic Fibrosis and PCOS H Chen, JH Guo, YC Lu, GL Ding, MK Yu, LL Tsang, KL Fok, XM Liu, XH Zhang, YW Chung, P Huang, H Huang, HC Chan	The Journal of clinical endocrinology and metabolism	2012
Glucose depletion in the airway surface liquid is essential for sterility of the airways AA Pezzulo, J Gutiérrez, KS Duschner, KS McConnell, PJ Taft, SE Ernst, TL Yahr, K Rahmouni, J Klesney-Tait, DA Stoltz, J Zabner	PloS one	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Cystic Fibrosis MD Amaral, K Kunzelmann	Cystic Fibrosis	2011
Listeria monocytogenes exploits cystic fibrosis transmembrane conductance regulator (CFTR) to escape the phagosome AL Radtke, KL Anderson, MJ Davis, MJ DiMagno, JA Swanson, MX O'Riordan	Proceedings of the National Academy of Sciences	2011
The distribution and expression of CFTR restricts electrogenic anion secretion to the ileum of the brushtail possum, Trichosurus vulpecula M Gill, RC Bartolo, K Demmers, N Harfoot, S Fan, G Butt	The Journal of experimental biology	2011
Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice LS Ostedgaard, DK Meyerholz, DW Vermeer, PH Karp, L Schneider, CD Sigmund, MJ Welsh	Proceedings of the National Academy of Sciences	2011
Will we ever model PSC? – “It's hard to be a PSC model!” MJ Pollheimer, M Trauner, P Fickert	Clinics and Research in Hepatology and Gastroenterology	2011
Decreased renal accumulation of aminoglycoside reflects defective receptor-mediated endocytosis in cystic fibrosis and Dent’s disease C Raggi, K Fujiwara, T Leal, F Jouret, O Devuyst, S Terryn	Pflügers Archiv - European Journal of Physiology	2011
Lipids in cystic fibrosis KA Becker, B Henry, R Ziobro, J Riethmüller, E Gulbins	Expert Review of Respiratory Medicine	2011
Rescue of ΔF508-CFTR Trafficking via a GRASP-Dependent Unconventional Secretion Pathway HY Gee, SH Noh, BL Tang, KH Kim, MG Lee	Cell	2011
Pathogenesis of primary sclerosing cholangitis MJ Pollheimer, E Halilbasic, P Fickert, M Trauner	Best practice & research. Clinical gastroenterology	2011
Calcium Modulated Chloride Pathways Contribute to Chloride Flux in Murine CF-Affected Macrophages A Shenoy, S Kopic, M Murek, C Caputo, JP Geibel, ME Egan	Pediatric Research	2011
Elevated Incidence of Dental Caries in a Mouse Model of Cystic Fibrosis MA Catalán, K Scott-Anne, MI Klein, H Koo, WH Bowen, JE Melvin, M Semple	PloS one	2011
Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium CA Hodges, BR Grady, K Mishra, CU Cotton, ML Drumm	AJP Gastrointestinal and Liver Physiology	2011
Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane conductance regulator expression in mouse lung A Lazrak, A Jurkuvenaite, L Chen, KM Keeling, JF Collawn, DM Bedwell, S Matalon	American journal of physiology. Lung cellular and molecular physiology	2011
Inhibition of acinar apoptosis occurs during acute pancreatitis in the human homologue DeltaF508 cystic fibrosis mouse MJ DiMagno, SH Lee, C Owyang, S Zhou	American journal of physiology. Gastrointestinal and liver physiology	2010
Increased plasma membrane cholesterol in cystic fibrosis cells correlates with CFTR genotype and depends on de novo cholesterol synthesis D Fang, RH West, ME Manson, J Ruddy, D Jiang, SF Previs, ND Sonawane, JD Burgess, TJ Kelley	Respiratory Research	2010
Cftr and ENaC ion channels mediate NaCl absorption in the mouse submandibular gland: Functional interplay between Cftr and ENaC MA Catalán, T Nakamoto, M Gonzalez-Begne, JM Camden, SM Wall, LL Clarke, JE Melvin	The Journal of Physiology	2010
Pyrazolylthiazole as ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator Correctors with Improved Hydrophilicity Compared to Bithiazoles L Ye, JM Knapp, P Sangwung, JC Fettinger, AS Verkman, MJ Kurth	Journal of Medicinal Chemistry	2010
The primary folding defect and rescue of ΔF508 CFTR emerge during translation of the mutant domain H Hoelen, B Kleizen, A Schmidt, J Richardson, P Charitou, PJ Thomas, I Braakman	PloS one	2010
A new role for bicarbonate secretion in cervico-uterine mucus release RW Muchekehu, PM Quinton	The Journal of Physiology	2010
Osteopenia in Cftr-deltaF508 mice J Paradis, M Wilke, CK Haston	Journal of Cystic Fibrosis	2010
Strain-dependent differences in electrogenic secretion of electrolytes across mouse colon epithelium: Colonic epithelial electrogenic electrolyte secretion CA Flores, LP Cid, FV Sepúlveda	Experimental Physiology	2010
Pathophysiological preconditions promoting mixed “black” pigment plus cholesterol gallstones in a ΔF508 mouse model of cystic fibrosis F Freudenberg, MR Leonard, SA Liu, JN Glickman, MC Carey	AJP Gastrointestinal and Liver Physiology	2010
Inhibition of acinar apoptosis occurs during acute pancreatitis in the human homologue ΔF508 cystic fibrosis mouse MJ DiMagno, SH Lee, C Owyang, S Zhou	AJP Gastrointestinal and Liver Physiology	2010
Mechanosensitive gating of CFTR WK Zhang, D Wang, Y Duan, MM Loy, HC Chan, P Huang	Nature Cell Biology	2010
Ducts isolated from the pancreas of CFTR-null mice secrete fluid P Pascua, M García, MP Fernández-Salazar, MP Hernández-Lorenzo, JJ Calvo, WH Colledge, RM Case, MC Steward, JI Román	Pflügers Archiv - European Journal of Physiology	2009
CFTR Functions as a Bicarbonate Channel in Pancreatic Duct Cells H Ishiguro, MC Steward, S Naruse, SB Ko, H Goto, RM Case, T Kondo, A Yamamoto	The Journal of General Physiology	2009
Ducts isolated from the pancreas of CFTR-null mice secrete fluid P Pascua, M García, MP Fernández-Salazar, MP Hernández-Lorenzo, JJ Calvo, WH Colledge, RM Case, MC Steward, JI san Román	Pflügers Archiv - European Journal of Physiology	2009
Airway Delivery of Low-Dose Miglustat Normalizes Nasal Potential Difference in F508del Cystic Fibrosis Mice B Lubamba, J Lebacq, P Lebecque, R Vanbever, A Leonard, P Wallemacq, T Leal	American journal of respiratory and critical care medicine	2009
Chloride channelopathies R Planells-Cases, TJ Jentsch	Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease	2009
Partial Correction of Cystic Fibrosis Defects with PLGA Nanoparticles Encapsulating Curcumin MS Cartiera, EC Ferreira, C Caputo, ME Egan, MJ Caplan, WM Saltzman	Molecular Pharmaceutics	2009
Production of CFTR-Null and CFTR-deltaF508 Heterozygous Pigs by AAV-Mediated Gene Targeting and Somatic Cell Nuclear Transfer Christopher S. Rogers, Yanhong Hao, Tatiana Rokhlina, Melissa Samuel, David A. Stoltz, Yuhong Li, Elena Petroff, Daniel W. Vermeer, Amanda C. Kabel, Ziying Yan, Lee Spate, David Wax, Clifton N. Murphy, August Rieke, Kristin Whitworth, Michael L. Linville, Scott W. Korte, John F. Engelhardt, Michal J. Welsh, Randall S. Prather	Journal of Clinical Investigation	2008
Pathophysiological basis of liver disease in cystic fibrosis employing a DeltaF508 mouse model F Freudenberg, AL Broderick, BB Yu, MR Leonard, JN Glickman, MC Carey	American journal of physiology. Gastrointestinal and liver physiology	2008
CFTR and defective endocytosis: new insights in the renal phenotype of cystic fibrosis F Jouret, O Devuyst	Pflügers Archiv - European Journal of Physiology	2008
Generation of a conditional null allele forCftrin mice CA Hodges, CU Cotton, MR Palmert, ML Drumm	genesis	2008
HCO3- transport in interlobular pancreatic ducts isolated from ΔF mice and slc26a6 null mice H ISHIGURO, A YAMAMOTO, T KONDO	Suizo	2008
Infertility in females with cystic fibrosis is multifactorial: evidence from mouse models CA Hodges, MR Palmert, ML Drumm	Endocrinology	2008
Preclinical Evidence that Sildenafil and Vardenafil Activate Chloride Transport in Cystic Fibrosis B Lubamba, H Lecourt, J Lebacq, P Lebecque, HD Jonge, P Wallemacq, T Leal	American journal of respiratory and critical care medicine	2008
Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice: Structural and functional tracheal abnormalities in CF mice E Bonvin, PL Rouzic, JF Bernaudin, CH Cottart, C Vandebrouck, A Crié, T Leal, A Clement, M Bonora	The Journal of Physiology	2008
Animal models of chronic lung infection with Pseudomonas aeruginosa : useful tools for cystic fibrosis studies I Kukavica-Ibrulj, RC Levesque	Laboratory Animals	2008
The role of the UPS in cystic fibrosis EL Turnbull, MF Rosser, DM Cyr	BMC Biochemistry	2007
Processing and function of CFTR-DeltaF508 are species-dependent LS Ostedgaard, CS Rogers, Q Dong, CO Randak, DW Vermeer, T Rokhlina, PH Karp, MJ Welsh	Proceedings of the National Academy of Sciences	2007
What have we learned from mouse models for cystic fibrosis? I Carvalho-Oliveira, BJ Scholte, D Penque	Expert Review of Molecular Diagnostics	2007
Ursodeoxycholic Acid Stimulates Cholangiocyte Fluid Secretion in Mice via CFTR-Dependent ATP Secretion R Fiorotto, C Spirlì, L Fabris, M Cadamuro, L Okolicsanyi, M Strazzabosco	Gastroenterology	2007
Cystic Fibrosis Mouse Models C Guilbault, Z Saeed, GP Downey, D Radzioch	American journal of respiratory cell and molecular biology	2007
Olfactory epithelia exhibit progressive functional and morphological defects in CF mice BR Grubb, TD Rogers, HM Kulaga, KA Burns, RL Wonsetler, RR Reed, LE Ostrowski	American journal of physiology. Cell physiology	2007
Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis S Beharry, C Ackerley, M Corey, G Kent, YM Heng, H Christensen, C Luk, RK Yantiss, IA Nasser, M Zaman, SD Freedman, PR Durie	AJP Gastrointestinal and Liver Physiology	2007
CFTR is required for the cAMP/PKA-regulated, ATP sensitivity of ROMK channels in mouse kidney Ming Lu, Qiang Leng, Marie E. Egan, Michael J. Caplan, Emile L. Boulpaep, Gerhard H. Giebisch and Steven C. Hebert	Journal of Clinical Investigation	2006
Bioelectric properties of chloride channels in human, pig, ferret, and mouse airway epithelia X Liu, M Luo, L Zhang, W Ding, Z Yan, JF Engelhardt	American journal of respiratory cell and molecular biology	2006
Pulmonary neuroendocrine cells, airway innervation, and smooth muscle are altered in Cftr null mice J Pan, C Luk, G Kent, E Cutz, H Yeger	American journal of respiratory cell and molecular biology	2006
Regulation of the ClC-2 lung epithelial chloride channel by glycosylation of SP1 N Vij, PL Zeitlin	American journal of respiratory cell and molecular biology	2006
Mouse as a Model of Growth Retardation in Cystic Fibrosis LA Rosenberg, MD Schluchter, AF Parlow, ML Drumm	Pediatric Research	2006
The cystic fibrosis transmembrane conductance regulator (Cftr) modulates the timing of puberty in mice R Jin, CA Hodges, ML Drumm, MR Palmert	Journal of medical genetics	2006
Azithromycin reduces spontaneous and induced inflammation in ΔF508 cystic fibrosis mice R Legssyer, F Huaux, J Lebacq, M Delos, E Marbaix, P Lebecque, D Lison, BJ Scholte, P Wallemacq, T Leal	Respiratory Research	2006
Distinct pattern of lung gene expression in the Cftr -KO mice developing spontaneous lung disease compared with their littermate controls C Guilbault, JP Novak, P Martin, ML Boghdady, Z Saeed, MC Guiot, TJ Hudson, D Radzioch	Physiological genomics	2006
Culture of murine nasal epithelia: model for cystic fibrosis BR Grubb, TD Rogers, PC Diggs, RC Boucher, LE Ostrowski	American journal of physiology. Lung cellular and molecular physiology	2006
ΔF508 Mutation Results in Impaired Gastric Acid Secretion SM Sidani, P Kirchhoff, T Socrates, L Stelter, E Ferreira, C Caputo, KE Roberts, RL Bell, ME Egan, JP Geibel	The Journal of biological chemistry	2006
Epithelial myosin light chain kinase-dependent barrier dysfunction mediates T cell activation-induced diarrhea in vivo. Daniel R. Clayburgh, Terrence A. Barrett, Yueming Tang, Jon B. Meddings, Linda J. Van Eldik, D. Martin Watterson, Lane L. Clarke, Randall J. Mrsny, Jerrold R. Turner	Journal of Clinical Investigation	2005
The role of epithelial P2Y2and P2Y4receptors in the regulation of intestinal chloride secretion E Ghanem, B Robaye, T Leal, J Leipziger, WV Driessche, R Beauwens, JM Boeynaems	British Journal of Pharmacology	2005
Species-specific differences in mouse and human airway epithelial biology of recombinant adeno-associated virus transduction X Liu, Z Yan, M Luo, JF Engelhardt	American journal of respiratory cell and molecular biology	2005
Base Treatment Corrects Defects Due to Misfolding of Mutant Cystic Fibrosis Transmembrane Conductance Regulator W Namkung, KH Kim, MG Lee	Gastroenterology	2005
Transfection of multiple pulmonary cell types following intravenous injection of PEI-DNA in normal and CFTR mutant mice F Dif, C Djediat, O Alegria, B Demeneix, G Levi	The Journal of Gene Medicine	2005
SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis BR Grubb, SE Gabriel, A Mengos, M Gentzsch, SH Randell, AM van Heeckeren, MR Knowles, ML Drumm, JR Riordan, RC Boucher	American journal of respiratory cell and molecular biology	2005
Viscoelastic Gel Formulations Enhance Airway Epithelial Gene Transfer with Viral Vectors PL Sinn, AJ Shah, MD Donovan, PB McCray	American journal of respiratory cell and molecular biology	2005
Effect of Macrolides on In Vivo Ion Transport across Cystic Fibrosis Nasal Epithelium PM Barker, DJ Gillie, MS Schechter, BK Rubin	American journal of respiratory and critical care medicine	2005
CFTR ΔF508 mutation has minimal effect on the gene expression profile of differentiated human airway epithelia J Zabner, TE Scheetz, HG Almabrazi, TL Casavant, J Huang, S Keshavjee, PB McCray	American journal of physiology. Lung cellular and molecular physiology	2005
Chloride conductance of CFTR facilitates basal Cl − /HCO 3− exchange in the villous epithelium of intact murine duodenum JE Simpson, LR Gawenis, NM Walker, KT Boyle, LL Clarke	AJP Gastrointestinal and Liver Physiology	2005
Commercial applications of nuclear transfer cloning: three examples EJ Forsberg	Reproduction, Fertility and Development	2005
Persistent Gene Expression in Mouse Nasal Epithelia following Feline Immunodeficiency Virus-Based Vector Gene Transfer PL Sinn, ER Burnight, MA Hickey, GW Blissard, PB McCray	Journal of virology	2005
Talniflumate Increases Survival in a Cystic Fibrosis Mouse Model of Distal Intestinal Obstructive Syndrome NM Walker, JE Simpson, RC Levitt, KT Boyle, LL Clarke	The Journal of pharmacology and experimental therapeutics	2005
A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia LL Clarke, LR Gawenis, TC Hwang, NM Walker, DB Gruis, EM Price	American journal of physiology. Cell physiology	2004
Severe osteopenia in CFTR-null mice F Dif, C Marty, C Baudoin, MC Vernejoul, G Levi	Bone	2004
Cystic fibrosis gene mutation reduces epithelial cell acidification and injury in acid-perfused mouse duodenum M Hirokawa, T Takeuchi, S Chu, Y Akiba, V Wu, PH Guth, E Engel, MH Montrose, JD Kaunitz	Gastroenterology	2004
Gene transfer to the tracheobronchial tree: implications for fetal gene therapy for cystic fibrosis SG Keswani, TM Crombleholme	Seminars in Pediatric Surgery	2004
Characteristic Multiorgan Pathology of Cystic Fibrosis in a Long-Living Cystic Fibrosis Transmembrane Regulator Knockout Murine Model PR Durie, G Kent, MJ Phillips, CA Ackerley	The American Journal of Pathology	2004
Instability of the insertional mutation in CftrTgH(neoim)Hgu cystic fibrosis mouse model N Charizopoulou, S Jansen, M Dorsch, F Stanke, JR Dorin, HJ Hedrich, B Tümmler	BMC genetics	2004
Animal models of cystic fibrosis BJ Scholte, DJ Davidson, M Wilke, HR de Jonge	Journal of Cystic Fibrosis	2004
Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice AM van Heeckeren, MD Schluchter, ML Drumm, PB Davis	American journal of physiology. Lung cellular and molecular physiology	2004
CFTR involvement in nasal potential differences in mice and pigs studied using a thiazolidinone CFTR inhibitor DB Salinas, N Pedemonte, C Muanprasat, WF Finkbeiner, DW Nielson, AS Verkman	American journal of physiology. Lung cellular and molecular physiology	2004
Additional Disruption of the ClC-2 Cl - Channel Does Not Exacerbate the Cystic Fibrosis Phenotype of Cystic Fibrosis Transmembrane Conductance Regulator Mouse Models AA Zdebik, JE Cuffe, M Bertog, C Korbmacher, TJ Jentsch	The Journal of biological chemistry	2004
No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis A Werner, ME Bongers, MJ Bijvelds, HR de Jonge, HJ Verkade	Journal of lipid research	2004
Curcumin, a Major Constituent of Turmeric, Corrects Cystic Fibrosis Defects ME Egan, M Pearson, SA Weiner, V Rajendran, D Rubin, J Glöckner-Pagel, S Canny, K Du, GL Lukacs, MJ Caplan	Science	2004
cAMP inhibition of murine intestinal Na+/H+ exchange requires CFTR-mediated cell shrinkage of villus epithelium1 1The authors thank Dr. Martin Katz, Dr. Jeff Lakritz, Cheryl Jensen, Matt Harline, and Howard Wilson for expert technical assistance LR Gawenis, CL Franklin, JE Simpson, BA Palmer, NM Walker, TM Wiggins, LL Clarke	Gastroenterology	2003
Newer Approaches to Genetic Modeling in Mice HD Xiao, S Fuchs, K Frenzel, JM Cole, KE Bernstein	The American Journal of Pathology	2003
Hypersusceptibility of cystic fibrosis mice to chronic Pseudomonas aeruginosa oropharyngeal colonization and lung infection FT Coleman, S Mueschenborn, G Meluleni, C Ray, VJ Carey, SO Vargas, CL Cannon, FM Ausubel, GB Pier	Proceedings of the National Academy of Sciences	2003
Oxidative Stress and Disease: Significance or Triviality? B Morrissey, A der Vliet, J Eiserich, C Cross	Oxidative Stress and Disease: Significance or Triviality?	2003
Regulation of Cl- secretion by 2-adrenergic receptors in mouse colonic epithelium RS Lam, EM App, D Nahirney, AJ Szkotak, MA Vieira-Coelho, M King, M Duszyk	The Journal of Physiology	2003
In vivo microdialysis for determination of nasal liquid ion composition BR Grubb, JL Chadburn, RC Boucher	American journal of physiology. Cell physiology	2002
Metabolic Liver Disease DH Perlmutter	Journal of Pediatric Gastroenterology and Nutrition	2002
Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells ME Egan, J Glöckner-Pagel, CA Ambrose, PA Cahill, L Pappoe, N Balamuth, E Cho, S Canny, CA Wagner, J Geibel, MJ Caplan	Nature Medicine	2002
CFTR with a partially deleted R domain corrects the cystic fibrosis chloride transport defect in human airway epithelia in vitro and in mouse nasal mucosa in vivo LS Ostedgaard, J Zabner, DW Vermeer, T Rokhlina, PH Karp, AA Stecenko, C Randak, MJ Welsh	Proceedings of the National Academy of Sciences	2002
Cystic fibrosis and related diseases of the pancreas S Naruse*, M Kitagawa, H Ishiguro, K Fujiki, T Hayakawa	Best Practice & Research Clinical Gastroenterology	2002
The Mousetrap: What We Can Learn When the Mouse Model Does Not Mimic the Human Disease SH Elsea, RE Lucas	ILAR journal / National Research Council, Institute of Laboratory Animal Resources	2002
Chloride channels in the kidney: lessons learned from knockout animals O Devuyst, WB Guggino	American journal of physiology. Renal physiology	2002
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Partial correction of defective Cl − secretion in cystic fibrosis epithelial cells by an analog of squalamine C Jiang, ER Lee, MB Lane, YF Xiao, DJ Harris, SH Cheng	American journal of physiology. Lung cellular and molecular physiology	2001
Partial restoration of defective chloride conductance in ΔF508 CF mice by trimethylamine oxide H Fischer, N Fukuda, P Barbry, B Illek, C Sartori, MA Matthay	American journal of physiology. Lung cellular and molecular physiology	2001
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Transgenic Cystic Fibrosis Mice Exhibit Reduced Early Clearance of Pseudomonas aeruginosa from the Respiratory Tract TH Schroeder, N Reiniger, G Meluleni, M Grout, FT Coleman, GB Pier	Journal of immunology (Baltimore, Md. : 1950)	2001
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Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections GB Pier	Proceedings of the National Academy of Sciences	2000
Airway gene transfer in mouse nasal-airways: importance of identification of epithelial type for assessment of gene transfer DW Parsons, PJ Hopkins, AJ Bourne, RC Boucher, AJ Martin	Gene Therapy	2000
Distribution of ion transport mRNAs throughout murine nose and lung LG Rochelle, DC Li, H Ye, E Lee, CR Talbot, RC Boucher	American journal of physiology. Lung cellular and molecular physiology	2000
Expression of nucleotide-regulated Cl − currents in CF and normal mouse tracheal epithelial cell lines EJ Thomas, SE Gabriel, M Makhlina, SP Hardy, MI Lethem	American journal of physiology. Cell physiology	2000
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Enhancing the efficiency of introducing precise mutations into the mouse genome by hit and run gene targeting P Dickinson, W L Kimber, F M Kilanowski, S Webb, B J Stevenson, D J Porteous, J R Dorin	Transgenic Research	2000
ΔF508 CFTR protein expression in tissues from patients with cystic fibrosis N Kälin, A Claass, M Sommer, E Puchelle, B Tümmler	Journal of Clinical Investigation	1999
Feline immunodeficiency virus vectors persistently transduce nondividing airway epithelia and correct the cystic fibrosis defect G Wang, V Slepushkin, J Zabner, S Keshavjee, JC Johnston, SL Sauter, DJ Jolly, TW Dubensky, BL Davidson, PB McCray	Journal of Clinical Investigation	1999
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Nonviral Vectors for Gene Therapy U Griesenbach, DM Geddes, EW Alton	Nonviral Vectors for Gene Therapy	1999
Efficient killing of inhaled bacteria in ΔF508 mice: role of airway surface liquid composition PB McCray, J Zabner, HP Jia, MJ Welsh, PS Thorne	American journal of physiology. Lung cellular and molecular physiology	1999
Pathophysiology of Gene-Targeted Mouse Models for Cystic Fibrosis BR Grubb, RC Boucher	Physiological reviews	1999
Cystic Fibrosis Transmembrane Conductance Regulator Regulates Luminal Cl − /HCO 3− Exchange in Mouse Submandibular and Pancreatic Ducts MG Lee, JY Choi, X Luo, E Strickland, PJ Thomas, S Muallem	The Journal of biological chemistry	1999
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A mouse model to test the in vivo efficacy of chemical chaperones C Bai, J Biwersi, AS Verkman, MA Matthay	Journal of Pharmacological and Toxicological Methods	1998
Regulation of amiloride-sensitive sodium absorption in murine airway epithelium by C-type natriuretic peptide TJ Kelley, CU Cotton, ML Drumm	American journal of physiology. Lung cellular and molecular physiology	1998
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