From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations von Veit, Gudio, Avramescu, Radu G, Chiang, Annette N, Houck, Scott A, Cai, Zhiwei, Peters, Kathryn W, Hong, Jeong S, Pollard, Harvey B, Guggino, William B, Balch, William E, Skach, William R, Cutting, Garry R, Frizzell, Raymond A, Sheppard, David N, Cyr, Douglas M, Sorscher, Eric J, Brodsky, Jeffrey L, Lukacs, Gergely L

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Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography von Liu, Linbo, Chu, Kengyeh K, Houser, Grace H, Diephuis, Bradford J, Li, Yao, Wilsterman, Eric J, Shastry, Suresh, Dierksen, Gregory, Birket, Susan E, Mazur, Marina, Byan-Parker, Suzanne, Grizzle, William E, Sorscher, Eric J, Rowe, Steven M, Tearney, Guillermo J

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A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease von Sloane, Peter A, Shastry, Suresh, Wilhelm, Andrew, Courville, Clifford, Tang, Li Ping, Backer, Kyle, Levin, Elina, Raju, S Vamsee, Li, Yao, Mazur, Marina, Byan-Parker, Suzanne, Grizzle, William, Sorscher, Eric J, Dransfield, Mark T, Rowe, Steven M

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Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats von Tuggle, Katherine L, Birket, Susan E, Cui, Xiaoxia, Hong, Jeong, Warren, Joe, Reid, Lara, Chambers, Andre, Ji, Diana, Gamber, Kevin, Chu, Kengyeh K, Tearney, Guillermo, Tang, Li Ping, Fortenberry, James A, Du, Ming, Cadillac, Joan M, Bedwell, David M, Rowe, Steven M, Sorscher, Eric J, Fanucchi, Michelle V

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Mutation accumulation in H. sapiens F508del CFTR countermands dN/dS type genomic analysis von Hong, Jeong S, Tindall, Janice M, Tindall, Samuel R, Sorscher, Eric J

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A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion von Sharma, Jyoti, Du, Ming, Wong, Eric, Mutyam, Venkateshwar, Li, Yao, Chen, Jianguo, Wangen, Jamie, Thrasher, Kari, Fu, Lianwu, Peng, Ning, Tang, Liping, Liu, Kaimao, Mathew, Bini, Bostwick, Robert J., Augelli-Szafran, Corinne E., Bihler, Hermann, Liang, Feng, Mahiou, Jerome, Saltz, Josef, Rab, Andras, Hong, Jeong, Sorscher, Eric J., Mendenhall, Eric M., Coppola, Candice J., Keeling, Kim M., Green, Rachel, Mense, Martin, Suto, Mark J., Rowe, Steven M., Bedwell, David M.

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Translation velocity determines the efficacy of engineered suppressor tRNAs on pathogenic nonsense mutations von Bharti, Nikhil, Santos, Leonardo, Davyt, Marcos, Behrmann, Stine, Eichholtz, Marie, Jimenez-Sanchez, Alejandro, Hong, Jeong S., Rab, Andras, Sorscher, Eric J., Albers, Suki, Ignatova, Zoya

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Relationships between genomic dissipation and de novo SNP evolution von Plyler, Zackery E, McAtee, Christopher W, Hill, Aubrey E, Crowley, Michael R, Tindall, Janice M, Tindall, Samuel R, Joshi, Disha, Sorscher, Eric J

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Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR von Birket, Susan E., Davis, Joy M., Fernandez-Petty, Courtney M., Henderson, Alexander G., Oden, Ashley M., Tang, LiPing, Wen, Hui, Hong, Jeong, Fu, Lianwu, Chambers, Andre, Fields, Alvin, Zhao, Gojun, Tearney, Guillermo J., Sorscher, Eric J., Rowe, Steven M.

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Myofibroblast differentiation and enhanced TGF-B signaling in cystic fibrosis lung disease von Harris, William T, Kelly, David R, Zhou, Yong, Wang, Dezhi, MacEwen, Mark, Macewen, Mark, Hagood, James S, Clancy, J P, Ambalavanan, Namasivayam, Sorscher, Eric J

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Targeted correction and restored function of the CFTR gene in cystic fibrosis induced pluripotent stem cells von Crane, Ana M, Kramer, Philipp, Bui, Jacquelin H, Chung, Wook Joon, Li, Xuan Shirley, Gonzalez-Garay, Manuel L, Hawkins, Finn, Liao, Wei, Mora, Daniela, Choi, Sangbum, Wang, Jianbin, Sun, Helena C, Paschon, David E, Guschin, Dmitry Y, Gregory, Philip D, Kotton, Darrell N, Holmes, Michael C, Sorscher, Eric J, Davis, Brian R

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Robust Stimulation of W1282X-CFTR Channel Activity by a Combination of Allosteric Modulators von Wang, Wei, Hong, Jeong S, Rab, Andras, Sorscher, Eric J, Kirk, Kevin L

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Sinupret activates CFTR and TMEM16A-dependent transepithelial chloride transport and improves indicators of mucociliary clearance von Zhang, Shaoyan, Skinner, Daniel, Hicks, Stephen Bradley, Bevensee, Mark O, Sorscher, Eric J, Lazrak, Ahmed, Matalon, Sadis, McNicholas, Carmel M, Woodworth, Bradford A

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Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications von McClure, Michelle L, Barnes, Stephen, Brodsky, Jeffrey L, Sorscher, Eric J

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A medium composition containing normal resting glucose that supports differentiation of primary human airway cells von Morgan, Rachel, Manfredi, Candela, Easley, Kristen F., Watkins, Lionel D., Hunt, William R., Goudy, Steven L., Sorscher, Eric J., Koval, Michael, Molina, Samuel A.

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Reduced bone length, growth plate thickness, bone content, and IGF-I as a model for poor growth in the CFTR-deficient rat von Stalvey, Michael S, Havasi, Viktoria, Tuggle, Katherine L, Wang, Dezhi, Birket, Susan, Rowe, Steve M, Sorscher, Eric J

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Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis von Balch, William E, Hutt, Darren M, Herman, David, Rodrigues, Ana P C, Noel, Sabrina, Pilewski, Joseph M, Matteson, Jeanne, Hoch, Ben, Kellner, Wendy, Kelly, Jeffery W, Schmidt, Andre, Thomas, Philip J, Matsumura, Yoshihiro, Skach, William R, Gentzsch, Martina, Riordan, John R, Sorscher, Eric J, Okiyoneda, Tsukasa, Yates, John R, Lukacs, Gergely L, Frizzell, Raymond A, Manning, Gerard, Gottesfeld, Joel M

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Assessing cell-specific effects of genetic variations using tRNA microarrays von Polte, Christine, Wedemeyer, Daniel, Oliver, Kathryn E, Wagner, Johannes, Bijvelds, Marcel J C, Mahoney, John, de Jonge, Hugo R, Sorscher, Eric J, Ignatova, Zoya

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Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect von Veit, Guido, Oliver, Kathryn, Apaja, Pirjo M, Perdomo, Doranda, Bidaud-Meynard, Aurélien, Lin, Sheng-Ting, Guo, Jingyu, Icyuz, Mert, Sorscher, Eric J, Hartman, IV, John L, Lukacs, Gergely L

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Cigarette smoke condensate inhibits transepithelial chloride transport and ciliary beat frequency von Cohen, Noam A., Zhang, Shaoyan, Sharp, Dawn B., Tamashiro, Edwin, Chen, Bei, Sorscher, Eric J., Woodworth, Bradford A.

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