Eric Sorscher MD
- Department of Pediatrics
Professor
- (404) 727-3293
- esorscher@emory.edu
-
Emory University
Department of Pediatrics
1760 Haygood Drive, Suite E276
Overview
Dr. Sorscher has maintained a research focus on cystic fibrosis (CF) related science and clinical intervention for more than 25 years. He has contributed to studies of cellular and molecular biology describing aminoglycoside suppression of CFTR truncation alleles, clinical significance of mild CFTR mutations and the bioelectric phenotype, acquired loss of CFTR function in vivo, and mechanisms underlying activation of CFTR by potentiator and corrector compounds. In this manner, he has participated in recent advances with regard to CF molecular pathogenesis and experimental therapeutics. Studies of other orphan conditions are also pursued by Sorscher and colleagues using lessons learned from CF. For example, a major interest involves characterization of a mechanism for tumor sensitization using the E. coli PNP gene. In this approach, tumors are rendered hundreds or thousands of times more sensitive to conventional chemotherapy by expression of a prokaryotic enzyme that cleaves nontoxic nucleoside prodrugs to a very active form. The research involves analysis of the crystal structure of E. coli PNP, and structure-based drug design of new compounds that are effectively cleaved in vivo.
Academic Appointment
- Professor, Cystic Fibrosis Center of Excellence, Department of Pediatrics, Emory University, Atlanta
Education
Degrees
- M.D. from Harvard University, Cambridge
- B.S. from Yale University, New Haven
Research
Publications
-
Nanoparticle delivery of a prodrug-activating bacterial enzyme leads to anti-tumor responses.
Nat Commun Volume: 16 Page(s): 3490
04/12/2025 Authors: Huayamares SG; Lian L; Rab R; Hou Y; Radmand A; Kim H; Zenhausern R; Achyut BR; Gilbert Ross M; Lokugamage MP -
Evaluating elexacaftor/tezacaftor/ivacaftor (ETI; Trikafta) for treatment of patients with non-cystic fibrosis bronchiectasis (NCFBE): A clinical study protocol.
PLoS One Volume: 20 Page(s): e0316721
01/01/2025 Authors: Swenson CE; Hunt WR; Manfredi C; Beltran DJ; Hong JS; Davis BR; Suzuki S; Barill C; Rab A; Chico C -
Evaluation of elexacaftor-tezacaftor-ivacaftor treatment in individuals with cystic fibrosis and CFTRN1303K in the USA: a prospective, multicentre, open-label, single-arm trial.
Lancet Respir Med Volume: 12 Page(s): 947 - 957
12/01/2024 Authors: Solomon GM; Linnemann RW; Rich R; Streby A; Buehler B; Hunter E; Vijaykumar K; Hunt WR; Brewington JJ; Rab A -
Development of robust antiviral assays using relevant apical-out human airway organoids.
bioRxiv
07/12/2024 Authors: Lee J-H; LeCher JC; Parigoris E; Shinagawa N; Sentosa J; Manfredi C; Goh SL; De R; Tao S; Zandi K -
Nucleic acid-based drugs for patients with solid tumours.
Nat Rev Clin Oncol Volume: 21 Page(s): 407 - 427
06/01/2024 Authors: Huayamares SG; Loughrey D; Kim H; Dahlman JE; Sorscher EJ -
Translation velocity determines the efficacy of engineered suppressor tRNAs on pathogenic nonsense mutations.
Nat Commun Volume: 15 Page(s): 2957
04/05/2024 Authors: Bharti N; Santos L; Davyt M; Behrmann S; Eichholtz M; Jimenez-Sanchez A; Hong JS; Rab A; Sorscher EJ; Albers S -
Mutation accumulation in H. sapiens F508del CFTR countermands dN/dS type genomic analysis.
PLoS One Volume: 19 Page(s): e0305832
01/01/2024 Authors: Hong JS; Tindall JM; Tindall SR; Sorscher EJ -
Relationships between genomic dissipation and de novo SNP evolution.
PLoS One Volume: 19 Page(s): e0303257
01/01/2024 Authors: Plyler ZE; McAtee CW; Hill AE; Crowley MR; Tindall JM; Tindall SR; Joshi D; Sorscher EJ -
Mechanisms by which the cystic fibrosis transmembrane conductance regulator may influence SARS-CoV-2 infection and COVID-19 disease severity.
FASEB J Volume: 37 Page(s): e23220
11/01/2023 Authors: Tedbury PR; Manfredi C; Degenhardt F; Conway J; Horwath MC; McCracken C; Sorscher AJ; Moreau S; Wright C; Edwards C -
A Novel 7H-[1,2,4]Triazolo[3,4-b]thiadiazine-based Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Directed toward Treatment of Cystic Fibrosis.
ACS Med Chem Lett Volume: 14 Page(s): 1338 - 1343
10/12/2023 Authors: Rab A; Yang X; Tracy WF; Hong JS; Joshi D; Manfredi C; Ponnaluri SS; Kolykhalov AA; Qui M; Fu H -
Elexacaftor/VX-445-mediated CFTR interactome remodeling reveals differential correction driven by mutation-specific translational dynamics.
J Biol Chem Volume: 299 Page(s): 105242
10/01/2023 Authors: Kim M; McDonald EF; Sabusap CMP; Timalsina B; Joshi D; Hong JS; Rab A; Sorscher EJ; Plate L -
Engineered tRNAs suppress nonsense mutations in cells and in vivo.
Nature Volume: 618 Page(s): 842 - 848
06/01/2023 Authors: Albers S; Allen EC; Bharti N; Davyt M; Joshi D; Perez-Garcia CG; Santos L; Mukthavaram R; Delgado-Toscano MA; Molina B -
High-throughput screens identify a lipid nanoparticle that preferentially delivers mRNA to human tumors in vivo.
J Control Release Volume: 357 Page(s): 394 - 403
05/01/2023 Authors: Huayamares SG; Lokugamage MP; Rab R; Da Silva Sanchez AJ; Kim H; Radmand A; Loughrey D; Lian L; Hou Y; Achyut BR -
Elexacaftor/VX-445-mediated CFTR interactome remodeling reveals differential correction driven by mutation-specific translational dynamics.
bioRxiv
02/04/2023 Authors: Kim M; McDonald EF; Sabusap CMP; Timalsina B; Joshi D; Hong JS; Rab A; Sorscher EJ; Plate L -
Evaluating antitumor activity of Escherichia coli purine nucleoside phosphorylase against head and neck patient-derived xenografts.
Cancer Rep (Hoboken) Volume: 6 Page(s): e1708
02/01/2023 Authors: Rab R; Ehrhardt A; Achyut BR; Joshi D; Gilbert-Ross M; Huang C; Floyd K; Borovjagin AV; Parker WB; Sorscher EJ -
Mutation profiling of the c.1521_1523delCTT (p.Phe508del, F508del) cystic fibrosis transmembrane conductance regulator allele using haplotype-resolved long-read next generation sequencing.
Hum Mutat Volume: 43 Page(s): 595 - 603
05/01/2022 Authors: Dilernia D; Amin P; Flores J; Stecenko A; Sorscher E -
Marine Natural Products as Leads against SARS-CoV-2 Infection.
J Nat Prod Volume: 85 Page(s): 657 - 665
03/25/2022 Authors: Chhetri BK; Tedbury PR; Sweeney-Jones AM; Mani L; Soapi K; Manfredi C; Sorscher E; Sarafianos SG; Kubanek J -
A medium composition containing normal resting glucose that supports differentiation of primary human airway cells.
Sci Rep Volume: 12 Page(s): 1540
01/27/2022 Authors: Morgan R; Manfredi C; Easley KF; Watkins LD; Hunt WR; Goudy SL; Sorscher EJ; Koval M; Molina SA -
Targeted Gene Insertion for Functional CFTR Restoration in Airway Epithelium.
Front Genome Ed Volume: 4 Page(s): 847645
01/01/2022 Authors: Barill C; Suzuki S; Rab A; Sorscher EJ; Davis BR -
Improved correction of F508del-CFTR biogenesis with a folding facilitator and an inhibitor of protein ubiquitination.
Bioorg Med Chem Lett Volume: 48 Page(s): 128243
09/15/2021 Authors: Goeckeler-Fried JL; Aldrin Denny R; Joshi D; Hill C; Larsen MB; Chiang AN; Frizzell RA; Wipf P; Sorscher EJ; Brodsky JL -
Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10kb C-to-T splicing mutation.
J Cyst Fibros Volume: 20 Page(s): 865 - 875
09/01/2021 Authors: Oren YS; Irony-Tur Sinai M; Golec A; Barchad-Avitzur O; Mutyam V; Li Y; Hong J; Ozeri-Galai E; Hatton A; Leibson C -
Association of cystic fibrosis transmembrane conductance regulator with epithelial sodium channel subunits carrying Liddle's syndrome mutations.
Am J Physiol Lung Cell Mol Physiol Volume: 321 Page(s): L308 - L320
08/01/2021 Authors: Rooj AK; Cormet-Boyaka E; Clark EB; Qadri YJ; Lee W; Boddu R; Agarwal A; Tambi R; Uddin M; Parpura V -
A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion.
Nat Commun Volume: 12 Page(s): 4358
07/16/2021 Authors: Sharma J; Du M; Wong E; Mutyam V; Li Y; Chen J; Wangen J; Thrasher K; Fu L; Peng N -
LPS decreases CFTR open probability and mucociliary transport through generation of reactive oxygen species.
Redox Biol Volume: 43 Page(s): 101998
07/01/2021 Authors: Cho DY; Zhang S; Lazrak A; Skinner D; Thompson HM; Grayson J; Guroji P; Aggarwal S; Bebok Z; Rowe SM -
Stability Prediction for Mutations in the Cytosolic Domains of Cystic Fibrosis Transmembrane Conductance Regulator.
J Chem Inf Model Volume: 61 Page(s): 1762 - 1777
04/26/2021 Authors: Bahia MS; Khazanov N; Zhou Q; Yang Z; Wang C; Hong JS; Rab A; Sorscher EJ; Brouillette CG; Hunt JF -
Positive epistasis between disease-causing missense mutations and silent polymorphism with effect on mRNA translation velocity.
Proc Natl Acad Sci U S A Volume: 118
01/26/2021 Authors: Rauscher R; Bampi GB; Guevara-Ferrer M; Santos LA; Joshi D; Mark D; Strug LJ; Rommens JM; Ballmann M; Sorscher EJ -
Derivation of Airway Basal Stem Cells from Human Pluripotent Stem Cells.
Cell Stem Cell Volume: 28 Page(s): 79 - 95.e8
01/07/2021 Authors: Hawkins FJ; Suzuki S; Beermann ML; Barill C; Wang R; Villacorta-Martin C; Berical A; Jean JC; Le Suer J; Matte T -
The CFTR P67L variant reveals a key role for N-terminal lasso helices in channel folding, maturation, and pharmacologic rescue.
J Biol Chem Volume: 296 Page(s): 100598
01/01/2021 Authors: Sabusap CM; Joshi D; Simhaev L; Oliver KE; Senderowitz H; van Willigen M; Braakman I; Rab A; Sorscher EJ; Hong JS -
Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR.
Am J Respir Crit Care Med Volume: 202 Page(s): 1271 - 1282
11/01/2020 Authors: Birket SE; Davis JM; Fernandez-Petty CM; Henderson AG; Oden AM; Tang L; Wen H; Hong J; Fu L; Chambers A -
Global assessment of the integrated stress response in CF patient-derived airway and intestinal tissues.
J Cyst Fibros Volume: 19 Page(s): 1021 - 1026
11/01/2020 Authors: Bampi GB; Rauscher R; Kirchner S; Oliver KE; Bijvelds MJC; Santos LA; Wagner J; Frizzell RA; de Jonge HR; Sorscher EJ -
CYSTIC FIBROSIS CARRIER STATUS AND PULMONARY TUBERCULOSIS IN THE TBILISI, GEORGIA REGIONS OF EUROPE
Volume: 55 Page(s): S115 - S115
10/01/2020 Authors: Hong JS; Ziegler TR; Figueroa J; Alvarez JA; Tangpricha V; Tukvadze N; Blumberg HM; McCracken C; Sorscher EJ -
POSITIVE EPISTATIC INTERACTIONS BETWEEN CF-CAUSING VARIANTS AND A SILENT POLYMORPHISM ARE ELICTED THROUGH EFFECTS ON RIBOSOME VELOCITY
Volume: 55 Page(s): S71 - S71
10/01/2020 Authors: Rauscher R; Bampi GB; Ferrer MG; Joshi D; Mark D; Strug LJ; Rommens J; Ballman M; Sorscher EJ; Oliver KE -
DEVELOPMENT AND VALIDATION OF A NOVEL PACBIO-BASED ANALYSIS FOR FULL-LENGTH SEQUENCING FROM COMPLEX CFTR ALLELES
Volume: 55 Page(s): S74 - S74
10/01/2020 Authors: Amin P; Flores J; Sorscher EJ; Stecenko A; Dilernia D -
UTILIZING YEAST PHENOMICS TO DISCOVER GENE INTERACTION NETWORKS THAT INFLUENCE BIOGENESIS OF CFTR NONSENSE ALLELES
Volume: 55 Page(s): S118 - S119
10/01/2020 Authors: Oliver KE; Mao Y; Laflin S; Linscott K; Gaines E; Ali H; Hong JS; Rab A; Sorscher EJ; Hartman JL -
DEFINING MODULATOR RESPONSE IN A SUBJECT WITH N1303K/G27V CFTR THROUGH MULTIPLE MODEL SYSTEMS
Volume: 55 Page(s): S185 - S185
10/01/2020 Authors: Hong JS; Manfredi C; Hadjiliadis D; Ostmann A; O'Shaughnessy RA; Strecker LM; Moncivaiz JD; Sorscher EJ; Brewington JJ -
APPLICATION OF IPSC-DERIVED AIRWAY EPITHELIUM FOR EVALUATING THE RESPONSE OF RARE OR UNKNOWN CFTR MUTATIONS TO MODULATORS
Volume: 55 Page(s): S193 - S193
10/01/2020 Authors: Barilla C; Suzuki S; Rab A; Goller K; Hong JS; Driggers WC; Flores J; Feldman RJ; Solomon GM; Stecenko A -
AIRWAY BASAL CELLS: MAINTAINING STEM CELL PROPERTIES DURING DERIVATION AND EXPANSION
Volume: 55 Page(s): S236 - S236
10/01/2020 Authors: Suzuki S; Barilla C; Hawkins F; Rab A; Beermann M; Matthias N; Randell S; Sorscher EJ; Kotton D; Davis BR -
IMPROVED FUNCTIONAL RESCUE OF G551D CFTR BY TRIPLE COMBINATION THERAPY
Volume: 55 Page(s): S188 - S189
10/01/2020 Authors: Rab A; Suzuki S; Disha J; Barilla C; Goller K; Manfredi C; Flores JK; Streby A; Stecenko A; Feldman RJ -
IPS-DERIVED AIRWAY CELL RESPONSE TO CFTR MODULATION: STUDY OF TRIPLE COMBINATION THERAPY IN CF PATIENTS WITHOUT AN APPROVED MODULATOR
Volume: 55 Page(s): S207 - S207
10/01/2020 Authors: Linnemann RW; Streby A; Rab A; Solomon GM; Hunter E; Hailemichael T; Driggers WC; Slaten K; Hathorne H; Hong JS -
Type I and Type III Interferons Restrict SARS-CoV-2 Infection of Human Airway Epithelial Cultures.
J Virol Volume: 94
09/15/2020 Authors: Vanderheiden A; Ralfs P; Chirkova T; Upadhyay AA; Zimmerman MG; Bedoya S; Aoued H; Tharp GM; Pellegrini KL; Manfredi C -
Highly Efficient Gene Editing of Cystic Fibrosis Patient-Derived Airway Basal Cells Results in Functional CFTR Correction.
Mol Ther Volume: 28 Page(s): 1684 - 1695
07/08/2020 Authors: Suzuki S; Crane AM; Anirudhan V; Barill C; Matthias N; Randell SH; Rab A; Sorscher EJ; Kerschner JL; Yin S -
The use of Trichomonas vaginalis purine nucleoside phosphorylase to activate fludarabine in the treatment of solid tumors.
Cancer Chemother Pharmacol Volume: 85 Page(s): 573 - 583
03/01/2020 Authors: Parker WB; Allan PW; Waud WR; Hong J; Gilbert-Ross M; Achyut BR; Joshi D; Behbahani T; Rab R; Ealick SE -
Modeling Cystic Fibrosis (CF) with Induced Pluripotent Stem Cells
Volume: 201
01/01/2020 Authors: Wang R; Agrawal P; Schlaeger T; Simone-Roach C; Martino CV; Rab A; Tang Y; Zhang Y; Suzuki S; Davis BR -
Slowing ribosome velocity restores folding and function of mutant CFTR.
J Clin Invest Volume: 129 Page(s): 5236 - 5253
12/02/2019 Authors: Oliver KE; Rauscher R; Mijnders M; Wang W; Wolpert MJ; Maya J; Sabusap CM; Kesterson RA; Kirk KL; Rab A -
An Ancient CFTR Ortholog Informs Molecular Evolution in ABC Transporters.
Dev Cell Volume: 51 Page(s): 421 - 430.e3
11/18/2019 Authors: Cui G; Hong J; Chung-Davidson Y-W; Infield D; Xu X; Li J; Simhaev L; Khazanov N; Stauffer B; Imhoff B -
Cystic fibrosis precision therapeutics: Emerging considerations.
Pediatr Pulmonol Volume: 54 Suppl 3 Page(s): S13 - S17
11/01/2019 Authors: Joshi D; Ehrhardt A; Hong JS; Sorscher EJ -
LAMPREY CFTR: AN ANCIENT CFTR ORTHOLOG INFORMS MOLECULAR EVOLUTION IN ABC TRANSPORTERS
Volume: 54 Page(s): S195 - S195
10/01/2019 Authors: Cui G; Hong JS; Chung-Davidson Y; Infield D; Xu X; Simhaev L; Khazanov N; Stauffer BB; Imhoff B; Cottrill KA -
FINDING NEMO: PROGRESS IN THE SEARCH FOR THE CRITICAL POST-TRANSLATIONAL MODIFICATIONS OF CFTR
Volume: 54 Page(s): S83 - S84
10/01/2019 Authors: Barnes S; Wilson LS; McClure M; Sorscher EJ; Aller S; Zhang S; Kim H; Bebok Z; Woodworth BA -
DIRECTED DIFFERENTIATION OF IPSC INTO AIRWAY BASAL CELLS
Volume: 54 Page(s): S211 - S212
10/01/2019 Authors: Suzuki S; Hawkins F; Barilla C; Crane A; Huang X; Beermann M; Rab A; Sorscher EJ; Kotton D; Davis BR -
GENE EDITING OF CYSTIC FIBROSIS AIRWAY BASAL CELLS
Volume: 54 Page(s): S211 - S211
10/01/2019 Authors: Suzuki S; Crane A; Anirudhan V; Matthias N; Rab A; Sorscher EJ; Kerschner JL; Yin S; Harris A; Conway A -
CFTR MRNA EXPRESSION ANALYSIS UTILIZING A REPOSITORY OF BANKED PRIMARY AIRWAY EPITHELIAL SAMPLES
Volume: 54 Page(s): S248 - S248
10/01/2019 Authors: Manfredi C; Rab A; Sorscher EJ; Hong JS -
METHYLOMIC ANALYSIS OF MAMMALIAN DNA UNDER CONDITIONS THAT RESCUE F508DEL-CFTR
Volume: 54 Page(s): S195 - S195
10/01/2019 Authors: Joshi D; Xue J; Cui X; Qin Z; Sorscher EJ -
GENE EDITING OF W1282X CFTR ONTO A NATIVE GENETIC BACKGROUND, AND IMPLICATIONS FOR IONOCYTE-DEPENDENT TRANSEPITHELIAL TRANSPORT
Volume: 54 Page(s): S216 - S216
10/01/2019 Authors: Rab A; Waller L; Gustin J; Manfredi C; Disha J; Lipman C; Hong JS; Sorscher EJ -
YEAST PHENOMIC ANALYSIS OF RPL12 EPISTASIS IN THE RESCUE OF F670DEL-YOR1 MISFOLDING
Volume: 54 Page(s): S243 - S244
10/01/2019 Authors: Santos S; Icyuz M; Sorscher EJ; Hartman JL -
PROTEOSTASIS PROFILING OF CLASS II CFTR MUTATIONS AS A MECHANISM FOR PREDICTING LUMACAFTOR THERATYPE
Volume: 54 Page(s): S199 - S199
10/01/2019 Authors: Sabusap CP; McDonald E; Hong JS; Sorscher EJ; Plate L -
PARTIAL RESCUE OF G542X-AND W1282X-CFTR IS ACHIEVED FOLLOWING SUPPRESSION OF SPECIFIC RIBOSOMAL COMPONENTS
Volume: 54 Page(s): S199 - S200
10/01/2019 Authors: Oliver KE; Ali H; Rauscher R; Bampi GB; Santos S; Rab A; Hong JS; Ignatova Z; Sorscher EJ; Hartman JL -
EPISTATIC EFFECTS OF COMPLEX ALLELES ON CYSTIC FIBROSIS PHENOTYPE - A PROTEIN TRANSLATIONAL PERSPECTIVE
Volume: 54 Page(s): S198 - S199
10/01/2019 Authors: Bampi GB; Rauscher R; Oliver KE; Ferrer MG; Joshi D; Mark D; Sutharsan S; Ballman M; Sorscher EJ; Ignatova Z -
VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.
Sci Rep Volume: 9 Page(s): 13460
09/17/2019 Authors: Cui G; Stauffer BB; Imhoff BR; Rab A; Hong JS; Sorscher EJ; McCarty NA -
Making precision medicine personal for cystic fibrosis.
Science Volume: 365 Page(s): 220 - 221
07/19/2019 Authors: Manfredi C; Tindall JM; Hong JS; Sorscher EJ -
Assessing cell-specific effects of genetic variations using tRNA microarrays.
BMC Genomics Volume: 20 Page(s): 549
07/16/2019 Authors: Polte C; Wedemeyer D; Oliver KE; Wagner J; Bijvelds MJC; Mahoney J; de Jonge HR; Sorscher EJ; Ignatova Z -
Intratumoral generation of 2-fluoroadenine to treat solid malignancies of the head and neck.
Head Neck Volume: 41 Page(s): 1979 - 1983
06/01/2019 Authors: Behbahani TE; Rosenthal EL; Parker WB; Sorscher EJ -
Non-obstructive vas deferens and epididymis loss in cystic fibrosis rats.
Mech Dev Volume: 155 Page(s): 15 - 26
02/01/2019 Authors: Plyler ZE; Birket SE; Schultz BD; Hong JS; Rowe SM; Petty CF; Crowley MR; Crossman DK; Schoeb TR; Sorscher EJ -
CFTR modulator theratyping: Current status, gaps and future directions.
J Cyst Fibros Volume: 18 Page(s): 22 - 34
01/01/2019 Authors: Clancy JP; Cotton CU; Donaldson SH; Solomon GM; VanDevanter DR; Boyle MP; Gentzsch M; Nick JA; Illek B; Wallenburg JC -
EVALUATION OF W1282X CFTR IN THE FRT MODEL AS A MEANS TO TEST SMALL MOLECULE INTERVENTIONS
Volume: 53 Page(s): 213 - 213
09/01/2018 Authors: Manfredi C; Rab A; Hong JS; Joshi D; Mahiou J; Mense M; Sorscher EJ -
STRUCTURE OF CFTR N-TERMINAL LASSO HELICES AS A GUIDE FOR EVALUATING MUTANT CFTR CHANNEL FOLDING, MATURATION, AND PHARMACOLOGIC RESCUE
Volume: 53 Page(s): 152 - 152
09/01/2018 Authors: Sabusap CP; Joshi D; Rab A; Solomon GM; Simhaev L; Sorscher EJ; Senderowitz H; Hong JS -
THERATYPING RARE AND COMMON CFTR MISSENSE VARIANTS BASED ON RESIDUAL FUNCTION AND PHENOTYPE
Volume: 53 Page(s): 262 - 263
09/01/2018 Authors: Raraigh KS; Han ST; Rab A; Matthew P; Evans TA; Davis E; McCague A; Joynt A; Lu Z; Sharma N -
RIBOSOMAL PROTEIN L12 IS A MOLECULAR TARGET SUITABLE FOR RESCUE OF CFTR DEFECTS WITH MULTIPLE DISEASE SUBCATEGORIES
Volume: 53 Page(s): 148 - 148
09/01/2018 Authors: Oliver KE; Rauscher R; Ali H; Icyuz M; Rab A; Hong JS; Hartman JL; Ignatova Z; Sorscher EJ -
INTEGRATION OF YEAST GENE INTERACTION NETWORK MODELS TO PREDICT MODIFIERS OF CFTR MOLECULAR PHENOTYPE
Volume: 53 Page(s): 207 - 208
09/01/2018 Authors: Santos S; Oliver KE; Sorscher EJ; Hartman JL -
CFTR PHOSPHORYLATION LEVEL MODULATES THE EFFICACY OF VX-770-MEDIATED POTENTIATION OF NUMEROUS HUMAN CFTR DISEASE-ASSOCIATED VARIANTS
Volume: 53 Page(s): 110 - 111
09/01/2018 Authors: Cui G; Stauffer BB; Imhoff B; Rab A; Hong JS; Sorscher EJ; McCarty NA -
SUPPRESSION OF PEROXISOME AND RIBOSOMAL CONSTITUENTS PARTIALLY RESTORES PLASMA MEMBRANE LOCALIZATION AND FUNCTION OF W1282X-CFTR
Volume: 53 Page(s): 209 - 210
09/01/2018 Authors: Ali H; Icyuz M; Kelly S; Rab A; Hong JS; Bedwell D; Hartman JL; Sorscher EJ; Oliver KE -
Residual function of cystic fibrosis mutants predicts response to small molecule CFTR modulators.
JCI Insight Volume: 3
07/26/2018 Authors: Han ST; Rab A; Pellicore MJ; Davis EF; McCague AF; Evans TA; Joynt AT; Lu Z; Cai Z; Raraigh KS -
Development of an airway mucus defect in the cystic fibrosis rat.
JCI Insight Volume: 3
01/11/2018 Authors: Birket SE; Davis JM; Fernandez CM; Tuggle KL; Oden AM; Chu KK; Tearney GJ; Fanucchi MV; Sorscher EJ; Rowe SM -
A Comparison between Two Pathophysiologically Different yet Microbiologically Similar Lung Diseases: Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Int J Respir Pulm Med Volume: 5
01/01/2018 Authors: Fenker DE; McDaniel CT; Panmanee W; Panos RJ; Sorscher EJ; Sabusap C; Clancy JP; Hassett DJ -
High-Throughput Yeast Phenomics Identifies Genetic Modifiers that Partially Rescue Human W1282X-CFTR
Volume: 197
01/01/2018 Authors: Ali H; Oliver KE; Icyuz M; Kelly S; Rab A; Hong JS; Bedwell DM; Hartman JL; Sorscher EJ -
Use of E. coli Purine Nucleoside Phosphorylase in the Treatment of Solid Tumors.
Curr Pharm Des
11/08/2017 Authors: Parker WB; Sorscher EJ -
A HUMANIZED G551D RAT MODEL TO EVALUATE CF AIRWAY DISEASE
Volume: 52 Page(s): S274 - S274
09/01/2017 Authors: Birket S; Davis JM; Oden AM; Hong JS; Tearney G; Sorscher EJ; Rowe SM -
SLOWING TRANSLATION STABILIZES CFTR TRANSMEMBRANE DOMAINS, INCREASES OPEN CHANNEL PROBABILITY AND ENHANCES FOLDING IN VIVO
Volume: 52 Page(s): S220 - S220
09/01/2017 Authors: Oliver KE; Rauscher R; Mijnders M; Wang W; Maya J; Kirk KL; Kesterson RA; Hong JS; Braakman I; Hartman JL -
THERAPY-BASED CLASSIFICATION OF RARE CFTR MUTATIONS
Volume: 52 Page(s): S330 - S330
09/01/2017 Authors: Rab A; Manfredi C; Joshi D; Sorscher EJ; Hong JS -
NON-OBSTRUCTIVE LOSS OF MALE REPRODUCTIVE TRACT IN CYSTIC FIBROSIS RATS
Volume: 52 Page(s): S280 - S280
09/01/2017 Authors: Plyler ZE; Schoeb TR; Hong JS; Rowe SM; Schultz B; Birket S; Sorscher EJ -
CFTR2 MUTATIONS IN NBD1: CORRELATION BETWEEN IN VITRO THERMAL INSTABILITY AND SEVERITY OF FOLDING DEFECT
Volume: 52 Page(s): S235 - S235
09/01/2017 Authors: Yang Z; Zhou Q; Rab A; Hong JS; Khazanov N; Senderowitz H; Sorscher EJ -
MODULATOR RESPONSIVENESS OF CFTR VARIANTS ASSOCIATED WITH REPLACEMENT AT THE SAME POSITION BY DISTINCT AMINO ACIDS: S549N AND S549R
Volume: 52 Page(s): S234 - S234
09/01/2017 Authors: Rab A; Al-Salmani M; Hong JS; Cai Z; Fass UW; Sorscher EJ; Sheppard DN -
IDENTIFICATION AND PROFILING OF SMALL MOLECULES DISPLAYING CFTR FUNCTIONAL RESCUE ACTIVITY IN FRT CELLS STABLY TRANSFECTED WITH Y122X, G542X, OR W1282X cDNA
Volume: 52 Page(s): S328 - S328
09/01/2017 Authors: Bihler H; Harrington J; Musisi I; Cheng Y; Mercadante D; Hong JS; Rab A; Sorscher EJ; Saltz J; Mahiou J -
THE P67L CFTR BIOGENESIS DEFECT AND ROLE OF N-TERMINAL LASSO HELICES THAT IMPACT CFTR FOLDING AND MATURATION
Volume: 52 Page(s): S232 - S232
09/01/2017 Authors: Sabusap CP; Rab A; Oliver KE; Hong J; Sorscher EJ -
MOLECULAR STUDIES AND DRUG DEVELOPMENT FOR THE CFTR W1282X VARIANT
Volume: 52 Page(s): S327 - S327
09/01/2017 Authors: Manfredi C; Rab A; Molina SA; Joshi D; Hong J; Ehrhardt A; Sorscher EJ -
NOVEL MODULATORS OF PROTEIN UBIQUITINATION IMPROVE VX-809-DEPENDENT RESCUE OF F508DEL-CFTR
Volume: 52 Page(s): S214 - S214
09/01/2017 Authors: Goeckeler-Fried J; Chiang A; Larsen M; Chung W; Denny R; Weissman A; Watkins SC; Sorscher EJ; Frizzell RA; Brodsky JL -
YEAST PHENOMIC MODELS OF CF-RELEVANT NONSENSE MUTATIONS REVEAL GENE MODIFIER NETWORKS PROMOTING PREMATURE TERMINATION CODON SUPPRESSION
Volume: 52 Page(s): S270 - S270
09/01/2017 Authors: Icyuz M; Kelly S; Oliver KE; Mutyam V; Rowe SM; Sorscher EJ; Bedwell DM; Hartman JL -
Transformative therapies for rare CFTR missense alleles.
Curr Opin Pharmacol Volume: 34 Page(s): 76 - 82
06/01/2017 Authors: Oliver KE; Han ST; Sorscher EJ; Cutting GR -
Reduced bone length, growth plate thickness, bone content, and IGF-I as a model for poor growth in the CFTR-deficient rat.
PLoS One Volume: 12 Page(s): e0188497
01/01/2017 Authors: Stalvey MS; Havasi V; Tuggle KL; Wang D; Birket S; Rowe SM; Sorscher EJ -
COMBINATIONS OF TRAFFICKING/FOLDING CORRECTORS AND READTHROUGH MODULATORS AS THERAPEUTIC APPROACH FOR CFTR PTC MUTATIONS
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 208 - 209
10/01/2016 Authors: Liang F; Wong E; Jordan NJ; Haibo S; Fitzpatrick LM; Hong JS; Rab A; Sorscher EJ; Mense M -
AUGMENTATION OF THE AVAILABLE POOL OF CFTR FOR SMALL MOLECULE CORRECTION BY INHIBITING THE E3 LIGASE, GP78
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 212 - 213
10/01/2016 Authors: Chung W; Fortenberry JA; Sokirniy I; Wu J; Chiang A; Goeckeler-Fried J; Sterner D; Mattern M; Brodsky JL; Sorscher EJ -
Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications.
Am J Physiol Lung Cell Mol Physiol Volume: 311 Page(s): L719 - L733
10/01/2016 Authors: McClure ML; Barnes S; Brodsky JL; Sorscher EJ -
HIGH-THROUGHPUT SCREENING FOR READTHROUGH MODULATORS OF CFTR PTC MUTATIONS Y122X AND W1282X
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 293 - 293
10/01/2016 Authors: Haibo S; Wong E; Jordan NJ; Fitzpatrick LM; Hong JS; Rab A; Sorscher EJ; Liang F; Mense M -
MOLECULAR ANALYSIS OF THE CFTR W1282X MUTATION
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 293 - 294
10/01/2016 Authors: Manfredi C; Rab A; Joshi D; Wang W; Kirk K; Saltz J; Mense M; Mahiou J; Sorscher EJ; Hong JS -
MODEL SYSTEMS FOR INVESTIGATING MOLECULAR PATHOGENESIS OF RARE CFTR ALLELES
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 256 - 257
10/01/2016 Authors: Rab A; Wen H; Manfredi C; Ehrhardt A; Joshi D; Sorscher EJ; Hong JS -
FUNCTIONAL CHARACTERIZATION OF ESTABLISHED AND NOVEL NONSENSE MUTATION READTHROUGH MODULATORS IN ENGINEERED FRT CELL LINES AND PRIMARY HUMAN BRONCHIAL EPITHELIAL (HBE) CELLS
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 264 - 264
10/01/2016 Authors: Bihler H; Harrington J; Musisi I; Mercadante D; Hong JS; Rab A; Sorscher EJ; Saltz J; Mahiou J; Wong E -
RIBOSOMAL TARGETING CORRECTS CFTR CLASS I AND II DEFECTS
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 209 - 209
10/01/2016 Authors: Oliver KE; Wang W; Wolpert MJ; Maya J; Icyuz M; Hong JS; Hartman JL; Sorscher EJ -
CHARACTERIZATION OF P67L CFTR AS A MODEL OF "PRECISION" THERAPEUTICS FOR RARE CFTR ALLELES
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 196 - 196
10/01/2016 Authors: Sabusap CP; Fu L; McNicholas-Bevensee CM; Wang W; Chung W; Wen H; Mazur M; Kirk K; Collawn JF; Hong JS -
ANALYSIS OF VAS DEFERENS ATRESIA IN THE CF RAT
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 256 - 256
10/01/2016 Authors: Plyler ZE; Schoeb TR; Hong JS; Tuggle KL; Birket S; Schultz BD; Sorscher EJ -
GENOME-WIDE ANALYSIS IN YEAST TO IDENTIFY MOLECULAR TARGETS PROMOTING READTHROUGH
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 252 - 252
10/01/2016 Authors: Icyuz M; Mutyam V; Rowe SM; Sorscher EJ; Hartman JL -
THE MUCUS TRANSPORT DEFECT IN THE CF RAT AIRWAY IS NORMALIZED BY ADDITION OF BICARBONATE
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 221 - 222
10/01/2016 Authors: Birket S; Tuggle KL; Oden A; Fernandez CM; Chu KK; Tearney GJ; Fanucchi M; Sorscher EJ; Rowe SM -
IDENTIFICATION OF NOVEL INHIBITORS OF UBIQUITINATION THAT IMPROVE RESCUE OF F508DEL-CFTR BY VX-809
PEDIATRIC PULMONOLOGY Volume: 51 Page(s): 206 - 206
10/01/2016 Authors: Goeckeler-Fried J; Estabrooks SK; Chiang A; Chung W; Ye Z; Denny RA; Weissman AM; Camacho CJ; Sorscher EJ; Brodsky JL -
Analysis of cystic fibrosis-associated P67L CFTR illustrates barriers to personalized therapeutics for orphan diseases.
JCI Insight Volume: 1
09/08/2016 Authors: Sabusap CM; Wang W; McNicholas CM; Chung WJ; Fu L; Wen H; Mazur M; Kirk KL; Collawn JF; Hong JS -
Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy.
Am J Physiol Lung Cell Mol Physiol Volume: 310 Page(s): L928 - L939
05/15/2016 Authors: Birket SE; Chu KK; Houser GH; Liu L; Fernandez CM; Solomon GM; Lin V; Shastry S; Mazur M; Sloane PA -
Ribosomal Stalk Protein Silencing Partially Corrects the F508-CFTR Functional Expression Defect.
PLoS Biol Volume: 14 Page(s): e1002462
05/01/2016 Authors: Veit G; Oliver K; Apaja PM; Perdomo D; Bidaud-Meynard A; Lin S-T; Guo J; Icyuz M; Sorscher EJ; Hartman JL -
Cystic fibrosis transmembrane conductance regulator activation by the solvent ethanol: implications for topical drug delivery.
Int Forum Allergy Rhinol Volume: 6 Page(s): 178 - 184
02/01/2016 Authors: Cho D-Y; Skinner D; Zhang S; Fortenberry J; Sorscher EJ; Dean NR; Woodworth BA -
From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.
Mol Biol Cell Volume: 27 Page(s): 424 - 433
02/01/2016 Authors: Veit G; Avramescu RG; Chiang AN; Houck SA; Cai Z; Peters KW; Hong JS; Pollard HB; Guggino WB; Balch WE -
Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.
J Biol Chem Volume: 291 Page(s): 1854 - 1865
01/22/2016 Authors: Ehrhardt A; Chung WJ; Pyle LC; Wang W; Nowotarski K; Mulvihill CM; Ramjeesingh M; Hong J; Velu SE; Lewis HA -
Robust Stimulation of W1282X-CFTR Channel Activity by a Combination of Allosteric Modulators.
PLoS One Volume: 11 Page(s): e0152232
01/01/2016 Authors: Wang W; Hong JS; Rab A; Sorscher EJ; Kirk KL -
Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics.
PLoS One Volume: 11 Page(s): e0163615
01/01/2016 Authors: Chung WJ; Goeckeler-Fried JL; Havasi V; Chiang A; Rowe SM; Plyler ZE; Hong JS; Mazur M; Piazza GA; Keeton AB -
PRE-CLINICAL AND CLINICAL VALIDATION OF AN ANTI-CANCER MODALITY THAT ABLATES REFRACTORY, LOW GROWTH FRACTION TUMORS.
Trans Am Clin Climatol Assoc Volume: 127 Page(s): 59 - 70
01/01/2016 Authors: Sorscher EJ; Hong JS; Parker WB -
DISTINCT PREMATURE TERMINATION CODON MUTATIONS OF CFTR MAY BENEFIT FROM SPECIFIC THERAPEUTIC APPROACHES TO CORRECTION AND READ-THROUGH MODULATION
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 202 - 202
10/01/2015 Authors: Liang F; Jordan NJ; Shang H; Wong E; Schneider C; Hong JS; Rab A; Sorscher EJ; Bihler H; Mense M -
INCREASING THE POOL OF CFTR AVAILABLE FOR CORRECTION: DISCOVERY OF NEW CF EXPERIMENTAL THERAPEUTICS THAT INHIBIT UBIQUITIN LIGASE
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 197 - 197
10/01/2015 Authors: Chung W; Fortenberry JA; Zhang Z; Wu J; Chiang A; Goeckeler-Fried J; Sterner D; Kodrasov M; Agarwal S; Kumar S -
THE INVESTIGATION OF QUALITY OF LIFE AND ADHERENCE IN PATIENTS WITH THE G551D MUTATION RECEIVING IVACAFTOR THERAPY
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 427 - 427
10/01/2015 Authors: Hathorne H; Brand KM; Britton LJ; Jackson T; Heltshe S; Kohler C; Cheong J; Harrington K; Sorscher EJ; Rowe SM -
EPITHELIAL CELL MODELS EXPRESSING DIVERSE CFTR MUTATIONS FOR STUDIES OF DISEASE MECHANISM AND DRUG DISCOVERY
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 259 - 259
10/01/2015 Authors: Rab A; Hong JS; Wen H; Sorscher EJ -
ANALYSIS OF P67L CFTR ILLUSTRATES BARRIERS TO THERAPEUTIC DEVELOPMENT FOR RARE CF MUTATIONS
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 279 - 279
10/01/2015 Authors: Sabusap CP; Fu L; McNicholas-Bevensee C; Wang W; Chung W; Wen H; Mazur M; Kirk K; Collawn J; Hong JS -
ANALYZYING THE IMMUNE REPERTOIRE IN CF AND NON-CF LUNGS
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 226 - 227
10/01/2015 Authors: Harris WT; Sorscher EJ; Han J -
TARGETING THE RIBOSOME AS A NOVEL APPROACH TO CORRECTING CFTR CLASS II TRAFFICKING DEFECTS
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 197 - 197
10/01/2015 Authors: Oliver KE; Veit G; Wolpert M; Guo J; Icyuz M; Hong JS; Wen H; Sorscher EJ; Lukacs GL; Schneider DA -
GENOME-WIDE ANALYSIS IN YEAST TO IDENTIFY MOLECULAR TARGETS PROMOTING READTHROUGH OF CFTR-G542X
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 293 - 294
10/01/2015 Authors: Icyuz M; Sorscher EJ; Hartman JL -
INHIBITORS OF CFTR UBIQUITINATION ACT SYNERGISTICALLY WITH SMALL MOLECULE CORRECTORS TO ENHANCE F508DEL-CFTR MATURATION AND ACTIVITY
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 196 - 196
10/01/2015 Authors: Goeckeler-Fried J; Chung W; Chiang A; Denny RA; Weissman AM; Sorscher EJ; Brodsky JL -
CHARACTERIZATION OF VAS DEFERENS ATRESIA IN THE CYSTIC FIBROSIS RAT MODEL
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 258 - 258
10/01/2015 Authors: Plyler ZE; Schoeb TR; Schultz BD; Sorscher EJ -
YEAST PHENOMIC ANALYSIS OF THE RPL12 MECHANISM THAT MODULATES ABCC-Delta F PROTEIN BIOGENESIS
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 254 - 254
10/01/2015 Authors: Icyuz M; Oliver KE; Schneider DA; Sorscher EJ; Hartman JL -
ANALYSIS OF THE EFFECTS OF RARE CYSTIC FIBROSIS MUTATIONS ON CFTR EXPRESSION, STABILITY AND FUNCTION
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 193 - 194
10/01/2015 Authors: Cai Z; Millen L; Gottschalk LB; Cutting GR; Hong JS; Lukacs GL; Sheppard DN; Sorscher EJ; Thomas PJ; Tuggle KL -
THE CFTR-/- RAT IS SUSCEPTIBLE TO PSEUDOMONAS AERUGINOSA INFECTION
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 242 - 242
10/01/2015 Authors: Birket S; Tuggle KL; Chu KK; Harris WT; Tearney G; Fanucchi M; Sorscher EJ; Rowe SM -
MECHANISTIC ANALYSIS OF RARE CFTR2 ALLELES
PEDIATRIC PULMONOLOGY Volume: 50 Page(s): 265 - 265
10/01/2015 Authors: Sorscher EJ; Balch WE; Braakman I; Brodsky J; Cry D; Frizzell R; Guggino W; Lukacs GL; Pollard H; Skach WR -
SNP Formation Bias in the Murine Genome Provides Evidence for Parallel Evolution.
Genome Biol Evol Volume: 7 Page(s): 2506 - 2519
08/06/2015 Authors: Plyler ZE; Hill AE; McAtee CW; Cui X; Moseley LA; Sorscher EJ -
Chlorogenic Acid Activates CFTR-Mediated Cl- Secretion in Mice and Humans: Therapeutic Implications for Chronic Rhinosinusitis.
Otolaryngol Head Neck Surg Volume: 153 Page(s): 291 - 297
08/01/2015 Authors: Illing EA; Cho D-Y; Zhang S; Skinner DF; Dunlap QA; Sorscher EJ; Woodworth BA -
Phase I dose-escalating trial of Escherichia coli purine nucleoside phosphorylase and fludarabine gene therapy for advanced solid tumors.
Ann Oncol Volume: 26 Page(s): 1481 - 1487
07/01/2015 Authors: Rosenthal EL; Chung TK; Parker WB; Allan PW; Clemons L; Lowman D; Hong J; Hunt FR; Richman J; Conry RM -
First-in-human dose-escalating trial of E. coli purine nucleoside phosphorylase and fludarabine gene therapy for advanced solid tumors.
Volume: 33
05/20/2015 Authors: Chung TK; Rosenthal E; Parker WB; Allan P; Clemons L; Lowman D; Hong J; Hunt FR; Richman J; Conry RM -
Targeted Correction and Restored Function of CFTR Gene in Cystic Fibrosis Induced Pluripotent Stem Cells
Volume: 23 Page(s): S74 - S74
05/01/2015 Authors: Davis BR; Crane AM; Kramer P; Bui JH; Chung WJ; Li XS; Gonzalez-Garay ML; Hawkins F; Liao W; Mora D -
Targeted correction and restored function of the CFTR gene in cystic fibrosis induced pluripotent stem cells.
Stem Cell Reports Volume: 4 Page(s): 569 - 577
04/14/2015 Authors: Crane AM; Kramer P; Bui JH; Chung WJ; Li XS; Gonzalez-Garay ML; Hawkins F; Liao W; Mora D; Choi S -
An autoregulatory mechanism governing mucociliary transport is sensitive to mucus load.
Am J Respir Cell Mol Biol Volume: 51 Page(s): 485 - 493
10/01/2014 Authors: Liu L; Shastry S; Byan-Parker S; Houser G; K Chu K; Birket SE; Fernandez CM; Gardecki JA; Grizzle WE; Wilsterman EJ -
AN HTS-COMPATIBLE PLATE FOR HIGHLY MINIATURIZED CULTURES OF PRIMARY HUMAN BRONCHIAL EPITHELIAL CELLS AT AIR-LIQUID INTERFACE
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 316 - 316
09/01/2014 Authors: Vu E; Sorscher EJ; Lowery R; Hayes S -
IMPACT OF PALMITOYLATION ON CLINICALLY SIGNIFICANT MUTATIONS CHARACTERIZED BY CFTR2
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 241 - 242
09/01/2014 Authors: Sabusap C; Hong JS; McClure M; Chung W; Wen H; Sorscher EJ -
IN VITRO AND IN VIVO MODELS OF ACUTE INFLAMMATION DEMONSTRATE ACQUIRED DEFECTS IN TRANSEPITHELIAL CL- TRANSPORT
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 245 - 245
09/01/2014 Authors: Grayson J; Zhang S; Skinner D; Fortenberry J; Sorscher EJ; Woodworth BA -
PULMONARY REMODELING PATHWAYS IN HUMAN CF AND THE PORCINE MODEL
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 279 - 280
09/01/2014 Authors: Harris WT; Jilling T; Halloran B; Zhang W; MacEwen M; Ambalavanan N; Sorscher EJ -
INHIBITORS OF CFTR UBIQUITINATION FUNCTION SYNERGISTICALLY WITH SMALL MOLECULE CORRECTORS TO ENHANCE Delta F508-CFTR MATURATION AND ACTIVITY
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 233 - 234
09/01/2014 Authors: Goeckeler-Fried J; Chung W; Chiang A; Denny RA; Weissman A; Sorscher EJ; Brodsky JL -
INVOLUTION OF VAS DEFERENS AS A MODEL FOR EPITHELIAL DISRUPTION IN THE CF RAT
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 282 - 282
09/01/2014 Authors: Plyler ZE; Fanucchi M; Tuggle KL; Pierucci-Alves F; Harris WT; Schultz BD; Sorscher EJ -
MODULATING THE DYNAMICS OF RIBOSOME FUNCTION RESCUES F508DEL CFTR MATURATIONAL ARREST
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 217 - 217
09/01/2014 Authors: Oliver KE; Veit G; Icyuz M; Guo J; McClure ML; Hong JS; Wen H; Sorscher EJ; Lukacs GL; Hartman JL -
INITIAL CHARACTERIZATION OF NBD1-TARGETING Delta F508 CORRECTORS
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 229 - 229
09/01/2014 Authors: Liu L; Park I; Fan Y; Bakey MT; Carver JR; Kirby EF; Chettiar SN; Regan N; Bhasin D; Li P -
ION TRANSPORT PHENOTYPE OF UPPER AIRWAY EPITHELIUM IN THE CFTR-/- RAT
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 279 - 279
09/01/2014 Authors: Skinner D; Zhang S; Dunlap Q; Tuggle KL; Sorscher EJ; Woodworth BA -
SEQUENCE-SPECIFIC GENETIC CORRECTION AND RESTORED FUNCTION OF CFTR GENE IN CYSTIC FIBROSIS INDUCED PLURIPOTENT STEM CELLS
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 274 - 274
09/01/2014 Authors: Crane AM; Kramer P; Bui JH; Chung WJ; Li XS; Gonzalez-Garay ML; Hawkins F; Liao W; Mora D; Wang J -
CFTR-/- RAT EXHIBITS DELAYED MUCOCILIARY CLEARANCE CHARACTERISTIC OF CYSTIC FIBROSIS AIRWAY DISEASE
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 246 - 246
09/01/2014 Authors: Birket SE; Tuggle KL; Chu KK; Tearney GJ; Fanucchi M; Sorscher EJ; Rowe SM -
CHARACTERIZATION OF CFTR-RELATED BONE DISEASE IN THE CF RAT MODEL
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 422 - 422
09/01/2014 Authors: Havasi V; Tuggle KL; Birket SE; Sorscher EJ; Stalvey MS -
DEFINING ALLELIC HETEROGENEITY AMONG CLINICALLY IMPORTANT MUTATIONS IN CFTR
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 275 - 276
09/01/2014 Authors: Hong JS; Sorscher EJ; Plyler ZE; Keiles S -
FUNCTIONAL CLASSIFICATION OF CFTR CORRECTORS BY PARALLEL TESTING IN TWO ISOGENIC FRT CELL LINES EXPRESSING CFTR F508DEL PROTEIN WITH SPECIFIC SUPPRESSOR MUTATIONS
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 237 - 237
09/01/2014 Authors: Finegan JF; Harrington JM; Musisi I; Bihler HJ; Wong E; Liang F; Hong JS; Sorscher EJ; Mense M -
EPITHELIAL MODELS ENCODING DIVERSE CFTR2 MUTATIONS FOR STUDIES OF DISEASE MECHANISM AND DRUG DISCOVERY
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 277 - 278
09/01/2014 Authors: Hong JS; Mahiou J; Liang F; Bihler HJ; Mense M; Lukacs GL; Wen H; Sorscher EJ -
RESVERATROL OVERCOMES TRANSEPITHELIAL FLUID AND ELECTROLYTE IMBALANCE IN A HYPOXIA-INDUCED MODEL OF ACQUIRED CFTR DEFICIENCY
PEDIATRIC PULMONOLOGY Volume: 49 Page(s): 237 - 238
09/01/2014 Authors: Zhang S; Chaaban M; Skinner D; Birket SE; Rowe SM; Sorscher EJ; Woodworth BA -
A functional anatomic defect of the cystic fibrosis airway.
Am J Respir Crit Care Med Volume: 190 Page(s): 421 - 432
08/15/2014 Authors: Birket SE; Chu KK; Liu L; Houser GH; Diephuis BJ; Wilsterman EJ; Dierksen G; Mazur M; Shastry S; Li Y -
Development and maintenance of a biospecimen repository for clinical samples derived from pulmonary patients.
Clin Transl Sci Volume: 7 Page(s): 336 - 341
08/01/2014 Authors: Schwiebert LM; Estell K; Meadows T; Thannickal VJ; Rowe S; Sorscher EJ; Harris WT; Gaggar A; Dransfield M; de Andrade JA -
Interference with ubiquitination in CFTR modifies stability of core glycosylated and cell surface pools.
Mol Cell Biol Volume: 34 Page(s): 2554 - 2565
07/01/2014 Authors: Lee S; Henderson MJ; Schiffhauer E; Despanie J; Henry K; Kang PW; Walker D; McClure ML; Wilson L; Sorscher EJ -
Porcine nasal epithelial cultures for studies of cystic fibrosis sinusitis.
Int Forum Allergy Rhinol Volume: 4 Page(s): 565 - 570
07/01/2014 Authors: Dean N; Ranganath NK; Jones B; Zhang S; Skinner D; Rowe SM; Sorscher EJ; Woodworth BA -
Marked repression of CFTR mRNA in the transgenic Cftr(tm1kth) mouse model.
J Cyst Fibros Volume: 13 Page(s): 351 - 352
05/01/2014 Authors: Zhang S; Ranganath NK; Skinner D; Bedwell DM; Buckley-Lanier JA; Sorscher EJ; Woodworth BA -
The unfolded protein response affects readthrough of premature termination codons.
EMBO Mol Med Volume: 6 Page(s): 685 - 701
05/01/2014 Authors: Oren YS; McClure ML; Rowe SM; Sorscher EJ; Bester AC; Manor M; Kerem E; Rivlin J; Zahdeh F; Mann M -
S-palmitoylation regulates biogenesis of core glycosylated wild-type and F508del CFTR in a post-ER compartment.
Biochem J Volume: 459 Page(s): 417 - 425
04/15/2014 Authors: McClure ML; Wen H; Fortenberry J; Hong JS; Sorscher EJ -
Longevity and plasticity of CFTR provide an argument for noncanonical SNP organization in hominid DNA.
PLoS One Volume: 9 Page(s): e109186
01/01/2014 Authors: Hill AE; Plyler ZE; Tiwari H; Patki A; Tully JP; McAtee CW; Moseley LA; Sorscher EJ -
Sinupret activates CFTR and TMEM16A-dependent transepithelial chloride transport and improves indicators of mucociliary clearance.
PLoS One Volume: 9 Page(s): e104090
01/01/2014 Authors: Zhang S; Skinner D; Hicks SB; Bevensee MO; Sorscher EJ; Lazrak A; Matalon S; McNicholas CM; Woodworth BA -
Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats.
PLoS One Volume: 9 Page(s): e91253
01/01/2014 Authors: Tuggle KL; Birket SE; Cui X; Hong J; Warren J; Reid L; Chambers A; Ji D; Gamber K; Chu KK -
Transcriptomic Profiling Indicates Increased Profibrotic Signaling In Cf Lung Parenchyma
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE Volume: 189
01/01/2014 Authors: Harris WT; Crossman D; Cui X; Tamas J; Halloran B; MacEwen M; Ambalavanan N; Sorscher EJ -
Myofibroblast Proliferation And Tissue Remodeling In The Porcine Model Of Cf Lung Disease
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE Volume: 189
01/01/2014 Authors: Harris WT; Jilling T; Halloran B; Zhang W; MacEwen M; Ambalavanan N; Sorscher E -
The silent codon change I507-ATC->ATT contributes to the severity of the F508 CFTR channel dysfunction.
FASEB J Volume: 27 Page(s): 4630 - 4645
11/01/2013 Authors: Lazrak A; Fu L; Bali V; Bartoszewski R; Rab A; Havasi V; Keiles S; Kappes J; Kumar R; Lefkowitz E -
S-PALMITOYLATION REGULATES STABILITY OF CORE GLYCOSYLATED WILD-TYPE AND F508DEL CFTR IN A POST-ER COMPARTMENT
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 221 - 221
10/01/2013 Authors: McClure M; Hong JS; Wen H; Sorscher EJ -
INTERFERENCE WITH UBIQUITINATION OF LYSINES IN THE NTAIL, R, TM10, ICL4, OR NBD2 DOMAINS MODIFIES STABILITY OF WT CFTR
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 207 - 207
10/01/2013 Authors: Lee S; McClure M; Wilson L; Sorscher E; Zeitlin P -
DEVELOPING A MODEL OF FIBROTIC LUNG DISEASE IN THE CF MOUSE
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 270 - 271
10/01/2013 Authors: Harris WT; Liu R; Kong M; Halloran B; Zhang W; MacEwen M; Ambalavanan N; Sorscher EJ -
CFTR KNOCKOUT RATS HAVE REDUCED GROWTH AND IGF-I CONCENTRATIONS
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 419 - 419
10/01/2013 Authors: Tuggle KL; Havasi V; Sorscher EJ; Stalvey MS -
HIGH THROUGHPUT SCREENING FOR COMPOUNDS THAT AUGMENT THE POOL OF F508DEL CFTR SUITABLE FOR PHARMACOLOGIC CORRECTION
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 228 - 228
10/01/2013 Authors: Chung W; Goeckeler-Fried J; Brodsky J; Havasi V; Rasmussen L; White E; Sorscher EJ -
A MURINE MODEL OF CYSTIC FIBROSIS AND RESPIRATORY SYNCYTIAL VIRUS INFECTION
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 320 - 321
10/01/2013 Authors: Kong M; Peng N; Namasivayam A; Schoeb T; Sorscher EJ; Clancy JP -
CHARACTERIZATION OF THE CFTR-KNOCKOUT RAT
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 271 - 271
10/01/2013 Authors: Tuggle KL; Birket S; Fortenberry J; Du M; Tang L; Hong J; Bedwell DM; Rowe SM; Sorscher EJ; Fanucchi M -
TRANSLATIONAL RELEVANCE OF THE YEAST YOR1-Delta F670 MODEL FOR Delta F508-CFTR BIOGENESIS
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 211 - 211
10/01/2013 Authors: Veit G; Okiyoneda T; Oliver K; Perdomo D; Sorscher EJ; Hartman JL; Lukacs GL -
PORCINE NASAL PRIMARY CULTURES FOR STUDIES OF CYSTIC FIBROSIS
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 275 - 275
10/01/2013 Authors: Skinner D; Ranganath N; Zhang S; Jones B; Sorscher EJ; Woodworth BA -
MUTATIONS IN YOR1 TO MODEL DIFFERENT ASPECTS OF CFTR BIOGENESIS
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 276 - 276
10/01/2013 Authors: Icyuz M; McClure M; Sorscher EJ; Hartman JL -
CONSTITUTIVE EXPRESSION OF CFTR SUPPRESSOR MUTATIONS AS A TOOL FOR INVESTIGATING F508DEL CORRECTION
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 274 - 274
10/01/2013 Authors: Hong JS; Tuggle KL; Wen H; Sorscher EJ -
DERIVATION AND CHARACTERIZATION OF A CFTR EXPRESSING CELL LINE AMENABLE FOR ION TRANSPORT STUDIES
PEDIATRIC PULMONOLOGY Volume: 48 Page(s): 277 - 277
10/01/2013 Authors: Jackson L; Tang L; Wen H; Bridges RJ; Sorscher EJ; Rowe SM; Hong JS -
Respiratory syncytial virus infection disrupts monolayer integrity and function in cystic fibrosis airway cells.
Viruses Volume: 5 Page(s): 2260 - 2271
09/19/2013 Authors: Kong M; Maeng P; Hong J; Szczesniak R; Sorscher E; Sullender W; Clancy JP -
Heterozygosity for the F508del mutation in the cystic fibrosis transmembrane conductance regulator anion channel attenuates influenza severity.
J Infect Dis Volume: 208 Page(s): 780 - 789
09/01/2013 Authors: Aeffner F; Abdulrahman B; Hickman-Davis JM; Janssen PM; Amer A; Bedwell DM; Sorscher EJ; Davis IC -
Comparison of cystic fibrosis transmembrane conductance regulator (CFTR) and ciliary beat frequency activation by the CFTR Modulators Genistein, VRT-532, and UCCF-152 in primary sinonasal epithelial cultures.
JAMA Otolaryngol Head Neck Surg Volume: 139 Page(s): 822 - 827
08/01/2013 Authors: Conger BT; Zhang S; Skinner D; Hicks SB; Sorscher EJ; Rowe SM; Woodworth BA -
Massive microRNA sequence conservation and prevalence in human and chimpanzee introns.
Front Biosci (Elite Ed) Volume: 5 Page(s): 814 - 822
06/01/2013 Authors: Hill AE; Sorscher EJ -
Resveratrol enhances airway surface liquid depth in sinonasal epithelium by increasing cystic fibrosis transmembrane conductance regulator open probability.
PLoS One Volume: 8 Page(s): e81589
01/01/2013 Authors: Zhang S; Blount AC; McNicholas CM; Skinner DF; Chestnut M; Kappes JC; Sorscher EJ; Woodworth BA -
Myofibroblast differentiation and enhanced TGF-B signaling in cystic fibrosis lung disease.
PLoS One Volume: 8 Page(s): e70196
01/01/2013 Authors: Harris WT; Kelly DR; Zhou Y; Wang D; MacEwen M; Hagood JS; Clancy JP; Ambalavanan N; Sorscher EJ -
Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography.
PLoS One Volume: 8 Page(s): e54473
01/01/2013 Authors: Liu L; Chu KK; Houser GH; Diephuis BJ; Li Y; Wilsterman EJ; Shastry S; Dierksen G; Birket SE; Mazur M -
Influenza-Induced Cardiopulmonary Dysfunction And Lung Injury Are Attenuated In Mice Heterozygous For The F508del Mutation In The Cystic Fibrosis Transmembrane Conductance Regulator Anion Channel
Volume: 187
01/01/2013 Authors: Davis IC; Aeffner F; Abdulrahman B; Hickman-Davis JM; Janssen P; Hayes D; Amer A; Bedwell DM; Sorscher EJ -
A yeast phenomic model for the gene interaction network modulating CFTR-F508 protein biogenesis.
Genome Med Volume: 4 Page(s): 103
12/27/2012 Authors: Louie RJ; Guo J; Rodgers JW; White R; Shah N; Pagant S; Kim P; Livstone M; Dolinski K; McKinney BA -
IDENTIFICATION OF EFFICACIOUS F508DEL CFTR CORRECTORS USING A NOVEL HOMOLOGY MODEL
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 226 - 227
09/01/2012 Authors: Aller S; Tang L; Rajpur J; Sloane P; Sorscher EJ; Rowe SM -
AVAILABILITY OF F508DEL CFTR FOR SMALL MOLECULE CORRECTION
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 224 - 224
09/01/2012 Authors: Chung W; Goeckeler-Fried J; Brodsky J; Havasi V; Rowe SM; Piazza GA; Keeton AB; Sorscher EJ -
ASSOCIATION OF CFTR WITH ENAC SUBUNITS CARRYING LIDDLE'S SYNDROME MUTATIONS
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 245 - 245
09/01/2012 Authors: Rooj AK; Cormet-Boyaka E; Clark EB; Qadri YJ; Sorscher EJ; Fuller CM; Berdiev BK -
POST-TRANSLATIONAL MODIFICATIONS OF CFTR GLYCOFORMS IDENTIFIED BY HIGH RESOLUTION, HIGH MASS ACCURACY MASS SPECTROMETRY
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 246 - 246
09/01/2012 Authors: McClure M; Wilson L; Ray M; DeLucas LJ; Rowe S; Wu X; Dai Q; Hong J; Sorscher EJ; Kappes JC -
DEVELOPING ANTIBODIES CAPABLE OF MONITORING CONFORMATION AND MATURATION OF CFTR
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 224 - 224
09/01/2012 Authors: Chung W; Fortenberry JA; Lukacs G; Roldan HA; Kappes JC; Brouillette C; DeLucas LJ; Ray MJ; Sorscher EJ; Accavitti-Loper M -
GENERATION OF A CFTR-KNOCKOUT RAT MODEL FOR CYSTIC FIBROSIS
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 279 - 279
09/01/2012 Authors: Tuggle KL; Gamber K; Cui X; Ji D; Chambers A; Bedwell DM; Rowe SM; Sorscher EJ; Fanucchi M -
RSV STIMULATES MMP-9 RELEASE FROM CF EPITHELIAL CELLS AND INCREASES MMP ACTIVITY IN HUMAN LUNGS
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 316 - 316
09/01/2012 Authors: Kong M; Peng N; Clancy JP; Blalock E; Gaggar A; Sorscher EJ -
PRIMARY RABBIT NASAL SEPTAL EPITHELIAL CULTURES FOR STUDIES OF CYSTIC FIBROSIS SINUS DISEASE
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 288 - 288
09/01/2012 Authors: Skinner D; Zhang S; Chaaban M; Sorscher E; Woodworth BA -
CFTR ACTIVATION BY THE SOLVENT ETHANOL: IMPLICATIONS FOR CF DRUG TESTING AND DELIVERY
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 248 - 248
09/01/2012 Authors: Zhang S; Skinner D; Chaaban M; Sorscher E; Woodworth BA -
S-PALMITOYLATION MEDIATES TRAFFICKING OF CFTR TO THE CELL SURFACE
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 246 - 246
09/01/2012 Authors: McClure M; Wilson L; Ray M; DeLucas LJ; Rowe S; Wu X; Dai Q; Hong JS; Kappes JC; Barnes S -
ADVANCING A YEAST PHENOMICS MODEL FOR GENE INTERACTION NETWORK ANALYSIS OF F508DEL-CFTR BIOGENESIS
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 287 - 287
09/01/2012 Authors: Guo J; Sorscher EJ; Hartman JL -
MYOFIBROBLAST PROLIFERATION AND FIBROSIS IN CF LUNG DISEASE
PEDIATRIC PULMONOLOGY Volume: 47 Page(s): 259 - 259
09/01/2012 Authors: Harris WT; Kelly D; Hagood J; Ambalavanan N; Sorscher E -
In vivo antitumor activity of intratumoral fludarabine phosphate in refractory tumors expressing E. coli purine nucleoside phosphorylase.
Cancer Chemother Pharmacol Volume: 70 Page(s): 321 - 329
08/01/2012 Authors: Sorscher EJ; Hong JS; Allan PW; Waud WR; Parker WB -
Cystic fibrosis transmembrane conductance regulator modulation by the tobacco smoke toxin acrolein.
Volume: 122 Page(s): 1193 - 1197
06/01/2012 Authors: Alexander NS; Blount A; Zhang S; Skinner D; Hicks SB; Chestnut M; Kebbel FA; Sorscher EJ; Woodworth BA -
Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report.
Am J Respir Crit Care Med Volume: 185 Page(s): 887 - 892
04/15/2012 Authors: Ramsey BW; Banks-Schlegel S; Accurso FJ; Boucher RC; Cutting GR; Engelhardt JF; Guggino WB; Karp CL; Knowles MR; Kolls JK -
Purification of CFTR for mass spectrometry analysis: identification of palmitoylation and other post-translational modifications.
Protein Eng Des Sel Volume: 25 Page(s): 7 - 14
01/01/2012 Authors: McClure M; DeLucas LJ; Wilson L; Ray M; Rowe SM; Wu X; Dai Q; Hong JS; Sorscher EJ; Kappes JC -
A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease.
PLoS One Volume: 7 Page(s): e39809
01/01/2012 Authors: Sloane PA; Shastry S; Wilhelm A; Courville C; Tang LP; Backer K; Levin E; Raju SV; Li Y; Mazur M -
Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators.
Am J Physiol Lung Cell Mol Physiol Volume: 301 Page(s): L587 - L597
10/01/2011 Authors: Pyle LC; Ehrhardt A; Mitchell LH; Fan L; Ren A; Naren AP; Li Y; Clancy JP; Bolger GB; Sorscher EJ -
Transepithelial ion transport is suppressed in hypoxic sinonasal epithelium.
Laryngoscope Volume: 121 Page(s): 1929 - 1934
09/01/2011 Authors: Blount A; Zhang S; Chestnut M; Hixon B; Skinner D; Sorscher EJ; Woodworth BA -
A breath of fresh air.
Sci Am Volume: 305 Page(s): 68 - 73
08/01/2011 Authors: Rowe SM; Clancy JP; Sorscher EJ -
Resveratrol has salutary effects on mucociliary transport and inflammation in sinonasal epithelium.
Laryngoscope Volume: 121 Page(s): 1313 - 1319
06/01/2011 Authors: Alexander NS; Hatch N; Zhang S; Skinner D; Fortenberry J; Sorscher EJ; Woodworth BA -
Effect of expression of adenine phosphoribosyltransferase on the in vivo anti-tumor activity of prodrugs activated by E. coli purine nucleoside phosphorylase.
Cancer Gene Ther Volume: 18 Page(s): 390 - 398
06/01/2011 Authors: Parker WB; Allan PW; Waud WR; Hong JS; Sorscher EJ -
Spontaneous inactivating p53 mutations and the "selfish cell".
Aging (Albany NY) Volume: 3 Page(s): 181
03/01/2011 Authors: Sorscher SM; Hill AE; Belizaire R; Sorscher EJ -
S-PALMITOYLATION IS A REGULATOR OF CFTR PROCESSING
PEDIATRIC PULMONOLOGY Page(s): 233 - 233
01/01/2011 Authors: McClure M; Wilson L; DeLucas LJ; Ray M; Rowe SM; Wu X; Dai Q; Hong JS; Kappes JC; Barnes S -
A CONSERVED BIOGENESIS NETWORK FOR YEAST YOR1-Delta F AND CFTR-Delta F
PEDIATRIC PULMONOLOGY Page(s): 277 - 277
01/01/2011 Authors: Guo J; Louie R; Rodgers JW; White R; Hong J; Sorscher EJ; Bryan JA; Miller EA; Hartman JL -
DEVELOPING A CFTR DEFICIENT PKD MOUSE MODEL
PEDIATRIC PULMONOLOGY Page(s): 279 - 280
01/01/2011 Authors: Havasi V; Buckley-Lanier J; Childress-Roper V; Fortenberry JA; Schoeb TR; Bedwell DM; Yoder BK; Sorscher EJ -
TRANSEPITHELIAL ION TRANSPORT THROUGH CFTR AND TMEM16A IS SUPPRESSED IN HYPOXIC SINONASAL EPITHELIUM
PEDIATRIC PULMONOLOGY Page(s): 250 - 250
01/01/2011 Authors: Zhang S; Blount A; Chestnut M; Hixon B; Skinner D; Sorscher EJ; Woodworth BA -
Quercetin increases cystic fibrosis transmembrane conductance regulator-mediated chloride transport and ciliary beat frequency: therapeutic implications for chronic rhinosinusitis.
Am J Rhinol Allergy Volume: 25 Page(s): 307 - 312
01/01/2011 Authors: Zhang S; Smith N; Schuster D; Azbell C; Sorscher EJ; Rowe SM; Woodworth BA -
RESVERATROL HAS SALUTARY EFFECTS ON MUCOCILIARY TRANSPORT AND INFLAMMATION IN SINONASAL EPITHELIUM
PEDIATRIC PULMONOLOGY Page(s): 249 - 249
01/01/2011 Authors: Skinner D; Alexander NS; Hatch N; Zhang S; Fortenberry J; Sorscher EJ; Woodworth BA -
COMPARISON OF CFTR AND CILIARY BEAT FREQUENCY ACTIVATION BY THE CFTR MODULATORS VX-770, VRT532, AND UCCF-152 IN PRIMARY SINONASAL EPITHELIAL CULTURES
PEDIATRIC PULMONOLOGY Page(s): 251 - 252
01/01/2011 Authors: Woodworth BA; Zhang S; Skinner D; Sorscher EJ; Rowe SM -
A PRO-BIOGENESIS COMPLEX FOR F508DEL-CFTR DISCOVERED WITH A YEAST MODEL AND VALIDATED IN HUMAN CELLS
PEDIATRIC PULMONOLOGY Page(s): 274 - 274
01/01/2011 Authors: Hong J; Guo J; Louie R; Wen H; Sorscher EJ; Miller E; Hartman JL -
HIGH CONTENT CELL-BASED IMAGING TO IDENTIFY SMALL MOLECULE CORRECTORS OF F508DEL-CFTR
PEDIATRIC PULMONOLOGY Page(s): 220 - 221
01/01/2011 Authors: Chung W; Rowe SM; Sorscher EJ; Piazza GA; Keeton AB -
PHARMACOLOGIC RESCUE OF ACQUIRED CFTR DYSFUNCTION IN CHRONIC OBSTRUCTIVE PULMONARY DISEASE
PEDIATRIC PULMONOLOGY Page(s): 300 - 301
01/01/2011 Authors: Sloane PA; Shastry S; Wilhelm A; Courville C; Tang L; Mehta N; Backer K; Levin E; Mazur M; Van Goor F -
CFTR Folding Consortium: methods available for studies of CFTR folding and correction.
Methods Mol Biol Volume: 742 Page(s): 335 - 353
01/01/2011 Authors: Peters KW; Okiyoneda T; Balch WE; Braakman I; Brodsky JL; Guggino WB; Penland CM; Pollard HB; Sorscher EJ; Skach WR -
IDENTIFICATION OF CFTR POST-TRANSLATIONAL MODIFICATION BY MASS SPECTROMETRY
PEDIATRIC PULMONOLOGY Page(s): 233 - 233
01/01/2011 Authors: McClure M; Wilson L; DeLucas LJ; Ray M; Rowe SM; Wu X; Dai Q; Hong J; Kappes JC; Sorscher EJ -
TOWARDS DEVELOPMENT OF CONFORMATIONALLY SENSITIVE MONOCLONAL ANTIBODIES AGAINST CFTR
PEDIATRIC PULMONOLOGY Page(s): 220 - 220
01/01/2011 Authors: Chung W; Fortenberry JA; Kappes JC; DeLucas LJ; Marjorie RJ; Alverson P; Sorscher EJ; Accavitti-Loper M -
Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens.
Fertil Steril Volume: 94 Page(s): 2122 - 2127
11/01/2010 Authors: Havasi V; Rowe SM; Kolettis PN; Dayangac D; Sahin A; Grangeia A; Carvalho F; Barros A; Sousa M; Bassas L -
Enhanced efficiency of prodrug activation therapy by tumor-selective replicating retrovirus vectors armed with the Escherichia coli purine nucleoside phosphorylase gene.
Cancer Gene Ther Volume: 17 Page(s): 614 - 623
09/01/2010 Authors: Tai C-K; Wang W; Lai Y-H; Logg CR; Parker WB; Li Y-F; Hong JS; Sorscher EJ; Chen TC; Kasahara N -
Hesperidin stimulates cystic fibrosis transmembrane conductance regulator-mediated chloride secretion and ciliary beat frequency in sinonasal epithelium.
Otolaryngol Head Neck Surg Volume: 143 Page(s): 397 - 404
09/01/2010 Authors: Azbell C; Zhang S; Skinner D; Fortenberry J; Sorscher EJ; Woodworth BA -
DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.
Pulm Pharmacol Ther Volume: 23 Page(s): 268 - 278
08/01/2010 Authors: Rowe SM; Pyle LC; Jurkevante A; Varga K; Collawn J; Sloane PA; Woodworth B; Mazur M; Fulton J; Fan L -
Exposure to cigarette smoke condensate reduces calcium activated chloride channel transport in primary sinonasal epithelial cultures.
Laryngoscope Volume: 120 Page(s): 1465 - 1469
07/01/2010 Authors: Virgin FW; Azbell C; Schuster D; Sunde J; Zhang S; Sorscher EJ; Woodworth BA -
The bioflavonoid compound, sinupret, stimulates transepithelial chloride transport in vitro and in vivo.
Laryngoscope Volume: 120 Page(s): 1051 - 1056
05/01/2010 Authors: Virgin F; Zhang S; Schuster D; Azbell C; Fortenberry J; Sorscher EJ; Woodworth BA -
Epigenetics and proteostasis in correction of misfolding disease
BIOCHEMISTRY AND CELL BIOLOGY-BIOCHIMIE ET BIOLOGIE CELLULAIRE Volume: 88 Page(s): 408 - 408
04/01/2010 Authors: Balch W; Hutt D; Herman D; Koulov A; Lapointe P; Coppinger J; Pankow S; Lu B; Rodriquez A; Noel S -
HESPERIDIN STIMULATES CFTR-MEDIATED CHLORIDE SECRETION AND CILIARY BEAT FREQUENCY IN SINONASAL EPITHELIUM
PEDIATRIC PULMONOLOGY Page(s): 267 - 267
01/01/2010 Authors: Skinner D; Zhang S; Azbell C; Fortenberry J; Sorscher EJ; Woodworth BA -
SINUPRET (R) STIMULATES CILIARY BEAT FREQUENCY & TRANSEPITHELIAL CHLORIDE TRANSPORT THROUGH CFTR DEPENDENT & INDEPENDENT MECHANISMS
PEDIATRIC PULMONOLOGY Page(s): 267 - 267
01/01/2010 Authors: Zhang S; Skinner D; Hicks B; Bevensee M; Sorscher EJ; Woodworth BA -
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.
Nat Chem Biol Volume: 6 Page(s): 25 - 33
01/01/2010 Authors: Hutt DM; Herman D; Rodrigues APC; Noel S; Pilewski JM; Matteson J; Hoch B; Kellner W; Kelly JW; Schmidt A -
A BIOCHEMICAL ASSAY FOR DISCOVERY OF GATING MODULATORS BASED ON INTRAMOLECULAR INTERACTIONS BETWEEN NUCLEOTIDE BINDING DOMAIN 1 AND THE FIRST CYTOPLASMIC LOOP OF CFTR
PEDIATRIC PULMONOLOGY Page(s): 236 - 236
01/01/2010 Authors: Chung W; Ehrhardt A; Pyle LC; Wang W; Bear CE; Lukacs G; Kirk KL; Sorscher EJ -
SCREENING OF CANDIDATE ADENOVIRUS VECTORS EXPRESSING SHRNAS FOR FUNCTIONAL RECOVERY OF Delta F508 CFTR
PEDIATRIC PULMONOLOGY Page(s): 222 - 223
01/01/2010 Authors: Sorscher EJ; Hong JS; Hutt DM; Chalfant M; Roth DM; Balch W; Noel S; Frizzell R; Okiyoneda T; Veit G -
YOR1-Delta F AS A CFTR-Delta F508-LIKE MUTATION TO IDENTIFY MODULATORS IN S. CEREVISIAE
PEDIATRIC PULMONOLOGY Page(s): 290 - 290
01/01/2010 Authors: Guo J; Louie R; Rodgers JW; Miller E; Sorscher EJ; Hartman JL -
DEVELOPING A CELLULAR ROADMAP FOR CORRECTORS OF CFTR MISFOLDING
PEDIATRIC PULMONOLOGY Page(s): 222 - 222
01/01/2010 Authors: Pyle LC; Balch W; Lukacs G; Braakman I; Guggino W; Thomas PJ; Penland C; Pollard H; Brodsky J; Frizzell R -
LPS & PSEUDOMONAS AERUGINOSA FILTRATE REDUCE CALCIUM ACTIVATED CHLORIDE CHANNEL TRANSPORT IN PRIMARY SINONASAL EPITHELIAL CULTURES
PEDIATRIC PULMONOLOGY Page(s): 356 - 356
01/01/2010 Authors: Zhang S; Skinner D; Sorscher EJ; Woodworth BA -
INTESTINAL MUCOSAL INFLAMMATION IN CYSTIC FIBROSIS
PEDIATRIC PULMONOLOGY Page(s): 427 - 427
01/01/2010 Authors: Dharmalingam P; Kurundkar A; Harris WT; Clancy JP; Sorscher EJ; Maheshwari A -
POST-TRANSLATIONAL MODIFICATIONS OF CFTR WITH RELEVANCE TO GATING AND PROCESSING
PEDIATRIC PULMONOLOGY Page(s): 241 - 241
01/01/2010 Authors: McClure M; DeLucas LJ; Wilson L; Ray M; Hong JS; Kappes JC; Sorscher EJ; Barnes S -
DEVELOPMENT OF A CFTR DEFICIENT CILIOPATHIC MOUSE MODEL
PEDIATRIC PULMONOLOGY Page(s): 295 - 295
01/01/2010 Authors: Havasi V; Buckley-Lanier JA; Roper C; Fortenberry JA; Bedwell DM; Sorscher EJ; Yoder BK -
A truncated CFTR protein rescues endogenous DeltaF508-CFTR and corrects chloride transport in mice.
FASEB J Volume: 23 Page(s): 3743 - 3751
11/01/2009 Authors: Cormet-Boyaka E; Hong JS; Berdiev BK; Fortenberry JA; Rennolds J; Clancy JP; Benos DJ; Boyaka PN; Sorscher EJ -
Cigarette smoke condensate inhibits transepithelial chloride transport and ciliary beat frequency.
Laryngoscope Volume: 119 Page(s): 2269 - 2274
11/01/2009 Authors: Cohen NA; Zhang S; Sharp DB; Tamashiro E; Chen B; Sorscher EJ; Woodworth BA -
Correlation of microRNA levels during hypoxia with predicted target mRNAs through genome-wide microarray analysis.
BMC Med Genomics Volume: 2 Page(s): 15
03/25/2009 Authors: Guimbellot JS; Erickson SW; Mehta T; Wen H; Page GP; Sorscher EJ; Hong JS -
PROTEOMIC ANALYSIS OF SALIVARY FLUID IN CYSTIC FIBROSIS
PEDIATRIC PULMONOLOGY Page(s): 303 - 304
01/01/2009 Authors: Troxler RB; Mobley JA; Bowersock G; Parrott M; Roach M; Sorscher EJ -
ANALYSIS OF SYNONYMOUS CFTR POLYMORPHISMS AS CONTRIBUTORS TO CBAVD
PEDIATRIC PULMONOLOGY Page(s): 276 - 276
01/01/2009 Authors: Plyler ZE; Bernard KF; Havasi V; Keiles SB; Hong JS; Wen H; Sorscher EJ -
DEVELOPMENT OF CONFORMATIONALLY SENSITIVE MONOCLONAL ANTIBODIES RAISED AGAINST FULL-LENGTH CFTR
PEDIATRIC PULMONOLOGY Page(s): 278 - 278
01/01/2009 Authors: Chung W; Fortenberry JA; Kappes JC; Accavittee-Loper MA; DeLucas LJ; Ray MJ; Alverson P; Sorscher EJ -
GENOME-WIDE EPISTASTS ANALYSIS OF A CFTR-Delta F508-LIKE MUTATION IN S. CEREVISIAE
PEDIATRIC PULMONOLOGY Page(s): 283 - 284
01/01/2009 Authors: Guo J; Louie R; Rodgers JW; Miller E; Sorscher EJ; Hartman JL -
MARKED REPRESSION OF CFTR MRNA IN THE TRANSGENIC CFTR boolean AND TM1KTH MOUSE MODEL
PEDIATRIC PULMONOLOGY Page(s): 306 - 306
01/01/2009 Authors: Zhang S; Schuster D; Bedwell D; Buckley-Lanier J; Sorscher EJ; Woodworth BA -
ACTIVATION OF CILIARY BEAT FREQUENCY IN PRIMARY MURINE NASAL EPITHELIAL CULTURES BY THE CFTR POTENTIATOR, QUERCETIN
PEDIATRIC PULMONOLOGY Page(s): 226 - 226
01/01/2009 Authors: Zhang S; Smith N; Schuster D; Sorscher EJ; Rowe SM; Woodworth BA -
Comparison of vectorial ion transport in primary murine airway and human sinonasal air-liquid interface cultures, models for studies of cystic fibrosis, and other airway diseases.
Am J Rhinol Allergy Volume: 23 Page(s): 149 - 152
01/01/2009 Authors: Zhang S; Fortenberry JA; Cohen NA; Sorscher EJ; Woodworth BA -
RESTORING Delta F508-CFTR TRAFFICKING AND ACTIVITY WITH EPIGENETIC MODIFIERS
PEDIATRIC PULMONOLOGY Page(s): 215 - 215
01/01/2009 Authors: Hutt DM; Herman DM; Rodrigues AP; Noel S; Gentzsch M; Pilewski JM; Riordan JR; Sorscher EJ; Kelly JW; Frizzell RA -
COMPREHENSIVE SPECTRUM OF ACTIVITY ANALYSIS FOR Delta F508-CFTR CORRECTORS
PEDIATRIC PULMONOLOGY Page(s): 291 - 291
01/01/2009 Authors: Pyle LC; Fan L; Fortenberry J; Tang L; Backer K; Mazur M; Clancy JP; Sorscher EJ; Rowe SM -
Evolutionary maintenance of oncogenesis.
J Cancer Res Clin Oncol Volume: 135 Page(s): 159 - 162
01/01/2009 Authors: Sorscher SM; Hill A; Sorscher EJ -
A NOVEL CELL-BASED IMAGING ASSAY FOR HIGH THROUGHPUT SCREENS TO IDENTIFY SMALL MOLECULE CORRECTORS OF Delta F508-CFTR
PEDIATRIC PULMONOLOGY Page(s): 278 - 278
01/01/2009 Authors: Chung W; Rowe SM; Keeton AB; Shevin R; Piazza GA; Sorscher EJ -
Genetic modification of adeno-associated viral vector type 2 capsid enhances gene transfer efficiency in polarized human airway epithelial cells.
Hum Gene Ther Volume: 19 Page(s): 1407 - 1414
12/01/2008 Authors: White AF; Mazur M; Sorscher EJ; Zinn KR; Ponnazhagan S -
The role of the F508C mutation in congenital bilateral absence of the vas deferens.
Genet Med Volume: 10 Page(s): 910 - 914
12/01/2008 Authors: Havasi V; Keiles S; Hambuch T; Sorscher EJ; Kammesheidt A -
Role of oxygen availability in CFTR expression and function.
Am J Respir Cell Mol Biol Volume: 39 Page(s): 514 - 521
11/01/2008 Authors: Guimbellot JS; Fortenberry JA; Siegal GP; Moore B; Wen H; Venglarik C; Chen Y-F; Oparil S; Sorscher EJ; Hong JS -
Is oncogenesis a normal cellular defense mechanism?
Cancer Chemother Pharmacol Volume: 62 Page(s): 1113
11/01/2008 Authors: Sorscher S; Hill AE; Sorscher EJ -
Potential role of high-mobility group box 1 in cystic fibrosis airway disease.
Am J Respir Crit Care Med Volume: 178 Page(s): 822 - 831
10/15/2008 Authors: Rowe SM; Jackson PL; Liu G; Hardison M; Livraghi A; Solomon GM; McQuaid DB; Noerager BD; Gaggar A; Clancy JP -
Inhibitory effects of hypertonic saline on P. aeruginosa motility.
J Cyst Fibros Volume: 7 Page(s): 267 - 269
07/01/2008 Authors: Havasi V; Hurst CO; Briles TC; Yang F; Bains DG; Hassett DJ; Sorscher E -
Luminal fluid tonicity regulates airway ciliary beating by altering membrane stretch and intracellular calcium.
Cell Motil Cytoskeleton Volume: 65 Page(s): 469 - 475
06/01/2008 Authors: Horvth G; Sorscher EJ -
FRET assessment of CFTR molecular assembly
FASEB JOURNAL Volume: 22
04/01/2008 Authors: Berdiev BK; Tousson A; Oosterveld-Hut HMJ; Qadri YJ; Cormet-Boyaka E; Hong JS; Fuller CM; Sorscher EJ; Lukacs GL; Benos DJ -
Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones.
Biochem J Volume: 410 Page(s): 555 - 564
03/15/2008 Authors: Varga K; Goldstein RF; Jurkuvenaite A; Chen L; Matalon S; Sorscher EJ; Bebok Z; Collawn JF -
Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer.
J Biol Chem Volume: 282 Page(s): 36481 - 36488
12/14/2007 Authors: Berdiev BK; Cormet-Boyaka E; Tousson A; Qadri YJ; Oosterveld-Hut HMJ; Hong JS; Gonzales PA; Fuller CM; Sorscher EJ; Lukacs GL -
Lymphoma chemovirotherapy: CD20-targeted and convertase-armed measles virus can synergize with fludarabine.
Cancer Res Volume: 67 Page(s): 10939 - 10947
11/15/2007 Authors: Ungerechts G; Springfeld C; Frenzke ME; Lampe J; Johnston PB; Parker WB; Sorscher EJ; Cattaneo R -
An immunocompetent murine model for oncolysis with an armed and targeted measles virus.
Mol Ther Volume: 15 Page(s): 1991 - 1997
11/01/2007 Authors: Ungerechts G; Springfeld C; Frenzke ME; Lampe J; Parker WB; Sorscher EJ; Cattaneo R -
Restoration of W1282X CFTR activity by enhanced expression.
Am J Respir Cell Mol Biol Volume: 37 Page(s): 347 - 356
09/01/2007 Authors: Rowe SM; Varga K; Rab A; Bebok Z; Byram K; Li Y; Sorscher EJ; Clancy JP -
Bioelectric effects of quinine on polarized airway epithelial cells.
J Cyst Fibros Volume: 6 Page(s): 351 - 359
09/01/2007 Authors: Bates E; Miller S; Alexander M; Mazur M; Fortenberry JA; Bebok Z; Sorscher EJ; Rowe SM -
Novel approaches for expression, stabilization and crystallization of CFTR
Page(s): 101 - 102
01/01/2007 Authors: Kappes J; Wu X; Ray M; Sorscher EJ; Brouillette C; Collawn J; Rowe S; DeLucas L -
Mechanisms underlying potentiator activation of CFTR
PEDIATRIC PULMONOLOGY Page(s): 305 - 305
01/01/2007 Authors: Pyle LC; Ehrhardt A; Wang W; Varga K; Nowatarski KJ; Rowe SM; Sorscher EJ -
Processing correction and direct activation of Delta F508 CFTR by the flavonoid equol in vitro
PEDIATRIC PULMONOLOGY Page(s): 299 - 299
01/01/2007 Authors: Rowe SM; Varga K; Bebok Z; Li Y; Fan L; Nowartowski K; Prasin J; Barnes S; Clancy JP; Sorscher EJ -
Designed zinc finger nucleases to generate cells carrying the Delta 508 genotype at the endogenous CFTR locus
PEDIATRIC PULMONOLOGY Page(s): 283 - 283
01/01/2007 Authors: Sun HC; Miller JC; Moehle EA; Rock JM; Holmes MC; Rebar EJ; Hong JS; Sorscher EJ; Urnov F -
CFTR molecular assembly monitored by FRET
PEDIATRIC PULMONOLOGY Page(s): 227 - 227
01/01/2007 Authors: Berdiev BK; Tousson A; Oosterveld-Hut HM; Hong JS; Qadri YJ; Pyle LC; Fuller CM; Lukacs GL; Sorscher EJ; Benos DJ -
Novel mediators of persistent neutrophilic inflammation in cystic fibrosis
PEDIATRIC PULMONOLOGY Page(s): 229 - 230
01/01/2007 Authors: Jackson PL; Livraghi A; O'Neal W; Noerager B; Solomon GM; McQuaid DB; Liu G; Gaggar A; Clancy JP; Sorscher EJ -
Develoment of a murine model to investigate the role of TGF beta-1 signaling in cystic fibrosis pathogenesis
PEDIATRIC PULMONOLOGY Page(s): 284 - 284
01/01/2007 Authors: Havasi V; Bartoszewski R; Guimbellot JS; Fortenberry J; Nicola T; Hagood JS; Serra RA; Murphy-Ulrich J; Buckley-Lanier JA; Bedwell DM -
Role of tissue growth factor beta-1 polymorphisms in congenital bilateral absence of the vas deferens
PEDIATRIC PULMONOLOGY Page(s): 276 - 276
01/01/2007 Authors: Havasi V; Rowe SM; Kolettis P; Grangeia A; Carvalho F; Barros A; Sousa M; Dayangac D; Casals T; Pierucci-Alves F -
Hypoxia-induced PGE(2) release from airway cells is mediated by adenosine and A2B adenosine receptors
PEDIATRIC PULMONOLOGY Page(s): 223 - 224
01/01/2007 Authors: Li Y; Fan L; Sorscher EJ; Clancy JP -
Comparison of maneuvers to correct Delta F508 CFTR: Chemical agents and low temperature produce surface targeted protein with similar activation profiles
PEDIATRIC PULMONOLOGY Page(s): 299 - 300
01/01/2007 Authors: Rowe SM; Bebok Z; Varga K; Collawn J; Sorscher EJ -
Implications of R-domain phosphorylation by CFTR potentiators on Delta F508 CFTR channel activity
PEDIATRIC PULMONOLOGY Page(s): 304 - 305
01/01/2007 Authors: Pyle LC; Fan L; Fortenberry J; Sthanam M; Sorscher EJ; Clancy JP; Rowe SM -
Phage biopanning to monitor Delta 508 CFTR folding and surface expression
PEDIATRIC PULMONOLOGY Page(s): 224 - 225
01/01/2007 Authors: Mazur M; Cobb M; Sorscher EJ; Engler J -
Analysis of microrna expression and CFTR repression during hypoxia
PEDIATRIC PULMONOLOGY Page(s): 224 - 224
01/01/2007 Authors: Guimbellot JS; Wen H; Erickson SW; Page GP; Sorscher EJ; Hong JS -
Small molecular correctors increase the surface stability of rescued Delta F508 CFTR
PEDIATRIC PULMONOLOGY Page(s): 288 - 288
01/01/2007 Authors: Varga K; Jurkuvenaite A; Goldstein R; Bartoszewski R; Sorscher EJ; Bebok Z; Collawn J -
Delta F508 CFTR activity in polarizing epithelial cells - Distinct differences in cyclic amp dependent CL transport
PEDIATRIC PULMONOLOGY Page(s): 285 - 285
01/01/2007 Authors: Rowe SM; Bebok Z; Bartoszewski R; Varga K; Collawn J; Sorscher EJ; Clancy JP -
Interregulation of proton-gated Na(+) channel 3 and cystic fibrosis transmembrane conductance regulator.
J Biol Chem Volume: 281 Page(s): 36960 - 36968
12/01/2006 Authors: Su X; Li Q; Shrestha K; Cormet-Boyaka E; Chen L; Smith PR; Sorscher EJ; Benos DJ; Matalon S; Ji H-L -
The non-random distribution of intronless human genes across molecular function categories.
FEBS Lett Volume: 580 Page(s): 4303 - 4305
08/07/2006 Authors: Hill AE; Sorscher EJ -
Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model.
J Mol Med (Berl) Volume: 84 Page(s): 573 - 582
07/01/2006 Authors: Du M; Keeling KM; Fan L; Liu X; Kovas T; Sorscher E; Bedwell DM -
Micro-RNA-like effects of complete intronic sequences.
Front Biosci Volume: 11 Page(s): 1998 - 2006
05/01/2006 Authors: Hill AE; Hong JS; Wen H; Teng L; McPherson DT; McPherson SA; Levasseur DN; Sorscher EJ -
Comparison of Antitumor Activity of Cytosine Deaminase:: Uracil Phosphoribosyl Transferase (CD:: UPRT) and Purine Nucleoside Phosphorylase (PNP) Suicide Genes Using Replicative but Non-Disseminative Adenovirus Vectors
MOLECULAR THERAPY Volume: 13 Page(s): S8 - S8
05/01/2006 Authors: Jaime J; Jabbour N; Parker W; Sorscher E; Nalbantoglu J; Massie B -
Activators of viral gene expression in polarized epithelial monolayers identified by rapid-throughput drug screening.
Gene Ther Volume: 13 Page(s): 781 - 788
05/01/2006 Authors: Sorscher EJ; Harris J; Alexander M; Rottgers A; Hardy K; Ponnazhagan S; Collawn JF; McClintock J; Amsler CD; Webster A -
Leflunomide prevents alveolar fluid clearance inhibition by respiratory syncytial virus.
Am J Respir Crit Care Med Volume: 173 Page(s): 673 - 682
03/15/2006 Authors: Davis IC; Lazarowski ER; Hickman-Davis JM; Fortenberry JA; Chen F-P; Zhao X; Sorscher E; Graves LM; Sullender WM; Matalon S -
Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis.
J Biol Chem Volume: 281 Page(s): 3329 - 3334
02/10/2006 Authors: Jurkuvenaite A; Varga K; Nowotarski K; Kirk KL; Sorscher EJ; Li Y; Clancy JP; Bebok Z; Collawn JF -
Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers.
J Physiol Volume: 569 Page(s): 601 - 615
12/01/2005 Authors: Bebok Z; Collawn JF; Wakefield J; Parker W; Li Y; Varga K; Sorscher EJ; Clancy JP -
Human genome -- from pieces to patterns.
Front Biosci Volume: 10 Page(s): 2576 - 2584
09/01/2005 Authors: Sakharkar MK; Kangueane P; Perumal BS; Chow VTK; Sorscher E; Sakharkar KR; Hill A -
Cystic fibrosis.
N Engl J Med Volume: 352 Page(s): 1992 - 2001
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Increased prevalence of the 5t allele of CFTR in cholestatic liver disease
Volume: 128 Page(s): A773 - A773
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Antibiotic-mediated chemoprotection enhances adaptation of E. coli PNP for herpes simplex virus-based glioma therapy.
Hum Gene Ther Volume: 16 Page(s): 339 - 347
03/01/2005 Authors: Bharara S; Sorscher EJ; Gillespie GY; Lindsey JR; Hong JS; Curlee KV; Allan PW; Gadi VK; Alexander SA; Secrist JA -
Synthesis and biological activity of 2-fluoro adenine and 6-methyl purine nucleoside analogs as prodrugs for suicide gene therapy of cancer.
Volume: 24 Page(s): 881 - 885
01/01/2005 Authors: Silamkoti AV; Allan PW; Hassan AEA; Fowler AT; Sorscher EJ; Parker WB; Secrist JA -
Design and evaluation of 5'-modified nucleoside analogs as prodrugs for an E. coli purine nucleoside phosphorylase mutant.
Volume: 24 Page(s): 387 - 392
01/01/2005 Authors: Parker WB; Allan PW; Ealick SE; Sorscher EJ; Hassan AEA; Silamkoti AV; Fowler AT; Waud WR; Secrist JA -
PNP anticancer gene therapy.
Curr Top Med Chem Volume: 5 Page(s): 1259 - 1274
01/01/2005 Authors: Zhang Y; Parker WB; Sorscher EJ; Ealick SE -
Excellent in vivo bystander activity of fludarabine phosphate against human glioma xenografts that express the escherichia coli purine nucleoside phosphorylase gene.
Cancer Res Volume: 64 Page(s): 6610 - 6615
09/15/2004 Authors: Hong JS; Waud WR; Levasseur DN; Townes TM; Wen H; McPherson SA; Moore BA; Bebok Z; Allan PW; Secrist JA -
Activation of airway cl- secretion in human subjects by adenosine.
Am J Respir Cell Mol Biol Volume: 31 Page(s): 140 - 146
08/01/2004 Authors: Hentchel-Franks K; Lozano D; Eubanks-Tarn V; Cobb B; Fan L; Oster R; Sorscher E; Clancy JP -
Assessment of CFTR localisation in native airway epithelial cells obtained by nasal brushing.
J Cyst Fibros Volume: 3 Suppl 2 Page(s): 43 - 48
08/01/2004 Authors: Harris CM; Mendes F; Dragomir A; Doull IJM; Carvalho-Oliveira I; Bebok Z; Clancy JP; Eubanks V; Sorscher EJ; Roomans GM -
Common structural patterns in human genes.
Bioinformatics Volume: 20 Page(s): 1632 - 1635
07/10/2004 Authors: Hill A; Sorscher E -
Hepatocyte growth factor stimulates adenoviral-mediated gene transfer across the apical membrane of epithelial cells.
J Gene Med Volume: 6 Page(s): 624 - 630
06/01/2004 Authors: Burrus JK; Matheson WD; Hong JS; Sorscher EJ; Balkovetz DF -
Efficient intracellular processing of the endogenous cystic fibrosis transmembrane conductance regulator in epithelial cell lines.
J Biol Chem Volume: 279 Page(s): 22578 - 22584
05/21/2004 Authors: Varga K; Jurkuvenaite A; Wakefield J; Hong JS; Guimbellot JS; Venglarik CJ; Niraj A; Mazur M; Sorscher EJ; Collawn JF -
Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry.
J Biol Chem Volume: 279 Page(s): 10720 - 10729
03/12/2004 Authors: Zsembery A; Fortenberry JA; Liang L; Bebok Z; Tucker TA; Boyce AT; Braunstein GM; Welty E; Bell PD; Sorscher EJ -
Tumor sensitization to purine analogs by E. coli PNP.
Methods Mol Med Volume: 90 Page(s): 223 - 245
01/01/2004 Authors: Curlee KV; Parker WB; Sorscher EJ -
Designer gene therapy using an Escherichia coli purine nucleoside phosphorylase/prodrug system.
Chem Biol Volume: 10 Page(s): 1173 - 1181
12/01/2003 Authors: Bennett EM; Anand R; Allan PW; Hassan AEA; Hong JS; Levasseur DN; McPherson DT; Parker WB; Secrist JA; Sorscher EJ -
Activation of chloride secretion in cystic fibrosis cells and tissues by the substituted imidazole SRI 2931.
Biochemistry Volume: 42 Page(s): 13241 - 13249
11/18/2003 Authors: Jones JR; Schwiebert EM; DuVall MD; Venglarik CJ; Wen H; Kovacs T; Mazur M; Clancy JP; Braunstein G; Bates E -
Adenosine receptors and phosphodiesterase inhibitors stimulate Cl- secretion in Calu-3 cells.
Am J Respir Cell Mol Biol Volume: 29 Page(s): 410 - 418
09/01/2003 Authors: Cobb BR; Fan L; Kovacs TE; Sorscher EJ; Clancy JP -
Membrane transplantation to correct integral membrane protein defects.
J Mol Med (Berl) Volume: 81 Page(s): 511 - 520
08/01/2003 Authors: Curlee KV; Hong JS; Clancy JP; King SA; Hunter E; Berdiev B; Benos D; Sommerfelt MA; Sorscher EJ; Sakalian M -
Excellent in vivo bystander activity of fludarabine phosphate against D54 tumors that express the E. coli purine nucleoside phosphorylase suicide gene
Volume: 7 Page(s): S277 - S278
05/01/2003 Authors: Parker WB; Hong J; Allan PW; Sorscher EJ; Lavasseur D; Townes TM; Secrist JA; Waud WR -
A long-acting suicide gene toxin, 6-methylpurine, inhibits slow growing tumors after a single administration.
J Pharmacol Exp Ther Volume: 304 Page(s): 1280 - 1284
03/01/2003 Authors: Gadi VK; Alexander SD; Waud WR; Allan PW; Parker WB; Sorscher EJ -
A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA.
Proc Natl Acad Sci U S A Volume: 100 Page(s): 342 - 346
01/07/2003 Authors: Naren AP; Cobb B; Li C; Roy K; Nelson D; Heda GD; Liao J; Kirk KL; Sorscher EJ; Hanrahan J -
Elevation of hepatic sulfotransferase (SULT) activities in mice with resistance to cystic fibrosis (CF)
Volume: 35 Page(s): 197 - 197
01/01/2003 Authors: Falany JL; Greer H; Sorscher EJ; Falany CN -
Antitumor activity of 2-fluoro-2'-deoxyadenosine against tumors that express Escherichia coli purine nucleoside phosphorylase.
Cancer Gene Ther Volume: 10 Page(s): 23 - 29
01/01/2003 Authors: Parker WB; Allan PW; Hassan AEA; Secrist JA; Sorscher EJ; Waud WR -
Ablation of internalization signals in the carboxyl-terminal tail of the cystic fibrosis transmembrane conductance regulator enhances cell surface expression.
J Biol Chem Volume: 277 Page(s): 49952 - 49957
12/20/2002 Authors: Peter K; Varga K; Bebok Z; McNicholas-Bevensee CM; Schwiebert L; Sorscher EJ; Schwiebert EM; Collawn JF -
Reactive oxygen nitrogen species decrease cystic fibrosis transmembrane conductance regulator expression and cAMP-mediated Cl- secretion in airway epithelia.
J Biol Chem Volume: 277 Page(s): 43041 - 43049
11/08/2002 Authors: Bebok Z; Varga K; Hicks JK; Venglarik CJ; Kovacs T; Chen L; Hardiman KM; Collawn JF; Sorscher EJ; Matalon S -
Role for phospholipid interactions in the trafficking defect of Delta F508-CFTR.
Biochemistry Volume: 41 Page(s): 11161 - 11170
09/17/2002 Authors: Eidelman O; BarNoy S; Razin M; Zhang J; McPhie P; Lee G; Huang Z; Sorscher EJ; Pollard HB -
Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.
J Mol Med (Berl) Volume: 80 Page(s): 595 - 604
09/01/2002 Authors: Du M; Jones JR; Lanier J; Keeling KM; Lindsey JR; Tousson A; Bebk Z; Whitsett JA; Dey CR; Colledge WH -
Elevation of hepatic sulphotransferase activities in mice with resistance to cystic fibrosis.
Biochem J Volume: 364 Page(s): 115 - 120
05/15/2002 Authors: Falany JL; Greer H; Kovacs T; Sorscher EJ; Falany CN -
Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis.
J Pediatr Volume: 140 Page(s): 439 - 444
04/01/2002 Authors: Eubanks V; Koppersmith N; Wooldridge N; Clancy JP; Lyrene R; Arani RB; Lee J; Moldawer L; Atchison J; Sorscher EJ -
Chronic iNOS inhibition blocks amiloride-sensitivity of respiratory epithelia in mice in vivo and in vitro
FASEB JOURNAL Volume: 16 Page(s): A403 - A403
03/20/2002 Authors: Hardiman KM; Fortenberry JA; Sorscher E; Matalon S -
Enhancement of adenovirus-mediated gene transfer in lungs and epithelial cells by EGTA.
Chest Volume: 121 Page(s): 35S
03/01/2002 Authors: Myles C; Sorscher E; Matalon S -
Design of gene therapy clinical trials in CF patients.
Methods Mol Med Volume: 70 Page(s): 575 - 584
01/01/2002 Authors: Curlee KV; Sorscher EJ -
A(2) adenosine receptors regulate CFTR through PKA and PLA(2).
Am J Physiol Lung Cell Mol Physiol Volume: 282 Page(s): L12 - L25
01/01/2002 Authors: Cobb BR; Ruiz F; King CM; Fortenberry J; Greer H; Kovacs T; Sorscher EJ; Clancy JP -
Chlorzoxazone or 1-EBIO increases Na(+) absorption across cystic fibrosis airway epithelial cells.
Am J Physiol Lung Cell Mol Physiol Volume: 281 Page(s): L1123 - L1129
11/01/2001 Authors: Gao L; Yankaskas JR; Fuller CM; Sorscher EJ; Matalon S; Forman HJ; Venglarik CJ -
Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis.
Am J Respir Crit Care Med Volume: 163 Page(s): 1683 - 1692
06/01/2001 Authors: Clancy JP; Bebk Z; Ruiz F; King C; Jones J; Walker L; Greer H; Hong J; Wing L; Macaluso M -
A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis.
Hum Gene Ther Volume: 12 Page(s): 751 - 761
05/01/2001 Authors: Ruiz FE; Clancy JP; Perricone MA; Bebok Z; Hong JS; Cheng SH; Meeker DP; Young KR; Schoumacher RA; Weatherly MR -
In vivo sensitization of ovarian tumors to chemotherapy by expression of E. coli purine nucleoside phosphorylase in a small fraction of cells.
Gene Ther Volume: 7 Page(s): 1738 - 1743
10/01/2000 Authors: Gadi VK; Alexander SD; Kudlow JE; Allan P; Parker WB; Sorscher EJ -
R-domain interactions with distal regions of CFTR lead to phosphorylation and activation.
Biochemistry Volume: 39 Page(s): 9868 - 9875
08/15/2000 Authors: King SA; Sorscher EJ -
Augmenation of adenovirus mediated gene transfer in epithelial cells by calcium chelation
FASEB JOURNAL Volume: 14 Page(s): A651 - A651
03/15/2000 Authors: Myles C; Sorscher E; Matalon S -
Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera
MOLECULAR BIOLOGY OF THE CELL Volume: 10 Page(s): 86A - 86A
11/01/1999 Authors: Garcia-Mata R; Bebok Z; Sorscher EJ; Sztul ES -
Epithelial P2X purinergic receptor channel expression and function.
J Clin Invest Volume: 104 Page(s): 875 - 884
10/01/1999 Authors: Taylor AL; Schwiebert LM; Smith JJ; King C; Jones JR; Sorscher EJ; Schwiebert EM -
CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands.
Am J Physiol Volume: 277 Page(s): L694 - L699
10/01/1999 Authors: Ballard ST; Trout L; Bebk Z; Sorscher EJ; Crews A -
Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera.
J Cell Biol Volume: 146 Page(s): 1239 - 1254
09/20/1999 Authors: Garca-Mata R; Bebk Z; Sorscher EJ; Sztul ES -
Components of human papillomavirus that activate transcription and support plasmid replication in human airway cells.
Am J Respir Cell Mol Biol Volume: 20 Page(s): 1001 - 1006
05/01/1999 Authors: Gadi VK; Zou N; Liu JS; Cheng S; Broker TR; Sorscher EJ; Chow LT -
Effects of the delta F508 mutation on domain interactions and the processing of CFTR.
FASEB JOURNAL Volume: 13 Page(s): A730 - A730
03/15/1999 Authors: King SA; Sorscher EJ -
The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis.
EMBO J Volume: 18 Page(s): 1492 - 1505
03/15/1999 Authors: Meacham GC; Lu Z; King S; Sorscher E; Tousson A; Cyr DM -
CFTR processing and degradation at the endoplasmic reticulum
FASEB JOURNAL Volume: 13 Page(s): A730 - A730
03/15/1999 Authors: Bebok Z; Hicks JK; King S; Hong JS; Sorscher EJ -
Adenosine elevates cAMP in IB-3 and COS-7 cells, activates surface localized delta F508 CFTR through an A2b adenosine receptor and stimulates Cl- secretion across the murine nasal mucosa.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE Volume: 159 Page(s): A678 - A678
03/01/1999 Authors: Jones J; Ruiz FE; King C; Cobb B; Sorscher EJ; Clancy JP -
Adenosine and its nucleotides activate wild-type and R117H CFTR through an A2B receptor-coupled pathway.
Am J Physiol Volume: 276 Page(s): C361 - C369
02/01/1999 Authors: Clancy JP; Ruiz FE; Sorscher EJ -
Gene therapy of cancer: activation of nucleoside prodrugs with E. coli purine nucleoside phosphorylase.
Volume: 18 Page(s): 745 - 757
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The mechanism underlying cystic fibrosis transmembrane conductance regulator transport from the endoplasmic reticulum to the proteasome includes Sec61beta and a cytosolic, deglycosylated intermediary.
J Biol Chem Volume: 273 Page(s): 29873 - 29878
11/06/1998 Authors: Bebk Z; Mazzochi C; King SA; Hong JS; Sorscher EJ -
Hdj-2 is an ER localized Hsp40 chaperone protein capable of directing cytosolic Hsp70 to facilitate early steps in CFTR biogenesis
MOLECULAR BIOLOGY OF THE CELL Volume: 9 Page(s): 347A - 347A
11/01/1998 Authors: Meacham G; Lu Z; Tousson A; King S; Sorscher E; Cyr DM -
Cystic fibrosis transmembrane conductance regulator (CFTR) nucleotide-binding domain 1 (NBD-1) and CFTR truncated within NBD-1 target to the epithelial plasma membrane and increase anion permeability.
Biochemistry Volume: 37 Page(s): 15222 - 15230
10/27/1998 Authors: Clancy JP; Hong JS; Bebk Z; King SA; Demolombe S; Bedwell DM; Sorscher EJ -
Activation of F508 CFTR in an epithelial monolayer.
Am J Physiol Cell Physiol Volume: 275 Page(s): C599 - C607
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Activation of DeltaF508 CFTR in an epithelial monolayer.
Am J Physiol Volume: 275 Page(s): C599 - C607
08/01/1998 Authors: Bebk Z; Venglarik CJ; Pnczl Z; Jilling T; Kirk KL; Sorscher EJ -
Cell to cell contact is not required for bystander cell killing by Escherichia coli purine nucleoside phosphorylase.
J Biol Chem Volume: 273 Page(s): 2322 - 2328
01/23/1998 Authors: Hughes BW; King SA; Allan PW; Parker WB; Sorscher EJ -
Recombinant synthesis of cystic fibrosis transmembrane conductance regulator and functional nucleotide-binding domains.
Methods Enzymol Volume: 292 Page(s): 686 - 697
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Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line.
Nat Med Volume: 3 Page(s): 1280 - 1284
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Purification, characterization, and expression of CFTR nucleotide-binding domains.
J Bioenerg Biomembr Volume: 29 Page(s): 475 - 482
10/01/1997 Authors: Clancy JP; Bebk Z; Sorscher EJ -
In vivo gene therapy of cancer with E. coli purine nucleoside phosphorylase.
Hum Gene Ther Volume: 8 Page(s): 1637 - 1644
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Adenoviral gene delivery elicits distinct pulmonary-associated T helper cell responses to the vector and to its transgene.
J Immunol Volume: 159 Page(s): 685 - 693
07/15/1997 Authors: van Ginkel FW; McGhee JR; Liu C; Simecka JW; Yamamoto M; Frizzell RA; Sorscher EJ; Kiyono H; Pascual DW -
8-cyclopentyl-1,3-dipropylxanthine and other xanthines differentially bind to the wild-type and delta F508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator.
Biochemistry Volume: 36 Page(s): 6455 - 6461
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Modulation of adenovirus-mediated gene transfer by nitric oxide.
Am J Respir Cell Mol Biol Volume: 16 Page(s): 501 - 509
05/01/1997 Authors: Haddad IY; Sorscher EJ; Garver RI; Hong J; Tzeng E; Matalon S -
Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR.
Am J Respir Crit Care Med Volume: 155 Page(s): 1684 - 1689
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Efficiency of plasmid delivery and expression after lipid-mediated gene transfer to human cells in vitro.
J Pharmacol Exp Ther Volume: 279 Page(s): 1462 - 1469
12/01/1996 Authors: Bebk Z; Abai AM; Dong JY; King SA; Kirk KL; Berta G; Hughes BW; Kraft AS; Burgess SW; Shaw W -
A method for the rapid detection of recombinant CFTR during gene therapy in cystic fibrosis.
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08/01/1996 Authors: Demolombe S; Bar I; Bebok Z; Clancy JP; Sorscher EJ; Thomas-Soumarmon A; Pavirani A; Escande D -
Delta F508-CFTR expression in polarized LLC-PK1 cells.
FASEB JOURNAL Volume: 10 Page(s): 437 - 437
03/08/1996 Authors: Venglarik CJ; Sorscher EJ; Senyk O; Benos DJ; Kirk KL; Jilling T -
cAMP and Cl--dependent regulation of apical protein secretion by colonic epithelial cells.
FASEB JOURNAL Volume: 10 Page(s): 538 - 538
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Interactions of the NBF-1 domain of CFTR with phospholipid membranes.
BIOPHYSICAL JOURNAL Volume: 70 Page(s): WP249 - WP249
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Development of drug targeting based on recombinant expression of the chicken avidin gene.
J Drug Target Volume: 4 Page(s): 41 - 49
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Interaction between cystic fibrosis transmembrane conductance regulator and outwardly rectified chloride channels.
J Biol Chem Volume: 270 Page(s): 29194 - 29200
12/08/1995 Authors: Jovov B; Ismailov II; Berdiev BK; Fuller CM; Sorscher EJ; Dedman JR; Kaetzel MA; Benos DJ -
Bystander killing of melanoma cells using the human tyrosinase promoter to express the Escherichia coli purine nucleoside phosphorylase gene.
Cancer Res Volume: 55 Page(s): 3339 - 3345
08/01/1995 Authors: Hughes BW; Wells AH; Bebok Z; Gadi VK; Garver RI; Parker WB; Sorscher EJ -
Severe phenotype in mice with termination mutation in exon 2 of cystic fibrosis gene.
Somat Cell Mol Genet Volume: 21 Page(s): 177 - 187
05/01/1995 Authors: Hasty P; O'Neal WK; Liu KQ; Morris AP; Bebok Z; Shumyatsky GB; Jilling T; Sorscher EJ; Bradley A; Beaudet AL -
Purification of recombinant protein derived from the baculovirus expression system using glutathione affinity agarose.
Methods Mol Biol Volume: 39 Page(s): 337 - 348
01/01/1995 Authors: Sorscher EJ; Sommerfelt MA -
Cationic lipids for reporter gene and CFTR transfer to rat pulmonary epithelium.
Gene Ther Volume: 2 Page(s): 38 - 49
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Informed consent to participate in a research study -- gene therapy for cystic fibrosis using cationic liposome mediated gene transfer: a phase I trial of safety and efficacy in the nasal airway.
Hum Gene Ther Volume: 5 Page(s): 1271 - 1277
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Gene therapy for cystic fibrosis using cationic liposome mediated gene transfer: a phase I trial of safety and efficacy in the nasal airway.
Hum Gene Ther Volume: 5 Page(s): 1259 - 1277
10/01/1994 Authors: Sorscher EJ; Logan JJ; Frizzell RA; Lyrene RK; Bebok Z; Dong JY; Duvall MD; Felgner PL; Matalon S; Walker L -
Cystic fibrosis transmembrane conductance regulator mutations that disrupt nucleotide binding.
J Clin Invest Volume: 94 Page(s): 228 - 236
07/01/1994 Authors: Logan J; Hiestand D; Daram P; Huang Z; Muccio DD; Hartman J; Haley B; Cook WJ; Sorscher EJ -
Tumor cell bystander killing in colonic carcinoma utilizing the Escherichia coli DeoD gene to generate toxic purines.
Gene Ther Volume: 1 Page(s): 233 - 238
07/01/1994 Authors: Sorscher EJ; Peng S; Bebok Z; Allan PW; Bennett LL; Parker WB -
Expression and polarized targeting of a rab3 isoform in epithelial cells.
J Cell Biol Volume: 125 Page(s): 583 - 594
05/01/1994 Authors: Weber E; Berta G; Tousson A; St John P; Green MW; Gopalokrishnan U; Jilling T; Sorscher EJ; Elton TS; Abrahamson DR -
USE OF 2-N3ATP TO IDENTIFY THE SITE OF ATP INTERACTION ON NUCLEOTIDE-BINDING DOMAIN-2 FROM THE CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR
FASEB JOURNAL Volume: 8 Page(s): A1356 - A1356
04/19/1994 Authors: HIESTAND DM; SORSCHER EJ; HALEY BE -
Strategy for achieving selective killing of carcinomas.
Gene Ther Volume: 1 Page(s): 46 - 50
01/01/1994 Authors: Garver RI; Goldsmith KT; Rodu B; Hu PC; Sorscher EJ; Curiel DT -
Use of fluorescence-activated cell sorting for rapid isolation of insect cells harboring recombinant baculovirus.
Methods Cell Biol Volume: 42 Pt B Page(s): 563 - 574
01/01/1994 Authors: Sommerfelt MA; Sorscher EJ -
One-step affinity isolation of recombinant protein using the baculovirus/insect cell expression system.
Protein Expr Purif Volume: 4 Page(s): 95 - 100
04/01/1993 Authors: Peng S; Sommerfelt M; Logan J; Huang Z; Jilling T; Kirk K; Hunter E; Sorscher E -
EXPRESSION AND POLARIZED TARGETING OF A RAB-3 ISOFORM IN SECRETORY EPITHELIAL-CELLS
FASEB JOURNAL Volume: 7 Page(s): A809 - A809
02/23/1993 Authors: KIRK KL; WEAVERGREEN M; BERTA G; WEBER E; JILLING T; SORSCHER E; ELTON TS -
3-ISOBUTYL-1-METHYLXANTHINE (IBMX) BLOCKS BINDING OF ATP ANALOGS TO THE CFTR 1ST NUCLEOTIDE BINDING DOMAIN (NBD-1)
FASEB JOURNAL Volume: 7 Page(s): A426 - A426
02/19/1993 Authors: LOGAN J; HIESTAND D; HUANG Z; MUCCIO D; HALEY B; SORSCHER EJ -
MUTATIONS IN CFTR NUCLEOTIDE BINDING DOMAINS (NBDS) DISRUPT TRINITROPHENYL ATP BINDING
FASEB JOURNAL Volume: 7 Page(s): A426 - A426
02/19/1993 Authors: SORSCHER EJ; DARAM P; HIESTAND D; HUANG Z; PENG S; MUCCIO D; HALEY B; LOGAN J -
Rapid purification of recombinant baculovirus using fluorescence-activated cell sorting.
Biotechniques Volume: 14 Page(s): 274 - 277
02/01/1993 Authors: Peng S; Sommerfelt MA; Berta G; Berry AK; Kirk KL; Hunter E; Sorscher EJ -
Regulation of plasma membrane recycling by CFTR.
Science Volume: 256 Page(s): 530 - 532
04/24/1992 Authors: Bradbury NA; Jilling T; Berta G; Sorscher EJ; Bridges RJ; Kirk KL -
Recombinant synthesis, purification, and nucleotide binding characteristics of the first nucleotide binding domain of the cystic fibrosis gene product.
J Biol Chem Volume: 267 Page(s): 6455 - 6458
04/05/1992 Authors: Hartman J; Huang Z; Rado TA; Peng S; Jilling T; Muccio DD; Sorscher EJ -
Affinity purification of insoluble recombinant fusion proteins containing glutathione-S-transferase.
Biotechnol Bioeng Volume: 39 Page(s): 828 - 832
04/05/1992 Authors: Hartman J; Daram P; Frizzell RA; Rado T; Benos DJ; Sorscher EJ -
Intrinsic anion channel activity of the recombinant first nucleotide binding fold domain of the cystic fibrosis transmembrane regulator protein.
Proc Natl Acad Sci U S A Volume: 89 Page(s): 1539 - 1543
03/01/1992 Authors: Arispe N; Rojas E; Hartman J; Sorscher EJ; Pollard HB -
IDENTIFICATION OF A MEMBRANE-PROTEIN FROM T84 CELLS USING ANTIBODIES MADE AGAINST A DIDS-BINDING PEPTIDE
AMERICAN JOURNAL OF PHYSIOLOGY Volume: 262 Page(s): C136 - C147
01/01/1992 Authors: SORSCHER EJ; FULLER CM; BRIDGES RJ; TOUSSON A; MARCHASE RB; BRINKLEY BR; FRIZZELL RA; BENOS DJ -
Identification of a membrane protein from T84 cells using antibodies made against a DIDS-binding peptide.
Am J Physiol Volume: 262 Page(s): C136 - C147
01/01/1992 Authors: Sorscher EJ; Fuller CM; Bridges RJ; Tousson A; Marchase RB; Brinkley BR; Frizzell RA; Benos DJ -
Antisense oligodeoxynucleotide to the cystic fibrosis gene inhibits anion transport in normal cultured sweat duct cells.
Proc Natl Acad Sci U S A Volume: 88 Page(s): 7759 - 7762
09/01/1991 Authors: Sorscher EJ; Kirk KL; Weaver ML; Jilling T; Blalock JE; LeBoeuf RD -
Diagnosis of genetic disease by primer-specified restriction map modification, with application to cystic fibrosis and retinitis pigmentosa.
Lancet Volume: 337 Page(s): 1115 - 1118
05/11/1991 Authors: Sorscher EJ; Huang Z -
PURIFICATION OF A RECOMBINANT FUSION PROTEIN CONTAINING THE 1ST NUCLEOTIDE BINDING FOLD OF THE CYSTIC-FIBROSIS GENE-PRODUCT AND EVIDENCE THAT IT BINDS ATP
FASEB JOURNAL Volume: 5 Page(s): A1760 - A1760
03/19/1991 Authors: HARTMAN J; FRIZZELL R; RADO T; BENOS D; SORSCHER E -
Immunochemical localization of amiloride-sensitive sodium channels in sodium-transporting epithelia.
J Cell Sci Volume: 93 ( Pt 2) Page(s): 349 - 362
06/01/1989 Authors: Tousson A; Alley CD; Sorscher EJ; Brinkley BR; Benos DJ -
Immunocytochemical localization of Na+ channels in rat kidney medulla.
Am J Physiol Volume: 256 Page(s): F366 - F369
02/01/1989 Authors: Brown D; Sorscher EJ; Ausiello DA; Benos DJ -
Antibodies against purified epithelial sodium channel protein from bovine renal papilla.
Am J Physiol Volume: 255 Page(s): C835 - C843
12/01/1988 Authors: Sorscher EJ; Accavitti MA; Keeton D; Steadman E; Frizzell RA; Benos DJ -
Phosphorylation of a single subunit of the epithelial Na+ channel protein following vasopressin treatment of A6 cells.
J Biol Chem Volume: 263 Page(s): 13875 - 13879
09/25/1988 Authors: Sariban-Sohraby S; Sorscher EJ; Brenner BM; Benos DJ -
Cystic fibrosis: a disorder of calcium-stimulated secretion and transepithelial sodium transport?
Lancet Volume: 1 Page(s): 368 - 370
02/13/1982 Authors: Sorscher EJ; Breslow JL