| Year |
Citation |
Score |
| 2024 |
Liou TG, Argel N, Asfour F, Brown PS, Chatfield BA, Cox DR, Daines CL, Durham D, Francis JA, Glover B, Helms M, Heynekamp T, Hoidal JR, Jensen JL, Kartsonaki C, ... ... Clancy JP, et al. Airway inflammation accelerates pulmonary exacerbations in cystic fibrosis. Iscience. 27: 108835. PMID 38384849 DOI: 10.1016/j.isci.2024.108835 |
0.363 |
|
| 2023 |
Hisert KB, Birket SE, Clancy JP, Downey DG, Engelhardt JF, Fajac I, Gray RD, Lachowicz-Scroggins ME, Mayer-Hamblett N, Thibodeau P, Tuggle KL, Wainwright CE, De Boeck K. Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy. The Lancet. Respiratory Medicine. 11: 916-931. PMID 37699420 DOI: 10.1016/S2213-2600(23)00324-7 |
0.343 |
|
| 2023 |
Kramer EL, Hudock KM, Davidson CR, Clancy JP. CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity. Respiratory Research. 24: 198. PMID 37568151 DOI: 10.1186/s12931-023-02495-2 |
0.364 |
|
| 2023 |
Putman MS, Norris AW, Hull RL, Rickels MR, Sussel L, Blackman SM, Chan CL, Ode KL, Daley T, Stecenko AA, Moran A, Helmick MJ, Cray S, Alvarez JA, Stallings VA, ... ... Clancy JP, et al. Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes. Diabetes Care. PMID 37125948 DOI: 10.2337/dc23-0380 |
0.309 |
|
| 2023 |
Putman MS, Norris AW, Hull RL, Rickels MR, Sussel L, Blackman SM, Chan CL, Ode KL, Daley T, Stecenko AA, Moran A, Helmick MJ, Cray S, Alvarez JA, Stallings VA, ... ... Clancy JP, et al. Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes. Diabetes. PMID 37125945 DOI: 10.2337/db22-0949 |
0.309 |
|
| 2023 |
Nichols DP, Morgan SJ, Skalland M, Vo AT, Van Dalfsen JM, Singh SB, Ni W, Hoffman LR, McGeer K, Heltshe SL, Clancy JP, Rowe SM, Jorth PK, Singh PK. Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density but lung infections generally persist. The Journal of Clinical Investigation. PMID 36976651 DOI: 10.1172/JCI167957 |
0.386 |
|
| 2022 |
Britto CJ, Ratjen F, Clancy JP. Emerging Approaches to Monitor and Modify Care in the Era of Cystic Fibrosis Transmembrane Conductance Regulators. Clinics in Chest Medicine. 43: 631-646. PMID 36344071 DOI: 10.1016/j.ccm.2022.06.006 |
0.337 |
|
| 2022 |
Gabel ME, Wang H, Gelfond D, Roach C, Rowe SM, Clancy JP, Sagel SD, Borowitz D. Changes in Glucose Breath Test in Cystic Fibrosis Patients Treated with one Month of Lumacaftor/ivacaftor. Journal of Pediatric Gastroenterology and Nutrition. PMID 35442228 DOI: 10.1097/MPG.0000000000003459 |
0.374 |
|
| 2021 |
Gecili E, Brokamp C, Palipana A, Huang R, Andrinopoulou ER, Pestian T, Rasnick E, Keogh RH, Ni Y, Clancy JP, Ryan P, Szczesniak RD. Seasonal variation of lung function in cystic fibrosis: longitudinal modeling to compare a Midwest US cohort to international populations. The Science of the Total Environment. 776. PMID 35125553 DOI: 10.1016/j.scitotenv.2021.145905 |
0.302 |
|
| 2021 |
Gecili E, Su W, Brokamp C, Andrinopoulou ER, Iii FJL, Pestian T, Clancy JP, Solomon GM, Brewington JJ, Szczesniak RD. Rapid cystic fibrosis lung-function decline and in-vitro CFTR modulation. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 33958278 DOI: 10.1016/j.jcf.2021.04.001 |
0.352 |
|
| 2021 |
Arora K, Yang F, Brewington JJ, McPhail G, Cortez AR, Sundaram N, Ramananda Y, Ogden H, Helmrath MA, Clancy JP, Naren AP. Patient personalized translational tools in Cystic fibrosis to transform data from bench to bed-side and back. American Journal of Physiology. Gastrointestinal and Liver Physiology. PMID 33949881 DOI: 10.1152/ajpgi.00095.2021 |
0.36 |
|
| 2020 |
Sagel SD, Khan U, Heltshe SL, Clancy JP, Borowitz D, Gelfond D, Donaldson SH, Moran A, Ratjen F, VanDalfsen JM, Rowe SM. Clinical Effectiveness of Lumacaftor/Ivacaftor in Cystic Fibrosis Patients Homozygous for F508del-CFTR. Annals of the American Thoracic Society. PMID 32644818 DOI: 10.1513/Annalsats.202002-144Oc |
0.485 |
|
| 2020 |
Kasam RK, Gajjala PR, Jegga AG, Courtney JA, Randell SH, Kramer EL, Clancy JP, Madala SK. Fibrocyte accumulation in the lungs of cystic fibrosis patients. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 32593509 DOI: 10.1016/J.Jcf.2020.06.011 |
0.477 |
|
| 2020 |
Gardner AI, Haq IJ, Simpson AJ, Becker KA, Gallagher J, Saint-Criq V, Verdon B, Mavin E, Trigg A, Gray MA, Koulman A, McDonnell MJ, Fisher AJ, Kramer EL, Clancy JP, et al. Recombinant Acid Ceramidase Reduces Inflammation and Infection in Cystic Fibrosis. American Journal of Respiratory and Critical Care Medicine. PMID 32569477 DOI: 10.1164/Rccm.202001-0180Oc |
0.422 |
|
| 2020 |
Szczesniak RD, Pestian T, Duan LL, Li D, Stamper S, Ferrara B, Kramer E, Clancy JP, Grossoehme D. Data driven decision making to characterize clinical personas of parents of children with cystic fibrosis: a mixed methods study. Bmc Pulmonary Medicine. 20: 174. PMID 32552880 DOI: 10.1186/S12890-020-01202-X |
0.352 |
|
| 2020 |
Shaw M, Khan U, Clancy JP, Donaldson SH, Sagel SD, Rowe SM, Ratjen F. Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 32513528 DOI: 10.1016/J.Jcf.2020.05.010 |
0.342 |
|
| 2020 |
Andrinopoulou ER, Clancy JP, Szczesniak RD. Multivariate joint modeling to identify markers of growth and lung function decline that predict cystic fibrosis pulmonary exacerbation onset. Bmc Pulmonary Medicine. 20: 142. PMID 32429862 DOI: 10.1186/S12890-020-1177-Z |
0.447 |
|
| 2020 |
Thomen RP, Walkup LL, Roach DJ, Higano N, Schapiro A, Brody A, Clancy JP, Cleveland ZI, Woods JC. Regional Structure-function in Cystic Fibrosis Lung Disease Using Hyperpolarized Xe and Ultrashort Echo Magnetic Resonance Imaging. American Journal of Respiratory and Critical Care Medicine. PMID 32243189 DOI: 10.1164/Rccm.202001-0031Le |
0.357 |
|
| 2020 |
Kramer EL, Madala SK, Hudock KM, Davidson C, Clancy JP. Subacute TGFβ Exposure Drives Airway Hyperresponsiveness in CF Mice through the PI3K Pathway. American Journal of Respiratory Cell and Molecular Biology. PMID 31922900 DOI: 10.1165/rcmb.2019-0158OC |
0.386 |
|
| 2019 |
Sagel SD, Wagner BD, Ziady A, Kelley T, Clancy JP, Narvaez-Rivas M, Pilewski J, Joseloff E, Sha W, Zelnick L, Setchell KDR, Heltshe SL, Muhlebach MS. Utilizing centralized biorepository samples for biomarkers of cystic fibrosis lung disease severity. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 31870630 DOI: 10.1016/J.Jcf.2019.12.007 |
0.439 |
|
| 2019 |
Woods JC, Wild JM, Wielpütz MO, Clancy JP, Hatabu H, Kauczor HU, van Beek EJR, Altes TA. Current state of the art MRI for the longitudinal assessment of cystic fibrosis. Journal of Magnetic Resonance Imaging : Jmri. PMID 31846139 DOI: 10.1002/Jmri.27030 |
0.402 |
|
| 2019 |
Szczesniak RD, Su W, Brokamp C, Keogh RH, Pestian JP, Seid M, Diggle PJ, Clancy JP. Dynamic predictive probabilities to monitor rapid cystic fibrosis disease progression. Statistics in Medicine. PMID 31816119 DOI: 10.1002/Sim.8443 |
0.374 |
|
| 2019 |
Bilton D, Pressler T, Fajac I, Clancy JP, Sands D, Minic P, Cipolli M, Galeva I, Solé A, Quittner AL, Liu K, McGinnis JP, Eagle G, Gupta R, Konstan MW, et al. Amikacin liposome inhalation suspension for chronic Pseudomonas aeruginosa infection in cystic fibrosis. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 31451351 DOI: 10.1016/J.Jcf.2019.08.001 |
0.439 |
|
| 2019 |
Theprungsirikul J, Skopelja-Gardner S, Meagher RE, Clancy JP, Zemanick ET, Ashare A, Rigby WFC. Dissociation of systemic and mucosal autoimmunity in cystic fibrosis. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 31262645 DOI: 10.1016/J.Jcf.2019.06.006 |
0.406 |
|
| 2019 |
Borcherding DC, Siefert ME, Lin S, Brewington J, Sadek H, Clancy JP, Plafker SM, Ziady AG. Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia. The Journal of Clinical Investigation. 130. PMID 31145101 DOI: 10.1172/Jci96273 |
0.471 |
|
| 2019 |
Liou TG, Adler FR, Argel N, Asfour F, Brown PS, Chatfield BA, Daines CL, Durham D, Francis JA, Glover B, Heynekamp T, Hoidal JR, Jensen JL, Keogh R, Kopecky CM, ... ... Clancy JP, et al. Prospective multicenter randomized patient recruitment and sample collection to enable future measurements of sputum biomarkers of inflammation in an observational study of cystic fibrosis. Bmc Medical Research Methodology. 19: 88. PMID 31027503 DOI: 10.1186/S12874-019-0705-0 |
0.406 |
|
| 2019 |
Jorth P, Ehsan Z, Rezayat A, Caldwell E, Pope C, Brewington JJ, Goss CH, Benscoter D, Clancy JP, Singh PK. Direct Lung Sampling Indicates That Established Pathogens Dominate Early Infections in Children with Cystic Fibrosis. Cell Reports. 27: 1190-1204.e3. PMID 31018133 DOI: 10.1016/J.Celrep.2019.03.086 |
0.461 |
|
| 2019 |
Southern KW, Clancy JP, Ranganathan S. Aerosolized agents for airway clearance in cystic fibrosis. Pediatric Pulmonology. PMID 30884217 DOI: 10.1002/Ppul.24306 |
0.412 |
|
| 2019 |
Szczesniak RD, Brokamp C, Su W, Mcphail GL, Pestian J, Clancy JP. Improving Detection of Rapid Cystic Fibrosis Disease Progression-Early Translation of a Predictive Algorithm Into a Point-of-Care Tool. Ieee Journal of Translational Engineering in Health and Medicine. 7: 2800108. PMID 30800534 DOI: 10.1109/Jtehm.2018.2878534 |
0.36 |
|
| 2018 |
Wu AC, Kiley JP, Noel PJ, Amur S, Burchard EG, Clancy JP, Galanter J, Inada M, Jones TK, Kropski JA, Loyd JE, Nogee LM, Raby BA, Rogers AJ, Schwartz DA, et al. Current Status and Future Opportunities in Lung Precision Medicine Research with a Focus on Biomarkers. An American Thoracic Society/National Heart, Lung, and Blood Institute Research Statement. American Journal of Respiratory and Critical Care Medicine. 198: e116-e136. PMID 30640517 DOI: 10.1164/Rccm.201810-1895St |
0.34 |
|
| 2018 |
Fenker DE, McDaniel CT, Panmanee W, Panos RJ, Sorscher EJ, Sabusap C, Clancy JP, Hassett DJ. A Comparison between Two Pathophysiologically Different yet Microbiologically Similar Lung Diseases: Cystic Fibrosis and Chronic Obstructive Pulmonary Disease. International Journal of Respiratory and Pulmonary Medicine. 5. PMID 30627668 DOI: 10.23937/2378-3516/1410098 |
0.351 |
|
| 2018 |
Sermet-Gaudelus I, Clancy JP, Nichols DP, Nick JA, De Boeck K, Solomon GM, Mall MA, Bolognese J, Bouisset F, den Hollander W, Paquette-Lamontagne N, Tomkinson N, Henig N, Elborn JS, Rowe SM. Antisense oligonucleotide eluforsen improves CFTR function in F508del cystic fibrosis. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 30467074 DOI: 10.1016/J.Jcf.2018.10.015 |
0.48 |
|
| 2018 |
Clancy JP. Rapid therapeutic advances in CFTR modulator science. Pediatric Pulmonology. 53: S4-S11. PMID 30289627 DOI: 10.1002/Ppul.24157 |
0.48 |
|
| 2018 |
Brewington JJ, Filbrandt ET, LaRosa FJ, Moncivaiz JD, Ostmann AJ, Strecker LM, Clancy JP. Brushed nasal epithelial cells are a surrogate for bronchial epithelial CFTR studies. Jci Insight. 3. PMID 29997283 DOI: 10.1172/Jci.Insight.99385 |
0.444 |
|
| 2018 |
Clancy JP, Cotton CU, Donaldson SH, Solomon GM, VanDevanter DR, Boyle MP, Gentzsch M, Nick JA, Illek B, Wallenburg JC, Sorscher EJ, Amaral MD, Beekman JM, Naren AP, Bridges RJ, et al. CFTR modulator theratyping: Current status, gaps and future directions. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 29934203 DOI: 10.1016/J.Jcf.2018.05.004 |
0.463 |
|
| 2018 |
Kramer EL, Hardie WD, Madala SK, Davidson CR, Clancy JP. Subacute TGFβ Expression Drives Inflammation, Goblet Cell Hyperplasia, and Pulmonary Function Abnormalities in Mice with Effects Dependent on CFTR Function. American Journal of Physiology. Lung Cellular and Molecular Physiology. PMID 29877096 DOI: 10.1152/Ajplung.00530.2017 |
0.488 |
|
| 2018 |
Brewington JJ, Filbrandt ET, LaRosa FJ, Moncivaiz JD, Ostmann AJ, Strecker LM, Clancy JP. Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study. Journal of Visualized Experiments : Jove. PMID 29708545 DOI: 10.3791/57492 |
0.425 |
|
| 2018 |
Sagel SD, Khan U, Jain R, Graff G, Daines CL, Dunitz JM, Borowitz D, Orenstein DM, Abdulhamid I, Noe J, Clancy JP, Slovis B, Rock MJ, McCoy KS, Strausbaugh S, et al. Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis: Randomized, Controlled, Multicenter Trial. American Journal of Respiratory and Critical Care Medicine. PMID 29688760 DOI: 10.1164/Rccm.201801-0105Oc |
0.344 |
|
| 2018 |
McCarthy C, Brewington JJ, Harkness B, Clancy JP, Trapnell BC. Personalised CFTR Pharmacotherapeutic Response Testing and Therapy of Cystic Fibrosis. The European Respiratory Journal. PMID 29563174 DOI: 10.1183/13993003.02457-2017 |
0.358 |
|
| 2018 |
Brewington JJ, Backstrom J, Feldman A, Kramer EL, Moncivaiz JD, Ostmann AJ, Zhu X, Lu LJ, Clancy JP. Chronic β2AR stimulation limits CFTR activation in human airway epithelia. Jci Insight. 3. PMID 29467332 DOI: 10.1172/Jci.Insight.93029 |
0.431 |
|
| 2018 |
Pennati F, Roach DJ, Clancy JP, Brody AS, Fleck RJ, Aliverti A, Woods JC. Assessment of pulmonary structure-function relationships in young children and adolescents with cystic fibrosis by multivolume proton-MRI and CT. Journal of Magnetic Resonance Imaging : Jmri. PMID 29457316 DOI: 10.1002/Jmri.25978 |
0.436 |
|
| 2017 |
Hudock KM, Clancy JP. An update on new and emerging therapies for cystic fibrosis. Expert Opinion On Emerging Drugs. 22: 331-346. PMID 29264936 DOI: 10.1080/14728214.2017.1418324 |
0.485 |
|
| 2017 |
Li D, Keogh R, Clancy JP, Szczesniak RD. Flexible semiparametric joint modeling: an application to estimate individual lung function decline and risk of pulmonary exacerbations in cystic fibrosis. Emerging Themes in Epidemiology. 14: 13. PMID 29201130 DOI: 10.1186/S12982-017-0067-1 |
0.41 |
|
| 2017 |
Kramer E, Clancy JP. TGFβ as a therapeutic target in cystic fibrosis. Expert Opinion On Therapeutic Targets. PMID 29168406 DOI: 10.1080/14728222.2018.1406922 |
0.484 |
|
| 2017 |
Zemanick ET, Wagner BD, Robertson CE, Ahrens RC, Chmiel JF, Clancy JP, Gibson RL, Harris WT, Kurland G, Laguna TA, McColley SA, McCoy K, Retsch-Bogart G, Sobush KT, Zeitlin PL, et al. Airway microbiota across age and disease spectrum in cystic fibrosis. The European Respiratory Journal. 50. PMID 29146601 DOI: 10.1183/13993003.00832-2017 |
0.46 |
|
| 2017 |
Arora K, Huang Y, Mun K, Yarlagadda S, Sundaram N, Kessler MM, Hannig G, Kurtz CB, Silos-Santiago I, Helmrath M, Palermo JJ, Clancy JP, Steinbrecher KA, Naren AP. Guanylate cyclase 2C agonism corrects CFTR mutants. Jci Insight. 2. PMID 28978796 DOI: 10.1172/Jci.Insight.93686 |
0.5 |
|
| 2017 |
Brewington JJ, Filbrandt ET, LaRosa FJ, Ostmann AJ, Strecker LM, Szczesniak RD, Clancy JP. Detection of CFTR function and modulation in primary human nasal cell spheroids. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 28712885 DOI: 10.1016/J.Jcf.2017.06.010 |
0.353 |
|
| 2017 |
Lechtzin N, Mayer-Hamblett N, West NE, Allgood S, Wilhelm E, Khan U, Aitken ML, Ramsey BW, Boyle MP, Mogayzel PJ, Gibson RL, Orenstein D, Milla C, Clancy JP, Antony V, et al. Home Monitoring in CF to Identify and Treat Acute Pulmonary Exacerbations: eICE Study Results. American Journal of Respiratory and Critical Care Medicine. PMID 28608719 DOI: 10.1164/Rccm.201610-2172Oc |
0.46 |
|
| 2017 |
Szczesniak RD, Li D, Su W, Brokamp C, Pestian J, Seid M, Clancy JP. Phenotypes of Rapid Cystic Fibrosis Lung Disease Progression during Adolescence and Young Adulthood. American Journal of Respiratory and Critical Care Medicine. PMID 28410569 DOI: 10.1164/Rccm.201612-2574Oc |
0.429 |
|
| 2017 |
Xu X, Abdalla T, Bratcher PE, Jackson PL, Sabbatini G, Wells JM, Lou XY, Quinn R, Blalock JE, Clancy JP, Gaggar A. Doxycycline improves clinical outcomes during cystic fibrosis exacerbations. The European Respiratory Journal. 49. PMID 28381428 DOI: 10.1183/13993003.01102-2016 |
0.372 |
|
| 2017 |
Zak SM, Clancy JP, Brewington JJ. CFTR functional assays in drug development Expert Opinion On Orphan Drugs. 5: 889-898. DOI: 10.1080/21678707.2017.1393413 |
0.402 |
|
| 2017 |
Szczesniak R, Su W, Keogh R, Clancy JP. EPS5.8 Dynamic prediction modeling to identify rapid lung function decline in cystic fibrosis Journal of Cystic Fibrosis. 16. DOI: 10.1016/S1569-1993(17)30319-3 |
0.401 |
|
| 2017 |
Derichs N, Taylor-Cousar J, Tullis E, Davies J, Nazareth D, Downey DG, Rosenbluth D, Fajac I, Malfroot A, Bakker M, Clancy JP, Uluer A, Castellani C, Sermet-Gaudelus I, Ahrens RC, et al. EPS1.3 Safety, tolerability and early signs of efficacy with riociguat for the treatment of adult Phe508del homozygous cystic fibrosis patients: study design and rationale for the Rio-CF study Journal of Cystic Fibrosis. 16. DOI: 10.1016/S1569-1993(17)30276-X |
0.391 |
|
| 2017 |
Rowe S, Sermet-Gaudelus I, Clancy JP, Nichols D, Nick J, Boeck KD, Solomon G, Elborn JS, Mall MA, Bolognese J, Bouisset F, Hollander Wd, Lamontagne N, Tomkinson N, Henig N. WS13.1 QR-010, an investigational RNA therapeutic, improves CFTR activity in cystic fibrosis subjects homozygous for the F508del mutation Journal of Cystic Fibrosis. 16. DOI: 10.1016/S1569-1993(17)30231-X |
0.349 |
|
| 2017 |
Jorth P, Rezayat A, Ehsan Z, Benscoter D, Clancy J, Singh P. WS03.5 Invasive sampling that bypasses the oropharynx provides insights into the lung microbiota of children with CF and mild lung disease Journal of Cystic Fibrosis. 16: S5-S6. DOI: 10.1016/S1569-1993(17)30172-8 |
0.398 |
|
| 2017 |
Mun KS, Arora K, Abu-El-Haija M, Nathan JD, Clancy J, Palermo JJ, Naren AP. Patient-Derived Pancreatic Ductal Organoids to Study CFTR Function Gastroenterology. 152: S17-S18. DOI: 10.1016/S0016-5085(17)30432-8 |
0.324 |
|
| 2017 |
Clancy J, Benitez DS, Fajac I, Jain M, Rowe SM, Sawicki G. Applying recent advances in the science of CFTR-based therapeutics to improve outcomes in patients with cystic fibrosis Journal of Cystic Fibrosis. 16: e9-e10. DOI: 10.1016/J.Jcf.2017.03.002 |
0.395 |
|
| 2016 |
Szczesniak R, Su W, Clancy JP. Dynamics of Disease Progression and Gastrostomy Tube Placement in Children and Adolescents with Cystic Fibrosis: Application of Joint Models for Longitudinal and Time-to-Event Data. Internal Medicine Review (Washington, D.C. : Online). 2. PMID 28232958 DOI: 10.18103/Imr.V2I9.163 |
0.404 |
|
| 2016 |
Muhlebach MS, Clancy JP, Heltshe SL, Ziady A, Kelley T, Accurso F, Pilewski J, Mayer-Hamblett N, Joseloff E, Sagel SD. Biomarkers for cystic fibrosis drug development. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 15: 714-723. PMID 28215711 DOI: 10.1016/J.Jcf.2016.10.009 |
0.329 |
|
| 2016 |
Kramer EL, Clancy JP. CFTR Modulator Therapies in Pediatric Cystic Fibrosis: Focus on Ivacaftor. Expert Opinion On Orphan Drugs. 4: 1033-1042. PMID 28042521 DOI: 10.1080/21678707.2016.1226800 |
0.488 |
|
| 2016 |
Harris WT, Boyd JT, McPhail GL, Brody AS, Szczesniak RD, Korbee LL, Baker ML, Clancy JP. Constrictive Bronchiolitis in Adolescents with Cystic Fibrosis with Refractory Pulmonary Decline. Annals of the American Thoracic Society. PMID 27684511 DOI: 10.1513/Annalsats.201412-594Oc |
0.423 |
|
| 2016 |
Downes KJ, Dong M, Fukuda T, Clancy JP, Haffner C, Bennett MR, Vinks AA, Goldstein SL. Urinary kidney injury biomarkers and tobramycin clearance among children and young adults with cystic fibrosis: a population pharmacokinetic analysis. The Journal of Antimicrobial Chemotherapy. PMID 27585963 DOI: 10.1093/Jac/Dkw351 |
0.384 |
|
| 2016 |
Roach DJ, Crémillieux Y, Fleck RJ, Brody AS, Serai SD, Szczesniak RD, Kerlakian S, Clancy JP, Woods JC. Ultrashort Echo-Time Magnetic Resonance Imaging Is a Sensitive Method for the Evaluation of Early Cystic Fibrosis Lung Disease. Annals of the American Thoracic Society. PMID 27551814 DOI: 10.1513/Annalsats.201603-203Oc |
0.379 |
|
| 2016 |
Walkup LL, Thomen RP, Akinyi TG, Watters E, Ruppert K, Clancy JP, Woods JC, Cleveland ZI. Feasibility, tolerability and safety of pediatric hyperpolarized (129)Xe magnetic resonance imaging in healthy volunteers and children with cystic fibrosis. Pediatric Radiology. PMID 27492388 DOI: 10.1007/S00247-016-3672-1 |
0.316 |
|
| 2016 |
Thomen RP, Walkup LL, Roach DJ, Cleveland ZI, Clancy JP, Woods JC. Hyperpolarized (129)Xe for investigation of mild cystic fibrosis lung disease in pediatric patients. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 27477942 DOI: 10.1016/J.Jcf.2016.07.008 |
0.436 |
|
| 2016 |
Flume PA, Clancy JP, Retsch-Bogart GZ, Tullis DE, Bresnik M, Derchak PA, Lewis SA, Ramsey BW. Continuous alternating inhaled antibiotics for chronic pseudomonal infection in cystic fibrosis. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 27233377 DOI: 10.1016/J.Jcf.2016.05.001 |
0.368 |
|
| 2016 |
Spielberg DR, Clancy JP. Cystic Fibrosis and Its Management Through Established and Emerging Therapies. Annual Review of Genomics and Human Genetics. PMID 26905785 DOI: 10.1146/Annurev-Genom-090314-050024 |
0.437 |
|
| 2016 |
Clancy JP. Cystic Fibrosis Transmembrane Conductance Regulator Function in Airway Smooth Muscle. A Novel Role in Cystic Fibrosis Airway Obstruction. American Journal of Respiratory and Critical Care Medicine. 193: 352-3. PMID 26871669 DOI: 10.1164/Rccm.201510-2088Ed |
0.431 |
|
| 2016 |
Brewington J, Clancy JP. Diagnostic Testing in Cystic Fibrosis. Clinics in Chest Medicine. 37: 31-46. PMID 26857766 DOI: 10.1016/J.Ccm.2015.10.005 |
0.42 |
|
| 2016 |
Pennati F, Aliverti A, Roach D, Clancy J, Woods J. Specific cystic fibrosis (CF) abnormalities studied by multi-volume CT and proton MRI (1H-MRI) European Respiratory Journal. 48. DOI: 10.1183/13993003.Congress-2016.Pa2257 |
0.362 |
|
| 2016 |
Pennati F, Salito C, Roach D, Clancy J, Brody A, Fleck R, Aliverti A, Woods J. Structure-function relationships in the lungs of cystic fibrosis (CF) patients using multi-volume high-resolution CT (HRCT) and proton MRI (1H-MRI) European Respiratory Journal. 48. DOI: 10.1183/13993003.Congress-2016.Pa2245 |
0.368 |
|
| 2015 |
Szczesniak RD, McPhail GL, Li D, Amin RS, Clancy JP. Predicting future lung function decline in cystic fibrosis patients: Statistical methods and clinical connections. Pediatric Pulmonology. PMID 26677806 DOI: 10.1002/Ppul.23357 |
0.415 |
|
| 2015 |
Brewington JJ, McPhail GL, Clancy JP. Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction. Expert Review of Respiratory Medicine. 1-13. PMID 26581802 DOI: 10.1586/17476348.2016.1122527 |
0.431 |
|
| 2015 |
Ehsan Z, Clancy JP. Management of Pseudomonas aeruginosa infection in cystic fibrosis patients using inhaled antibiotics with a focus on nebulized liposomal amikacin. Future Microbiology. PMID 26573178 DOI: 10.2217/Fmb.15.117 |
0.471 |
|
| 2015 |
Sinha C, Zhang W, Moon CS, Actis M, Yarlagadda S, Arora K, Woodroofe K, Clancy JP, Lin S, Ziady AG, Frizzell R, Fujii N, Naren AP. Capturing the Direct Binding of CFTR Correctors to CFTR by Using Click Chemistry. Chembiochem : a European Journal of Chemical Biology. PMID 26227551 DOI: 10.1002/Cbic.201500123 |
0.434 |
|
| 2015 |
Kotha K, Szczesniak RD, Naren AP, Fenchel MC, Duan LL, McPhail GL, Clancy JP. Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 26210165 DOI: 10.1016/J.Jcf.2015.07.002 |
0.436 |
|
| 2015 |
Powers SW, Stark LJ, Chamberlin LA, Filigno SS, Sullivan SM, Lemanek KL, Butcher JL, Driscoll KA, Daines CL, Brody AS, Schindler T, Konstan MW, McCoy KS, Nasr SZ, Castile RG, ... ... Clancy JP, et al. Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial. Jama Pediatrics. 169: e150636. PMID 25938655 DOI: 10.1001/Jamapediatrics.2015.0636 |
0.344 |
|
| 2015 |
Downes KJ, Patil NR, Rao MB, Koralkar R, Harris WT, Clancy JP, Goldstein SL, Askenazi DJ. Risk factors for acute kidney injury during aminoglycoside therapy in patients with cystic fibrosis. Pediatric Nephrology (Berlin, Germany). PMID 25912993 DOI: 10.1007/S00467-015-3097-3 |
0.336 |
|
| 2015 |
Conrad C, Lymp J, Thompson V, Dunn C, Davies Z, Chatfield B, Nichols D, Clancy J, Vender R, Egan ME, Quittell L, Michelson P, Antony V, Spahr J, Rubenstein RC, et al. Long-term treatment with oral N-acetylcysteine: affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 14: 219-27. PMID 25228446 DOI: 10.1016/J.Jcf.2014.08.008 |
0.409 |
|
| 2015 |
Tiddens HA, De Boeck K, Clancy JP, Fayon M, H G M A, Bresnik M, Derchak A, Lewis SA, Oermann CM. Open label study of inhaled aztreonam for Pseudomonas eradication in children with cystic fibrosis: The ALPINE study. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 14: 111-9. PMID 25091537 DOI: 10.1016/J.Jcf.2014.06.003 |
0.402 |
|
| 2015 |
Ehsan Z, Clancy J. T100: Nebulized-concentrated tobramycin formulation for treatment of Pseudomonas aeruginosa infection in cystic fibrosis patients Expert Opinion On Orphan Drugs. 3: 933-943. DOI: 10.1517/21678707.2015.1064308 |
0.413 |
|
| 2015 |
Pennati F, Salito C, Roach D, Clancy JP, Woods J, Aliverti A. Regional ventilation in infants quantified by multi-volume high resolution computed tomography (HRCT) and multi-volume proton magnetic resonance imaging (MRI) European Respiratory Journal. 46. DOI: 10.1183/13993003.Congress-2015.Oa2949 |
0.321 |
|
| 2015 |
McPhail G, Chini B, Siracusa C, Fenchel M, Szczesniak R, Clancy J. 211 Vitamin D insufficiency is associated with pulmonary exacerbations in children with cystic fibrosis Journal of Cystic Fibrosis. 14: S112. DOI: 10.1016/S1569-1993(15)30387-8 |
0.308 |
|
| 2015 |
Bilton D, Pressler T, Fajac I, Clancy JP, Minic P, Cipolli M, Galeva I, Solé A, Dupont LJ, Mayer-Hamblett N, Torchio S, McGinnis JP, Eagle G, Konstan M. 112 Analysis of long-term use of liposomal amikacin for inhalation (LAI) in patients with cystic fibrosis (CF) who have chronic infection from Pseudomonas aeruginosa Journal of Cystic Fibrosis. 14. DOI: 10.1016/S1569-1993(15)30289-7 |
0.361 |
|
| 2015 |
Tiddens H, De Boeck K, Clancy J, Fayon M, Arets H, Bresnik M, Derchak P, Lewis S, Oermann C. 110 Aztreonam for inhalation solution (AZLI) for eradication of new onset Pseudomonas aeruginosa ( PA ) infection in children with cystic fibrosis (CF): Evaluation of treatment failures Journal of Cystic Fibrosis. 14: S85. DOI: 10.1016/S1569-1993(15)30287-3 |
0.304 |
|
| 2015 |
Tiddens H, De Boeck K, Clancy J, Fayon M, Arets H, Bresnik M, Derchak P, Lewis S, Oermann C. WS02.2 Aztreonam for inhalation solution (AZLI) for eradication of new onset Pseudomonas aeruginosa ( PA ) infection in children with cystic fibrosis (CF): Results of facemask vs. nebulizer mouthpiece use Journal of Cystic Fibrosis. 14: S3. DOI: 10.1016/S1569-1993(15)30008-4 |
0.344 |
|
| 2015 |
Schuchman EH, Clancy J, Dimango E, Petrache I, Gulbins E, He X. Novel use of the lysosomal enzyme acid ceramidase for the treatment of inflammatory lung diseases, including cystic fibrosis Molecular Genetics and Metabolism. 114: S104-S105. DOI: 10.1016/J.Ymgme.2014.12.236 |
0.382 |
|
| 2015 |
Stuart Elborn J, Sermet-Gaudelus I, Flume PA, Madge S, Cerny F, Clancy JP, Bichl S, Morton A. Advanced curriculum for cystic fibrosis: Integrating genomic-driven data into patient-centered treatment strategies Journal of Cystic Fibrosis. DOI: 10.1016/J.Jcf.2015.12.013 |
0.413 |
|
| 2014 |
Sun H, Harris WT, Kortyka S, Kotha K, Ostmann AJ, Rezayat A, Sridharan A, Sanders Y, Naren AP, Clancy JP. Tgf-beta downregulation of distinct chloride channels in cystic fibrosis-affected epithelia. Plos One. 9: e106842. PMID 25268501 DOI: 10.1371/Journal.Pone.0106842 |
0.351 |
|
| 2014 |
Clancy JP. CFTR potentiators: not an open and shut case. Science Translational Medicine. 6: 246fs27. PMID 25101884 DOI: 10.1126/Scitranslmed.3009674 |
0.348 |
|
| 2014 |
Downes KJ, Rao MB, Kahill L, Nguyen H, Clancy JP, Goldstein SL. Daily serum creatinine monitoring promotes earlier detection of acute kidney injury in children and adolescents with cystic fibrosis. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 13: 435-41. PMID 24718099 DOI: 10.1016/J.Jcf.2014.03.005 |
0.316 |
|
| 2014 |
Accurso FJ, Van Goor F, Zha J, Stone AJ, Dong Q, Ordonez CL, Rowe SM, Clancy JP, Konstan MW, Hoch HE, Heltshe SL, Ramsey BW, Campbell PW, Ashlock MA. Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 13: 139-47. PMID 24660233 DOI: 10.1016/J.Jcf.2013.09.007 |
0.416 |
|
| 2014 |
Clancy JP, Johnson SG, Yee SW, McDonagh EM, Caudle KE, Klein TE, Cannavo M, Giacomini KM. Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines for ivacaftor therapy in the context of CFTR genotype. Clinical Pharmacology and Therapeutics. 95: 592-7. PMID 24598717 DOI: 10.1038/Clpt.2014.54 |
0.382 |
|
| 2014 |
Ehsan Z, Wetzel JD, Clancy JP. Nebulized liposomal amikacin for the treatment of Pseudomonas aeruginosa infection in cystic fibrosis patients. Expert Opinion On Investigational Drugs. 23: 743-9. PMID 24597573 DOI: 10.1517/13543784.2014.895322 |
0.465 |
|
| 2014 |
Kong MY, Clancy JP, Peng N, Li Y, Szul TJ, Xu X, Oster R, Sullender W, Ambalavanan N, Blalock JE, Gaggar A. Pulmonary matrix metalloproteinase-9 activity in mechanically ventilated children with respiratory syncytial virus. The European Respiratory Journal. 43: 1086-96. PMID 24311764 DOI: 10.1183/09031936.00105613 |
0.324 |
|
| 2014 |
Bilton D, Pressler T, Fajac I, Clancy JP, Sands D, Minic P, Cipolli M, Rosa ML, Galeva I, Solé A, Dupont L, Goss CH, Mayer-Hamblett N, Quittner AL, Constantine S, et al. WS7.3 Once-daily liposomal amikacin for inhalation is noninferior to twice-daily tobramycin inhalation solution in improving pulmonary function in cystic fibrosis patients with chronic infection due to Pseudomonas aeruginosa Journal of Cystic Fibrosis. 13. DOI: 10.1016/S1569-1993(14)60048-5 |
0.409 |
|
| 2014 |
Clancy J, Amin R, Szczesniak R, Mcphail G, Fleck R. WS3.3 MRI-based pulmonary blood flow and lung function in CF patients – flow changes with pulmonary decline Journal of Cystic Fibrosis. 13: S6. DOI: 10.1016/S1569-1993(14)60021-7 |
0.386 |
|
| 2013 |
Szczesniak RD, McPhail GL, Duan LL, Macaluso M, Amin RS, Clancy JP. A semiparametric approach to estimate rapid lung function decline in cystic fibrosis. Annals of Epidemiology. 23: 771-7. PMID 24103586 DOI: 10.1016/J.Annepidem.2013.08.009 |
0.44 |
|
| 2013 |
Kong M, Maeng P, Hong J, Szczesniak R, Sorscher E, Sullender W, Clancy JP. Respiratory syncytial virus infection disrupts monolayer integrity and function in cystic fibrosis airway cells. Viruses. 5: 2260-71. PMID 24056672 DOI: 10.3390/V5092260 |
0.374 |
|
| 2013 |
Raju SV, Jackson PL, Courville CA, McNicholas CM, Sloane PA, Sabbatini G, Tidwell S, Tang LP, Liu B, Fortenberry JA, Jones CW, Boydston JA, Clancy JP, Bowen LE, Accurso FJ, et al. Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function. American Journal of Respiratory and Critical Care Medicine. 188: 1321-30. PMID 24040746 DOI: 10.1164/Rccm.201304-0733Oc |
0.338 |
|
| 2013 |
Clancy JP, Szczesniak RD, Ashlock MA, Ernst SE, Fan L, Hornick DB, Karp PH, Khan U, Lymp J, Ostmann AJ, Rezayat A, Starner TD, Sugandha SP, Sun H, Quinney N, et al. Multicenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function. Plos One. 8: e73905. PMID 24040112 DOI: 10.1371/Journal.Pone.0073905 |
0.451 |
|
| 2013 |
Kotha K, Clancy JP. Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence. Therapeutic Advances in Respiratory Disease. 7: 288-96. PMID 24004658 DOI: 10.1177/1753465813502115 |
0.483 |
|
| 2013 |
Harris WT, Kelly DR, Zhou Y, Wang D, MacEwen M, Macewen M, Hagood JS, Clancy JP, Ambalavanan N, Sorscher EJ. Myofibroblast differentiation and enhanced TGF-B signaling in cystic fibrosis lung disease. Plos One. 8: e70196. PMID 23950911 DOI: 10.1371/Journal.Pone.0070196 |
0.428 |
|
| 2013 |
Rowe SM, Liu B, Hill A, Hathorne H, Cohen M, Beamer JR, Accurso FJ, Dong Q, Ordoñez CL, Stone AJ, Olson ER, Clancy JP. Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation. Plos One. 8: e66955. PMID 23922647 DOI: 10.1371/Journal.Pone.0066955 |
0.355 |
|
| 2013 |
Rymut SM, Harker A, Corey DA, Burgess JD, Sun H, Clancy JP, Kelley TJ. Reduced microtubule acetylation in cystic fibrosis epithelial cells. American Journal of Physiology. Lung Cellular and Molecular Physiology. 305: L419-31. PMID 23873844 DOI: 10.1152/Ajplung.00411.2012 |
0.419 |
|
| 2013 |
Clancy JP, Dupont L, Konstan MW, Billings J, Fustik S, Goss CH, Lymp J, Minic P, Quittner AL, Rubenstein RC, Young KR, Saiman L, Burns JL, Govan JR, Ramsey B, et al. Phase II studies of nebulised Arikace in CF patients with Pseudomonas aeruginosa infection. Thorax. 68: 818-25. PMID 23749840 DOI: 10.1136/Thoraxjnl-2012-202230 |
0.396 |
|
| 2013 |
Fleck R, McPhail G, Szczesniak R, Knowlton J, Radhakrishnan R, Clancy J, Amin R. Aortopulmonary collateral flow in cystic fibrosis assessed with phase-contrast MRI. Pediatric Radiology. 43: 1279-86. PMID 23677424 DOI: 10.1007/S00247-013-2708-Z |
0.431 |
|
| 2013 |
McPhail GL, Clancy JP. Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis. Drugs of Today (Barcelona, Spain : 1998). 49: 253-60. PMID 23616952 DOI: 10.1358/dot.2013.49.4.1940984 |
0.392 |
|
| 2013 |
Rowe SM, Reeves G, Hathorne H, Solomon GM, Abbi S, Renard D, Lock R, Zhou P, Danahay H, Clancy JP, Waltz DA. Reduced sodium transport with nasal administration of the prostasin inhibitor camostat in subjects with cystic fibrosis. Chest. 144: 200-7. PMID 23412700 DOI: 10.1378/Chest.12-2431 |
0.374 |
|
| 2013 |
Harris WT, Kelly DR, Zhou Y, Wang D, MacEwen M, Hagood JS, Clancy JP, Ambalavanan N, Sorscher EJ. Correction: Myofibroblast Differentiation and Enhanced Tgf-B Signaling in Cystic Fibrosis Lung Disease Plos One. 8. DOI: 10.1371/Annotation/D0132De3-56Ca-4258-9119-Bdab0Ceb2Cff |
0.366 |
|
| 2012 |
Grossoehme DH, Ragsdale JR, Cotton S, Meyers MA, Clancy JP, Seid M, Joseph PM. Using spirituality after an adult CF diagnosis: cognitive reframing and adherence motivation. Journal of Health Care Chaplaincy. 18: 110-20. PMID 23094612 DOI: 10.1080/08854726.2012.720544 |
0.348 |
|
| 2012 |
Rowe SM, Borowitz DS, Burns JL, Clancy JP, Donaldson SH, Retsch-Bogart G, Sagel SD, Ramsey BW. Progress in cystic fibrosis and the CF Therapeutics Development Network. Thorax. 67: 882-90. PMID 22960984 DOI: 10.1136/Thoraxjnl-2012-202550 |
0.466 |
|
| 2012 |
Clancy JP, Rowe SM, Accurso FJ, Aitken ML, Amin RS, Ashlock MA, Ballmann M, Boyle MP, Bronsveld I, Campbell PW, De Boeck K, Donaldson SH, Dorkin HL, Dunitz JM, Durie PR, et al. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax. 67: 12-8. PMID 21825083 DOI: 10.1136/Thoraxjnl-2011-200393 |
0.438 |
|
| 2012 |
Kong M, Peng N, Jackson P, Clancy J, Blalock E, Gaggar A. 174: Dysregulated Matrix Metalloproteinase-9 Activity And Elevated Proline-Glycine-Proline (Pgp) Levels Are Observed In Pediatric Rsv-Induced Lung Injury Critical Care Medicine. 40: 1-328. DOI: 10.1097/01.Ccm.0000424394.89408.E6 |
0.322 |
|
| 2011 |
Kong MY, Li Y, Oster R, Gaggar A, Clancy JP. Early elevation of matrix metalloproteinase-8 and -9 in pediatric ARDS is associated with an increased risk of prolonged mechanical ventilation. Plos One. 6: e22596. PMID 21857935 DOI: 10.1371/Journal.Pone.0022596 |
0.324 |
|
| 2011 |
Rowe SM, Clancy JP, Sorscher EJ. A breath of fresh air. Scientific American. 305: 68-73. PMID 21827128 DOI: 10.1038/Scientificamerican0811-68 |
0.363 |
|
| 2011 |
Pyle LC, Ehrhardt A, Mitchell LH, Fan L, Ren A, Naren AP, Li Y, Clancy JP, Bolger GB, Sorscher EJ, Rowe SM. Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators. American Journal of Physiology. Lung Cellular and Molecular Physiology. 301: L587-97. PMID 21724857 DOI: 10.1152/Ajplung.00465.2010 |
0.403 |
|
| 2011 |
Rowe SM, Clancy JP, Wilschanski M. Nasal potential difference measurements to assess CFTR ion channel activity. Methods in Molecular Biology (Clifton, N.J.). 741: 69-86. PMID 21594779 DOI: 10.1007/978-1-61779-117-8_6 |
0.361 |
|
| 2011 |
Harris WT, Muhlebach MS, Oster RA, Knowles MR, Clancy JP, Noah TL. Plasma TGF-β₁ in pediatric cystic fibrosis: potential biomarker of lung disease and response to therapy. Pediatric Pulmonology. 46: 688-95. PMID 21337732 DOI: 10.1002/Ppul.21430 |
0.439 |
|
| 2010 |
Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. The New England Journal of Medicine. 363: 1991-2003. PMID 21083385 DOI: 10.1056/Nejmoa0909825 |
0.471 |
|
| 2010 |
Solomon GM, Konstan MW, Wilschanski M, Billings J, Sermet-Gaudelus I, Accurso F, Vermeulen F, Levin E, Hathorne H, Reeves G, Sabbatini G, Hill A, Mayer-Hamblett N, Ashlock M, Clancy JP, et al. An international randomized multicenter comparison of nasal potential difference techniques. Chest. 138: 919-28. PMID 20472865 DOI: 10.1378/Chest.10-0179 |
0.341 |
|
| 2010 |
Rowe SM, Pyle LC, Jurkevante A, Varga K, Collawn J, Sloane PA, Woodworth B, Mazur M, Fulton J, Fan L, Li Y, Fortenberry J, Sorscher EJ, Clancy JP. DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers. Pulmonary Pharmacology & Therapeutics. 23: 268-78. PMID 20226262 DOI: 10.1016/J.Pupt.2010.02.001 |
0.388 |
|
| 2010 |
Jackson PL, Xu X, Wilson L, Weathington NM, Clancy JP, Blalock JE, Gaggar A. Human neutrophil elastase-mediated cleavage sites of MMP-9 and TIMP-1: implications to cystic fibrosis proteolytic dysfunction. Molecular Medicine (Cambridge, Mass.). 16: 159-66. PMID 20111696 DOI: 10.2119/Molmed.2009.00109 |
0.409 |
|
| 2010 |
Pyle LC, Fulton JC, Sloane PA, Backer K, Mazur M, Prasain J, Barnes S, Clancy JP, Rowe SM. Activation of the cystic fibrosis transmembrane conductance regulator by the flavonoid quercetin: potential use as a biomarker of ΔF508 cystic fibrosis transmembrane conductance regulator rescue. American Journal of Respiratory Cell and Molecular Biology. 43: 607-16. PMID 20042712 DOI: 10.1165/Rcmb.2009-0281Oc |
0.415 |
|
| 2009 |
Rowe SM, Clancy JP. Pharmaceuticals targeting nonsense mutations in genetic diseases: progress in development. Biodrugs : Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy. 23: 165-74. PMID 19627168 DOI: 10.2165/00063030-200923030-00003 |
0.383 |
|
| 2009 |
Cormet-Boyaka E, Hong JS, Berdiev BK, Fortenberry JA, Rennolds J, Clancy JP, Benos DJ, Boyaka PN, Sorscher EJ. A truncated CFTR protein rescues endogenous DeltaF508-CFTR and corrects chloride transport in mice. Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 23: 3743-51. PMID 19620404 DOI: 10.1096/Fj.08-127878 |
0.443 |
|
| 2009 |
Kong MY, Gaggar A, Li Y, Winkler M, Blalock JE, Clancy JP. Matrix metalloproteinase activity in pediatric acute lung injury. International Journal of Medical Sciences. 6: 9-17. PMID 19159011 DOI: 10.7150/Ijms.6.9 |
0.308 |
|
| 2009 |
Accurso F, Rowe S, Durie P, Konstan M, Dunitz J, Hornick D, Sagel S, Boyle M, Uluer A, Moss R, Ramsey B, Freedman S, Dong Q, Zha J, Stone A, ... ... Clancy J, et al. Final results of a 14- and 28-day study of VX-770 in subjects with CF Journal of Cystic Fibrosis. 8: S25. DOI: 10.1016/S1569-1993(09)60101-6 |
0.333 |
|
| 2008 |
Rowe SM, Jackson PL, Liu G, Hardison M, Livraghi A, Solomon GM, McQuaid DB, Noerager BD, Gaggar A, Clancy JP, O'Neal W, Sorscher EJ, Abraham E, Blalock JE. Potential role of high-mobility group box 1 in cystic fibrosis airway disease. American Journal of Respiratory and Critical Care Medicine. 178: 822-31. PMID 18658107 DOI: 10.1164/Rccm.200712-1894Oc |
0.433 |
|
| 2008 |
Gaggar A, Jackson PL, Noerager BD, O'Reilly PJ, McQuaid DB, Rowe SM, Clancy JP, Blalock JE. A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation. Journal of Immunology (Baltimore, Md. : 1950). 180: 5662-9. PMID 18390751 DOI: 10.4049/Jimmunol.180.8.5662 |
0.425 |
|
| 2008 |
Chen L, Bosworth CA, Pico T, Collawn JF, Varga K, Gao Z, Clancy JP, Fortenberry JA, Lancaster JR, Matalon S. DETANO and nitrated lipids increase chloride secretion across lung airway cells. American Journal of Respiratory Cell and Molecular Biology. 39: 150-62. PMID 18314534 DOI: 10.1165/Rcmb.2008-0005Oc |
0.362 |
|
| 2008 |
Rennolds J, Tower C, Musgrove L, Fan L, Maloney K, Clancy JP, Kirk KL, Sztul E, Cormet-Boyaka E. Cystic fibrosis transmembrane conductance regulator trafficking is mediated by the COPI coat in epithelial cells. The Journal of Biological Chemistry. 283: 833-9. PMID 17932045 DOI: 10.1074/Jbc.M706504200 |
0.411 |
|
| 2007 |
Moss RB, Milla C, Colombo J, Accurso F, Zeitlin PL, Clancy JP, Spencer LT, Pilewski J, Waltz DA, Dorkin HL, Ferkol T, Pian M, Ramsey B, Carter BJ, Martin DB, et al. Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial. Human Gene Therapy. 18: 726-32. PMID 17685853 DOI: 10.1089/Hum.2007.022 |
0.452 |
|
| 2007 |
Rowe SM, Accurso F, Clancy JP. Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials. Proceedings of the American Thoracic Society. 4: 387-98. PMID 17652506 DOI: 10.1513/pats.200703-043BR |
0.302 |
|
| 2007 |
Rowe SM, Varga K, Rab A, Bebok Z, Byram K, Li Y, Sorscher EJ, Clancy JP. Restoration of W1282X CFTR activity by enhanced expression. American Journal of Respiratory Cell and Molecular Biology. 37: 347-56. PMID 17541014 DOI: 10.1165/Rcmb.2006-0176Oc |
0.408 |
|
| 2007 |
Gaggar A, Li Y, Weathington N, Winkler M, Kong M, Jackson P, Blalock JE, Clancy JP. Matrix metalloprotease-9 dysregulation in lower airway secretions of cystic fibrosis patients. American Journal of Physiology. Lung Cellular and Molecular Physiology. 293: L96-L104. PMID 17384080 DOI: 10.1152/Ajplung.00492.2006 |
0.308 |
|
| 2007 |
Clancy JP, Rowe SM, Bebok Z, Aitken ML, Gibson R, Zeitlin P, Berclaz P, Moss R, Knowles MR, Oster RA, Mayer-Hamblett N, Ramsey B. No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations. American Journal of Respiratory Cell and Molecular Biology. 37: 57-66. PMID 17347447 DOI: 10.1165/Rcmb.2006-0173Oc |
0.433 |
|
| 2007 |
Kishnani PS, Corzo D, Nicolino M, Byrne B, Mandel H, Hwu WL, Leslie N, Levine J, Spencer C, McDonald M, Li J, Dumontier J, Halberthal M, Chien YH, Hopkin R, ... ... Clancy JP, et al. Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease. Neurology. 68: 99-109. PMID 17151339 DOI: 10.1212/01.Wnl.0000251268.41188.04 |
0.309 |
|
| 2006 |
Rowe SM, Clancy JP. Advances in cystic fibrosis therapies. Current Opinion in Pediatrics. 18: 604-13. PMID 17099358 DOI: 10.1097/Mop.0B013E3280109B90 |
0.375 |
|
| 2006 |
Li Y, Wang W, Parker W, Clancy JP. Adenosine regulation of cystic fibrosis transmembrane conductance regulator through prostenoids in airway epithelia. American Journal of Respiratory Cell and Molecular Biology. 34: 600-8. PMID 16399952 DOI: 10.1165/Rcmb.2005-0421Oc |
0.428 |
|
| 2006 |
Hallows KR, Fitch AC, Richardson CA, Reynolds PR, Clancy JP, Dagher PC, Witters LA, Kolls JK, Pilewski JM. Up-regulation of AMP-activated kinase by dysfunctional cystic fibrosis transmembrane conductance regulator in cystic fibrosis airway epithelial cells mitigates excessive inflammation. The Journal of Biological Chemistry. 281: 4231-41. PMID 16361706 DOI: 10.1074/Jbc.M511029200 |
0.467 |
|
| 2006 |
Jurkuvenaite A, Varga K, Nowotarski K, Kirk KL, Sorscher EJ, Li Y, Clancy JP, Bebok Z, Collawn JF. Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis. The Journal of Biological Chemistry. 281: 3329-34. PMID 16339147 DOI: 10.1074/Jbc.M508131200 |
0.357 |
|
| 2005 |
Bebok Z, Collawn JF, Wakefield J, Parker W, Li Y, Varga K, Sorscher EJ, Clancy JP. Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers. The Journal of Physiology. 569: 601-15. PMID 16210354 DOI: 10.1113/Jphysiol.2005.096669 |
0.407 |
|
| 2005 |
Wang W, Li G, Clancy JP, Kirk KL. Activating cystic fibrosis transmembrane conductance regulator channels with pore blocker analogs. The Journal of Biological Chemistry. 280: 23622-30. PMID 15857825 DOI: 10.1074/Jbc.M503118200 |
0.357 |
|
| 2004 |
Harris CM, Mendes F, Dragomir A, Doull IJ, Carvalho-Oliveira I, Bebok Z, Clancy JP, Eubanks V, Sorscher EJ, Roomans GM, Amaral MD, McPherson MA, Penque D, Dormer RL. Assessment of CFTR localisation in native airway epithelial cells obtained by nasal brushing. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 43-8. PMID 15463924 DOI: 10.1016/J.Jcf.2004.05.009 |
0.413 |
|
| 2004 |
Hentchel-Franks K, Lozano D, Eubanks-Tarn V, Cobb B, Fan L, Oster R, Sorscher E, Clancy JP. Activation of airway cl- secretion in human subjects by adenosine. American Journal of Respiratory Cell and Molecular Biology. 31: 140-6. PMID 15039139 DOI: 10.1165/Rcmb.2004-0012Oc |
0.339 |
|
| 2004 |
Moss RB, Rodman D, Spencer LT, Aitken ML, Zeitlin PL, Waltz D, Milla C, Brody AS, Clancy JP, Ramsey B, Hamblett N, Heald AE. Repeated adeno-associated virus serotype 2 aerosol-mediated cystic fibrosis transmembrane regulator gene transfer to the lungs of patients with cystic fibrosis: a multicenter, double-blind, placebo-controlled trial. Chest. 125: 509-21. PMID 14769732 DOI: 10.1378/Chest.125.2.509 |
0.418 |
|
| 2004 |
Zsembery A, Fortenberry JA, Liang L, Bebok Z, Tucker TA, Boyce AT, Braunstein GM, Welty E, Bell PD, Sorscher EJ, Clancy JP, Schwiebert EM. Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry. The Journal of Biological Chemistry. 279: 10720-9. PMID 14701827 DOI: 10.1074/Jbc.M313391200 |
0.449 |
|
| 2003 |
Jones JR, Schwiebert EM, DuVall MD, Venglarik CJ, Wen H, Kovacs T, Mazur M, Clancy JP, Braunstein G, Bates E, Greer H, Maddry JA, Sorscher EJ. Activation of chloride secretion in cystic fibrosis cells and tissues by the substituted imidazole SRI 2931. Biochemistry. 42: 13241-9. PMID 14609335 DOI: 10.1021/Bi0340447 |
0.432 |
|
| 2003 |
Cobb BR, Fan L, Kovacs TE, Sorscher EJ, Clancy JP. Adenosine receptors and phosphodiesterase inhibitors stimulate Cl- secretion in Calu-3 cells. American Journal of Respiratory Cell and Molecular Biology. 29: 410-8. PMID 12714375 DOI: 10.1165/Rcmb.2002-0247Oc |
0.337 |
|
| 2003 |
Naren AP, Cobb B, Li C, Roy K, Nelson D, Heda GD, Liao J, Kirk KL, Sorscher EJ, Hanrahan J, Clancy JP. A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA. Proceedings of the National Academy of Sciences of the United States of America. 100: 342-6. PMID 12502786 DOI: 10.1073/Pnas.0135434100 |
0.304 |
|
| 2002 |
Cobb BR, Ruiz F, King CM, Fortenberry J, Greer H, Kovacs T, Sorscher EJ, Clancy JP. A(2) adenosine receptors regulate CFTR through PKA and PLA(2). American Journal of Physiology. Lung Cellular and Molecular Physiology. 282: L12-25. PMID 11741811 DOI: 10.1152/Ajplung.2002.282.1.L12 |
0.358 |
|
| 2001 |
Clancy JP, Bebök Z, Ruiz F, King C, Jones J, Walker L, Greer H, Hong J, Wing L, Macaluso M, Lyrene R, Sorscher EJ, Bedwell DM. Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis. American Journal of Respiratory and Critical Care Medicine. 163: 1683-92. PMID 11401894 DOI: 10.1164/Ajrccm.163.7.2004001 |
0.434 |
|
| 2001 |
Ruiz FE, Clancy JP, Perricone MA, Bebok Z, Hong JS, Cheng SH, Meeker DP, Young KR, Schoumacher RA, Weatherly MR, Wing L, Morris JE, Sindel L, Rosenberg M, van Ginkel FW, et al. A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis. Human Gene Therapy. 12: 751-61. PMID 11339892 DOI: 10.1089/104303401750148667 |
0.32 |
|
| 2001 |
Braunstein GM, Roman RM, Clancy JP, Kudlow BA, Taylor AL, Shylonsky VG, Jovov B, Peter K, Jilling T, Ismailov II, Benos DJ, Schwiebert LM, Fitz JG, Schwiebert EM. Cystic fibrosis transmembrane conductance regulator facilitates ATP release by stimulating a separate ATP release channel for autocrine control of cell volume regulation. The Journal of Biological Chemistry. 276: 6621-30. PMID 11110786 DOI: 10.1074/Jbc.M005893200 |
0.328 |
|
| 1999 |
Clancy JP, Ruiz FE, Sorscher EJ. Adenosine and its nucleotides activate wild-type and R117H CFTR through an A2B receptor-coupled pathway. The American Journal of Physiology. 276: C361-9. PMID 9950763 DOI: 10.1152/Ajpcell.1999.276.2.C361 |
0.331 |
|
| 1999 |
Prince LS, Peter K, Hatton SR, Zaliauskiene L, Cotlin LF, Clancy JP, Marchase RB, Collawn JF. Efficient endocytosis of the cystic fibrosis transmembrane conductance regulator requires a tyrosine-based signal. The Journal of Biological Chemistry. 274: 3602-9. PMID 9920908 DOI: 10.1074/Jbc.274.6.3602 |
0.371 |
|
| 1998 |
Clancy JP, Hong JS, Bebök Z, King SA, Demolombe S, Bedwell DM, Sorscher EJ. 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. 37: 15222-30. PMID 9790686 DOI: 10.1021/Bi980436F |
0.356 |
|
| 1997 |
Bedwell DM, Kaenjak A, Benos DJ, Bebok Z, Bubien JK, Hong J, Tousson A, Clancy JP, Sorscher EJ. Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line. Nature Medicine. 3: 1280-4. PMID 9359706 DOI: 10.1038/Nm1197-1280 |
0.426 |
|
| 1997 |
Walker LC, Venglarik CJ, Aubin G, Weatherly MR, McCarty NA, Lesnick B, Ruiz F, Clancy JP, Sorscher EJ. Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR. American Journal of Respiratory and Critical Care Medicine. 155: 1684-9. PMID 9154877 DOI: 10.1164/Ajrccm.155.5.9154877 |
0.473 |
|
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