J. Clancy

发表

Flexible semiparametric joint modeling: an application to estimate individual lung function decline and risk of pulmonary exacerbations in cystic fibrosis

R. Szczesniak, Dan Li, R. Keogh, 2017, Emerging Themes in Epidemiology.

Cystic Fibrosis Transmembrane Conductance Regulator Facilitates ATP Release by Stimulating a Separate ATP Release Channel for Autocrine Control of Cell Volume Regulation*

J. Clancy, D. Benos, E. Schwiebert, 2001, The Journal of Biological Chemistry.

Improving Detection of Rapid Cystic Fibrosis Disease Progression–Early Translation of a Predictive Algorithm Into a Point-of-Care Tool

R. Szczesniak, J. Clancy, J. Pestian, 2019, IEEE Journal of Translational Engineering in Health and Medicine.

Early detection of rapid cystic fibrosis disease progression tailored to point of care: A proof-of-principle study

R. Szczesniak, J. Clancy, J. Pestian, 2017, 2017 IEEE Healthcare Innovations and Point of Care Technologies (HI-POCT).

Subacute TGFβ expression drives inflammation, goblet cell hyperplasia, and pulmonary function abnormalities in mice with effects dependent on CFTR function.

J. Clancy, S. Madala, W. Hardie, 2018, American journal of physiology. Lung cellular and molecular physiology.

Fibrocyte accumulation in the lungs of cystic fibrosis patients.

J. Clancy, A. Jegga, S. Randell, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Myofibroblast Differentiation and Enhanced Tgf-B Signaling in Cystic Fibrosis Lung Disease

W. T. Harris, J. Clancy, N. Ambalavanan, 2013, PloS one.

CRISPRi-mediated functional analysis of lung disease-associated loci at non-coding regions

J. Clancy, M. Guo, F. Lim, 2020, NAR genomics and bioinformatics.

Data driven decision making to characterize clinical personas of parents of children with cystic fibrosis: a mixed methods study

R. Szczesniak, Dan Li, J. Clancy, 2020, BMC Pulmonary Medicine.

Dynamic predictive probabilities to monitor rapid cystic fibrosis disease progression

P. Diggle, R. Szczesniak, R. Keogh, 2019, Statistics in medicine.

Joint Hierarchical Gaussian Process Model with Application to Forecast in Medical Monitoring

R. Szczesniak, J. Clancy, L. Duan, 2014, 1408.4660.

Cystic Fibrosis Point of Personalized Detection (CFPOPD): An Interactive Web Application

P. Diggle, R. Szczesniak, R. Keogh, 2020, JMIR medical informatics.

Long-term amikacin liposome inhalation suspension in cystic fibrosis patients with chronic P. aeruginosa infection.

J. Clancy, A. Quittner, M. Konstan, 2021, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Aerosolized agents for airway clearance in cystic fibrosis

J. Clancy, K. Southern, S. Ranganathan, 2019, Pediatric pulmonology.

Amikacin liposome inhalation suspension for chronic Pseudomonas aeruginosa infection in cystic fibrosis.

J. Clancy, A. Quittner, M. Konstan, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

AGGRESSIVE TREATMENT STRATEGIES TO OPTIMIZE PATIENT OUTCOMES

J. Clancy, 2009 .

Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line

J. Clancy, D. Benos, Z. Bebők, 1997, Nature Medicine.

Restoration of W1282X CFTR activity by enhanced expression.

J. Clancy, Z. Bebők, S. Rowe, 2007, American journal of respiratory cell and molecular biology.

An international randomized multicenter comparison of nasal potential difference techniques.

J. Clancy, F. Accurso, F. Vermeulen, 2010, Chest.

Reduced microtubule acetylation in cystic fibrosis epithelial cells.

J. Clancy, Deborah A. Corey, T. Kelley, 2013, American journal of physiology. Lung cellular and molecular physiology.

Matrix Metalloproteinase-9 Mediates RSV Infection in Vitro and in Vivo

J. Clancy, A. Gaggar, N. Ambalavanan, 2015, Viruses.

Diagnostic Testing in Cystic Fibrosis.

J. Clancy, J. Brewington, John P. Clancy, 2016, Clinics in chest medicine.

A truncated CFTR protein rescues endogenous ∆F508‐CFTR and corrects chloride transport in mice

J. Clancy, D. Benos, Jeong-Soo Hong, 2009, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.

An update on new and emerging therapies for cystic fibrosis

J. Clancy, K. Hudock, Kristin M. Hudock, 2017, Expert opinion on emerging drugs.

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.

J. Clancy, S. Freedman, F. Accurso, 2010, The New England journal of medicine.

Ultrashort Echo-Time Magnetic Resonance Imaging Is a Sensitive Method for the Evaluation of Early Cystic Fibrosis Lung Disease.

R. Szczesniak, J. Clancy, S. Serai, 2016, Annals of the American Thoracic Society.

DETANO and nitrated lipids increase chloride secretion across lung airway cells.

J. Clancy, S. Matalon, J. Collawn, 2008, American journal of respiratory cell and molecular biology.

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

E. Burchard, J. Galanter, J. Clancy, 2018, American journal of respiratory and critical care medicine.

Associations of Sputum Biomarkers with Clinical Outcomes in People with Cystic Fibrosis

R. Szczesniak, J. Clancy, A. Quittner, 2022, medRxiv.

Evaluating the Impact of Stopping Chronic Therapies after Modulator Drug Therapy in Cystic Fibrosis: The SIMPLIFY Clinical Trial Study Design

J. Clancy, G. Sawicki, K. Riekert, 2021, Annals of the American Thoracic Society.

Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction

J. Clancy, G. McPhail, J. Brewington, 2016, Expert review of respiratory medicine.

Home Monitoring of Patients with Cystic Fibrosis to Identify and Treat Acute Pulmonary Exacerbations. eICE Study Results

R. Gibson, C. Goss, J. Clancy, 2017, American journal of respiratory and critical care medicine.

Doxycycline improves clinical outcomes during cystic fibrosis exacerbations

J. Clancy, A. Gaggar, J. Blalock, 2017, European Respiratory Journal.

Adenosine regulation of cystic fibrosis transmembrane conductance regulator through prostenoids in airway epithelia.

J. Clancy, Wei Wang, Yao Li, 2006, American journal of respiratory cell and molecular biology.

Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function.

J. Clancy, J. Blalock, M. Dransfield, 2013, American journal of respiratory and critical care medicine.

Phase II studies of nebulised Arikace in CF patients with Pseudomonas aeruginosa infection

C. Goss, J. Clancy, L. Saiman, 2013, Thorax.

Cystic Fibrosis Transmembrane Conductance Regulator Function in Airway Smooth Muscle. A Novel Role in Cystic Fibrosis Airway Obstruction.

J. Clancy, 2016, American journal of respiratory and critical care medicine.

CFTR modulator therapies in pediatric cystic fibrosis: focus on ivacaftor

J. Clancy, E. Kramer, 2016, Expert opinion on orphan drugs.

Feasibility, tolerability and safety of pediatric hyperpolarized 129Xe magnetic resonance imaging in healthy volunteers and children with cystic fibrosis

J. Clancy, R. Thomen, J. Woods, 2016, Pediatric Radiology.

Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials.

J. Clancy, F. Accurso, S. Rowe, 2007, Proceedings of the American Thoracic Society.

Adenosine and its nucleotides activate wild-type and R117H CFTR through an A2B receptor-coupled pathway.

J. Clancy, E. Sorscher, F. Ruiz, 1999, The American journal of physiology.

CFTR functional assays in drug development

J. Clancy, J. Brewington, Sara Zak, 2017 .

Diagnosing cystic fibrosis in patients with non-diagnostic results: the case for intestinal current measurements

J. Clancy, 2010, Thorax.

Hyperpolarized 129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients.

J. Clancy, D. Roach, R. Thomen, 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Subacute TGFβ Exposure Drives Airway Hyperresponsiveness in CF Mice through the PI3K Pathway.

J. Clancy, S. Madala, E. Kramer, 2020, American journal of respiratory cell and molecular biology.

Subacute TGFb Exposure Drives Airway Hyperresponsiveness in Cystic Fibrosis Mice through the PI3K Pathway

J. Clancy, S. Madala, E. Kramer, 2020 .

Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in Cystic Fibrosis

J. Clancy, L. Hunter, G. McPhail, 2020, medRxiv.

A macromolecular complex of β2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA

J. Clancy, K. Kirk, G. Heda, 2002, Proceedings of the National Academy of Sciences of the United States of America.

Pulmonary matrix metalloproteinase-9 activity in mechanically ventilated children with respiratory syncytial virus

J. Clancy, A. Gaggar, N. Ambalavanan, 2013, European Respiratory Journal.

A Novel Proteolytic Cascade Generates an Extracellular Matrix-Derived Chemoattractant in Chronic Neutrophilic Inflammation12

J. Clancy, A. Gaggar, J. Blalock, 2008, The Journal of Immunology.

Regional Structure-function in Cystic Fibrosis Lung Disease Using Hyperpolarized 129Xe and Ultrashort Echo Magnetic Resonance Imaging.

J. Clancy, D. Roach, R. Thomen, 2020, American journal of respiratory and critical care medicine.

Clinical Effectiveness of Lumacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for F508del-CFTR. A Clinical Trial

J. Clancy, S. Donaldson, F. Ratjen, 2021, Annals of the American Thoracic Society.

Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study.

J. Clancy, S. Donaldson, F. Ratjen, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Early Elevation of Matrix Metalloproteinase-8 and -9 in Pediatric ARDS Is Associated with an Increased Risk of Prolonged Mechanical Ventilation

J. Clancy, A. Gaggar, R. Oster, 2011, PloS one.

A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis.

J. Clancy, J. Morris, Z. Bebők, 2001, Human gene therapy.

Activation of airway cl- secretion in human subjects by adenosine.

J. Clancy, R. Oster, E. Sorscher, 2004, American journal of respiratory cell and molecular biology.

No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations.

R. Gibson, J. Clancy, Z. Bebők, 2007, American journal of respiratory cell and molecular biology.

Respiratory syncytial virus infection disrupts monolayer integrity and function in cystic fibrosis airway cells.

R. Szczesniak, J. Clancy, E. Sorscher, 2013, Viruses.

Response Chloride Channels in Cystic Fibrosis Patients

Matthew P. Anderson, J. Clancy, M. Welsh, 1990, Science.

Cystic Fibrosis Transmembrane Conductance Regulator Trafficking Is Mediated by the COPI Coat in Epithelial Cells*

J. Clancy, Cristy Tower, K. Kirk, 2008, Journal of Biological Chemistry.

Up-regulation of AMP-activated Kinase by Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Airway Epithelial Cells Mitigates Excessive Inflammation*

J. Clancy, P. Reynolds, K. Hallows, 2006, Journal of Biological Chemistry.

Recombinant human acid α-glucosidase

B. Byrne, J. Clancy, H. Mandel, 2007, Neurology.

Plasma TGF‐β1 in pediatric cystic fibrosis: Potential biomarker of lung disease and response to therapy

W. T. Harris, J. Clancy, T. Noah, 2011, Pediatric pulmonology.

MicroRNA-145, Cystic Fibrosis Transmembrane Conductance Regulator, and Transforming Growth Factor-β. An (Un)tangled Regulatory Web.

J. Clancy, E. Kramer, 2017, American journal of respiratory and critical care medicine.

A2 adenosine receptors regulate CFTR through PKA and PLA2

J. Clancy, H. Greer, T. Kovaçs, 2002 .

Urinary kidney injury biomarkers and tobramycin clearance among children and young adults with cystic fibrosis: a population pharmacokinetic analysis

J. Clancy, S. Goldstein, A. Vinks, 2017, The Journal of antimicrobial chemotherapy.

A(2) adenosine receptors regulate CFTR through PKA and PLA(2).

J. Clancy, H. Greer, T. Kovaçs, 2002, American journal of physiology. Lung cellular and molecular physiology.

Changes in Glucose Breath Test in Cystic Fibrosis Patients Treated With 1 Month of Lumacaftor/Ivacaftor

J. Clancy, S. Rowe, Hongyue Wang, 2022, Journal of pediatric gastroenterology and nutrition.

Mutations in the Amino Terminus of the Cystic Fibrosis Transmembrane Conductance Regulator Enhance Endocytosis*

J. Clancy, Z. Bebők, K. Kirk, 2006, Journal of Biological Chemistry.

Emerging Approaches to Monitor and Modify Care in the Era of Cystic Fibrosis Transmembrane Conductance Regulators.

J. Clancy, F. Ratjen, C. Britto, 2022, Clinics in chest medicine.

Brushed nasal epithelial cells are a surrogate for bronchial epithelial CFTR studies.

J. Clancy, J. Brewington, J. Moncivaiz, 2018, JCI insight.

PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy.

L. Hoffman, M. Rosenfeld, J. Clancy, 2021, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Clinical Effectiveness of Lumacaftor/Ivacaftor in Cystic Fibrosis Patients Homozygous for F508del-CFTR.

J. Clancy, S. Donaldson, F. Ratjen, 2020, Annals of the American Thoracic Society.

Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials.

Kapil M. Patel, D. Sanders, R. Gibson, 2022, The Lancet. Respiratory medicine.

Effects of an Antioxidant‐enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial

J. Clancy, K. McCoy, D. Orenstein, 2018, American journal of respiratory and critical care medicine.

Take it to the Bank.

J. Clancy, E. Joseloff, 2017, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Early Treatment With Alglucosidase Alfa Prolongs Long-Term Survival of Infants With Pompe Disease

J. Clancy, H. Mandel, N. Leslie, 2009, Pediatric Research.

Adenosine receptors, cystic fibrosis, and airway hydration.

J. Clancy, G. Com, 2009, Handbook of experimental pharmacology.

Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease.

B. Byrne, J. Clancy, H. Mandel, 2007, Neurology.

Open label study of inhaled aztreonam for Pseudomonas eradication in children with cystic fibrosis: The ALPINE study.

J. Clancy, S. Lewis, H. Tiddens, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI.

Phuong T. Vu, L. Hoffman, M. Rosenfeld, 2022, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Dissociation of systemic and mucosal autoimmunity in cystic fibrosis.

J. Clancy, A. Ashare, E. Zemanick, 2020, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Nasal potential difference measurements to assess CFTR ion channel activity.

J. Clancy, S. Rowe, M. Wilschanski, 2011, Methods in molecular biology.

Multivariate joint modeling to identify markers of growth and lung function decline that predict cystic fibrosis pulmonary exacerbation onset

E. Andrinopoulou, R. Szczesniak, J. Clancy, 2019, BMC Pulmonary Medicine.

Extracellular Zinc and ATP Restore Chloride Secretion across Cystic Fibrosis Airway Epithelia by Triggering Calcium Entry*

J. Clancy, Z. Bebők, E. Schwiebert, 2004, Journal of Biological Chemistry.

Molecular and Cell Biology of Adenosine Receptors

J. Clancy, B. Cobb, 2003 .

Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in a Pediatric Cystic Fibrosis Cohort.

J. Clancy, L. Hunter, M. P. Feeney, 2021, American journal of audiology.

Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis.

J. Clancy, D. Bedwell, M. Macaluso, 2001, American journal of respiratory and critical care medicine.

Pharmaceuticals Targeting Nonsense Mutations in Genetic Diseases

J. Clancy, S. Rowe, 2012, BioDrugs.

Bayesian Ensemble Trees (BET) for Clustering and Prediction in Heterogeneous Data

Leo L Duan, John P Clancy, Rhonda D Szczesniak, 2016, Journal of computational and graphical statistics : a joint publication of American Statistical Association, Institute of Mathematical Statistics, Interface Foundation of North America.

BET: Bayesian Ensemble Trees for Clustering and Prediction in Heterogeneous Data

R. Szczesniak, J. Clancy, L. Duan, 2014, 1408.4140.

TGFβ as a therapeutic target in cystic fibrosis

J. Clancy, E. Kramer, 2018, Expert opinion on therapeutic targets.

Chronic β2AR stimulation limits CFTR activation in human airway epithelia.

J. Clancy, Xiaoting Zhu, E. Kramer, 2018, JCI insight.

Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators.

J. Clancy, S. Rowe, G. Bolger, 2011, American journal of physiology. Lung cellular and molecular physiology.

Adenosine receptors and phosphodiesterase inhibitors stimulate Cl- secretion in Calu-3 cells.

J. Clancy, T. Kovaçs, E. Sorscher, 2003, American journal of respiratory cell and molecular biology.

CFTR modulator theratyping: Current status, gaps and future directions.

J. Wallenburg, J. Clancy, J. Nick, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Aortopulmonary collateral flow in cystic fibrosis assessed with phase-contrast MRI

R. Szczesniak, J. Clancy, R. Amin, 2013, Pediatric Radiology.

Multicenter Intestinal Current Measurements in Rectal Biopsies from CF and Non-CF Subjects to Monitor CFTR Function

R. Szczesniak, J. Clancy, S. Donaldson, 2013, PloS one.

Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence

J. Clancy, K. Kotha, 2013, Therapeutic advances in respiratory disease.

Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR.

J. Clancy, B. Lesnick, N. McCarty, 1997, American journal of respiratory and critical care medicine.

CFTR Modulator Therapies in Cystic Fibrosis

J. Clancy, D. Spielberg, C. Siracusa, 2017 .

Assessment of CFTR localisation in native airway epithelial cells obtained by nasal brushing.

J. Clancy, Z. Bebők, M. Amaral, 2004, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

A Comparison between Two Pathophysiologically Different yet Microbiologically Similar Lung Diseases: Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.

J. Clancy, D. Hassett, Warunya Panmanee, 2018, International journal of respiratory and pulmonary medicine.

Regulation of chloride channels by protein kinase C in normal and cystic fibrosis airway epithelia.

P. Greengard, A. Nairn, J. Clancy, 1989, Science.

Progress in cystic fibrosis and the CF Therapeutics Development Network

John P Clancy, J. Clancy, S. Donaldson, 2012, Thorax.

Measurement of ion transport function in rectal biopsies.

J. Clancy, N. Derichs, M. Hug, 2011, Methods in molecular biology.

Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial.

Thomas Ferkol, Carlos Milla, Frank Accurso, 2007, Human gene therapy.

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.

J. Clancy, D. Waltz, B. Ramsey, 2004, Chest.

Airway microbiota across age and disease spectrum in cystic fibrosis

W. T. Harris, R. Gibson, J. Clancy, 2017, European Respiratory Journal.

The Caregiver Quality of Life Cystic Fibrosis (CQOLCF) Scale: Modification and validation of an instrument to measure quality of life in cystic fibrosis family caregivers

J. Clancy, D. Macrina, W. Boling, 2003, Quality of Life Research.

Recombinant Acid Ceramidase Reduces Inflammation and Infection in Cystic Fibrosis

J. Clancy, E. Gulbins, A. Koulman, 2020, American journal of respiratory and critical care medicine.

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.

C. Goss, J. Clancy, L. Herzenberg, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Nucleosides accelerate inflammatory osteolysis, acting as distinct innate immune activators

J. Clancy, Pingar Yang, J. McDonald, 2011, Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research.

Evaluation Of Two Phase II Blinded and Placebo-Controlled Studies Of Nebulized Liposomal Amikacin (Arikace") In The Treatment Of Cystic Fibrosis Patients With Pseudomonas Aeruginosa Lung Infection

C. Goss, J. Clancy, L. Saiman, 2010, ATS 2010.

Matrix metalloprotease-9 dysregulation in lower airway secretions of cystic fibrosis patients.

J. Clancy, A. Gaggar, J. Blalock, 2007, American journal of physiology. Lung cellular and molecular physiology.

Potential role of high-mobility group box 1 in cystic fibrosis airway disease.

J. Clancy, A. Gaggar, J. Blalock, 2008, American journal of respiratory and critical care medicine.

Influences of environmental exposures on individuals living with cystic fibrosis

E. Andrinopoulou, R. Szczesniak, R. Keogh, 2020, Expert review of respiratory medicine.

Direct Lung Sampling Indicates That Established Pathogens Dominate Early Infections in Children with Cystic Fibrosis.

C. Goss, J. Clancy, Z. Ehsan, 2019, Cell reports.

Detection of CFTR function and modulation in primary human nasal cell spheroids.

R. Szczesniak, J. Clancy, J. Brewington, 2018, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.

J. Clancy, Y. Li, S. Rowe, 2010, Pulmonary pharmacology & therapeutics.

Failure of cAMP agonists to activate rescued ΔF508 CFTR in CFBE41o– airway epithelial monolayers

J. Wakefield, J. Clancy, Z. Bebők, 2005, The Journal of physiology.

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study.

J. Clancy, J. Brewington, J. Moncivaiz, 2018, Journal of visualized experiments : JoVE.

Phenotypic Alteration of an Established Human Airway Cell Line by Media Selection

J. Clancy, G. Livnat, J. Brewington, 2023, International journal of molecular sciences.

Personalised CFTR pharmacotherapeutic response testing and therapy of cystic fibrosis

J. Clancy, B. Trapnell, C. McCarthy, 2018, European Respiratory Journal.

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

J. Clancy, M. Ballmann, F. Accurso, 2011, Thorax.

Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial

L. Hoffman, M. Rosenfeld, J. Clancy, 2021, American journal of respiratory and critical care medicine.

PharmGKB summary: ivacaftor pathway, pharmacokinetics/pharmacodynamics

R. Altman, E. McDonagh, T. Klein, 2017, Pharmacogenetics and genomics.

TGF-Beta Downregulation of Distinct Chloride Channels in Cystic Fibrosis-Affected Epithelia

W. T. Harris, J. Clancy, Y. Sanders, 2014, PloS one.

Phenotypes of Rapid Cystic Fibrosis Lung Disease Progression during Adolescence and Young Adulthood

John Pestian, Michael Seid, Rhonda D. Szczesniak, 2017, American journal of respiratory and critical care medicine.

Neutrophil extracellular traps activate IL-8 and IL-1 expression in human bronchial epithelia.

J. Clancy, Yan Xu, B. Trapnell, 2020, American journal of physiology. Lung cellular and molecular physiology.

Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor.

J. Clancy, D. Drotar, Nanhua Zhang, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Rapid therapeutic advances in CFTR modulator science

J. Clancy, 2018, Pediatric pulmonology.

Current state of the art MRI for the longitudinal assessment of cystic fibrosis

H. Kauczor, E. V. van Beek, J. Clancy, 2019, Journal of magnetic resonance imaging : JMRI.

Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data.

J. Clancy, F. van Goor, F. Accurso, 2014, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

The impact of SARS-CoV-2 on the cystic fibrosis foundation therapeutics development network

C. Goss, J. Clancy, D. Nichols, 2020, Journal of Cystic Fibrosis.

Data driven decision making to characterize clinical personas of parents of children with cystic fibrosis: a mixed methods study

R. Szczesniak, Dan Li, J. Clancy, 2020, BMC Pulmonary Medicine.

Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis.

J. Clancy, L. Moldawer, Jeanette Lee, 2002, The Journal of pediatrics.

Clinical Pharmacogenetics Implementation Consortium (CPIC) Guidelines for Ivacaftor Therapy in the Context of CFTR Genotype

E. McDonagh, T. Klein, J. Clancy, 2014, Clinical pharmacology and therapy.

Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial.

R. Szczesniak, J. Clancy, S. Powers, 2015, JAMA pediatrics.

Behavioral and Nutritional Treatment for Preschool-Aged ChildrenWith Cystic Fibrosis

J. Clancy, J. Rausch, R. Castile, 2015 .

Efficient Endocytosis of the Cystic Fibrosis Transmembrane Conductance Regulator Requires a Tyrosine-based Signal*

R. Marchase, J. Clancy, L. Zaliauskiene, 1999, The Journal of Biological Chemistry.

T100: nebulized-concentrated tobramycin formulation for treatment of Pseudomonas aeruginosa infection in cystic fibrosis patients

J. Clancy, Z. Ehsan, 2015 .

Prospective multicenter randomized patient recruitment and sample collection to enable future measurements of sputum biomarkers of inflammation in an observational study of cystic fibrosis

R. Keogh, J. Clancy, A. Quittner, 2019, BMC Medical Research Methodology.

Seasonal variation of lung function in cystic fibrosis: longitudinal modeling to compare a Midwest US cohort to international populations.

E. Andrinopoulou, R. Szczesniak, R. Keogh, 2021, The Science of the total environment.

Advances in cystic fibrosis therapies

J. Clancy, S. Rowe, 2006, Current opinion in pediatrics.

Neutrophil Extracellular Trap Burden in CF and Disease Control BAL

J. Clancy, E. Kramer, K. Hudock, 2020 .

Reduced sodium transport with nasal administration of the prostasin inhibitor camostat in subjects with cystic fibrosis.

J. Clancy, D. Renard, H. Danahay, 2013, Chest.

Bayesian regularization for a nonstationary Gaussian linear mixed effects model

R. Szczesniak, J. Clancy, S. Sivaganesan, 2021, Statistics in medicine.

A semiparametric approach to estimate rapid lung function decline in cystic fibrosis.

Raouf S Amin, Leo L Duan, John P Clancy, 2013, Annals of epidemiology.

Built environment factors predictive of early rapid lung function decline in cystic fibrosis

E. Andrinopoulou, R. Szczesniak, R. Keogh, 2023, Pediatric pulmonology.

Risk factors for acute kidney injury during aminoglycoside therapy in patients with cystic fibrosis

W. T. Harris, J. Clancy, S. Goldstein, 2015, Pediatric Nephrology.

Cystic Fibrosis and Its Management Through Established and Emerging Therapies.

J. Clancy, D. Spielberg, 2016, Annual review of genomics and human genetics.

Continuous alternating inhaled antibiotics for chronic pseudomonal infection in cystic fibrosis.

J. Clancy, S. Lewis, D. E. Tullis, 2016, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Seasonality, mediation and comparison (SMAC) methods to identify influences on lung function decline

Ruth H. Keogh, Eleni-Rosalina Andrinopoulou, Yizhao Ni, 2021, MethodsX.

Purification, Characterization, and Expression of CFTR Nucleotide-Binding Domains

J. Clancy, E. Sorscher, Zs. Bebök, 1997, Journal of bioenergetics and biomembranes.

Personalized medicine in cystic fibrosis: dawning of a new era.

J. Clancy, M. Jain, 2012, American journal of respiratory and critical care medicine.

Activating Cystic Fibrosis Transmembrane Conductance Regulator Channels with Pore Blocker Analogs*

J. Clancy, K. Kirk, Wei Wang, 2005, Journal of Biological Chemistry.

Capturing the Direct Binding of CFTR Correctors to CFTR by Using Click Chemistry

Chandrima Sinha, Kavisha Arora, Anjaparavanda P. Naren, 2015, Chembiochem : a European journal of chemical biology.

Guanylate cyclase 2C agonism corrects CFTR mutants.

J. Clancy, M. Helmrath, Nambirajan Sundaram, 2017, JCI insight.

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.

J. Clancy, S. Demolombe, D. Bedwell, 1998, Biochemistry.

Management of Pseudomonas aeruginosa infection in cystic fibrosis patients using inhaled antibiotics with a focus on nebulized liposomal amikacin.

J. Clancy, Z. Ehsan, 2015, Future microbiology.

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

J. Clancy, L. Dupont, M. Konstan, 2015 .

ANALYSIS OF LONG-TERM LIPOSOMAL AMIKACIN FOR INHALATION IN PATIENTS WITH CYSTIC FIBROSIS AND CHRONIC INFECTION FROM PSEUDOMONAS AERUGINOSA

J. Clancy, L. Dupont, M. Konstan, 2014 .

Utilizing centralized biorepository samples for biomarkers of cystic fibrosis lung disease severity.

J. Clancy, K. Setchell, J. Pilewski, 2019, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia.

J. Clancy, S. Plafker, A. Ziady, 2019, The Journal of clinical investigation.

Assessment of pulmonary structure–function relationships in young children and adolescents with cystic fibrosis by multivolume proton‐MRI and CT

Andrea Aliverti, J. Clancy, A. Aliverti, 2018, Journal of magnetic resonance imaging : JMRI.

Cystic Fibrosis–Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes

J. Clancy, M. Rickels, L. Sussel, 2023, Diabetes care.

Cystic Fibrosis–Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes

J. Clancy, M. Rickels, L. Sussel, 2023, Diabetes.

Optimizing Nasal Potential Difference Analysis for CFTR Modulator Development: Assessment of Ivacaftor in CF Subjects with the G551D-CFTR Mutation

J. Clancy, F. Accurso, E. Olson, 2013, PloS one.

An Animated Functional Data Analysis Interface to Cluster Rapid Lung Function Decline and Enhance Center-Level Care in Cystic Fibrosis

R. Szczesniak, J. Clancy, W. Su, 2021, Journal of healthcare engineering.

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.

Rhonda Szczesniak, Weiji Su, R. Szczesniak, 2016, Internal medicine review.

Using Spirituality After an Adult CF Diagnosis: Cognitive Reframing and Adherence Motivation

Judith R. Ragsdale, J. Clancy, D. Grossoehme, 2012, Journal of health care chaplaincy.

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.

J. Clancy, S. Rowe, M. Mazur, 2010, American journal of respiratory cell and molecular biology.

PharmGKB summary: very important pharmacogene information for CFTR

R. Altman, E. McDonagh, T. Klein, 2015, Pharmacogenetics and genomics.

Membrane transplantation to correct integral membrane protein defects

J. Clancy, D. Benos, E. Hunter, 2003, Journal of Molecular Medicine.

Human Neutrophil Elastase-Mediated Cleavage Sites of MMP-9 and TIMP-1: Implications to Cystic Fibrosis Proteolytic Dysfunction

J. Clancy, A. Gaggar, J. Blalock, 2010, Molecular medicine.

Nebulized liposomal amikacin for the treatment of Pseudomonas aeruginosa infection in cystic fibrosis patients

J. Clancy, Z. Ehsan, J. D. Wetzel, 2014, Expert opinion on investigational drugs.

Constrictive Bronchiolitis in Cystic Fibrosis Adolescents with Refractory Pulmonary Decline.

W. T. Harris, R. Szczesniak, J. Clancy, 2016, Annals of the American Thoracic Society.

Daily serum creatinine monitoring promotes earlier detection of acute kidney injury in children and adolescents with cystic fibrosis.

J. Clancy, S. Goldstein, M. Rao, 2014, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Parallel effects of VX-770 on transepithelial potential difference in vitro and in vivo

J. Clancy, F. Accurso, M. Ashlock, 2010 .

Relationship between Pulmonary outcomes, biomarkers Of CF Disease, And Serum drug Levels in Subjects with The G551D-CFTR Mutation Treated With VX-770, An Investigational Oral Potentiator Of CFTR

J. Clancy, F. Accurso, M. Ashlock, 2010, ATS 2010.

Final results of a 14- and 28-day study of VX-770 in subjects with CF

J. Clancy, S. Freedman, F. Accurso, 2009 .

Improvement in ion transport biomarkers and spirometry with the investigational CFTR potentiator VX-770 in subjects with cystic fibrosis and the G551D-CFTR mutation

J. Clancy, F. Accurso, J. Pilewski, 2010 .

Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density, but lung infections generally persist

L. Hoffman, J. Clancy, S. Rowe, 2023, The Journal of clinical investigation.

Cystic fibrosis transmembrane conductance regulator modulators

J. Clancy, 2013 .

Antisense oligonucleotide eluforsen improves CFTR function in F508del cystic fibrosis

J. Clancy, J. Bolognese, J. Nick, 2018, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function.

R. Szczesniak, J. Clancy, L. Duan, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Fibrosis: A Multicenter, Double-Blind, Placebo-Controlled Trial Cystic Transmembrane Regulator Gene Transfer to the Lungs of Patients With Repeated Adeno-Associated Virus Serotype 2 Aerosol-Mediated Cystic Fibrosis

J. Clancy, D. Waltz, B. Ramsey, 2006 .

Calcium-dependent regulation of airway epithelial chloride channels.

J. Clancy, M. Welsh, M. Li, 1990, The American journal of physiology.

Safety and efficacy of alternative alglucosidase alfa regimens in Pompe disease

J. Clancy, J. Charrow, C. Tifft, 2015, Neuromuscular Disorders.

Brushed nasal epithelial cells are a surrogate for bronchial epithelial CFTR studies.

J. Clancy, J. Brewington, J. Moncivaiz, 2018, JCI insight.

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study.

J. Clancy, J. Brewington, J. Moncivaiz, 2018, Journal of visualized experiments : JoVE.

Correction: Myofibroblast Differentiation and Enhanced Tgf-B Signaling in Cystic Fibrosis Lung Disease

W. T. Harris, J. Clancy, N. Ambalavanan, 2013, PLoS ONE.

Clinically-approved CFTR modulators rescue Nrf2 dysfunction in cystic fibrosis airway epithelia.

J. Clancy, S. Plafker, A. Ziady, 2019, The Journal of clinical investigation.

Sweat Chloride Testing.

J. Clancy, C. McCarthy, J. Brewington, 2019, JAMA.

A Comparison between Two Pathophysiologically Different yet Microbiologically Similar Lung Diseases: Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.

J. Clancy, D. Hassett, Warunya Panmanee, 2018, International journal of respiratory and pulmonary medicine.

Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis.

J. Clancy, G. McPhail, 2013, Drugs of today.

Open label study of inhaled aztreonam for Pseudomonas eradication in children with cystic fibrosis: The ALPINE study.

J. Clancy, S. Lewis, C. Oermann, 2015, Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society.

Joint hierarchical Gaussian process model with application to personalized prediction in medical monitoring

Xia Wang, Leo L Duan, John P Clancy, 2018, Stat.

CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity

J. Clancy, E. Kramer, C. Davidson, 2023, Respiratory Research.

Predicting future lung function decline in cystic fibrosis patients: Statistical methods and clinical connections

Raouf S Amin, John P Clancy, Rhonda D Szczesniak, 2016, Pediatric pulmonology.

Phosphorylation‐Dependent Regulation of Apical Membrane Chloride Channels in Normal and Cystic Fibrosis Airway Epithelium a

J. Clancy, M. Welsh, J. D. McCann, 1989, Annals of the New York Academy of Sciences.