| Year |
Title |
Altmetric |
| 2022
|
Inhaled gene therapy of preclinical muco-obstructive lung diseases by nanoparticles capable of breaching the airway mucus barrier.
Thorax.
77:812-820.
2022
|
|
| 2022
|
COVID-19 Causes Ciliary Dysfunction as Demonstrated by Human Intranasal Micro-Optical Coherence Tomography Imaging.
2022
|
|
| 2022
|
Changes in Glucose Breath Test in Cystic Fibrosis Patients Treated With 1 Month of Lumacaftor/Ivacaftor.
Journal of Pediatric Gastroenterology and Nutrition.
75:42-47.
2022
|
|
| 2022
|
Evaluation of a novel CFTR potentiator in COPD ferrets with acquired CFTR dysfunction.
European Respiratory Journal.
60.
2022
|
|
| 2022
|
Reply to Martin et al..
American Journal of Respiratory and Critical Care Medicine.
205:1366-1367.
2022
|
|
| 2022
|
Ivacaftor restores delayed mucociliary transport caused by Pseudomonas aeruginosa–induced acquired cystic fibrosis transmembrane conductance regulator dysfunction in rabbit nasal epithelia.
International Forum of Allergy and Rhinology.
12:690-698.
2022
|
|
| 2022
|
Human distal airways contain a multipotent secretory cell that can regenerate alveoli.
Nature.
604:120-126.
2022
|
|
| 2022
|
Cystic Fibrosis Transmembrane Conductance Regulator: Roles in Chronic Obstructive Pulmonary Disease.
American Journal of Respiratory and Critical Care Medicine.
205:631-640.
2022
|
|
| 2022
|
Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis A Clinical Trial.
American Journal of Respiratory and Critical Care Medicine.
205:529-539.
2022
|
|
| 2022
|
Static mucus impairs bacterial clearance and allows chronic infection with Pseudomonas aeruginosa in the cystic fibrosis rat..
European Respiratory Journal.
2022
|
|
| 2022
|
Mucociliary Transport Deficiency and Disease Progression in Syrian Hamsters with SARS-CoV-2 Infection.
2022
|
|
| 2022
|
Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study.
Journal of Cystic Fibrosis.
2022
|
|
| 2022
|
Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study.
Journal of Cystic Fibrosis.
21:143-145.
2022
|
|
| 2022
|
Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D.
Journal of Cystic Fibrosis.
2022
|
|
| 2022
|
Poly (acetyl, arginyl) glucosamine disrupts Pseudomonas aeruginosa biofilms and enhances bacterial clearance in a rat lung infection model.
Microbiology.
168.
2022
|
|
| 2021
|
A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion.
Nature Communications.
12.
2021
|
|
| 2021
|
Inhaled high molecular weight hyaluronan ameliorates respiratory failure in acute COPD exacerbation: a pilot study.
Respiratory Research.
22.
2021
|
|
| 2021
|
Empire-CF study: A phase 2 clinical trial of leukotriene A4 hydrolase inhibitor acebilustat in adult subjects with cystic fibrosis.
Journal of Cystic Fibrosis.
20:1026-1034.
2021
|
|
| 2021
|
Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study.
Journal of Cystic Fibrosis.
20:965-971.
2021
|
|
| 2021
|
Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis.
Journal of Cystic Fibrosis.
20:1018-1025.
2021
|
|
| 2021
|
Minimally Invasive Image-Guided Gut Transport Function Measurement Probe.
Frontiers in Physics.
9.
2021
|
|
| 2021
|
Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation.
Journal of Cystic Fibrosis.
20:865-875.
2021
|
|
| 2021
|
Triple therapy for cystic fibrosis Phe508del-gating and -residual function genotypes.
New England Journal of Medicine.
385:815-825.
2021
|
|
| 2021
|
Cessation of smoke exposure improves pediatric CF outcomes: Longitudinal analysis of CF Foundation Patient Registry data.
Journal of Cystic Fibrosis.
20:618-624.
2021
|
|
| 2021
|
LPS decreases CFTR open probability and mucociliary transport through generation of reactive oxygen species.
Redox Biology.
43.
2021
|
|
| 2021
|
Long-term safety and efficacy of tezacaftor–ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
The Lancet Respiratory Medicine.
9:733-746.
2021
|
|
| 2021
|
Tobacco smoke exposure limits the therapeutic benefit of tezacaftor/ivacaftor in pediatric patients with cystic fibrosis.
Journal of Cystic Fibrosis.
20:612-617.
2021
|
|
| 2021
|
A phase 3 open-label study of elexacaftor/tezacaftor/ivacaftor in children 6 through 11 years of age with cystic fibrosis and at least one F508del allele.
American Journal of Respiratory and Critical Care Medicine.
203:1522-1532.
2021
|
|
| 2021
|
Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series.
Journal of Cystic Fibrosis.
20:399-401.
2021
|
|
| 2021
|
Novel correctors and potentiators enhance translational readthrough in CFTR nonsense mutations.
American Journal of Respiratory Cell and Molecular Biology.
64:604-616.
2021
|
|
| 2021
|
Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.
Journal of Cystic Fibrosis.
20:213-219.
2021
|
|
| 2021
|
Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients.
Journal of Cystic Fibrosis.
20:333-338.
2021
|
|
| 2021
|
PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy.
Journal of Cystic Fibrosis.
20:205-212.
2021
|
|
| 2021
|
Safety and efficacy of the cystic fibrosis transmembrane conductance regulator potentiator icenticaftor (QBW251).
Journal of Cystic Fibrosis.
20:250-256.
2021
|
|
| 2021
|
Tezacaftor/ivacaftor in people with cystic fibrosis who stopped lumacaftor/ivacaftor due to respiratory adverse events.
Journal of Cystic Fibrosis.
20:228-233.
2021
|
|
| 2021
|
Clinical Effectiveness of Lumacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for F508del-CFTR A Clinical Trial.
Annals of the American Thoracic Society.
18:75-83.
2021
|
|
| 2021
|
Korean Red Ginseng aqueous extract improves markers of mucociliary clearance by stimulating chloride secretion.
Journal of Ginseng Research.
45:66-74.
2021
|
|
| 2020
|
A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay.
Frontiers in Physiology.
11.
2020
|
|
| 2020
|
Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study.
Journal of Cystic Fibrosis.
19:931-933.
2020
|
|
| 2020
|
G551D mutation impairs PKA-dependent activation of CFTR channel that can be restored by novel GOF mutations.
American Journal of Physiology: Lung Cellular and Molecular Physiology.
319:L770-L785.
2020
|
|
| 2020
|
Ivacaftor reverses airway mucus abnormalities in a rat model harboring a humanized G551D-CFTR.
American Journal of Respiratory and Critical Care Medicine.
202:1271-1282.
2020
|
|
| 2020
|
Novel therapy of bicarbonate, glutathione, and ascorbic acid improves cystic fibrosis mucus transport.
American Journal of Respiratory Cell and Molecular Biology.
63:362-373.
2020
|
|
| 2020
|
Tobacco smoke exposure and socioeconomic factors are independent predictors of pulmonary decline in pediatric cystic fibrosis.
Journal of Cystic Fibrosis.
19:783-790.
2020
|
|
| 2020
|
Variable cellular ivacaftor concentrations in people with cystic fibrosis on modulator therapy.
Journal of Cystic Fibrosis.
19:742-745.
2020
|
|
| 2020
|
Transparency and diversity in cystic fibrosis research – Authors' reply.
The Lancet.
396:602.
2020
|
|
| 2020
|
Pharmacological approaches for targeting cystic fibrosis nonsense mutations.
European Journal of Medicinal Chemistry.
200.
2020
|
|
| 2020
|
Contribution of Short Chain Fatty Acids to the Growth of Pseudomonas aeruginosa in Rhinosinusitis.
Frontiers in Cellular and Infection Microbiology.
10.
2020
|
|
| 2020
|
Fibroblast Growth Factor Receptor 4 Deficiency Mediates Airway Inflammation in the Adult Healthy Lung?.
Frontiers in Medicine.
7.
2020
|
|
| 2020
|
Airway remodeling in ferrets with cigarette smoke-induced COPD using μCT imaging.
American Journal of Physiology: Lung Cellular and Molecular Physiology.
319:L11-L20.
2020
|
|
| 2020
|
Ataluren/ivacaftor combination therapy: Two N-of-1 trials in cystic fibrosis patients with nonsense mutations.
Pediatric Pulmonology.
55:1838-1842.
2020
|
|
| 2020
|
Efficacy and safety of ataluren in patients with nonsense-mutation cystic fibrosis not receiving chronic inhaled aminoglycosides: The international, randomized, double-blind, placebo-controlled Ataluren Confirmatory Trial in Cystic Fibrosis (ACT CF).
Journal of Cystic Fibrosis.
19:595-601.
2020
|
|
| 2020
|
Haemophilus influenzae persists in biofilm communities in a smoke-exposed ferret model of COPD..
ERJ Open Research.
6.
2020
|
|
| 2020
|
CFTR targeted therapies: Recent advances in cystic fibrosis and possibilities in other diseases of the airways.
European Respiratory Review.
29:1-13.
2020
|
|
| 2020
|
Brd4-p300 inhibition downregulates Nox4 and accelerates lung fibrosis resolution in aged mice.
JCI insight.
5.
2020
|
|
| 2020
|
Human nasal epithelial organoids for therapeutic development in cystic fibrosis.
Genes.
11:1-17.
2020
|
|
| 2020
|
Cystic fibrosis: Emergence of highly effective targeted therapeutics and potential clinical implications.
American Journal of Respiratory and Critical Care Medicine.
201:1193-1208.
2020
|
|
| 2020
|
Controlled delivery of ciprofloxacin and ivacaftor via sinus stent in a preclinical model of Pseudomonas sinusitis.
International Forum of Allergy and Rhinology.
10:481-488.
2020
|
|
| 2020
|
The impact of Lactococcus lactis (probiotic nasal rinse) co-culture on growth of patient-derived strains of Pseudomonas aeruginosa.
International Forum of Allergy and Rhinology.
10:444-449.
2020
|
|
| 2020
|
Disease modification and biomarker development in Parkinson disease: Revision or reconstruction?.
Neurology.
94:481-494.
2020
|
|
| 2020
|
Gaming Console Home-Based Exercise for Adults with Cystic Fibrosis: Study Protocol..
International Journal of Caring Sciences.
13:1530-1540.
2020
|
|
| 2020
|
Triple Therapy for Cystic Fibrosis with a Phe508del CFTR Mutation. Reply.
New England Journal of Medicine.
382:684.
2020
|
|
| 2020
|
Pulmonary artery enlargement is associated with pulmonary hypertension and decreased survival in severe cystic fibrosis: A cohort study.
PLoS One.
15.
2020
|
|
| 2020
|
The Effects of the Anti-aging Protein Klotho on Mucociliary Clearance.
Frontiers in Medicine.
6.
2020
|
|
| 2020
|
Changes in airway microbiome and inflammation with ivacaftor treatment in patients with cystic fibrosis and the G551D mutation.
Annals of the American Thoracic Society.
17:212-220.
2020
|
|
| 2020
|
Efficacy and safety of the cftr potentiator icenticaftor (Qbw251) in copd: Results from a phase 2 randomized trial.
The International Journal of Chronic Obstructive Pulmonary Disease.
15:2399-2409.
2020
|
|
| 2020
|
Excess mucus viscosity and airway dehydration impact COPD airway clearance.
European Respiratory Journal.
55.
2020
|
|
| 2020
|
Females with cystic fibrosis demonstrate a differential response profile to ivacaftor compared with males.
American Journal of Respiratory and Critical Care Medicine.
201:996-998.
2020
|
|
| 2020
|
The future of cystic fibrosis care: a global perspective.
The Lancet Respiratory Medicine.
8:65-124.
2020
|
|
| 2019
|
Prevalence of chronic rhinosinusitis in bronchiectasis patients suspected of ciliary dyskinesia.
International Forum of Allergy and Rhinology.
9:1430-1435.
2019
|
|
| 2019
|
Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial.
The Lancet.
394:1940-1948.
2019
|
|
| 2019
|
Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele.
New England Journal of Medicine.
381:1809-1819.
2019
|
|
| 2019
|
Area deprivation as a risk factor for methicillin-resistant Staphylococcus aureus infection in pediatric cystic fibrosis.
The Pediatric Infectious Disease Journal.
38:E285-E289.
2019
|
|
| 2019
|
Co-cultured microfluidic model of the airway optimized for microscopy and micro-optical coherence tomography imaging.
Biomedical Optics Express.
10:5414-5430.
2019
|
|
| 2019
|
Revealing the molecular signaling pathways of mucus stasis in cystic fibrosis.
Journal of Clinical Investigation.
129:4089-4090.
2019
|
|
| 2019
|
Geometry-Dependent Spectroscopic Contrast in Deep Tissues.
iScience.
19:965-975.
2019
|
|
| 2019
|
Intranasal micro-optical coherence tomography imaging for cystic fibrosis studies.
Science Translational Medicine.
11.
2019
|
|
| 2019
|
Vaporized e-cigarette liquids induce ion transport dysfunction in airway epithelia.
American Journal of Respiratory Cell and Molecular Biology.
61:162-173.
2019
|
|
| 2019
|
The effect of CFTR modulators on a cystic fibrosis patient presenting with recurrent pancreatitis in the absence of respiratory symptoms: A case report.
BMC Gastroenterology.
19.
2019
|
|
| 2019
|
Antisense oligonucleotide eluforsen improves CFTR function in F508del cystic fibrosis.
Journal of Cystic Fibrosis.
18:536-542.
2019
|
|
| 2019
|
Herbal dry extract BNO 1011 improves clinical and mucociliary parameters in a rabbit model of chronic rhinosinusitis.
International Forum of Allergy and Rhinology.
9:629-637.
2019
|
|
| 2019
|
Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles.
ERJ Open Research.
5.
2019
|
|
| 2019
|
Evaluation of 1,2,3-Triazoles as Amide Bioisosteres In Cystic Fibrosis Transmembrane Conductance Regulator Modulators VX-770 and VX-809.
Chemistry: A European Journal.
25:3662-3674.
2019
|
|
| 2019
|
Colocolonic intussusception in an adult cystic fibrosis patient.
Journal of Cystic Fibrosis.
18:e11-e13.
2019
|
|
| 2019
|
Non-obstructive vas deferens and epididymis loss in cystic fibrosis rats.
Mechanisms of Development.
155:15-26.
2019
|
|
| 2019
|
Objective versus self-reported adherence to airway clearance therapy in cystic fibrosis.
Respiratory Care: a monthly science journal.
64:176-181.
2019
|
|
| 2019
|
A glycopolymer improves vascoelasticity and mucociliary transport of abnormal cystic fibrosis mucus.
JCI insight.
4.
2019
|
|
| 2019
|
Acquired cystic fibrosis transmembrane conductance regulator dysfunction and radiographic bronchiectasis in current and former smokers: A cross-sectional study.
Annals of the American Thoracic Society.
16:150-153.
2019
|
|
| 2019
|
CFTR modulator theratyping: Current status, gaps and future directions.
Journal of Cystic Fibrosis.
18:22-34.
2019
|
|
| 2019
|
Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations.
Journal of Cystic Fibrosis.
18:102-109.
2019
|
|
| 2018
|
Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR.
JCI insight.
3.
2018
|
|
| 2018
|
Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice.
Nature Communications.
9.
2018
|
|
| 2018
|
The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis.
Current Opinion in Pharmacology.
43:152-165.
2018
|
|
| 2018
|
Heme scavenging reduces pulmonary endoplasmic reticulum stress, fibrosis, and emphysema.
JCI insight.
3.
2018
|
|
| 2018
|
Functional anatomic imaging of the airway surface.
Annals of the American Thoracic Society.
15:S177-S183.
2018
|
|
| 2018
|
VX-445-tezacaftor-ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles.
New England Journal of Medicine.
379:1612-1620.
2018
|
|
| 2018
|
VX-659-tezacaftor-ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles.
New England Journal of Medicine.
379:1599-1611.
2018
|
|
| 2018
|
Influenza-mediated reduction of lung epithelial ion channel activity leads to dysregulated pulmonary fluid homeostasis.
JCI insight.
3.
2018
|
|
| 2018
|
Ataluren, a new therapeutic for alpha-1 antitrypsin-deficient individuals with nonsense mutations.
American Journal of Respiratory and Critical Care Medicine.
198:1099-1102.
2018
|
|
| 2018
|
Standardized measurement of nasal membrane transepithelial potential difference (NPD).
Journal of Visualized Experiments.
2018.
2018
|
|
| 2018
|
EMPIRE-CF: A phase II randomized placebo-controlled trial of once-daily, oral acebilustat in adult patients with cystic fibrosis – Study design and patient demographics.
Contemporary Clinical Trials.
72:86-94.
2018
|
|
| 2018
|
Maternal smoking induces acquired CFTR dysfunction in neonatal rats.
American Journal of Respiratory and Critical Care Medicine.
198:672-674.
2018
|
|
| 2018
|
A revised airway epithelial hierarchy includes CFTR-expressing ionocytes.
Nature.
560:319-324.
2018
|
|
| 2018
|
Changes in lung clearance index in preschool-aged patients with cystic fibrosis treated with ivacaftor (GOAL): A clinical trial.
American Journal of Respiratory and Critical Care Medicine.
198:526-528.
2018
|
|
| 2018
|
An Adeno-Associated Viral Vector Capable of Penetrating the Mucus Barrier to Inhaled Gene Therapy.
Molecular Therapy - Methods and Clinical Development.
9:296-304.
2018
|
|
| 2018
|
Ivacaftor-treated patients with cystic fibrosis derive long-term benefit despite no short-term clinical improvement.
American Journal of Respiratory and Critical Care Medicine.
197:1483-1486.
2018
|
|
| 2018
|
Seeing cilia: Imaging modalities for ciliary motion and clinical connections.
American Journal of Physiology: Lung Cellular and Molecular Physiology.
314:L909-L921.
2018
|
|
| 2018
|
Sensitivity of ivacaftor to drug-drug interactions with rifampin, a cytochrome P450 3A4 inducer.
Pediatric Pulmonology.
53:E6-E8.
2018
|
|
| 2018
|
MicroRNA-145 antagonism reverses TGF-b inhibition of F508del CFTR correction in airway Epithelia.
American Journal of Respiratory and Critical Care Medicine.
197:632-643.
2018
|
|
| 2018
|
Implementation of a successful eradication protocol for Burkholderia Cepacia complex in cystic fibrosis patients.
BMC Pulmonary Medicine.
18.
2018
|
|
| 2018
|
Not simply the lesser of two evils.
American Journal of Physiology: Lung Cellular and Molecular Physiology.
314:L236-L238.
2018
|
|
| 2018
|
Sinus microanatomy and microbiota in a rabbit model of rhinosinusitis.
Frontiers in Cellular and Infection Microbiology.
7.
2018
|
|
| 2018
|
Development of an airway mucus defect in the cystic fibrosis rat.
JCI insight.
3.
2018
|
|
| 2017
|
Klotho Inhibits Interleukin-8 Secretion from Cystic Fibrosis Airway Epithelia.
Scientific Reports.
7.
2017
|
|
| 2017
|
Tezacaftor–ivacaftor in residual-function heterozygotes with cystic fibrosis.
New England Journal of Medicine.
377:2024-2035.
2017
|
|
| 2017
|
Reduced bone length, growth plate thickness, bone content, and IGF-I as a model for poor growth in the CFTR-deficient rat.
PLoS One.
12.
2017
|
|
| 2017
|
Toward inclusive therapy with CFTR modulators: Progress and challenges.
Pediatric Pulmonology.
52:S4-S14.
2017
|
|
| 2017
|
Use of ferrets for electrophysiologic monitoring of ion transport.
PLoS One.
12.
2017
|
|
| 2017
|
Roflumilast reverses CFTR-mediated ion transport dysfunction in cigarette smoke-exposed mice.
Respiratory Research.
18.
2017
|
|
| 2017
|
A multiple reader scoring system for Nasal Potential Difference parameters.
Journal of Cystic Fibrosis.
16:573-578.
2017
|
|
| 2017
|
Assessment of acquired mucociliary clearance defects using micro-optical coherence tomography.
International Forum of Allergy and Rhinology.
7:920-925.
2017
|
|
| 2017
|
Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences.
Human Molecular Genetics.
26:3116-3129.
2017
|
|
| 2017
|
The therapeutic potential of CFTR modulators for COPD and other airway diseases.
Current Opinion in Pharmacology.
34:132-139.
2017
|
|
| 2017
|
Assessment of ciliary phenotype in primary ciliary dyskinesia by micro-optical coherence tomography.
JCI insight.
2.
2017
|
|
| 2017
|
Protective and antifungal properties of Nanodisk-Amphotericin B over commercially available Amphotericin B..
World Journal of Otorhinolaryngology-Head and Neck Surgery.
3:2-8.
2017
|
|
| 2017
|
Flexible, high-resolution micro-optical coherence tomography endobronchial probe toward in vivo imaging of cilia.
Optics Letters.
42:867-870.
2017
|
|
| 2017
|
A little CFTR can change a lot: slowing cystic fibrosis progression.
The Lancet Respiratory Medicine.
5:86-87.
2017
|
|
| 2017
|
Growth in prepubertal children with cystic fibrosis treated with ivacaftor.
Pediatrics.
139.
2017
|
|
| 2017
|
Lumacaftor/ivacaftor treatment of patients with cystic fibrosis heterozygous for F508del-CFTR.
Annals of the American Thoracic Society.
14:213-219.
2017
|
|
| 2017
|
A Case Report of Pregnancy During Use of Targeted Therapeutics for Cystic Fibrosis.
Journal of Obstetric, Gynecologic, and Neonatal Nursing.
46:72-77.
2017
|
|
| 2017
|
Applying recent advances in the science of CFTR-based therapeutics to improve outcomes in patients with cystic fibrosis.
Journal of Cystic Fibrosis.
2017
|
|
| 2017
|
Impact of CFTR modulation on intestinal pH, motility, and clinical outcomes in patients with cystic fibrosis and the G551D mutation.
Clinical and Translational Gastroenterology.
8.
2017
|
|
| 2017
|
The cystic fibrosis transmembrane conductance regulator potentiator ivacaftor augments mucociliary clearance abrogating cystic fibrosis transmembrane conductance regulator inhibition by cigarette smoke.
American Journal of Respiratory Cell and Molecular Biology.
56:99-108.
2017
|
|
| 2017
|
Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation.
Journal of Cystic Fibrosis.
16:24-29.
2017
|
|
| 2016
|
Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression.
Proceedings of the National Academy of Sciences of USA.
113:12508-12513.
2016
|
|
| 2016
|
Discovery of clinically approved agents that promote suppression of cystic fibrosis transmembrane conductance regulator nonsense mutations.
American Journal of Respiratory and Critical Care Medicine.
194:1092-1103.
2016
|
|
| 2016
|
Codon bias and the folding dynamics of the cystic fibrosis transmembrane conductance regulator.
Cellular and Molecular Biology Letters: an international journal.
21.
2016
|
|
| 2016
|
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.
11.
2016
|
|
| 2016
|
A ferret model of COPD-related chronic bronchitis.
JCI insight.
1:e87536.
2016
|
|
| 2016
|
Particle-Tracking Microrheology Using Micro-Optical Coherence Tomography.
Biophysical Journal.
111:1053-1063.
2016
|
|
| 2016
|
Dual SMAD Signaling Inhibition Enables Long-Term Expansion of Diverse Epithelial Basal Cells.
Cell Stem Cell.
19:217-231.
2016
|
|
| 2016
|
Pulmonary artery enlargement and cystic fibrosis pulmonary exacerbations: a cohort study.
The Lancet Respiratory Medicine.
4:636-645.
2016
|
|
| 2016
|
In vivo imaging of airway cilia and mucus clearance with micro-optical coherence tomography.
Biomedical Optics Express.
7:2494-2505.
2016
|
|
| 2016
|
Mutation of Growth Arrest Specific 8 Reveals a Role in Motile Cilia Function and Human Disease.
PLoS Genetics.
12.
2016
|
|
| 2016
|
Pilot evaluation of ivacaftor for chronic bronchitis.
The Lancet Respiratory Medicine.
4:e32-e33.
2016
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| 2016
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Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy.
American Journal of Physiology: Lung Cellular and Molecular Physiology.
310:L928-L939.
2016
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| 2016
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Therapeutic approaches to acquired cystic fibrosis transmembrane conductance regulator dysfunction in Chronic bronchitis.
Annals of the American Thoracic Society.
13:S169-S176.
2016
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| 2016
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New and emerging targeted therapies for cystic fibrosis.
BMJ.
352.
2016
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| 2016
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Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance.
Clinics in Chest Medicine.
37:147-158.
2016
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| 2016
|
Alterations in blood leukocytes of G551D-bearing cystic fibrosis patients undergoing treatment with ivacaftor.
Journal of Cystic Fibrosis.
15:67-73.
2016
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| 2015
|
Recovery of acquired cystic fibrosis transmembrane conductance regulator dysfunction after smoking cessation.
American Journal of Respiratory and Critical Care Medicine.
192:1521-1524.
2015
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| 2015
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Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors.
Pediatric Pulmonology.
50:S3-S13.
2015
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| 2015
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Heme oxygenase-1-mediated autophagy protects against pulmonary endothelial cell death and development of emphysema in cadmium-treated mice.
American Journal of Physiology: Lung Cellular and Molecular Physiology.
309:L280-L292.
2015
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| 2015
|
Moderate intensity exercise mediates comparable increases in exhaled chloride as albuterol in individuals with cystic fibrosis.
Respiratory Medicine.
109:1001-1011.
2015
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| 2015
|
Lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for phe508del CFTR.
New England Journal of Medicine.
373:220-231.
2015
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| 2015
|
Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: A double-blind, randomised controlled trial.
The Lancet Respiratory Medicine.
3:524-533.
2015
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| 2015
|
Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator-knockout ferret lungs.
American Journal of Respiratory Cell and Molecular Biology.
52:683-694.
2015
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| 2015
|
Cystic fibrosis.
Nature Reviews Disease Primers.
1:15010.
2015
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| 2015
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▵F508 CFTR surface stability is regulated by DAB2 and CHIP-mediated ubiquitination in post-endocytic compartments.
PLoS One.
10.
2015
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| 2015
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Improved clinical and radiographic outcomes after treatment with ivacaftor in a young adult with cystic fibrosis with the P67L CFTR mutation.
Chest.
147:e79-e82.
2015
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| 2015
|
Novel outcome measures for clinical trials in cystic fibrosis.
Pediatric Pulmonology.
50:302-315.
2015
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| 2015
|
Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor.
Clinical Infectious Diseases.
60:703-712.
2015
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| 2014
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An autoregulatory mechanism governing mucociliary transport is sensitive to mucus load.
American Journal of Respiratory Cell and Molecular Biology.
51:485-493.
2014
|
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| 2014
|
A functional anatomic defect of the cystic fibrosis airway.
American Journal of Respiratory and Critical Care Medicine.
190:421-432.
2014
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| 2014
|
Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.
American Journal of Respiratory and Critical Care Medicine.
190:175-184.
2014
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| 2014
|
Characterization of defects in ion transport and tissue development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-knockout rats.
PLoS One.
9.
2014
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| 2014
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Sweat chloride as a biomarker of CFTR activity: Proof of concept and ivacaftor clinical trial data.
Journal of Cystic Fibrosis.
13:139-147.
2014
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| 2014
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Acquired defects in CFTR-dependent β-adrenergic sweat secretion in chronic obstructive pulmonary disease.
Respiratory Research.
15.
2014
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| 2014
|
Impact of heterozygote CFTR Mutations in COPD patients with Chronic Bronchitis.
Respiratory Research.
15.
2014
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| 2014
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A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: A phase 2 randomised controlled trial.
The Lancet Respiratory Medicine.
2:527-538.
2014
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| 2014
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Ataluren for the treatment of nonsense-mutation cystic fibrosis: A randomised, double-blind, placebo-controlled phase 3 trial.
The Lancet Respiratory Medicine.
2:539-547.
2014
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| 2014
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Cystic fibrosis transmembrane conductance regulator activation by roflumilast contributes to therapeutic benefit in chronic bronchitis.
American Journal of Respiratory Cell and Molecular Biology.
50:549-558.
2014
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| 2014
|
Development and maintenance of a biospecimen repository for clinical samples derived from pulmonary patients.
Clinical and Translational Science.
7:336-341.
2014
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| 2014
|
Evaluating the predictive ability of sweat chloride.
Journal of Cystic Fibrosis.
13:118.
2014
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| 2014
|
Overcoming the cystic fibrosis sputum barrier to leading adeno-associated virus gene therapy vectors.
Molecular Therapy.
22:1484-1493.
2014
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| 2014
|
Porcine nasal epithelial cultures for studies of cystic fibrosis sinusitis.
International Forum of Allergy and Rhinology.
4:565-570.
2014
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| 2014
|
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor.
American Journal of Respiratory Cell and Molecular Biology.
50:805-816.
2014
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| 2014
|
The unfolded protein response affects readthrough of premature termination codons.
EMBO Molecular Medicine.
6:685-701.
2014
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| 2013
|
Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function.
American Journal of Respiratory and Critical Care Medicine.
188:1321-1330.
2013
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| 2013
|
Cigarette smoke and CFTR: Implications in the pathogenesis of COPD.
American Journal of Physiology: Lung Cellular and Molecular Physiology.
305.
2013
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| 2013
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Multicenter Intestinal Current Measurements in Rectal Biopsies from CF and Non-CF Subjects to Monitor CFTR Function.
PLoS One.
8.
2013
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| 2013
|
Cystic fibrosis chronic rhinosinusitis: A comprehensive review.
American Journal of Rhinology and Allergy.
27:387-395.
2013
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| 2013
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IP-10 Is a Potential Biomarker of Cystic Fibrosis Acute Pulmonary Exacerbations.
PLoS One.
8.
2013
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| 2013
|
Cystic fibrosis transmembrane regulator correctors and potentiators.
Cold Spring Harbor perspectives in biology.
5.
2013
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| 2013
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Optimizing Nasal Potential Difference Analysis for CFTR Modulator Development: Assessment of Ivacaftor in CF Subjects with the G551D-CFTR Mutation.
PLoS One.
8.
2013
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| 2013
|
Cystic fibrosis transmembrane regulator correctors and potentiators.
Cold Spring Harbor Perspectives in Medicine.
3.
2013
|
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| 2013
|
Cystic fibrosis transmembrane regulator correctors and potentiators.
Cold Spring Harbor perspectives in biology.
5.
2013
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| 2013
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Method for Quantitative Study of Airway Functional Microanatomy Using Micro-Optical Coherence Tomography.
PLoS One.
8.
2013
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| 2013
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Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD.
Chest.
144:498-506.
2013
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| 2013
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CFTR biomarkers: Time for promotion to surrogate end-point?.
European Respiratory Journal.
41:203-216.
2013
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| 2013
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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 Otolaryngology-Head and Neck Surgery.
139:822-827.
2013
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| 2013
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Reduced sodium transport with nasal administration of the prostasin inhibitor camostat in subjects with cystic fibrosis.
Chest.
144:200-207.
2013
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| 2013
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Understanding the relationship between sweat chloride and lung function in cystic fibrosis.
Chest.
144:1418.
2013
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| 2012
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Clearance of initial mucoid Pseudomonas aeruginosa in patients with cystic fibrosis.
Pediatric Pulmonology.
47:1113-1122.
2012
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| 2012
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A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease.
PLoS One.
7.
2012
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| 2012
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Dab2 is a key regulator of endocytosis and post-endocytic trafficking of the cystic fibrosis transmembrane conductance regulator.
Biochemical Journal.
441:633-643.
2012
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| 2012
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CFTR potentiator treatment of cystic fibrosis.
Drugs of the Future.
37:167-174.
2012
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| 2012
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Extensive surgical and comprehensive postoperative medical management for cystic fibrosis chronic rhinosinusitis.
American Journal of Rhinology and Allergy.
26:70-75.
2012
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| 2012
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Progress in cystic fibrosis and the CF Therapeutics Development Network.
Thorax.
67:882-890.
2012
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| 2012
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Purification of CFTR for mass spectrometry analysis: Identification of palmitoylation and other post-translational modifications.
Protein Engineering Design and Selection.
25:7-14.
2012
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| 2012
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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.
67:12-18.
2012
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| 2011
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A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.
New England Journal of Medicine.
365:1663-1672.
2011
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| 2011
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Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54.
Journal of Molecular Medicine.
89:1149-1161.
2011
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| 2011
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Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators.
American Journal of Physiology: Lung Cellular and Molecular Physiology.
301.
2011
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| 2011
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Nasal potential difference measurements to assess CFTR ion channel activity..
Methods in Molecular Biology.
741:69-86.
2011
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| 2011
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Quercetin increases cystic fibrosis transmembrane conductance regulator-mediated chloride transport and ciliary beat frequency: Therapeutic implications for chronic rhinosinusitis.
American Journal of Rhinology and Allergy.
25:307-312.
2011
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| 2011
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Medicine: A breath of fresh air.
Scientific American.
305:48-53.
2011
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| 2011
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A breath of fresh air..
Scientific American.
305:68-73.
2011
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| 2010
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Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.
New England Journal of Medicine.
363:1991-2003.
2010
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| 2010
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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.
43:607-616.
2010
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| 2010
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Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens.
Fertility and Sterility.
94:2122-2127.
2010
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| 2010
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Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis.
Current Opinion in Pulmonary Medicine.
16:591-597.
2010
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| 2010
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A critical role for LTA4H in limiting chronic pulmonary neutrophilic inflammation.
Science.
330:90-94.
2010
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| 2010
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An international randomized multicenter comparison of nasal potential difference techniques.
Chest.
138:919-928.
2010
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| 2010
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ΔF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.
Pulmonary Pharmacology and Therapeutics.
23:268-278.
2010
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| 2010
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Proline-Glycine-Proline (PGP) and high mobility group box protein-1 (HMGB1): Potential mediators of cystic fibrosis airway inflammation.
Open Respiratory Medicine Journal.
4:32-38.
2010
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| 2009
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Extensive pulmonary laceration in pediatric trauma.
Respiration: international journal of thoracic medicine.
79:76.
2009
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| 2009
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Pharmaceuticals targeting nonsense mutations in genetic diseases: Progress in development.
BioDrugs: clinical immunotherapeutics, biopharmaceuticals and gene therapy.
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2009
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Authors' response.
Thorax.
63:1026.
2008
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| 2008
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Potential role of high-mobility group box 1 in cystic fibrosis airway disease.
American Journal of Respiratory and Critical Care Medicine.
178:822-831.
2008
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| 2008
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A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in Chronic neutrophilic inflammation.
Journal of Immunology.
180:5662-5669.
2008
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| 2008
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Use of β blockers and the risk of death in hospitalised patients with acute exacerbations of COPD.
Thorax.
63:301-305.
2008
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| 2008
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Authors' response.
Thorax.
63:1027.
2008
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| 2008
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Genetic and reproductive knowledge among adolescents and adults with cystic fibrosis.
Chest.
133:1533.
2008
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| 2007
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Bioelectric effects of quinine on polarized airway epithelial cells.
Journal of Cystic Fibrosis.
6:351-359.
2007
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| 2007
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Pulmonary aneurysms and intracardiac thrombi due to Behçet's disease in an African-American adolescent with oculocutaneous albinism.
Clinical Rheumatology.
26:1537-1539.
2007
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| 2007
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Restoration of W1282X CFTR activity by enhanced expression.
American Journal of Respiratory Cell and Molecular Biology.
37:347-356.
2007
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| 2007
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Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials.
Annals of the American Thoracic Society.
4:387-398.
2007
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| 2007
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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.
2007
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| 2006
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Advances in cystic fibrosis therapies.
Current Opinion in Pediatrics.
18:604-613.
2006
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| 2005
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Cystic fibrosis.
New England Journal of Medicine.
352:1992-2001.
2005
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| 2005
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Cystic fibrosis.
New England Journal of Medicine.
352:2039.
2005
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| 2002
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Foodborne disease outbreaks in United States schools.
The Pediatric Infectious Disease Journal.
21:623-628.
2002
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| 2000
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A foodborne outbreak of gastroenteritis associated with Norwalk-like viruses: First molecular traceback to deli sandwiches contaminated during preparation.
Journal of Infectious Diseases.
181:1467-1470.
2000
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| 1999
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What are the eating cognitions of children whose chronic diseases do and do not require attention to diet?.
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1999
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