Steven M. Rowe

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

Cystic fibrosis.

Pulmonary aneurysms and intracardiac thrombi due to Behçet's disease in an African-American adolescent with oculocutaneous albinism.

Advances in cystic fibrosis therapies.

Bioelectric effects of quinine on polarized airway epithelial cells.

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

Restoration of W1282X CFTR activity by enhanced expression.

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

A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation.

Extensive pulmonary laceration in pediatric trauma.

Genetic and reproductive knowledge among adolescents and adults with cystic fibrosis.

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

Pharmaceuticals targeting nonsense mutations in genetic diseases: progress in development.

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.

Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens.

Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): Potential Mediators of Cystic Fibrosis Airway Inflammation.

An international randomized multicenter comparison of nasal potential difference techniques.

A critical role for LTA4H in limiting chronic pulmonary neutrophilic inflammation.

Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis.

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

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

Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54.

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.

A breath of fresh air.

Dab2 is a key regulator of endocytosis and post-endocytic trafficking of the cystic fibrosis transmembrane conductance regulator.

Purification of CFTR for mass spectrometry analysis: identification of palmitoylation and other post-translational modifications.

Quercetin increases cystic fibrosis transmembrane conductance regulator-mediated chloride transport and ciliary beat frequency: therapeutic implications for chronic rhinosinusitis.

Extensive surgical and comprehensive postoperative medical management for cystic fibrosis chronic rhinosinusitis.

Clearance of initial mucoid Pseudomonas aeruginosa in patients with cystic fibrosis.

A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease.

Progress in cystic fibrosis and the CF Therapeutics Development Network.

Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography.

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

Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD.

Cystic fibrosis transmembrane regulator correctors and potentiators.

Evaluating the predictive ability of sweat chloride.

Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation.

Cigarette smoke and CFTR: implications in the pathogenesis of COPD.

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.

IP-10 is a potential biomarker of cystic fibrosis acute pulmonary exacerbations.

Multicenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function.

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

Understanding the relationship between sweat chloride and lung function in cystic fibrosis.

Cystic fibrosis transmembrane conductance regulator activation by roflumilast contributes to therapeutic benefit in chronic bronchitis.

Cystic fibrosis chronic rhinosinusitis: a comprehensive review.

Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor.

Impact of heterozygote CFTR mutations in COPD patients with chronic bronchitis.

Acquired defects in CFTR-dependent β-adrenergic sweat secretion in chronic obstructive pulmonary disease.

Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats.

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

Porcine nasal epithelial cultures for studies of cystic fibrosis sinusitis.

Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.

Overcoming the cystic fibrosis sputum barrier to leading adeno-associated virus gene therapy vectors.

Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.

An autoregulatory mechanism governing mucociliary transport is sensitive to mucus load.

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.

A functional anatomic defect of the cystic fibrosis airway.

Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator-knockout ferret lungs.

Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor.

Novel outcome measures for clinical trials in cystic fibrosis.

Improved clinical and radiographic outcomes after treatment with ivacaftor in a young adult with cystic fibrosis with the P67L CFTR mutation.

Alterations in blood leukocytes of G551D-bearing cystic fibrosis patients undergoing treatment with ivacaftor.

ΔF508 CFTR surface stability is regulated by DAB2 and CHIP-mediated ubiquitination in post-endocytic compartments.

Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.

Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial.

Heme oxygenase-1-mediated autophagy protects against pulmonary endothelial cell death and development of emphysema in cadmium-treated mice.

Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors.

Recovery of Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction after Smoking Cessation.

Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance.

Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy.

New and emerging targeted therapies for cystic fibrosis.

Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations.

Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis.

Pilot evaluation of ivacaftor for chronic bronchitis.

Cystic fibrosis.

Pulmonary artery enlargement and cystic fibrosis pulmonary exacerbations: a cohort study.

In vivo imaging of airway cilia and mucus clearance with micro-optical coherence tomography.

Mutation of Growth Arrest Specific 8 Reveals a Role in Motile Cilia Function and Human Disease.

The Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor Augments Mucociliary Clearance Abrogating Cystic Fibrosis Transmembrane Conductance Regulator Inhibition by Cigarette Smoke.

Particle-Tracking Microrheology Using Micro-Optical Coherence Tomography.

A ferret model of COPD-related chronic bronchitis.

Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression.

Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation.

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.

Lumacaftor/Ivacaftor Treatment of Patients with Cystic Fibrosis Heterozygous for F508del-CFTR.

A little CFTR can change a lot: slowing cystic fibrosis progression.

Growth in Prepubertal Children With Cystic Fibrosis Treated With Ivacaftor.

Flexible, high-resolution micro-optical coherence tomography endobronchial probe toward in vivo imaging of cilia.

Assessment of ciliary phenotype in primary ciliary dyskinesia by micro-optical coherence tomography.

Impact of CFTR Modulation on Intestinal pH, Motility, and Clinical Outcomes in Patients With Cystic Fibrosis and the G551D Mutation.

A multiple reader scoring system for Nasal Potential Difference parameters.

Codon bias and the folding dynamics of the cystic fibrosis transmembrane conductance regulator.

Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences.

Assessment of acquired mucociliary clearance defects using micro-optical coherence tomography.

Toward inclusive therapy with CFTR modulators: Progress and challenges.

Roflumilast reverses CFTR-mediated ion transport dysfunction in cigarette smoke-exposed mice.

Use of ferrets for electrophysiologic monitoring of ion transport.

Klotho Inhibits Interleukin-8 Secretion from Cystic Fibrosis Airway Epithelia.

Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis.

The therapeutic potential of CFTR modulators for COPD and other airway diseases.

Protective and antifungal properties of Nanodisk-Amphotericin B over commercially available Amphotericin B.

MicroRNA-145 Antagonism Reverses TGF-β Inhibition of F508del CFTR Correction in Airway Epithelia.

Ivacaftor-treated Patients with Cystic Fibrosis Derive Long-Term Benefit Despite No Short-Term Clinical Improvement.

Development of an airway mucus defect in the cystic fibrosis rat.

Not simply the lesser of two evils.

Sinus Microanatomy and Microbiota in a Rabbit Model of Rhinosinusitis.

Sensitivity of ivacaftor to drug-drug interactions with rifampin, a cytochrome P450 3A4 inducer.

Seeing cilia: imaging modalities for ciliary motion and clinical connections.

Changes in Lung Clearance Index in Preschool-aged Patients with Cystic Fibrosis Treated with Ivacaftor (GOAL): A Clinical Trial.

Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations.

Ataluren, a New Therapeutic for Alpha-1 Antitrypsin Deficient Individuals with Nonsense Mutations.

An Adeno-Associated Viral Vector Capable of Penetrating the Mucus Barrier to Inhaled Gene Therapy.

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.

A revised airway epithelial hierarchy includes CFTR-expressing ionocytes.