Steven M. Rowe

Department of Medicine and the Gregory Fleming James Cystic Fibrosis Center, University of Alabama at Birmingham

Affiliated withUniversity of Alabama at Birmingham

Research Area

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JoVE Journal Publications

ArticleTotal : 1	Year
Publication title Cited by 30	2018

Other Publications

Article	Year
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. The New England journal of medicine\| PubMed ID: 22047557	2011
Cystic fibrosis. The New England journal of medicine\| PubMed ID: 15888700	2005
Pulmonary aneurysms and intracardiac thrombi due to Behçet's disease in an African-American adolescent with oculocutaneous albinism. Clinical rheumatology\| PubMed ID: 17047893	2007
Advances in cystic fibrosis therapies. Current opinion in pediatrics\| PubMed ID: 17099358	2006
Bioelectric effects of quinine on polarized airway epithelial cells. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 17329172	2007
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\| PubMed ID: 17347447	2007
Restoration of W1282X CFTR activity by enhanced expression. American journal of respiratory cell and molecular biology\| PubMed ID: 17541014	2007
Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials. Proceedings of the American Thoracic Society\| PubMed ID: 17652506	2007
A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation. Journal of immunology (Baltimore, Md. : 1950)\| PubMed ID: 18390751	2008
Extensive pulmonary laceration in pediatric trauma. Respiration; international review of thoracic diseases\| PubMed ID: 18560207	2010
Genetic and reproductive knowledge among adolescents and adults with cystic fibrosis. Chest\| PubMed ID: 18574307	2008
Potential role of high-mobility group box 1 in cystic fibrosis airway disease. American journal of respiratory and critical care medicine\| PubMed ID: 18658107	2008
Pharmaceuticals targeting nonsense mutations in genetic diseases: progress in development. BioDrugs : clinical immunotherapeutics, biopharmaceuticals and gene therapy\| PubMed ID: 19627168	2009
Activation of the cystic fibrosis transmembrane conductance regulator by the flavonoid quercetin: potential use as a biomarker of ΔF508 cystic fibrosis transmembrane conductance regulator re... American journal of respiratory cell and molecular biology\| PubMed ID: 20042712	2010
Association of cystic fibrosis genetic modifiers with congenital bilateral absence of the vas deferens. Fertility and sterility\| PubMed ID: 20100616	2010
Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): Potential Mediators of Cystic Fibrosis Airway Inflammation. The open respiratory medicine journal\| PubMed ID: 20448817	2010
An international randomized multicenter comparison of nasal potential difference techniques. Chest\| PubMed ID: 20472865	2010
A critical role for LTA4H in limiting chronic pulmonary neutrophilic inflammation. Science (New York, N.Y.)\| PubMed ID: 20813919	2010
Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis. Current opinion in pulmonary medicine\| PubMed ID: 20829696	2010
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. The New England journal of medicine\| PubMed ID: 21083385	2010
Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators. American journal of physiology. Lung cellular and molecular physiology\| PubMed ID: 21724857	2011
Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54. Journal of molecular medicine (Berlin, Germany)\| PubMed ID: 21779978	2011
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\| PubMed ID: 21825083	2012
A breath of fresh air. Scientific American\| PubMed ID: 21827128	2011
Dab2 is a key regulator of endocytosis and post-endocytic trafficking of the cystic fibrosis transmembrane conductance regulator. The Biochemical journal\| PubMed ID: 21995445	2012
Purification of CFTR for mass spectrometry analysis: identification of palmitoylation and other post-translational modifications. Protein engineering, design & selection : PEDS\| PubMed ID: 22119790	2012
Quercetin increases cystic fibrosis transmembrane conductance regulator-mediated chloride transport and ciliary beat frequency: therapeutic implications for chronic rhinosinusitis. American journal of rhinology & allergy\| PubMed ID: 22186243	2011
Extensive surgical and comprehensive postoperative medical management for cystic fibrosis chronic rhinosinusitis. American journal of rhinology & allergy\| PubMed ID: 22391086	2012
Clearance of initial mucoid Pseudomonas aeruginosa in patients with cystic fibrosis. Pediatric pulmonology\| PubMed ID: 22496040	2012
A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease. PloS one\| PubMed ID: 22768130	2012
Progress in cystic fibrosis and the CF Therapeutics Development Network. Thorax\| PubMed ID: 22960984	2012
Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography. PloS one\| PubMed ID: 23372732	2013
Reduced sodium transport with nasal administration of the prostasin inhibitor camostat in subjects with cystic fibrosis. Chest\| PubMed ID: 23412700	2013
Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD. Chest\| PubMed ID: 23538783	2013
Cystic fibrosis transmembrane regulator correctors and potentiators. Cold Spring Harbor perspectives in medicine\| PubMed ID: 23818513	2013
Evaluating the predictive ability of sweat chloride. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 23920003	2014
Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation. PloS one\| PubMed ID: 23922647	2013
Cigarette smoke and CFTR: implications in the pathogenesis of COPD. American journal of physiology. Lung cellular and molecular physiology\| PubMed ID: 23934925	2013
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 ep... JAMA otolaryngology-- head & neck surgery\| PubMed ID: 23949358	2013
IP-10 is a potential biomarker of cystic fibrosis acute pulmonary exacerbations. PloS one\| PubMed ID: 23977293	2013
Multicenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function. PloS one\| PubMed ID: 24040112	2013
Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function. American journal of respiratory and critical care medicine\| PubMed ID: 24040746	2013
Understanding the relationship between sweat chloride and lung function in cystic fibrosis. Chest\| PubMed ID: 24081360	2013
Cystic fibrosis transmembrane conductance regulator activation by roflumilast contributes to therapeutic benefit in chronic bronchitis. American journal of respiratory cell and molecular biology\| PubMed ID: 24106801	2014
Cystic fibrosis chronic rhinosinusitis: a comprehensive review. American journal of rhinology & allergy\| PubMed ID: 24119602	2013
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor. American journal of respiratory cell and molecular biology\| PubMed ID: 24251786	2014
Impact of heterozygote CFTR mutations in COPD patients with chronic bronchitis. Respiratory research\| PubMed ID: 24517344	2014
Acquired defects in CFTR-dependent β-adrenergic sweat secretion in chronic obstructive pulmonary disease. Respiratory research\| PubMed ID: 24568560	2014
Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats. PloS one\| PubMed ID: 24608905	2014
Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 24660233	2014
Porcine nasal epithelial cultures for studies of cystic fibrosis sinusitis. International forum of allergy & rhinology\| PubMed ID: 24733748	2014
Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial. The Lancet. Respiratory medicine\| PubMed ID: 24836205	2014
Overcoming the cystic fibrosis sputum barrier to leading adeno-associated virus gene therapy vectors. Molecular therapy : the journal of the American Society of Gene Therapy\| PubMed ID: 24869933	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\| PubMed ID: 24927234	2014
An autoregulatory mechanism governing mucociliary transport is sensitive to mucus load. American journal of respiratory cell and molecular biology\| PubMed ID: 24937762	2014
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\| PubMed ID: 24973281	2014
A functional anatomic defect of the cystic fibrosis airway. American journal of respiratory and critical care medicine\| PubMed ID: 25029666	2014
Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator-knockout ferret lungs. American journal of respiratory cell and molecular biology\| PubMed ID: 25317669	2015
Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America\| PubMed ID: 25425629	2015
Novel outcome measures for clinical trials in cystic fibrosis. Pediatric pulmonology\| PubMed ID: 25641878	2015
Improved clinical and radiographic outcomes after treatment with ivacaftor in a young adult with cystic fibrosis with the P67L CFTR mutation. Chest\| PubMed ID: 25732475	2015
Alterations in blood leukocytes of G551D-bearing cystic fibrosis patients undergoing treatment with ivacaftor. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 25769931	2016
ΔF508 CFTR surface stability is regulated by DAB2 and CHIP-mediated ubiquitination in post-endocytic compartments. PloS one\| PubMed ID: 25879443	2015
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. The New England journal of medicine\| PubMed ID: 25981758	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\| PubMed ID: 26070913	2015
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\| PubMed ID: 26071551	2015
Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors. Pediatric pulmonology\| PubMed ID: 26097168	2015
Recovery of Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction after Smoking Cessation. American journal of respiratory and critical care medicine\| PubMed ID: 26669476	2015
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance. Clinics in chest medicine\| PubMed ID: 26857776	2016
Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy. American journal of physiology. Lung cellular and molecular physiology\| PubMed ID: 26968770	2016
New and emerging targeted therapies for cystic fibrosis. BMJ (Clinical research ed.)\| PubMed ID: 27030675	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\| PubMed ID: 27104944	2016
Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis. Annals of the American Thoracic Society\| PubMed ID: 27115953	2016
Pilot evaluation of ivacaftor for chronic bronchitis. The Lancet. Respiratory medicine\| PubMed ID: 27185048	2016
Cystic fibrosis. Nature reviews. Disease primers\| PubMed ID: 27189798	2015
Pulmonary artery enlargement and cystic fibrosis pulmonary exacerbations: a cohort study. The Lancet. Respiratory medicine\| PubMed ID: 27298019	2016
In vivo imaging of airway cilia and mucus clearance with micro-optical coherence tomography. Biomedical optics express\| PubMed ID: 27446685	2016
Mutation of Growth Arrest Specific 8 Reveals a Role in Motile Cilia Function and Human Disease. PLoS genetics\| PubMed ID: 27472056	2016
The Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor Augments Mucociliary Clearance Abrogating Cystic Fibrosis Transmembrane Conductance Regulator Inhibition by Ciga... American journal of respiratory cell and molecular biology\| PubMed ID: 27585394	2017
Particle-Tracking Microrheology Using Micro-Optical Coherence Tomography. Biophysical journal\| PubMed ID: 27602733	2016
A ferret model of COPD-related chronic bronchitis. JCI insight\| PubMed ID: 27699245	2016
Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 27702906	2016
Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 27707539	2017
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\| PubMed ID: 27732613	2016
Lumacaftor/Ivacaftor Treatment of Patients with Cystic Fibrosis Heterozygous for F508del-CFTR. Annals of the American Thoracic Society\| PubMed ID: 27898234	2017
A little CFTR can change a lot: slowing cystic fibrosis progression. The Lancet. Respiratory medicine\| PubMed ID: 28011036	2017
Growth in Prepubertal Children With Cystic Fibrosis Treated With Ivacaftor. Pediatrics\| PubMed ID: 28143919	2017
Flexible, high-resolution micro-optical coherence tomography endobronchial probe toward in vivo imaging of cilia. Optics letters\| PubMed ID: 28198885	2017
Assessment of ciliary phenotype in primary ciliary dyskinesia by micro-optical coherence tomography. JCI insight\| PubMed ID: 28289722	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\| PubMed ID: 28300821	2017
A multiple reader scoring system for Nasal Potential Difference parameters. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 28465124	2017
Codon bias and the folding dynamics of the cystic fibrosis transmembrane conductance regulator. Cellular & molecular biology letters\| PubMed ID: 28536625	2016
Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences. Human molecular genetics\| PubMed ID: 28575328	2017
Assessment of acquired mucociliary clearance defects using micro-optical coherence tomography. International forum of allergy & rhinology\| PubMed ID: 28658531	2017
Toward inclusive therapy with CFTR modulators: Progress and challenges. Pediatric pulmonology\| PubMed ID: 28881097	2017
Roflumilast reverses CFTR-mediated ion transport dysfunction in cigarette smoke-exposed mice. Respiratory research\| PubMed ID: 28923049	2017
Use of ferrets for electrophysiologic monitoring of ion transport. PloS one\| PubMed ID: 29077751	2017
Klotho Inhibits Interleukin-8 Secretion from Cystic Fibrosis Airway Epithelia. Scientific reports\| PubMed ID: 29085059	2017
Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis. The New England journal of medicine\| PubMed ID: 29099333	2017
The therapeutic potential of CFTR modulators for COPD and other airway diseases. Current opinion in pharmacology\| PubMed ID: 29132121	2017
Protective and antifungal properties of Nanodisk-Amphotericin B over commercially available Amphotericin B. World journal of otorhinolaryngology - head and neck surgery\| PubMed ID: 29204573	2017
MicroRNA-145 Antagonism Reverses TGF-β Inhibition of F508del CFTR Correction in Airway Epithelia. American journal of respiratory and critical care medicine\| PubMed ID: 29232160	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\| PubMed ID: 29256624	2018
Development of an airway mucus defect in the cystic fibrosis rat. JCI insight\| PubMed ID: 29321377	2018
Not simply the lesser of two evils. American journal of physiology. Lung cellular and molecular physiology\| PubMed ID: 29351443	2018
Sinus Microanatomy and Microbiota in a Rabbit Model of Rhinosinusitis. Frontiers in cellular and infection microbiology\| PubMed ID: 29376039	2017
Sensitivity of ivacaftor to drug-drug interactions with rifampin, a cytochrome P450 3A4 inducer. Pediatric pulmonology\| PubMed ID: 29488691	2018
Seeing cilia: imaging modalities for ciliary motion and clinical connections. American journal of physiology. Lung cellular and molecular physiology\| PubMed ID: 29493257	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\| PubMed ID: 29614238	2018
Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 29685811	2018
Ataluren, a New Therapeutic for Alpha-1 Antitrypsin Deficient Individuals with Nonsense Mutations. American journal of respiratory and critical care medicine\| PubMed ID: 30011228	2018
An Adeno-Associated Viral Vector Capable of Penetrating the Mucus Barrier to Inhaled Gene Therapy. Molecular therapy. Methods & clinical development\| PubMed ID: 30038933	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\| PubMed ID: 30056216	2018
A revised airway epithelial hierarchy includes CFTR-expressing ionocytes. Nature\| PubMed ID: 30069044	2018