Steven M. Rowe Department of Medicine and the Gregory Fleming James Cystic Fibrosis Center University of Alabama at Birmingham Biography Publications Institution JoVE Articles Steven M. Rowe has not added a biography. If you are Steven M. Rowe and would like to personalize this page please email our Author Liaison for assistance. Publications A Revised Airway Epithelial Hierarchy Includes CFTR-expressing Ionocytes Nature. Aug, 2018 | Pubmed ID: 30069044 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. Sep, 2018 | Pubmed ID: 30056216 An Adeno-Associated Viral Vector Capable of Penetrating the Mucus Barrier to Inhaled Gene Therapy Molecular Therapy. Methods & Clinical Development. Jun, 2018 | Pubmed ID: 30038933 Ataluren, a New Therapeutic for Alpha-1 Antitrypsin Deficient Individuals with Nonsense Mutations American Journal of Respiratory and Critical Care Medicine. Jul, 2018 | Pubmed ID: 30011228 Effectiveness of Ivacaftor in Cystic Fibrosis Patients with Non-G551D Gating Mutations Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. Apr, 2018 | Pubmed ID: 29685811 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. Aug, 2018 | Pubmed ID: 29614238 Seeing Cilia: Imaging Modalities for Ciliary Motion and Clinical Connections American Journal of Physiology. Lung Cellular and Molecular Physiology. Jun, 2018 | Pubmed ID: 29493257 Sensitivity of Ivacaftor to Drug-drug Interactions with Rifampin, a Cytochrome P450 3A4 Inducer Pediatric Pulmonology. May, 2018 | Pubmed ID: 29488691 Not Simply the Lesser of Two Evils American Journal of Physiology. Lung Cellular and Molecular Physiology. Feb, 2018 | Pubmed ID: 29351443 Development of an Airway Mucus Defect in the Cystic Fibrosis Rat JCI Insight. Jan, 2018 | Pubmed ID: 29321377 Ivacaftor-treated Patients with Cystic Fibrosis Derive Long-Term Benefit Despite No Short-Term Clinical Improvement American Journal of Respiratory and Critical Care Medicine. Jun, 2018 | Pubmed ID: 29256624 MicroRNA-145 Antagonism Reverses TGF-β Inhibition of F508del CFTR Correction in Airway Epithelia American Journal of Respiratory and Critical Care Medicine. Mar, 2018 | Pubmed ID: 29232160 Sinus Microanatomy and Microbiota in a Rabbit Model of Rhinosinusitis Frontiers in Cellular and Infection Microbiology. 2017 | Pubmed ID: 29376039 Protective and Antifungal Properties of Nanodisk-Amphotericin B over Commercially Available Amphotericin B World Journal of Otorhinolaryngology - Head and Neck Surgery. Mar, 2017 | Pubmed ID: 29204573 The Therapeutic Potential of CFTR Modulators for COPD and Other Airway Diseases Current Opinion in Pharmacology. 06, 2017 | Pubmed ID: 29132121 Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis The New England Journal of Medicine. 11, 2017 | Pubmed ID: 29099333 Klotho Inhibits Interleukin-8 Secretion from Cystic Fibrosis Airway Epithelia Scientific Reports. Oct, 2017 | Pubmed ID: 29085059 Use of Ferrets for Electrophysiologic Monitoring of Ion Transport PloS One. 2017 | Pubmed ID: 29077751 Roflumilast Reverses CFTR-mediated Ion Transport Dysfunction in Cigarette Smoke-exposed Mice Respiratory Research. 09, 2017 | Pubmed ID: 28923049 Toward Inclusive Therapy with CFTR Modulators: Progress and Challenges Pediatric Pulmonology. Nov, 2017 | Pubmed ID: 28881097 Assessment of Acquired Mucociliary Clearance Defects Using Micro-optical Coherence Tomography International Forum of Allergy & Rhinology. 09, 2017 | Pubmed ID: 28658531 Identification of the Amino Acids Inserted During Suppression of CFTR Nonsense Mutations and Determination of Their Functional Consequences Human Molecular Genetics. 08, 2017 | Pubmed ID: 28575328 A Multiple Reader Scoring System for Nasal Potential Difference Parameters Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. Sep, 2017 | Pubmed ID: 28465124 Impact of CFTR Modulation on Intestinal PH, Motility, and Clinical Outcomes in Patients With Cystic Fibrosis and the G551D Mutation Clinical and Translational Gastroenterology. Mar, 2017 | Pubmed ID: 28300821 Assessment of Ciliary Phenotype in Primary Ciliary Dyskinesia by Micro-optical Coherence Tomography JCI Insight. Mar, 2017 | Pubmed ID: 28289722 Flexible, High-resolution Micro-optical Coherence Tomography Endobronchial Probe Toward in Vivo Imaging of Cilia Optics Letters. Feb, 2017 | Pubmed ID: 28198885 Growth in Prepubertal Children With Cystic Fibrosis Treated With Ivacaftor Pediatrics. Feb, 2017 | Pubmed ID: 28143919 A Little CFTR Can Change a Lot: Slowing Cystic Fibrosis Progression The Lancet. Respiratory Medicine. 02, 2017 | Pubmed ID: 28011036 Lumacaftor/Ivacaftor Treatment of Patients with Cystic Fibrosis Heterozygous for F508del-CFTR Annals of the American Thoracic Society. Feb, 2017 | Pubmed ID: 27898234 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. Jan, 2017 | Pubmed ID: 27707539 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. Jan, 2017 | Pubmed ID: 27585394 Codon Bias and the Folding Dynamics of the Cystic Fibrosis Transmembrane Conductance Regulator Cellular & Molecular Biology Letters. 2016 | Pubmed ID: 28536625 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. 2016 | Pubmed ID: 27732613 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. 11, 2016 | Pubmed ID: 27702906 A Ferret Model of COPD-related Chronic Bronchitis JCI Insight. 09, 2016 | Pubmed ID: 27699245 Particle-Tracking Microrheology Using Micro-Optical Coherence Tomography Biophysical Journal. Sep, 2016 | Pubmed ID: 27602733 Mutation of Growth Arrest Specific 8 Reveals a Role in Motile Cilia Function and Human Disease PLoS Genetics. Jul, 2016 | Pubmed ID: 27472056 In Vivo Imaging of Airway Cilia and Mucus Clearance with Micro-optical Coherence Tomography Biomedical Optics Express. Jul, 2016 | Pubmed ID: 27446685 Pulmonary Artery Enlargement and Cystic Fibrosis Pulmonary Exacerbations: a Cohort Study The Lancet. Respiratory Medicine. 08, 2016 | Pubmed ID: 27298019 Pilot Evaluation of Ivacaftor for Chronic Bronchitis The Lancet. Respiratory Medicine. 06, 2016 | Pubmed ID: 27185048 Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis Annals of the American Thoracic Society. Apr, 2016 | Pubmed ID: 27115953 Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations American Journal of Respiratory and Critical Care Medicine. 11, 2016 | Pubmed ID: 27104944 New and Emerging Targeted Therapies for Cystic Fibrosis BMJ (Clinical Research Ed.). Mar, 2016 | Pubmed ID: 27030675 Combination Therapy with Cystic Fibrosis Transmembrane Conductance Regulator Modulators Augment the Airway Functional Microanatomy American Journal of Physiology. Lung Cellular and Molecular Physiology. 05, 2016 | Pubmed ID: 26968770 Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance Clinics in Chest Medicine. Mar, 2016 | Pubmed ID: 26857776 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. Jan, 2016 | Pubmed ID: 25769931 Cystic Fibrosis Nature Reviews. Disease Primers. 05, 2015 | Pubmed ID: 27189798 Recovery of Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction After Smoking Cessation American Journal of Respiratory and Critical Care Medicine. Dec, 2015 | Pubmed ID: 26669476 Breakthrough Therapies: Cystic Fibrosis (CF) Potentiators and Correctors Pediatric Pulmonology. Oct, 2015 | Pubmed ID: 26097168 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. Aug, 2015 | Pubmed ID: 26071551 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. Jul, 2015 | Pubmed ID: 26070913 Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR The New England Journal of Medicine. 07, 2015 | Pubmed ID: 25981758 ΔF508 CFTR Surface Stability is Regulated by DAB2 and CHIP-mediated Ubiquitination in Post-endocytic Compartments PloS One. 2015 | Pubmed ID: 25879443 Improved Clinical and Radiographic Outcomes After Treatment with Ivacaftor in a Young Adult with Cystic Fibrosis with the P67L CFTR Mutation Chest. Mar, 2015 | Pubmed ID: 25732475 Novel Outcome Measures for Clinical Trials in Cystic Fibrosis Pediatric Pulmonology. Mar, 2015 | Pubmed ID: 25641878 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. Mar, 2015 | Pubmed ID: 25425629 Defective Innate Immunity and Hyperinflammation in Newborn Cystic Fibrosis Transmembrane Conductance Regulator-knockout Ferret Lungs American Journal of Respiratory Cell and Molecular Biology. Jun, 2015 | Pubmed ID: 25317669 A Functional Anatomic Defect of the Cystic Fibrosis Airway American Journal of Respiratory and Critical Care Medicine. Aug, 2014 | Pubmed ID: 25029666 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. Jul, 2014 | Pubmed ID: 24973281 An Autoregulatory Mechanism Governing Mucociliary Transport is Sensitive to Mucus Load American Journal of Respiratory Cell and Molecular Biology. Oct, 2014 | Pubmed ID: 24937762 Clinical Mechanism of the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor in G551D-mediated Cystic Fibrosis American Journal of Respiratory and Critical Care Medicine. Jul, 2014 | Pubmed ID: 24927234 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. Aug, 2014 | Pubmed ID: 24869933 Ataluren for the Treatment of Nonsense-mutation Cystic Fibrosis: a Randomised, Double-blind, Placebo-controlled Phase 3 Trial The Lancet. Respiratory Medicine. Jul, 2014 | Pubmed ID: 24836205 Porcine Nasal Epithelial Cultures for Studies of Cystic Fibrosis Sinusitis International Forum of Allergy & Rhinology. Jul, 2014 | Pubmed ID: 24733748 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. Mar, 2014 | Pubmed ID: 24660233 Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-knockout Rats PloS One. 2014 | Pubmed ID: 24608905 Acquired Defects in CFTR-dependent β-adrenergic Sweat Secretion in Chronic Obstructive Pulmonary Disease Respiratory Research. Feb, 2014 | Pubmed ID: 24568560 Impact of Heterozygote CFTR Mutations in COPD Patients with Chronic Bronchitis Respiratory Research. Feb, 2014 | Pubmed ID: 24517344 Synthetic Aminoglycosides Efficiently Suppress Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations and Are Enhanced by Ivacaftor American Journal of Respiratory Cell and Molecular Biology. Apr, 2014 | Pubmed ID: 24251786 Cystic Fibrosis Transmembrane Conductance Regulator Activation by Roflumilast Contributes to Therapeutic Benefit in Chronic Bronchitis American Journal of Respiratory Cell and Molecular Biology. Mar, 2014 | Pubmed ID: 24106801 Evaluating the Predictive Ability of Sweat Chloride Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. Jan, 2014 | Pubmed ID: 23920003 Cystic Fibrosis Chronic Rhinosinusitis: a Comprehensive Review American Journal of Rhinology & Allergy. Sep-Oct, 2013 | Pubmed ID: 24119602 Understanding the Relationship Between Sweat Chloride and Lung Function in Cystic Fibrosis Chest. Oct, 2013 | Pubmed ID: 24081360 Cigarette Smoke Induces Systemic Defects in Cystic Fibrosis Transmembrane Conductance Regulator Function American Journal of Respiratory and Critical Care Medicine. Dec, 2013 | Pubmed ID: 24040746 Multicenter Intestinal Current Measurements in Rectal Biopsies from CF and Non-CF Subjects to Monitor CFTR Function PloS One. 2013 | Pubmed ID: 24040112 IP-10 is a Potential Biomarker of Cystic Fibrosis Acute Pulmonary Exacerbations PloS One. 2013 | Pubmed ID: 23977293 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 & Neck Surgery. Aug, 2013 | Pubmed ID: 23949358 Cigarette Smoke and CFTR: Implications in the Pathogenesis of COPD American Journal of Physiology. Lung Cellular and Molecular Physiology. Oct, 2013 | Pubmed ID: 23934925 Optimizing Nasal Potential Difference Analysis for CFTR Modulator Development: Assessment of Ivacaftor in CF Subjects with the G551D-CFTR Mutation PloS One. 2013 | Pubmed ID: 23922647 Cystic Fibrosis Transmembrane Regulator Correctors and Potentiators Cold Spring Harbor Perspectives in Medicine. Jul, 2013 | Pubmed ID: 23818513 Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in the Lower Airways in COPD Chest. Aug, 2013 | Pubmed ID: 23538783 Reduced Sodium Transport with Nasal Administration of the Prostasin Inhibitor Camostat in Subjects with Cystic Fibrosis Chest. Jul, 2013 | Pubmed ID: 23412700 Method for Quantitative Study of Airway Functional Microanatomy Using Micro-optical Coherence Tomography PloS One. 2013 | Pubmed ID: 23372732 Progress in Cystic Fibrosis and the CF Therapeutics Development Network Thorax. Oct, 2012 | Pubmed ID: 22960984 A Pharmacologic Approach to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Smoking Related Lung Disease PloS One. 2012 | Pubmed ID: 22768130 Clearance of Initial Mucoid Pseudomonas Aeruginosa in Patients with Cystic Fibrosis Pediatric Pulmonology. Nov, 2012 | Pubmed ID: 22496040 Extensive Surgical and Comprehensive Postoperative Medical Management for Cystic Fibrosis Chronic Rhinosinusitis American Journal of Rhinology & Allergy. Jan-Feb, 2012 | Pubmed ID: 22391086 Purification of CFTR for Mass Spectrometry Analysis: Identification of Palmitoylation and Other Post-translational Modifications Protein Engineering, Design & Selection : PEDS. Jan, 2012 | Pubmed ID: 22119790 Dab2 is a Key Regulator of Endocytosis and Post-endocytic Trafficking of the Cystic Fibrosis Transmembrane Conductance Regulator The Biochemical Journal. Jan, 2012 | Pubmed ID: 21995445 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. Jan, 2012 | Pubmed ID: 21825083 Quercetin Increases Cystic Fibrosis Transmembrane Conductance Regulator-mediated Chloride Transport and Ciliary Beat Frequency: Therapeutic Implications for Chronic Rhinosinusitis American Journal of Rhinology & Allergy. Sep-Oct, 2011 | Pubmed ID: 22186243 A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation The New England Journal of Medicine. Nov, 2011 | Pubmed ID: 22047557 A Breath of Fresh Air Scientific American. Aug, 2011 | Pubmed ID: 21827128 Suppression of CFTR Premature Termination Codons and Rescue of CFTR Protein and Function by the Synthetic Aminoglycoside NB54 Journal of Molecular Medicine (Berlin, Germany). Nov, 2011 | Pubmed ID: 21779978 Regulatory Domain Phosphorylation to Distinguish the Mechanistic Basis Underlying Acute CFTR Modulators American Journal of Physiology. Lung Cellular and Molecular Physiology. Oct, 2011 | Pubmed ID: 21724857 Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTR Mutation The New England Journal of Medicine. Nov, 2010 | Pubmed ID: 21083385 Cystic Fibrosis Transmembrane Conductance Regulator Protein Repair As a Therapeutic Strategy in Cystic Fibrosis Current Opinion in Pulmonary Medicine. Nov, 2010 | Pubmed ID: 20829696 A Critical Role for LTA4H in Limiting Chronic Pulmonary Neutrophilic Inflammation Science (New York, N.Y.). Oct, 2010 | Pubmed ID: 20813919 An International Randomized Multicenter Comparison of Nasal Potential Difference Techniques Chest. Oct, 2010 | Pubmed ID: 20472865 Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): Potential Mediators of Cystic Fibrosis Airway Inflammation The Open Respiratory Medicine Journal. Mar, 2010 | Pubmed ID: 20448817 Association of Cystic Fibrosis Genetic Modifiers with Congenital Bilateral Absence of the Vas Deferens Fertility and Sterility. Nov, 2010 | Pubmed ID: 20100616 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. Nov, 2010 | Pubmed ID: 20042712 Extensive Pulmonary Laceration in Pediatric Trauma Respiration; International Review of Thoracic Diseases. 2010 | Pubmed ID: 18560207 Pharmaceuticals Targeting Nonsense Mutations in Genetic Diseases: Progress in Development BioDrugs : Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy. 2009 | Pubmed ID: 19627168 Potential Role of High-mobility Group Box 1 in Cystic Fibrosis Airway Disease American Journal of Respiratory and Critical Care Medicine. Oct, 2008 | Pubmed ID: 18658107 Genetic and Reproductive Knowledge Among Adolescents and Adults with Cystic Fibrosis Chest. Jun, 2008 | Pubmed ID: 18574307 A Novel Proteolytic Cascade Generates an Extracellular Matrix-derived Chemoattractant in Chronic Neutrophilic Inflammation Journal of Immunology (Baltimore, Md. : 1950). Apr, 2008 | Pubmed ID: 18390751 Detection of Cystic Fibrosis Transmembrane Conductance Regulator Activity in Early-phase Clinical Trials Proceedings of the American Thoracic Society. Aug, 2007 | Pubmed ID: 17652506 Restoration of W1282X CFTR Activity by Enhanced Expression American Journal of Respiratory Cell and Molecular Biology. Sep, 2007 | Pubmed ID: 17541014 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. Jul, 2007 | Pubmed ID: 17347447 Bioelectric Effects of Quinine on Polarized Airway Epithelial Cells Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. Sep, 2007 | Pubmed ID: 17329172 Pulmonary Aneurysms and Intracardiac Thrombi Due to Behçet's Disease in an African-American Adolescent with Oculocutaneous Albinism Clinical Rheumatology. Sep, 2007 | Pubmed ID: 17047893 Advances in Cystic Fibrosis Therapies Current Opinion in Pediatrics. Dec, 2006 | Pubmed ID: 17099358 Cystic Fibrosis The New England Journal of Medicine. May, 2005 | Pubmed ID: 15888700 Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD) George M. Solomon1, Inez Bronsveld2, Kathryn Hayes3, Michael Wilschanski4, Paola Melotti5, Steven M. Rowe1, Isabelle Sermet-Gaudelus6,7 1Department of Medicine and the Gregory Fleming James Cystic Fibrosis Center, University of Alabama at Birmingham, 2Department of Pulmonology and Tuberculosis, University Medical Center Utrecht, 3Center for Experimental Medicine, Queens University, Northern Ireland, 4Hadassah Hebrew University Medical Center, Jerusalem, 5Centro Fibrosi Cistica, Azienda Ospedaliera Universitaria Integrata, 6Service de Pneumologie et Allergologie Pédiatriques and Center de Ressources et de Compétence de la Mucoviscidose, Hôpital Necker Enfants Malades, 7INSERM U 1151, Institut Necker Enfants Malades JoVE 57006 의학
Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD) George M. Solomon1, Inez Bronsveld2, Kathryn Hayes3, Michael Wilschanski4, Paola Melotti5, Steven M. Rowe1, Isabelle Sermet-Gaudelus6,7 1Department of Medicine and the Gregory Fleming James Cystic Fibrosis Center, University of Alabama at Birmingham, 2Department of Pulmonology and Tuberculosis, University Medical Center Utrecht, 3Center for Experimental Medicine, Queens University, Northern Ireland, 4Hadassah Hebrew University Medical Center, Jerusalem, 5Centro Fibrosi Cistica, Azienda Ospedaliera Universitaria Integrata, 6Service de Pneumologie et Allergologie Pédiatriques and Center de Ressources et de Compétence de la Mucoviscidose, Hôpital Necker Enfants Malades, 7INSERM U 1151, Institut Necker Enfants Malades JoVE 57006 의학