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Changes in mucociliary clearance over time in children with cystic fibrosis.
- Published in:
- Pediatric Pulmonology, 2020, v. 55, n. 9, p. 2307, doi. 10.1002/ppul.24858
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- Publication type:
- Article
Measurements of spontaneous CFTR-mediated ion transport without acute channel activation in airway epithelial cultures after modulator exposure.
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- Scientific Reports, 2021, v. 11, n. 1, p. 1, doi. 10.1038/s41598-021-02044-1
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- Publication type:
- Article
Author Correction: Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment.
- Published in:
- 2021
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- Publication type:
- Correction Notice
Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment.
- Published in:
- Scientific Reports, 2021, v. 11, n. 1, p. 1, doi. 10.1038/s41598-021-99184-1
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- Publication type:
- Article
Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment.
- Published in:
- Scientific Reports, 2021, v. 11, n. 1, p. 1, doi. 10.1038/s41598-021-99184-1
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- Publication type:
- Article
Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population.
- Published in:
- 2000
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- Publication type:
- journal article
ONGOING RESEARCH INTO OTHER EMERGING THERAPIES.
- Published in:
- Johns Hopkins Advanced Studies in Medicine, 2010, v. 10, n. 1, p. 19
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- Publication type:
- Article
Cystic fibrosis and estrogens: a perfect storm.
- Published in:
- 2008
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- Publication type:
- journal article
Transmembrane Mutations Alter the Channel Characteristics of the Cystic Fibrosis Transmembrane Conductance Regulator Expressed in Xenopus Oocytes.
- Published in:
- Cellular Physiology & Biochemistry (Karger AG), 1994, v. 4, n. 1/2, p. 10, doi. 10.1159/000154705
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- Publication type:
- Article
Expanding CFTR Modulator Testing to Carriers of CFTR Variants.
- Published in:
- 2021
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- Publication type:
- journal article
Effect of apical chloride concentration on the measurement of responses to CFTR modulation in airway epithelia cultured from nasal brushings.
- Published in:
- Physiological Reports, 2020, v. 8, n. 19, p. 1, doi. 10.14814/phy2.14603
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- Publication type:
- Article
Dietary supplement use in pediatric patients with cystic fibrosis.
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- American Journal of Health-System Pharmacy, 2008, v. 65, n. 6, p. 562, doi. 10.2146/ajhp070447
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- Publication type:
- Article
NHLBI training workshop report: The vanishing pediatric pulmonary investigator and recommendations for recovery.
- Published in:
- Pediatric Pulmonology, 2010, v. 45, n. 1, p. 25, doi. 10.1002/ppul.21155
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- Publication type:
- Article
Pulmonary function in adolescents with ataxia telangiectasia.
- Published in:
- Pediatric Pulmonology, 2008, v. 43, n. 1, p. 59, doi. 10.1002/ppul.20738
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- Publication type:
- Article
Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosis.
- Published in:
- Pediatric Pulmonology, 2007, v. 42, n. 7, p. 610, doi. 10.1002/ppul.20625
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- Publication type:
- Article
Acidic pH Hyperpolarizes Nasal Potential Difference.
- Published in:
- Pediatric Pulmonology, 2006, v. 41, n. 2, p. 151, doi. 10.1002/ppul.20328
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- Publication type:
- Article
Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: Results of a phase 1/phase 2 multicenter study in mild to moderate cystic fibrosis.
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- Pediatric Pulmonology, 2005, v. 39, n. 4, p. 339
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- Publication type:
- Article
Randomized, Double-Blind, Placebo-Controlled, Dose-Escalating Study of Aerosolized Interferon Gamma-1b in Patients With Mild to Moderate Cystic Fibrosis Lung DiseaseOur results were presented in part at the American Thoracic Society 2003 Annual Meeting (Moss et al., Am J Respir Crit Care Med 2003;167:923).Conflict of interest statement: Karen Starko, M.D., is an employee of Intermune, Inc., which partly sponsored this study, provided study agents, and participated in data analysis.
- Published in:
- Pediatric Pulmonology, 2005, v. 39, n. 3, p. 209, doi. 10.1002/ppul.20152
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- Publication type:
- Article
Standardized procedure for measurement of nasal potential difference: An outcome measure in multicenter cystic fibrosis clinical trials (Previous presentation: NACFF Poster, 2001. Standaert TA, Zeitlin PL, Brass L, Konstan M, Noone PG, Boitano L, Emerson J, Milgram L, Hunter J, Hammond K, Foy C, Berclaz P-Y, Davies Z, Knowles MR. Nasal potential difference measurements from 8 CF centers utilizing a recently developed standardized operating procedure. Pediatr Pulmonol 2001;32:22.)
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- Pediatric Pulmonology, 2004, v. 37, n. 5, p. 385, doi. 10.1002/ppul.10448
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- Publication type:
- Article
Future Pharmacological Treatment of Cystic Fibrosis.
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- Respiration, 2000, v. 67, n. 4, p. 351, doi. 10.1159/000029528
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- Publication type:
- Article
Cystic fibrosis transmembrane conductance regulator function, not TAS2R38 gene haplotypes, predict sinus surgery in children and young adults with cystic fibrosis.
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- International Forum of Allergy & Rhinology, 2020, v. 10, n. 6, p. 748, doi. 10.1002/alr.22548
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- Publication type:
- Article
CFTR Is a Negative Regulator of NFkB Mediated Innate Immune Response.
- Published in:
- PLoS ONE, 2009, v. 4, n. 2, p. 1, doi. 10.1371/journal.pone.0004664
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- Publication type:
- Article
Patients with Mutations in Gsα Have Reduced Activation of a Downstream Target in Epithelial Tissues due to Haploinsufficiency.
- Published in:
- 2007
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- Publication type:
- journal article
Modulation of ΔF508 Cystic Fibrosis Transmembrane Regulator Trafficking and Function with 4-Phenylbutyrate and Flavonoids.
- Published in:
- American Journal of Respiratory Cell & Molecular Biology, 2004, v. 31, n. 3, p. 351, doi. 10.1165/rcmb.2002-0086OC
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- Publication type:
- Article
Protein Processing and Degradation in Pulmonary Health and Disease.
- Published in:
- American Journal of Respiratory Cell & Molecular Biology, 2003, v. 29, n. 5, p. 642, doi. 10.1165/rcmb.2002-0200WS
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- Publication type:
- Article
Modulation of Sp1 and Sp3 in Lung Epithelial Cells Regulates ClC-2 Chloride Channel Expression.
- Published in:
- American Journal of Respiratory Cell & Molecular Biology, 2003, v. 29, n. 4, p. 499, doi. 10.1165/rcmb.2003-0030oc
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- Publication type:
- Article
Proteome of synaptosome-associated proteins in spinal cord dorsal horn after peripheral nerve injury.
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- Proteomics, 2009, v. 9, n. 5, p. 1241, doi. 10.1002/pmic.200800636
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- Publication type:
- Article
'Bronchitis' obliterans and prolonged transient hypogammaglobulinemia in a child.
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- Pediatric Pulmonology, 1993, v. 16, n. 6, p. 375, doi. 10.1002/ppul.1950160610
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- Publication type:
- Article
NHLBI Training Workshop Report: The Vanishing Pediatric Pulmonary Investigator and Recommendations for Recovery.
- Published in:
- Lung, 2009, v. 187, n. 6, p. 367, doi. 10.1007/s00408-009-9184-0
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- Publication type:
- Article
Etiology of Bronchopulmonary Dysplasia: Before Birth.
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- Pediatric Allergy, Immunology & Pulmonology, 2011, v. 24, n. 1, p. 21, doi. 10.1089/ped.2011.0074
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- Publication type:
- Article
Benralizumab does not impair antibody response to seasonal influenza vaccination in adolescent and young adult patients with moderate to severe asthma: results from the Phase IIIb ALIZE trial.
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- Journal of Asthma & Allergy, 2018, v. 11, p. 181, doi. 10.2147/JAA.S172338
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- Publication type:
- Article
Asthma inflammatory subtypes and new biologic therapies.
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- Revista Mexicana de Anestesiología, 2019, v. 42, n. S1, p. S287
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- Publication type:
- Article
CHOP Transcription Factor Mediates IL-8 Signaling in Cystic Fibrosis Bronchial Epithelial Cells.
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- American Journal of Respiratory Cell & Molecular Biology, 2008, v. 38, n. 2, p. 176, doi. 10.1165/rcmb.2007-0197OC
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- Publication type:
- Article
Respiratory Epithelial Gene Expression in Patients with Mild and Severe Cystic Fibrosis Lung Disease.
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- American Journal of Respiratory Cell & Molecular Biology, 2006, v. 35, n. 3, p. 327, doi. 10.1165/rcmb.2005-0359OC
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- Publication type:
- Article
Regulation of the CIC-2 Lung Epithelial Chloride Channel by Glycosylation of SP1.
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- American Journal of Respiratory Cell & Molecular Biology, 2006, v. 34, n. 6, p. 754, doi. 10.1165/rcmb.2005-0442OC
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- Publication type:
- Article
Alternative mRNA splice variants of the rat ClC-2 chloride channel gene are expressed in lung: genomic sequence and organization of ClC-2.
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- Nucleic Acids Research, 1997, v. 25, n. 20, p. 4153, doi. 10.1093/nar/25.20.4153
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- Publication type:
- Article
A Short ClC-2 mRNA Transcript Is Produced by Exon Skipping.
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- Nucleic Acids Research, 1996, v. 24, n. 17, p. 3453, doi. 10.1093/nar/24.17.3453
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- Publication type:
- Article
Regulation of ion channels in cystic fibrosis (869.8).
- Published in:
- FASEB Journal, 2014, v. 28, p. N.PAG, doi. 10.1096/fasebj.28.1_supplement.869.8
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- Publication type:
- Article
Multiple Molecular Chaperone-mediated Pharmacologic Rescue of δF508-CFTR from ERAD.
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- FASEB Journal, 2007, v. 21, n. 5, p. A420
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- Publication type:
- Article
Inflammatory and Microbiologic Markers in Induced Sputum after Intravenous Antibiotics in Cystic Fibrosis.
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- American Journal of Respiratory & Critical Care Medicine, 2003, v. 168, n. 12, p. 1471, doi. 10.1164/rccm.200306-731OC
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- Publication type:
- Article
Significant Microbiological Effect of Inhaled Tobramycin in Young Children with Cystic Fibrosis.
- Published in:
- American Journal of Respiratory & Critical Care Medicine, 2003, v. 167, n. 6, p. 841, doi. 10.1164/rccm.200208-855OC
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- Publication type:
- Article
Evaluation of Exposure and Health Care Worker Response to Nebulized Administration of tgAAVCF to Patients with Cystic Fibrosis.
- Published in:
- Annals of Occupational Hygiene, 2004, v. 48, n. 8, p. 673, doi. 10.1093/annhyg/meh066
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- Publication type:
- Article
Description of a Standardized Nutrition Classification Plan and its Relation to Nutritional Outcomes in Children with Cystic Fibrosis.
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- Journal of Pediatric Psychology, 2010, v. 35, n. 1, p. 6, doi. 10.1093/jpepsy/jsp029
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- Publication type:
- Article
N-acetylcysteine Enhances Cystic Fibrosis Sputum Penetration and Airway Gene Transfer by Highly Compacted DNA Nanoparticles.
- Published in:
- Molecular Therapy, 2011, v. 19, n. 11, p. 1981, doi. 10.1038/mt.2011.160
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- Publication type:
- Article
717. VCP Short Hairpin RNA Rescues ΔF508-CFTR and Suppresses IL8 Levels: Therapeutic Implications in Cystic Fibrosis.
- Published in:
- Molecular Therapy, 2006, v. 13, p. S276, doi. 10.1016/j.ymthe.2006.08.796
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- Publication type:
- Article
Evidence of CFTR Function in Cystic Fibrosis after Systemic Administration of 4-Phenylbutyrate
- Published in:
- Molecular Therapy, 2002, v. 6, n. 1, p. 119, doi. 10.1006/mthe.2002.0639
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- Publication type:
- Article
Digitoxin for Airway Inflammation in Cystic Fibrosis: Preliminary Assessment of Safety, Pharmacokinetics, and Dose Finding.
- Published in:
- 2017
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- Publication type:
- journal article
Membrane-associated heparan sulfate is not required for rAAV-2 infection of human respiratory epithelia.
- Published in:
- Virology Journal, 2006, v. 3, p. 29, doi. 10.1186/1743-422X-3-29
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- Publication type:
- Article
Atypical Cystic Fibrosis and CFTR-Related Diseases.
- Published in:
- Clinical Reviews in Allergy & Immunology, 2008, v. 35, n. 3, p. 116, doi. 10.1007/s12016-008-8083-0
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- Publication type:
- Article
Cystic Fibrosis Transmembrane Regulator Protein Mutations: 'Class' Opportunity for Novel Drug Innovation.
- Published in:
- Pediatric Drugs, 2007, v. 9, n. 1, p. 1, doi. 10.2165/00148581-200709010-00001
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- Publication type:
- Article