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Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTR Mutation

The New England journal of medicine, 2010-11, Vol.363 (21), p.1991-2003

2010

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Biomarkers for cystic fibrosis drug development

Journal of cystic fibrosis, 2016-11, Vol.15 (6), p.714-723

2016

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Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study

Journal of cystic fibrosis, 2021-11, Vol.20 (6), p.965-971

2021

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Plasma TGF-β1 in pediatric cystic fibrosis: Potential biomarker of lung disease and response to therapy

Pediatric pulmonology, 2011-07, Vol.46 (7), p.688-695

2011

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Repeated adeno-associated virus serotype 2 aerosol-mediated cystic fibrosis transmembrane regulator gene transfer to the lungs of patients with cystic fibrosis: A multicenter, double-blind, placebo-controlled trial

Chest, 2004-02, Vol.125 (2), p.509-521

2004

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Evidence that Systemic Gentamicin Suppresses Premature Stop Mutations in Patients with Cystic Fibrosis

American journal of respiratory and critical care medicine, 2001-06, Vol.163 (7), p.1683-1692

2001

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Adenosine receptors, cystic fibrosis, and airway hydration

Handbook of experimental pharmacology, 2009 (193), p.363

2009

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Adenosine Regulation of Cystic Fibrosis Transmembrane Conductance Regulator through Prostenoids in Airway Epithelia

American journal of respiratory cell and molecular biology, 2006-05, Vol.34 (5), p.600-608

2006

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Advances in cystic fibrosis therapies

Current opinion in pediatrics, 2006-12, Vol.18 (6), p.604-613

2006

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Matrix metalloproteinase activity in pediatric acute lung injury

International journal of medical sciences, 2009-01, Vol.6 (1), p.9-17

2009

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Up-regulation of AMP-activated Kinase by Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Airway Epithelial Cells Mitigates Excessive Inflammation

The Journal of biological chemistry, 2006-02, Vol.281 (7), p.4231-4241

2006

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A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis

Human gene therapy, 2001-05, Vol.12 (7), p.751-761

2001

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Extracellular Zinc and ATP Restore Chloride Secretion across Cystic Fibrosis Airway Epithelia by Triggering Calcium Entry

The Journal of biological chemistry, 2004-03, Vol.279 (11), p.10720-10729

2004

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Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line

Nature medicine, 1997-11, Vol.3 (11), p.1280-1284

1997

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Activation of Airway Cl- Secretion in Human Subjects by Adenosine

American journal of respiratory cell and molecular biology, 2004-08, Vol.31 (2), p.140-146

2004

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Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis

The Journal of pediatrics, 2002-04, Vol.140 (4), p.439-444

2002

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Adenosine Receptors and Phosphodiesterase Inhibitors Stimulate Cl- Secretion in Calu-3 Cells

American journal of respiratory cell and molecular biology, 2003-09, Vol.29 (3), p.410-418

2003

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A(2) adenosine receptors regulate CFTR through PKA and PLA(2)

American journal of physiology. Lung cellular and molecular physiology, 2002-01, Vol.282 (1), p.L12-L25

2002

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The Caregiver Quality of Life Cystic Fibrosis (CQOLCF) Scale: Modification and Validation of an Instrument to Measure Quality of Life in Cystic Fibrosis Family Caregivers

Quality of life research, 2003-12, Vol.12 (8), p.1119-1126

2003

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Endorectal repair of rectocele revisited

British journal of surgery, 1997-01, Vol.84 (1), p.89-91

1997

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Regulation of Chloride Channels by Protein Kinase C in Normal and Cystic Fibrosis Airway Epithelia

Science (American Association for the Advancement of Science), 1989-06, Vol.244 (4910), p.1353-1356

1989

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Activation of Chloride Secretion in Cystic Fibrosis Cells and Tissues by the Substituted Imidazole SRI 2931

Biochemistry (Easton), 2003-11, Vol.42 (45), p.13241-13249

2003

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Membrane transplantation to correct integral membrane protein defects

Journal of molecular medicine (Berlin, Germany), 2003-08, Vol.81 (8), p.511-520

2003

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Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR

American journal of respiratory and critical care medicine, 1997-05, Vol.155 (5), p.1684-1689

1997

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Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTR Mutation

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

Biomarkers for cystic fibrosis drug development

Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study

Plasma TGF-β1 in pediatric cystic fibrosis: Potential biomarker of lung disease and response to therapy

Repeated adeno-associated virus serotype 2 aerosol-mediated cystic fibrosis transmembrane regulator gene transfer to the lungs of patients with cystic fibrosis: A multicenter, double-blind, placebo-controlled trial

Evidence that Systemic Gentamicin Suppresses Premature Stop Mutations in Patients with Cystic Fibrosis

Adenosine receptors, cystic fibrosis, and airway hydration

Adenosine Regulation of Cystic Fibrosis Transmembrane Conductance Regulator through Prostenoids in Airway Epithelia

Advances in cystic fibrosis therapies

Matrix metalloproteinase activity in pediatric acute lung injury

Up-regulation of AMP-activated Kinase by Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Airway Epithelial Cells Mitigates Excessive Inflammation

A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis

Extracellular Zinc and ATP Restore Chloride Secretion across Cystic Fibrosis Airway Epithelia by Triggering Calcium Entry

Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line

Activation of Airway Cl- Secretion in Human Subjects by Adenosine

Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis

Adenosine Receptors and Phosphodiesterase Inhibitors Stimulate Cl- Secretion in Calu-3 Cells

A(2) adenosine receptors regulate CFTR through PKA and PLA(2)

The Caregiver Quality of Life Cystic Fibrosis (CQOLCF) Scale: Modification and Validation of an Instrument to Measure Quality of Life in Cystic Fibrosis Family Caregivers

Endorectal repair of rectocele revisited

Regulation of Chloride Channels by Protein Kinase C in Normal and Cystic Fibrosis Airway Epithelia

Activation of Chloride Secretion in Cystic Fibrosis Cells and Tissues by the Substituted Imidazole SRI 2931

Membrane transplantation to correct integral membrane protein defects

Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR

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