Citation Impact
Citing Papers
Advances in signalling by extracellular nucleotides
2000
Pulmonary Epithelial Sodium-Channel Dysfunction and Excess Airway Liquid in Pseudohypoaldosteronism
1999
A Pilot Study of Aerosolized Amiloride for the Treatment of Lung Disease in Cystic Fibrosis
1990
Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer
1990 Standout
Generation and characterization of a ΔF508 cystic fibrosis mouse model
1995 StandoutNobel
Nucleotide signalling during inflammation
2014 StandoutNature
Colonic and esophageal transepithelial potential difference in cystic fibrosis
1989
The Importance of Bacterial Culture to Food Microbiology in the Age of Genomics
2017
Molecular Mechanisms of Human Hypertension
2001 Standout
Denufosol: A review of studies with inhaled P2Y2 agonists that led to Phase 3
2008
Activation by Extracellular Nucleotides of Chloride Secretion in the Airway Epithelia of Patients with Cystic Fibrosis
1991
Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology
2015 Standout
In vivo transfer of the human cystic fibrosis transmembrane conductance regulator gene to the airway epithelium
1992
Molecular characteristics of amiloride-sensitive sodium channels
1992
Production of a severe cystic fibrosis mutation in mice by gene targeting
1993 StandoutNobel
A functional CFTR protein is required for mouse intestinal cAMP‐, cGMP‐ and Ca2+‐dependent HCO3− secretion
1997 StandoutNobel
TYGS is an automated high-throughput platform for state-of-the-art genome-based taxonomy
2019 Standout
Cystic Fibrosis
2005
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
2011 Standout
Mucus clearance as a primary innate defense mechanism for mammalian airways
2002
Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans
1994 Nature
Phase 2 Randomized Safety and Efficacy Trial of Nebulized Denufosol Tetrasodium in Cystic Fibrosis
2007
Effect of Aerosolized Recombinant Human DNase on Exacerbations of Respiratory Symptoms and on Pulmonary Function in Patients with Cystic Fibrosis
1994 Standout
A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis
1998
A constitutively open potassium channel formed by KCNQ1 and KCNE3
2000 StandoutNature
Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis
1992
Expression and Regulation of the Na+‐K+‐2Cl− Cotransporter NKCC1 in the Normal and CFTR‐Deficient Murine Colon
2003 StandoutNobel
Cystic fibrosis: molecular biology and therapeutic implications
1992 StandoutScience
Airway Mucus Function and Dysfunction
2010 Standout
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
1990 Standout
Mucin Granule Intraluminal Organization in Living Mucous/Goblet Cells
2005 StandoutNobel
Metabolism of P2 Receptor Agonists in Human Airways
2004
Cystic Fibrosis Transmembrane Conductance Regulator Mediates the Cyclic Adenosine Monophosphate–Induced Fluid Secretion But Not the Inhibition of Resorption in Mouse Gallbladder Epithelium
1997 StandoutNobel
International Union of Pharmacology LVIII: Update on the P2Y G Protein-Coupled Nucleotide Receptors: From Molecular Mechanisms and Pathophysiology to Therapy
2006
Degradation of CFTR by the ubiquitin-proteasome pathway
1995 Standout
Extracellular mRNA Induces Dendritic Cell Activation by Stimulating Tumor Necrosis Factor-α Secretion and Signaling through a Nucleotide Receptor
2002 StandoutNobel
The Nature of Small-Airway Obstruction in Chronic Obstructive Pulmonary Disease
2004 Standout
A mouse model for the delta F508 allele of cystic fibrosis.
1995 StandoutNobel
Mutations in the Cystic Fibrosis Gene in Patients with Congenital Absence of the Vas Deferens
1995 Standout
A Novel Mutation in the Cystic Fibrosis Gene in Patients with Pulmonary Disease but Normal Sweat Chloride Concentrations
1994
Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy
1993 StandoutNatureNobel
Importance of basolateral K+ conductance in maintaining Cl− secretion in murine nasal and colonic epithelia
1998
Immunogenic cell death and DAMPs in cancer therapy
2012 Standout
An Animal Model for Cystic Fibrosis Made by Gene Targeting
1992 StandoutScienceNobel
Aggresomes: A Cellular Response to Misfolded Proteins
1998 Standout
The kinase Grk2 regulates Nedd4/Nedd4-2-dependent control of epithelial Na + channels
2004 StandoutNobel
Biofilms: Survival Mechanisms of Clinically Relevant Microorganisms
2002 Standout
Identification of the Cystic Fibrosis Gene: Chromosome Walking and Jumping
1989 StandoutScience
Persistence of Abnormal Chloride Conductance Regulation in Transformed Cystic Fibrosis Epithelia
1989 Science
Regulated Airway Goblet Cell Mucin Secretion
2007
Expression cloning of an ATP receptor from mouse neuroblastoma cells.
1993 StandoutNobel
Activation of human eosinophils via P2 receptors: novel findings and future perspectives
2005
Abnormal apical cell membrane in cystic fibrosis respiratory epithelium. An in vitro electrophysiologic analysis.
1987
Altered intestinal chloride transport in cystic fibrosis
1988
Defective Epithelial Chloride Transport in a Gene-Targeted Mouse Model of Cystic Fibrosis
1992 StandoutScienceNobel
Enhanced Myocardial Function in Transgenic Mice Overexpressing the β 2 -Adrenergic Receptor
1994 StandoutScienceNobel
Cystic Fibrosis Gene Mutation in Two Sisters with Mild Disease and Normal Sweat Electrolyte Levels
1991
Cystic fibrosis decreases the apical membrane chloride permeability of monolayers cultured from cells of tracheal epithelium.
1985
Location of Crossovers during Gene Targeting with Insertion and Replacement Vectors
1993 StandoutNobel
Isolation of additional polymorphic clones from the cystic fibrosis region, using chromosome jumping from D7S8.
1989
Alginate synthesis by Pseudomonas aeruginosa: a key pathogenic factor in chronic pulmonary infections of cystic fibrosis patients
1991
Altered Regulation of Airway Epithelial Cell Chloride Channels in Cystic Fibrosis
1986 Science
Chloride secretion in the trachea of null cystic fibrosis mice: the effects of transfection with pTrial10‐CFTR2.
1997 StandoutNobel
Identification of the Cystic Fibrosis Gene: Genetic Analysis
1989 StandoutScience
Cell-type-specific and hypoxia-inducible expression of the human erythropoietin gene in transgenic mice.
1991 StandoutNobel
Pathophysiology of Gene-Targeted Mouse Models for Cystic Fibrosis
1999
Identification of markers linked to disease-resistance genes by bulked segregant analysis: a rapid method to detect markers in specific genomic regions by using segregating populations.
1991 Standout
Type 1 Neurofibromatosis Gene: Identification of a Large Transcript Disrupted in Three NF1 Patients
1990 StandoutScience
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
1989 StandoutScience
The genetic advantage hypothesis in cystic fibrosis heterozygotes: a murine study.
1995 StandoutNobel
Mucus clearance as a primary innate defense mechanism for mammalian airways
2002
Biogenesis of γ-secretase early in the secretory pathway
2007 StandoutNobel
Regulation of Cl- channels in normal and cystic fibrosis airway epithelial cells by extracellular ATP.
1992
Functional Significance of Cell Volume Regulatory Mechanisms
1998 Standout
Hyperabsorption of Na+ and raised Ca(2+)-mediated Cl- secretion in nasal epithelia of CF mice
1994
Benign missense variations in the cystic fibrosis gene.
2020
Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium
1983 Science
Cystic Fibrosis: a disease of ion channels?
1987
A C-terminal motif found in the β 2 -adrenergic receptor, P2Y1 receptor and cystic fibrosis transmembrane conductance regulator determines binding to the Na + /H + exchanger regulatory factor family of PDZ proteins
1998 StandoutNobel
Impaired cell volume regulation in intestinal crypt epithelia of cystic fibrosis mice.
1995 StandoutNobel
Sodium/Calcium Exchange: Its Physiological Implications
1999 Standout
Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation.
1986
Physiology of Microglia
2011 Standout
Overexpression of a Transporter Gene in a Multidrug-Resistant Human Lung Cancer Cell Line
1992 StandoutScience
Works of Michael Knowles being referenced
Acute safety and effects on mucociliary clearance of aerosolized uridine 5'-triphosphate +/- amiloride in normal human adults.
1996
Genomic Tools for Customized Recovery and Detection of Foodborne Shiga Toxigenic Escherichia coli
2016
Mild Cystic Fibrosis in a Consanguineous Family
1989
Relative ion permeability of normal and cystic fibrosis nasal epithelium.
1983