Citation Impact
Citing Papers
SuFEx-enabled, agnostic discovery of covalent inhibitors of human neutrophil elastase
2019 StandoutNobel
Tracking of Quantum Dot-labeled CFTR Shows Near Immobilization by C-Terminal PDZ Interactions
2006
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
2011
In vitro reconstitution of ER-stress induced ATF6 transport in COPII vesicles
2009 StandoutNobel
Diversity oriented clicking delivers β-substituted alkenyl sulfonyl fluorides as covalent human neutrophil elastase inhibitors
2022 StandoutNobel
Engineering precision nanoparticles for drug delivery
2020 Standout
Targeting microbial biofilms: current and prospective therapeutic strategies
2017 Standout
Sputum Biomarkers of Inflammation in Cystic Fibrosis Lung Disease
2007
Extensive posttranscriptional deletion of the coding sequences for part of nucleotide-binding fold 1 in respiratory epithelial mRNA transcripts of the cystic fibrosis transmembrane conductance regulator gene is not associated with the clinical manifestations of cystic fibrosis.
1992
Introducing sense into nonsense in treatments of human genetic diseases
2008
mRNA vaccines — a new era in vaccinology
2018 StandoutNobel
Rho Signaling Regulates Pannexin 1-mediated ATP Release from Airway Epithelia
2011
Generation and characterization of a ΔF508 cystic fibrosis mouse model
1995 StandoutNobel
Nucleotide signalling during inflammation
2014 StandoutNature
Purinergic Signaling in the Airways
2012
Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats
2014
Association between Pulmonary Function and Sputum Biomarkers in Cystic Fibrosis
2007
Suicide as an outcome for mental disorders
1997 Standout
Amyloidogenesis in Alzheimer's disease: some possible therapeutic opportunities
1992 StandoutNobel
New Therapeutic Approaches to Mendelian Disorders
2010
Glycopeptide-preferring Polypeptide GalNAc Transferase 10 (ppGalNAc T10), Involved in Mucin-type O-Glycosylation, Has a Unique GalNAc-O-Ser/Thr-binding Site in Its Catalytic Domain Not Found in ppGalNAc T1 or T2
2009 StandoutNobel
The C. elegans Cell Corpse Engulfment Gene ced-7 Encodes a Protein Similar to ABC Transporters
1998 StandoutNobel
Genotype and Phenotype in Cystic Fibrosis
2000
Activation of chloride channels in normal and cystic fibrosis airway epithelial cells by multifunctional calcium/calmodulin-dependent protein kinase
1991 Nature
Defective regulation of outwardly rectifying Cl− channels by protein kinase A corrected by insertion of CFTR
1992 Nature
Targeting oxidative stress in disease: promise and limitations of antioxidant therapy
2021 Standout
Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel
1991
Production of a severe cystic fibrosis mutation in mice by gene targeting
1993 StandoutNobel
Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial
2008
Differential roles of NHERF1, NHERF2, and PDZK1 in regulating CFTR-mediated intestinal anion secretion in mice
2009
Neutrophil-epithelial crosstalk at the intestinal lumenal surface mediated by reciprocal secretion of adenosine and IL-6
2001
Nanotechnologies in delivery of mRNA therapeutics using nonviral vector-based delivery systems
2017
New insights into cystic fibrosis: molecular switches that regulate CFTR
2006
Processing of CFTR: Traversing the cellular maze—How much CFTR needs to go through to avoid cystic fibrosis?
2005
In vivo genome editing using Staphylococcus aureus Cas9
2015 StandoutNature
Autophagy in Human Health and Disease
2013 Standout
Remodelling the extracellular matrix in development and disease
2014 Standout
CFTR Delivery to 25% of Surface Epithelial Cells Restores Normal Rates of Mucus Transport to Human Cystic Fibrosis Airway Epithelium
2009
Activation of K+ and Cl? channels in MDCK cells during volume regulation in hypotonic media
1992
Transmembrane Pickets Connect Cyto- and Pericellular Skeletons Forming Barriers to Receptor Engagement
2018 StandoutNobel
Cystic fibrosis genetics: from molecular understanding to clinical application
2014 Standout
A Mutation in the Cystic Fibrosis Transmembrane Conductance Regulator Generates a Novel Internalization Sequence and Enhances Endocytic Rates
2003
CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship
1993 Nature
Therapeutic in vivo gene transfer for genetic disease using AAV: progress and challenges
2011
Cystic fibrosis: molecular biology and therapeutic implications
1992 StandoutScience
A functional CFTR assay using primary cystic fibrosis intestinal organoids
2013
Surrogate Wnt agonists that phenocopy canonical Wnt and β-catenin signalling
2017 StandoutNatureNobel
Nonsense-mediated mRNA decayin health and disease
1999
Therapeutic suppression of premature termination codons: Mechanisms and clinical considerations (Review)
2014
Folding of CFTR Is Predominantly Cotranslational
2005
The CF Gene Product as a Member of a Membrane Transporter (TM6-NBF) Super Family
1991
Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy
2015
Aminoglycoside antibiotics restore dystrophin function to skeletal muscles of mdx mice
1999
A mouse model for the delta F508 allele of cystic fibrosis.
1995 StandoutNobel
The ΔF508-CFTR mutation results in increased biofilm formation byPseudomonas aeruginosaby increasing iron availability
2008
Lipid and polymer nanoparticles for drug delivery to bacterial biofilms
2014
Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy
1993 StandoutNatureNobel
Calcium channels activated by hydrogen peroxide mediate abscisic acid signalling in guard cells
2000 StandoutNature
Modeling Development and Disease with Organoids
2016 Standout
Organic Solutes Rescue the Functional Defect in ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator
2003
A Pilot Clinical Trial of Oral Sodium 4-Phenylbutyrate (Buphenyl) in Δ F508-Homozygous Cystic Fibrosis Patients
1998
Sputum Biomarkers of Inflammation and Lung Function Decline in Children with Cystic Fibrosis
2012
Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis
2003 Standout
A Pilot Study of the Effect of Gentamicin on Nasal Potential Difference Measurements in Cystic Fibrosis Patients Carrying Stop Mutations
2000
Synthetic Aminoglycosides Efficiently Suppress Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations and Are Enhanced by Ivacaftor
2013
The unfolded protein response affects readthrough of premature termination codons
2014
An Animal Model for Cystic Fibrosis Made by Gene Targeting
1992 StandoutScienceNobel
Identification of the Cystic Fibrosis Gene: Chromosome Walking and Jumping
1989 StandoutScience
Immunomodulation in Cystic Fibrosis: Why and How?
2020
Inflammation and its genesis in cystic fibrosis
2015
Excess Mortality in Panic Disorder
1982
Defective Epithelial Chloride Transport in a Gene-Targeted Mouse Model of Cystic Fibrosis
1992 StandoutScienceNobel
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis
2014
The Patch Clamp Technique
1995
Pain regulation by non-neuronal cells and inflammation
2016 StandoutScience
Ca2+ and nucleotide dependent regulation of voltage dependent anion channels in the plasma membrane of guard cells.
1990
Hypoxia-inducible factor 1-dependent expression of adenosine receptor 2B promotes breast cancer stem cell enrichment
2018 StandoutNobel
Organogenesis in a dish: Modeling development and disease using organoid technologies
2014 StandoutScience
Abnormal regulation of ion channels in cystic fibrosis epithelia
1990
The cystic fibrosis transmembrane conductance regulator is a dual ATP and chloride channel.
1994
Specific inhibition of gene expression by small double-stranded RNAs in invertebrate and vertebrate systems
2001 StandoutNobel
Moving forward: cystic fibrosis gene therapy
2013
Identification of the Cystic Fibrosis Gene: Genetic Analysis
1989 StandoutScience
Reversible Photocontrol of Biological Systems by the Incorporation of Molecular Photoswitches
2013 StandoutNobel
Cell-type-specific and hypoxia-inducible expression of the human erythropoietin gene in transgenic mice.
1991 StandoutNobel
Hydroxylation of the eukaryotic ribosomal decoding center affects translational accuracy
2014 StandoutNobel
Pathophysiology of Gene-Targeted Mouse Models for Cystic Fibrosis
1999
Identification of simple arylfluorosulfates as potent agents against resistant bacteria
2021 StandoutNobel
Control of CFTR Channel Gating by Phosphorylation and Nucleotide Hydrolysis
1999
CFTR protein expression in primary and cultured epithelia.
1992
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
Viral Vectors for Gene Therapy: Translational and Clinical Outlook
2015
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
A systematic dissection of determinants and consequences of snoRNA-guided pseudouridylation of human mRNA
2022 StandoutNobel
The Role of Serine Proteases and Antiproteases in the Cystic Fibrosis Lung
2015
Targeted gene knock-in by homology-directed genome editing using Cas9 ribonucleoprotein and AAV donor delivery
2017 StandoutNobel
Characterization of the cystic fibrosis transmembrane conductance regulator in a colonocyte cell line.
1992
Regulation of Cl- channels in normal and cystic fibrosis airway epithelial cells by extracellular ATP.
1992
Effects of C-terminal deletions on cystic fibrosis transmembrane conductance regulator function in cystic fibrosis airway epithelia
2003
Association of Anxiety-Related Traits with a Polymorphism in the Serotonin Transporter Gene Regulatory Region
1996 StandoutScience
The serine protease inhibitor SerpinA3N attenuates neuropathic pain by inhibiting T cell–derived leukocyte elastase
2015
Impaired cell volume regulation in intestinal crypt epithelia of cystic fibrosis mice.
1995 StandoutNobel
Current Trends in Development of Liposomes for Targeting Bacterial Biofilms
2016
Genetic determination of exocrine pancreatic function in cystic fibrosis.
1992
ATP transduces signals from ASGM1, a glycolipid that functions as a bacterial receptor
2001 StandoutNobel
Lys49 myotoxin from the Brazilian lancehead pit viper elicits pain through regulated ATP release
2017 StandoutNobel
Comparative study of platelet 3H-paroxetine and 3H-imipramine binding in panic disorder patients and healthy controls.
1994
Overexpression of a Transporter Gene in a Multidrug-Resistant Human Lung Cancer Cell Line
1992 StandoutScience
Works of John P. Clancy being referenced
A Novel Proteolytic Cascade Generates an Extracellular Matrix-Derived Chemoattractant in Chronic Neutrophilic Inflammation
2008
Extracellular Zinc and ATP Restore Chloride Secretion across Cystic Fibrosis Airway Epithelia by Triggering Calcium Entry
2004
Phosphorylation‐Dependent Regulation of Apical Membrane Chloride Channels in Normal and Cystic Fibrosis Airway Epitheliuma
1989
Predictors of serious suicide attempts among patients with panic disorder
1991
Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line
1997
Repeated Adeno-Associated Virus Serotype 2 Aerosol-Mediated Cystic Fibrosis Transmembrane Regulator Gene Transfer to the Lungs of Patients With Cystic Fibrosis
2004
Activation of the Cystic Fibrosis Transmembrane Conductance Regulator by the Flavonoid Quercetin
2009
Detection of Cystic Fibrosis Transmembrane Conductance Regulator Activity in Early-Phase Clinical Trials
2007
Potential Role of High-Mobility Group Box 1 in Cystic Fibrosis Airway Disease
2008
Up-regulation of AMP-activated Kinase by Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Airway Epithelial Cells Mitigates Excessive Inflammation
2005
An International Randomized Multicenter Comparison of Nasal Potential Difference Techniques
2010
Long-term treatment with oral N-acetylcysteine: Affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial
2014
Human Neutrophil Elastase-Mediated Cleavage Sites of MMP-9 and TIMP-1: Implications to Cystic Fibrosis Proteolytic Dysfunction
2010
Efficient Endocytosis of the Cystic Fibrosis Transmembrane Conductance Regulator Requires a Tyrosine-based Signal
1999
Phase II studies of nebulised Arikace in CF patients withPseudomonas aeruginosainfection
2013
Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR.
1997
Restoration of W1282X CFTR Activity by Enhanced Expression
2007
Adenosine Regulation of Cystic Fibrosis Transmembrane Conductance Regulator through Prostenoids in Airway Epithelia
2006
Personalized Medicine in Cystic Fibrosis
2012
Evidence that Systemic Gentamicin Suppresses Premature Stop Mutations in Patients with Cystic Fibrosis
2001
Activation of Airway Cl − Secretion in Human Subjects by Adenosine
2004
A Clinical Inflammatory Syndrome Attributable to Aerosolized Lipid–DNA Administration in Cystic Fibrosis
2001
Repeated Aerosolized AAV-CFTR for Treatment of Cystic Fibrosis: A Randomized Placebo-Controlled Phase 2B Trial
2007
Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators
2011
Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence
2013
Matrix metalloprotease-9 dysregulation in lower airway secretions of cystic fibrosis patients
2007
Anxiety neurosis and physical illness
1978
Regulation of Chloride Channels by Protein Kinase C in Normal and Cystic Fibrosis Airway Epithelia
1989 Science
Sweat chloride as a biomarker of CFTR activity: Proof of concept and ivacaftor clinical trial data
2013
Reduced microtubule acetylation in cystic fibrosis epithelial cells
2013
A macromolecular complex of β 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA
2002
Tritiated imipramine binding to platelets is decreased in patients with agoraphobia
1985
Adenosine and its nucleotides activate wild-type and R117H CFTR through an A2Breceptor-coupled pathway
1999