Christine E. Bear

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JoVE Journal Publications

ArticleTotal : 3	Year
Publication title Cited by 7	2015
Publication title Cited by 1	2022
Publication title	2026

Other Publications

Article	Year
A Bioengineered Three-Dimensional Cell Culture Platform Integrated with Microfluidics To Address Antimicrobial Resistance in Tuberculosis. mBio\| PubMed ID: 28174307	2017
Dimeric cystic fibrosis transmembrane conductance regulator exists in the plasma membrane. The Biochemical journal\| PubMed ID: 12820897	2003
The chloride channel ClC-4 co-localizes with cystic fibrosis transmembrane conductance regulator and may mediate chloride flux across the apical membrane of intestinal epithelia. The Journal of biological chemistry\| PubMed ID: 11675385	2002
Studies of the molecular basis for cystic fibrosis using purified reconstituted CFTR protein. Methods in molecular medicine\| PubMed ID: 11917519	2002
Epithelial cell chloride channel activity correlates with improved airway function in cystic fibrosis patients with the major mutant: Delta F508. Pediatric research\| PubMed ID: 12409505	2002
Evidence for a functional interaction between the ClC-2 chloride channel and the retrograde motor dynein complex. The Journal of biological chemistry\| PubMed ID: 12601004	2003
CFTR directly mediates nucleotide-regulated glutathione flux. The EMBO journal\| PubMed ID: 12727866	2003
The chloride channel ClC-4 contributes to endosomal acidification and trafficking. The Journal of biological chemistry\| PubMed ID: 12746443	2003
Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore. The Biochemical journal\| PubMed ID: 12892562	2003
Phosphorylation-induced conformational changes of cystic fibrosis transmembrane conductance regulator monitored by attenuated total reflection-Fourier transform IR spectroscopy and fluoresce... The Journal of biological chemistry\| PubMed ID: 14660584	2004
Molecular basis for the chloride channel activity of cystic fibrosis transmembrane conductance regulator and the consequences of disease-causing mutations. Current topics in developmental biology\| PubMed ID: 15094300	2004
A heteromeric complex of the two nucleotide binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) mediates ATPase activity. The Journal of biological chemistry\| PubMed ID: 15284228	2004
Methods to study CFTR protein in vitro. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 15463933	2004
The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 15463939	2004
Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 15463942	2004
ATPase assay of purified, reconstituted CFTR protein. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 15463945	2004
Role of intramolecular and intermolecular interactions in ClC channel and transporter function. Pflügers Archiv : European journal of physiology\| PubMed ID: 16167151	2006
Evaluation of the membrane-spanning domain of ClC-2. The Biochemical journal\| PubMed ID: 16526942	2006
Nucleotides bind to the C-terminus of ClC-5. The Biochemical journal\| PubMed ID: 16686597	2006
The Walker B motif of the second nucleotide-binding domain (NBD2) of CFTR plays a key role in ATPase activity by the NBD1-NBD2 heterodimer. The Biochemical journal\| PubMed ID: 16989640	2007
ATP depletion inhibits the endocytosis of ClC-2. Journal of cellular physiology\| PubMed ID: 17620322	2008
The intact CFTR protein mediates ATPase rather than adenylate kinase activity. The Biochemical journal\| PubMed ID: 18241200	2008
Probing structure-function relationships and gating mechanisms in the CorA Mg2+ transport system. The Journal of biological chemistry\| PubMed ID: 18276588	2008
Evidence for a superoxide permeability pathway in endosomal membranes. Molecular and cellular biology\| PubMed ID: 18378695	2008
Molecular basis for the ATPase activity of CFTR. Archives of biochemistry and biophysics\| PubMed ID: 18417076	2008
Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease. The Biochemical journal\| PubMed ID: 18945216	2009
A novel method for monitoring the cytosolic delivery of peptide cargo. Journal of controlled release : official journal of the Controlled Release Society\| PubMed ID: 19285529	2009
A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability. Molecular pharmacology\| PubMed ID: 19339490	2009
An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice. Journal of cell science\| PubMed ID: 19339555	2009
Functional rescue of DeltaF508-CFTR by peptides designed to mimic sorting motifs. Chemistry & biology\| PubMed ID: 19477416	2009
ClC transporters: discoveries and challenges in defining the mechanisms underlying function and regulation of ClC-5. Pflügers Archiv : European journal of physiology\| PubMed ID: 20049483	2010
A chemical corrector modifies the channel function of F508del-CFTR. Molecular pharmacology\| PubMed ID: 20501743	2010
Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise. Annals of neurology\| PubMed ID: 20517942	2010
ATP induces conformational changes in the carboxyl-terminal region of ClC-5. The Journal of biological chemistry\| PubMed ID: 21173145	2011
Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant. The Journal of biological chemistry\| PubMed ID: 21602569	2011
Targeting the regulation of CFTR channels. The Biochemical journal\| PubMed ID: 21726198	2011
Structural basis for alginate secretion across the bacterial outer membrane. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 21778407	2011
Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy. Essays in biochemistry\| PubMed ID: 21967060	2011
Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator. Journal of medicinal chemistry\| PubMed ID: 22074181	2011
Identification and validation of hits from high throughput screens for CFTR modulators. Current pharmaceutical design\| PubMed ID: 22229556	2012
Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein. Nature biotechnology\| PubMed ID: 22922672	2012
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent m... The Journal of biological chemistry\| PubMed ID: 22942289	2012
Functional Rescue of F508del-CFTR Using Small Molecule Correctors. Frontiers in pharmacology\| PubMed ID: 23055971	2012
50 years ago in the Journal of Pediatrics: the effect of N-acetylcysteine on the viscosity of tracheobronchial secretions in cystic fibrosis of the pancreas. The Journal of pediatrics\| PubMed ID: 23260312	2013
Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5. The Biochemical journal\| PubMed ID: 23566014	2013
Proton-dependent gating and proton uptake by Wzx support O-antigen-subunit antiport across the bacterial inner membrane. mBio\| PubMed ID: 24023388	2013
Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention. Genetics in medicine : official journal of the American College of Medical Genetics\| PubMed ID: 24556927	2014
VX-809 and related corrector compounds exhibit secondary activity stabilizing active F508del-CFTR after its partial rescue to the cell surface. Chemistry & biology\| PubMed ID: 24726831	2014
Lung arginase expression and activity is increased in cystic fibrosis mouse models. Journal of applied physiology (Bethesda, Md. : 1985)\| PubMed ID: 24925982	2014
The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor. Pflugers Archiv : European journal of physiology\| PubMed ID: 25277268	2014
The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation. Proteomics\| PubMed ID: 25330774	2015
Efficient generation of functional CFTR-expressing airway epithelial cells from human pluripotent stem cells. Nature protocols\| PubMed ID: 25654755	2015
Acellular Lung Scaffolds Direct Differentiation of Endoderm to Functional Airway Epithelial Cells: Requirement of Matrix-Bound HS Proteoglycans. Stem cell reports\| PubMed ID: 25660407	2015
Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity. PloS one\| PubMed ID: 26079370
Directed differentiation of cholangiocytes from human pluripotent stem cells. Nature biotechnology\| PubMed ID: 26167630
Facilitating Structure-Function Studies of CFTR Modulator Sites with Efficiencies in Mutagenesis and Functional Screening. Journal of biomolecular screening\| PubMed ID: 26385858
Finding new drugs to enhance anion secretion in cystic fibrosis: Toward suitable systems for better drug screening. Report on the pre-conference meeting to the 12th ECFS Basic Science Confer... Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 26474804	2015
Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop. The Journal of biological chemistry\| PubMed ID: 26627831
Testing gene therapy vectors in human primary nasal epithelial cultures. Molecular therapy. Methods & clinical development\| PubMed ID: 26730394
The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain. Biochemical pharmacology\| PubMed ID: 27614011
Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants. Cellular and molecular life sciences : CMLS\| PubMed ID: 27722768
Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface. The Journal of biological chemistry\| PubMed ID: 28003367
Cystic fibrosis gene modifier SLC26A9 modulates airway response to CFTR-directed therapeutics. Human molecular genetics\| PubMed ID: 28171547
Synergy of cAMP and calcium signaling pathways in CFTR regulation. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 28242698
Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia. NPJ genomic medicine\| PubMed ID: 28649446
Orkambi® and amplifier co-therapy improves function from a rare mutation in gene-edited cells and patient tissue. EMBO molecular medicine\| PubMed ID: 28667089
Editorial overview: Respiratory: Transformational therapies for cystic fibrosis. Current opinion in pharmacology\| PubMed ID: 29221574	2017
Is a Genetic Modifier of Cystic Fibrosis That Regulates Attachment to Human Bronchial Epithelial Cells. mBio\| PubMed ID: 29259090	2017
Structural effects of extracellular loop mutations in CFTR helical hairpins. Biochimica et biophysica acta. Biomembranes\| PubMed ID: 29307731
Transducing Airway Basal Cells with a Helper-Dependent Adenoviral Vector for Lung Gene Therapy. Human gene therapy\| PubMed ID: 29320887
Comprehensive mapping of cystic fibrosis mutations to CFTR protein identifies mutation clusters and molecular docking predicts corrector binding site. Proteins\| PubMed ID: 29569753
Correctors of the Major Cystic Fibrosis Mutant Interact through Membrane-Spanning Domains. Molecular pharmacology\| PubMed ID: 29618585
The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 29685812
Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del. Molecular pharmacology\| PubMed ID: 29903751
SLC6A14, an amino acid transporter, modifies the primary CF defect in fluid secretion. eLife\| PubMed ID: 30004386
Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators. Frontiers in pharmacology\| PubMed ID: 30022950
Activity of a novel antimicrobial peptide against Pseudomonas aeruginosa biofilms. Scientific reports\| PubMed ID: 30283025	2018
Augmentation of Cystic Fibrosis Transmembrane Conductance Regulator Function in Human Bronchial Epithelial Cells via SLC6A14-Dependent Amino Acid Uptake. Implications for Treatment of Cystic... American journal of respiratory cell and molecular biology\| PubMed ID: 31189070
Conversion of human and mouse fibroblasts into lung-like epithelial cells. Scientific reports\| PubMed ID: 31227724
Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Cells\| PubMed ID: 31370288
ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling ... Molecular pharmacology\| PubMed ID: 31427400
Functional rescue of c.3846G>A (W1282X) in patient-derived nasal cultures achieved by inhibition of nonsense mediated decay and protein modulators with complementary mechanisms of action. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 31831337
A Therapy for Most with Cystic Fibrosis. Cell\| PubMed ID: 31978337
Anti-Infectives Restore ORKAMBI Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of . Biomolecules\| PubMed ID: 32092967
Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis-causing mutation. FASEB bioAdvances\| PubMed ID: 32123813
The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor. Journal of personalized medicine\| PubMed ID: 32414100
A helper-dependent adenoviral vector rescues CFTR to wild-type functional levels in cystic fibrosis epithelial cells harbouring class I mutations. The European respiratory journal\| PubMed ID: 32457197
Allele-Specific Prevention of Nonsense-Mediated Decay in Cystic Fibrosis Using Homology-Independent Genome Editing. Molecular therapy. Methods & clinical development\| PubMed ID: 32490033
Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 32741662
Preclinical Studies of a Rare CF-Causing Mutation in the Second Nucleotide Binding Domain (c.3700A>G) Show Robust Functional Rescue in Primary Nasal Cultures by Novel CFTR Modulators. Journal of personalized medicine\| PubMed ID: 33167369
Rescue of multiple class II CFTR mutations by elexacaftor+ tezacaftor+ivacaftor mediated in part by the dual activities of Elexacaftor as both corrector and potentiator. The European respiratory journal\| PubMed ID: 33303536
One-Step Formation of Protein-Based Tubular Structures for Functional Devices and Tissues. Advanced healthcare materials\| PubMed ID: 33694327
Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTA. Journal of personalized medicine\| PubMed ID: 33920764	2021
Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator. iScience\| PubMed ID: 34142049	2021
Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 34419414	2021
A new platform for high-throughput therapy testing on iPSC-derived lung progenitor cells from cystic fibrosis patients. Stem cell reports\| PubMed ID: 34678210	2021
Perspectives on the translation of in-vitro studies to precision medicine in Cystic Fibrosis. EBioMedicine\| PubMed ID: 34740114	2021
Generation of functional ciliated cholangiocytes from human pluripotent stem cells. Nature communications\| PubMed ID: 34764255	2021
High-Throughput Functional Analysis of CFTR and Other Apically Localized Proteins in iPSC-Derived Human Intestinal Organoids. Cells\| PubMed ID: 34943927	2021
Antisense oligonucleotide splicing modulation as a novel Cystic Fibrosis therapeutic approach for the W1282X nonsense mutation. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 34972649	2021
Stage-Specific Generation of Human Pluripotent Stem Cell Derived Lung Models to Measure CFTR Function. Current protocols\| PubMed ID: 35025140	2022
CFTR interactome mapping using the mammalian membrane two-hybrid high-throughput screening system. Molecular systems biology\| PubMed ID: 35156780	2022
A protocol for identifying the binding sites of small molecules on the cystic fibrosis transmembrane conductance regulator (CFTR) protein. STAR protocols\| PubMed ID: 35434660	2022

Article	Year
A Bioengineered Three-Dimensional Cell Culture Platform Integrated with Microfluidics To Address Antimicrobial Resistance in Tuberculosis. mBio\| PubMed ID: 28174307	2017
Dimeric cystic fibrosis transmembrane conductance regulator exists in the plasma membrane. The Biochemical journal\| PubMed ID: 12820897	2003
The chloride channel ClC-4 co-localizes with cystic fibrosis transmembrane conductance regulator and may mediate chloride flux across the apical membrane of intestinal epithelia. The Journal of biological chemistry\| PubMed ID: 11675385	2002
Studies of the molecular basis for cystic fibrosis using purified reconstituted CFTR protein. Methods in molecular medicine\| PubMed ID: 11917519	2002
Epithelial cell chloride channel activity correlates with improved airway function in cystic fibrosis patients with the major mutant: Delta F508. Pediatric research\| PubMed ID: 12409505	2002
Evidence for a functional interaction between the ClC-2 chloride channel and the retrograde motor dynein complex. The Journal of biological chemistry\| PubMed ID: 12601004	2003
CFTR directly mediates nucleotide-regulated glutathione flux. The EMBO journal\| PubMed ID: 12727866	2003
The chloride channel ClC-4 contributes to endosomal acidification and trafficking. The Journal of biological chemistry\| PubMed ID: 12746443	2003
Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore. The Biochemical journal\| PubMed ID: 12892562	2003
Phosphorylation-induced conformational changes of cystic fibrosis transmembrane conductance regulator monitored by attenuated total reflection-Fourier transform IR spectroscopy and fluoresce... The Journal of biological chemistry\| PubMed ID: 14660584	2004
Molecular basis for the chloride channel activity of cystic fibrosis transmembrane conductance regulator and the consequences of disease-causing mutations. Current topics in developmental biology\| PubMed ID: 15094300	2004
A heteromeric complex of the two nucleotide binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) mediates ATPase activity. The Journal of biological chemistry\| PubMed ID: 15284228	2004
Methods to study CFTR protein in vitro. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 15463933	2004
The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 15463939	2004
Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 15463942	2004
ATPase assay of purified, reconstituted CFTR protein. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 15463945	2004
Role of intramolecular and intermolecular interactions in ClC channel and transporter function. Pflügers Archiv : European journal of physiology\| PubMed ID: 16167151	2006
Evaluation of the membrane-spanning domain of ClC-2. The Biochemical journal\| PubMed ID: 16526942	2006
Nucleotides bind to the C-terminus of ClC-5. The Biochemical journal\| PubMed ID: 16686597	2006
The Walker B motif of the second nucleotide-binding domain (NBD2) of CFTR plays a key role in ATPase activity by the NBD1-NBD2 heterodimer. The Biochemical journal\| PubMed ID: 16989640	2007
ATP depletion inhibits the endocytosis of ClC-2. Journal of cellular physiology\| PubMed ID: 17620322	2008
The intact CFTR protein mediates ATPase rather than adenylate kinase activity. The Biochemical journal\| PubMed ID: 18241200	2008
Probing structure-function relationships and gating mechanisms in the CorA Mg2+ transport system. The Journal of biological chemistry\| PubMed ID: 18276588	2008
Evidence for a superoxide permeability pathway in endosomal membranes. Molecular and cellular biology\| PubMed ID: 18378695	2008
Molecular basis for the ATPase activity of CFTR. Archives of biochemistry and biophysics\| PubMed ID: 18417076	2008
Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease. The Biochemical journal\| PubMed ID: 18945216	2009
A novel method for monitoring the cytosolic delivery of peptide cargo. Journal of controlled release : official journal of the Controlled Release Society\| PubMed ID: 19285529	2009
A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability. Molecular pharmacology\| PubMed ID: 19339490	2009
An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice. Journal of cell science\| PubMed ID: 19339555	2009
Functional rescue of DeltaF508-CFTR by peptides designed to mimic sorting motifs. Chemistry & biology\| PubMed ID: 19477416	2009
ClC transporters: discoveries and challenges in defining the mechanisms underlying function and regulation of ClC-5. Pflügers Archiv : European journal of physiology\| PubMed ID: 20049483	2010
A chemical corrector modifies the channel function of F508del-CFTR. Molecular pharmacology\| PubMed ID: 20501743	2010
Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise. Annals of neurology\| PubMed ID: 20517942	2010
ATP induces conformational changes in the carboxyl-terminal region of ClC-5. The Journal of biological chemistry\| PubMed ID: 21173145	2011
Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant. The Journal of biological chemistry\| PubMed ID: 21602569	2011
Targeting the regulation of CFTR channels. The Biochemical journal\| PubMed ID: 21726198	2011
Structural basis for alginate secretion across the bacterial outer membrane. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 21778407	2011
Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy. Essays in biochemistry\| PubMed ID: 21967060	2011
Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator. Journal of medicinal chemistry\| PubMed ID: 22074181	2011
Identification and validation of hits from high throughput screens for CFTR modulators. Current pharmaceutical design\| PubMed ID: 22229556	2012
Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein. Nature biotechnology\| PubMed ID: 22922672	2012
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent m... The Journal of biological chemistry\| PubMed ID: 22942289	2012
Functional Rescue of F508del-CFTR Using Small Molecule Correctors. Frontiers in pharmacology\| PubMed ID: 23055971	2012
50 years ago in the Journal of Pediatrics: the effect of N-acetylcysteine on the viscosity of tracheobronchial secretions in cystic fibrosis of the pancreas. The Journal of pediatrics\| PubMed ID: 23260312	2013
Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5. The Biochemical journal\| PubMed ID: 23566014	2013
Proton-dependent gating and proton uptake by Wzx support O-antigen-subunit antiport across the bacterial inner membrane. mBio\| PubMed ID: 24023388	2013
Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention. Genetics in medicine : official journal of the American College of Medical Genetics\| PubMed ID: 24556927	2014
VX-809 and related corrector compounds exhibit secondary activity stabilizing active F508del-CFTR after its partial rescue to the cell surface. Chemistry & biology\| PubMed ID: 24726831	2014
Lung arginase expression and activity is increased in cystic fibrosis mouse models. Journal of applied physiology (Bethesda, Md. : 1985)\| PubMed ID: 24925982	2014
The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor. Pflugers Archiv : European journal of physiology\| PubMed ID: 25277268	2014
The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation. Proteomics\| PubMed ID: 25330774	2015
Efficient generation of functional CFTR-expressing airway epithelial cells from human pluripotent stem cells. Nature protocols\| PubMed ID: 25654755	2015
Acellular Lung Scaffolds Direct Differentiation of Endoderm to Functional Airway Epithelial Cells: Requirement of Matrix-Bound HS Proteoglycans. Stem cell reports\| PubMed ID: 25660407	2015
Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity. PloS one\| PubMed ID: 26079370
Directed differentiation of cholangiocytes from human pluripotent stem cells. Nature biotechnology\| PubMed ID: 26167630
Facilitating Structure-Function Studies of CFTR Modulator Sites with Efficiencies in Mutagenesis and Functional Screening. Journal of biomolecular screening\| PubMed ID: 26385858
Finding new drugs to enhance anion secretion in cystic fibrosis: Toward suitable systems for better drug screening. Report on the pre-conference meeting to the 12th ECFS Basic Science Confer... Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 26474804	2015
Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop. The Journal of biological chemistry\| PubMed ID: 26627831
Testing gene therapy vectors in human primary nasal epithelial cultures. Molecular therapy. Methods & clinical development\| PubMed ID: 26730394
The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain. Biochemical pharmacology\| PubMed ID: 27614011
Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants. Cellular and molecular life sciences : CMLS\| PubMed ID: 27722768
Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface. The Journal of biological chemistry\| PubMed ID: 28003367
Cystic fibrosis gene modifier SLC26A9 modulates airway response to CFTR-directed therapeutics. Human molecular genetics\| PubMed ID: 28171547
Synergy of cAMP and calcium signaling pathways in CFTR regulation. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 28242698
Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia. NPJ genomic medicine\| PubMed ID: 28649446
Orkambi® and amplifier co-therapy improves function from a rare mutation in gene-edited cells and patient tissue. EMBO molecular medicine\| PubMed ID: 28667089
Editorial overview: Respiratory: Transformational therapies for cystic fibrosis. Current opinion in pharmacology\| PubMed ID: 29221574	2017
Is a Genetic Modifier of Cystic Fibrosis That Regulates Attachment to Human Bronchial Epithelial Cells. mBio\| PubMed ID: 29259090	2017
Structural effects of extracellular loop mutations in CFTR helical hairpins. Biochimica et biophysica acta. Biomembranes\| PubMed ID: 29307731
Transducing Airway Basal Cells with a Helper-Dependent Adenoviral Vector for Lung Gene Therapy. Human gene therapy\| PubMed ID: 29320887
Comprehensive mapping of cystic fibrosis mutations to CFTR protein identifies mutation clusters and molecular docking predicts corrector binding site. Proteins\| PubMed ID: 29569753
Correctors of the Major Cystic Fibrosis Mutant Interact through Membrane-Spanning Domains. Molecular pharmacology\| PubMed ID: 29618585
The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 29685812
Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del. Molecular pharmacology\| PubMed ID: 29903751
SLC6A14, an amino acid transporter, modifies the primary CF defect in fluid secretion. eLife\| PubMed ID: 30004386
Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators. Frontiers in pharmacology\| PubMed ID: 30022950
Activity of a novel antimicrobial peptide against Pseudomonas aeruginosa biofilms. Scientific reports\| PubMed ID: 30283025	2018
Augmentation of Cystic Fibrosis Transmembrane Conductance Regulator Function in Human Bronchial Epithelial Cells via SLC6A14-Dependent Amino Acid Uptake. Implications for Treatment of Cystic... American journal of respiratory cell and molecular biology\| PubMed ID: 31189070
Conversion of human and mouse fibroblasts into lung-like epithelial cells. Scientific reports\| PubMed ID: 31227724
Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Cells\| PubMed ID: 31370288
ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling ... Molecular pharmacology\| PubMed ID: 31427400
Functional rescue of c.3846G>A (W1282X) in patient-derived nasal cultures achieved by inhibition of nonsense mediated decay and protein modulators with complementary mechanisms of action. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 31831337
A Therapy for Most with Cystic Fibrosis. Cell\| PubMed ID: 31978337
Anti-Infectives Restore ORKAMBI Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of . Biomolecules\| PubMed ID: 32092967
Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis-causing mutation. FASEB bioAdvances\| PubMed ID: 32123813
The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor. Journal of personalized medicine\| PubMed ID: 32414100
A helper-dependent adenoviral vector rescues CFTR to wild-type functional levels in cystic fibrosis epithelial cells harbouring class I mutations. The European respiratory journal\| PubMed ID: 32457197
Allele-Specific Prevention of Nonsense-Mediated Decay in Cystic Fibrosis Using Homology-Independent Genome Editing. Molecular therapy. Methods & clinical development\| PubMed ID: 32490033
Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 32741662
Preclinical Studies of a Rare CF-Causing Mutation in the Second Nucleotide Binding Domain (c.3700A>G) Show Robust Functional Rescue in Primary Nasal Cultures by Novel CFTR Modulators. Journal of personalized medicine\| PubMed ID: 33167369
Rescue of multiple class II CFTR mutations by elexacaftor+ tezacaftor+ivacaftor mediated in part by the dual activities of Elexacaftor as both corrector and potentiator. The European respiratory journal\| PubMed ID: 33303536
One-Step Formation of Protein-Based Tubular Structures for Functional Devices and Tissues. Advanced healthcare materials\| PubMed ID: 33694327
Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTA. Journal of personalized medicine\| PubMed ID: 33920764	2021
Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator. iScience\| PubMed ID: 34142049	2021
Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society\| PubMed ID: 34419414	2021
A new platform for high-throughput therapy testing on iPSC-derived lung progenitor cells from cystic fibrosis patients. Stem cell reports\| PubMed ID: 34678210	2021
Perspectives on the translation of in-vitro studies to precision medicine in Cystic Fibrosis. EBioMedicine\| PubMed ID: 34740114	2021
Generation of functional ciliated cholangiocytes from human pluripotent stem cells. Nature communications\| PubMed ID: 34764255	2021
High-Throughput Functional Analysis of CFTR and Other Apically Localized Proteins in iPSC-Derived Human Intestinal Organoids. Cells\| PubMed ID: 34943927	2021
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