Christine E. Bear

Programme in Molecular Structure and Function, Hospital for Sick Children

Affiliated withHospital for Sick Children University of Toronto

Research Area

Biography

Christine E. Bear has not added Biography.
If you are Christine E. Bear and would like to personalize this page please email our Author Liaison for assistance.

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
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