Christine E. Bear Molecular Medicine Hospital for Sick Children Biography Publications Institution JoVE Articles Christine E. Bear has not added a biography. If you are Christine E. Bear and would like to personalize this page please email our Author Liaison for assistance. Publications A Protocol for Identifying the Binding Sites of Small Molecules on the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein STAR Protocols. Jun, 2022 | Pubmed ID: 35434660 CFTR Interactome Mapping Using the Mammalian Membrane Two-hybrid High-throughput Screening System Molecular Systems Biology. 02, 2022 | Pubmed ID: 35156780 Stage-Specific Generation of Human Pluripotent Stem Cell Derived Lung Models to Measure CFTR Function Current Protocols. Jan, 2022 | Pubmed ID: 35025140 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. Dec, 2021 | Pubmed ID: 34972649 High-Throughput Functional Analysis of CFTR and Other Apically Localized Proteins in IPSC-Derived Human Intestinal Organoids Cells. 12, 2021 | Pubmed ID: 34943927 Generation of Functional Ciliated Cholangiocytes from Human Pluripotent Stem Cells Nature Communications. 11, 2021 | Pubmed ID: 34764255 Perspectives on the Translation of In-vitro Studies to Precision Medicine in Cystic Fibrosis EBioMedicine. Nov, 2021 | Pubmed ID: 34740114 A New Platform for High-throughput Therapy Testing on IPSC-derived Lung Progenitor Cells from Cystic Fibrosis Patients Stem Cell Reports. 11, 2021 | Pubmed ID: 34678210 Riociguat for the Treatment of Phe508del Homozygous Adults with Cystic Fibrosis Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 11, 2021 | Pubmed ID: 34419414 Identification of Binding Sites for Ivacaftor on the Cystic Fibrosis Transmembrane Conductance Regulator IScience. Jun, 2021 | Pubmed ID: 34142049 Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTA Journal of Personalized Medicine. Apr, 2021 | Pubmed ID: 33920764 Activity of a Novel Antimicrobial Peptide Against Pseudomonas Aeruginosa Biofilms Scientific Reports. 10, 2018 | Pubmed ID: 30283025 Is a Genetic Modifier of Cystic Fibrosis That Regulates Attachment to Human Bronchial Epithelial Cells MBio. 12, 2017 | Pubmed ID: 29259090 Editorial Overview: Respiratory: Transformational Therapies for Cystic Fibrosis Current Opinion in Pharmacology. 06, 2017 | Pubmed ID: 29221574 A Bioengineered Three-Dimensional Cell Culture Platform Integrated with Microfluidics To Address Antimicrobial Resistance in Tuberculosis MBio. 02, 2017 | Pubmed ID: 28174307 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 Conference, Albufeira, 25-28 March 2015 Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. Nov, 2015 | Pubmed ID: 26474804 Acellular Lung Scaffolds Direct Differentiation of Endoderm to Functional Airway Epithelial Cells: Requirement of Matrix-Bound HS Proteoglycans Stem Cell Reports. Feb, 2015 | Pubmed ID: 25660407 Efficient Generation of Functional CFTR-expressing Airway Epithelial Cells from Human Pluripotent Stem Cells Nature Protocols. Mar, 2015 | Pubmed ID: 25654755 The Major Cystic Fibrosis Causing Mutation Exhibits Defective Propensity for Phosphorylation Proteomics. Jan, 2015 | Pubmed ID: 25330774 The Cystic Fibrosis Transmembrane Conductance Regulator is an Extracellular Chloride Sensor Pflugers Archiv : European Journal of Physiology. Oct, 2014 | Pubmed ID: 25277268 Lung Arginase Expression and Activity is Increased in Cystic Fibrosis Mouse Models Journal of Applied Physiology (Bethesda, Md. : 1985). Aug, 2014 | Pubmed ID: 24925982 VX-809 and Related Corrector Compounds Exhibit Secondary Activity Stabilizing Active F508del-CFTR After Its Partial Rescue to the Cell Surface Chemistry & Biology. May, 2014 | Pubmed ID: 24726831 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. Aug, 2014 | Pubmed ID: 24556927 Proton-dependent Gating and Proton Uptake by Wzx Support O-antigen-subunit Antiport Across the Bacterial Inner Membrane MBio. 2013 | Pubmed ID: 24023388 Conformational Defects Underlie Proteasomal Degradation of Dent's Disease-causing Mutants of ClC-5 The Biochemical Journal. Jun, 2013 | Pubmed ID: 23566014 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. Jan, 2013 | Pubmed ID: 23260312 Functional Rescue of F508del-CFTR Using Small Molecule Correctors Frontiers in Pharmacology. 2012 | Pubmed ID: 23055971 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 Manner The Journal of Biological Chemistry. Oct, 2012 | Pubmed ID: 22942289 Directed Differentiation of Human Pluripotent Stem Cells into Mature Airway Epithelia Expressing Functional CFTR Protein Nature Biotechnology. Sep, 2012 | Pubmed ID: 22922672 Identification and Validation of Hits from High Throughput Screens for CFTR Modulators Current Pharmaceutical Design. 2012 | Pubmed ID: 22229556 Synthesis and Properties of Molecular Probes for the Rescue Site on Mutant Cystic Fibrosis Transmembrane Conductance Regulator Journal of Medicinal Chemistry. Dec, 2011 | Pubmed ID: 22074181 Insights into the Mechanisms Underlying CFTR Channel Activity, the Molecular Basis for Cystic Fibrosis and Strategies for Therapy Essays in Biochemistry. Sep, 2011 | Pubmed ID: 21967060 Structural Basis for Alginate Secretion Across the Bacterial Outer Membrane Proceedings of the National Academy of Sciences of the United States of America. Aug, 2011 | Pubmed ID: 21778407 Targeting the Regulation of CFTR Channels The Biochemical Journal. Apr, 2011 | Pubmed ID: 21726198 Probing Conformational Rescue Induced by a Chemical Corrector of F508del-cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutant The Journal of Biological Chemistry. Jul, 2011 | Pubmed ID: 21602569 ATP Induces Conformational Changes in the Carboxyl-terminal Region of ClC-5 The Journal of Biological Chemistry. Feb, 2011 | Pubmed ID: 21173145 Cystic Fibrosis Transmembrane Conductance Regulator in Human Muscle: Dysfunction Causes Abnormal Metabolic Recovery in Exercise Annals of Neurology. Jun, 2010 | Pubmed ID: 20517942 A Chemical Corrector Modifies the Channel Function of F508del-CFTR Molecular Pharmacology. Sep, 2010 | Pubmed ID: 20501743 ClC Transporters: Discoveries and Challenges in Defining the Mechanisms Underlying Function and Regulation of ClC-5 Pflügers Archiv : European Journal of Physiology. Jul, 2010 | Pubmed ID: 20049483 Functional Rescue of DeltaF508-CFTR by Peptides Designed to Mimic Sorting Motifs Chemistry & Biology. May, 2009 | Pubmed ID: 19477416 An Essential Role for ClC-4 in Transferrin Receptor Function Revealed in Studies of Fibroblasts Derived from Clcn4-null Mice Journal of Cell Science. Apr, 2009 | Pubmed ID: 19339555 A Small-molecule Modulator Interacts Directly with DeltaPhe508-CFTR to Modify Its ATPase Activity and Conformational Stability Molecular Pharmacology. Jun, 2009 | Pubmed ID: 19339490 A Novel Method for Monitoring the Cytosolic Delivery of Peptide Cargo Journal of Controlled Release : Official Journal of the Controlled Release Society. Jul, 2009 | Pubmed ID: 19285529 Direct Interaction of a Small-molecule Modulator with G551D-CFTR, a Cystic Fibrosis-causing Mutation Associated with Severe Disease The Biochemical Journal. Feb, 2009 | Pubmed ID: 18945216 Molecular Basis for the ATPase Activity of CFTR Archives of Biochemistry and Biophysics. Aug, 2008 | Pubmed ID: 18417076 Evidence for a Superoxide Permeability Pathway in Endosomal Membranes Molecular and Cellular Biology. Jun, 2008 | Pubmed ID: 18378695 Probing Structure-function Relationships and Gating Mechanisms in the CorA Mg2+ Transport System The Journal of Biological Chemistry. Apr, 2008 | Pubmed ID: 18276588 The Intact CFTR Protein Mediates ATPase Rather Than Adenylate Kinase Activity The Biochemical Journal. Jun, 2008 | Pubmed ID: 18241200 ATP Depletion Inhibits the Endocytosis of ClC-2 Journal of Cellular Physiology. Jan, 2008 | Pubmed ID: 17620322 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. Jan, 2007 | Pubmed ID: 16989640 Nucleotides Bind to the C-terminus of ClC-5 The Biochemical Journal. Sep, 2006 | Pubmed ID: 16686597 Evaluation of the Membrane-spanning Domain of ClC-2 The Biochemical Journal. Jun, 2006 | Pubmed ID: 16526942 Role of Intramolecular and Intermolecular Interactions in ClC Channel and Transporter Function Pflügers Archiv : European Journal of Physiology. Mar, 2006 | Pubmed ID: 16167151 ATPase Assay of Purified, Reconstituted CFTR Protein Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. Aug, 2004 | Pubmed ID: 15463945 Determination of CFTR Chloride Channel Activity and Pharmacology Using Radiotracer Flux Methods Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. Aug, 2004 | Pubmed ID: 15463942 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. Aug, 2004 | Pubmed ID: 15463939 Methods to Study CFTR Protein in Vitro Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. Aug, 2004 | Pubmed ID: 15463933 A Heteromeric Complex of the Two Nucleotide Binding Domains of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mediates ATPase Activity The Journal of Biological Chemistry. Oct, 2004 | Pubmed ID: 15284228 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. 2004 | Pubmed ID: 15094300 Phosphorylation-induced Conformational Changes of Cystic Fibrosis Transmembrane Conductance Regulator Monitored by Attenuated Total Reflection-Fourier Transform IR Spectroscopy and Fluorescence Spectroscopy The Journal of Biological Chemistry. Feb, 2004 | Pubmed ID: 14660584 Stable Dimeric Assembly of the Second Membrane-spanning Domain of CFTR (cystic Fibrosis Transmembrane Conductance Regulator) Reconstitutes a Chloride-selective Pore The Biochemical Journal. Nov, 2003 | Pubmed ID: 12892562 Dimeric Cystic Fibrosis Transmembrane Conductance Regulator Exists in the Plasma Membrane The Biochemical Journal. Sep, 2003 | Pubmed ID: 12820897 The Chloride Channel ClC-4 Contributes to Endosomal Acidification and Trafficking The Journal of Biological Chemistry. Aug, 2003 | Pubmed ID: 12746443 CFTR Directly Mediates Nucleotide-regulated Glutathione Flux The EMBO Journal. May, 2003 | Pubmed ID: 12727866 Evidence for a Functional Interaction Between the ClC-2 Chloride Channel and the Retrograde Motor Dynein Complex The Journal of Biological Chemistry. May, 2003 | Pubmed ID: 12601004 Epithelial Cell Chloride Channel Activity Correlates with Improved Airway Function in Cystic Fibrosis Patients with the Major Mutant: Delta F508 Pediatric Research. Nov, 2002 | Pubmed ID: 12409505 Studies of the Molecular Basis for Cystic Fibrosis Using Purified Reconstituted CFTR Protein Methods in Molecular Medicine. 2002 | Pubmed ID: 11917519 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. Jan, 2002 | Pubmed ID: 11675385 One-Step Formation of Protein-Based Tubular Structures for Functional Devices and Tissues Advanced Healthcare Materials. | Pubmed ID: 33694327 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 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 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 Allele-Specific Prevention of Nonsense-Mediated Decay in Cystic Fibrosis Using Homology-Independent Genome Editing Molecular Therapy. Methods & Clinical Development. | Pubmed ID: 32490033 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 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 Activity of Lumacaftor is Not Conserved in Zebrafish Cftr Bearing the Major Cystic Fibrosis-causing Mutation FASEB BioAdvances. | Pubmed ID: 32123813 Anti-Infectives Restore ORKAMBI Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of Biomolecules. | Pubmed ID: 32092967 A Therapy for Most with Cystic Fibrosis Cell. | Pubmed ID: 31978337 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 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 to the Cystic Fibrosis Transmembrane Conductance Regulator Channel Molecular Pharmacology. | Pubmed ID: 31427400 Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Cells. | Pubmed ID: 31370288 Conversion of Human and Mouse Fibroblasts into Lung-like Epithelial Cells Scientific Reports. | Pubmed ID: 31227724 Augmentation of Cystic Fibrosis Transmembrane Conductance Regulator Function in Human Bronchial Epithelial Cells Via SLC6A14-Dependent Amino Acid Uptake. Implications for Treatment of Cystic Fibrosis American Journal of Respiratory Cell and Molecular Biology. | Pubmed ID: 31189070 Molecular Mechanism of Action of Trimethylangelicin Derivatives As CFTR Modulators Frontiers in Pharmacology. | Pubmed ID: 30022950 SLC6A14, an Amino Acid Transporter, Modifies the Primary CF Defect in Fluid Secretion ELife. | Pubmed ID: 30004386 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 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 Correctors of the Major Cystic Fibrosis Mutant Interact Through Membrane-Spanning Domains Molecular Pharmacology. | Pubmed ID: 29618585 Comprehensive Mapping of Cystic Fibrosis Mutations to CFTR Protein Identifies Mutation Clusters and Molecular Docking Predicts Corrector Binding Site Proteins. | Pubmed ID: 29569753 Transducing Airway Basal Cells with a Helper-Dependent Adenoviral Vector for Lung Gene Therapy Human Gene Therapy. | Pubmed ID: 29320887 Structural Effects of Extracellular Loop Mutations in CFTR Helical Hairpins Biochimica Et Biophysica Acta. Biomembranes. | Pubmed ID: 29307731 Orkambi® and Amplifier Co-therapy Improves Function from a Rare Mutation in Gene-edited Cells and Patient Tissue EMBO Molecular Medicine. | Pubmed ID: 28667089 Phenotypic Profiling of CFTR Modulators in Patient-derived Respiratory Epithelia NPJ Genomic Medicine. | Pubmed ID: 28649446 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 Cystic Fibrosis Gene Modifier SLC26A9 Modulates Airway Response to CFTR-directed Therapeutics Human Molecular Genetics. | Pubmed ID: 28171547 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 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 The Investigational Cystic Fibrosis Drug Trimethylangelicin Directly Modulates CFTR by Stabilizing the First Membrane-spanning Domain Biochemical Pharmacology. | Pubmed ID: 27614011 Testing Gene Therapy Vectors in Human Primary Nasal Epithelial Cultures Molecular Therapy. Methods & Clinical Development. | Pubmed ID: 26730394 Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop The Journal of Biological Chemistry. | Pubmed ID: 26627831 Facilitating Structure-Function Studies of CFTR Modulator Sites with Efficiencies in Mutagenesis and Functional Screening Journal of Biomolecular Screening. | Pubmed ID: 26385858 Directed Differentiation of Cholangiocytes from Human Pluripotent Stem Cells Nature Biotechnology. | Pubmed ID: 26167630 Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity PloS One. | Pubmed ID: 26079370 Un ensayo basado en fluorescencia del potencial de membrana para el estudio funcional de alto rendimiento de dos canales iónicos endógenos en dos líneas celulares epiteliales Sunny Xia1,3, Michelle Di Paola3, Nicola L. Jones2,4,5, Christine E. Bear1,3,5 1Molecular Medicine, Hospital for Sick Children, 2Cell Biology, Hospital for Sick Children, 3Department of Physiology, University of Toronto, 4Department of Paediatrics, University of Toronto, 5Department of Biochemistry, University of Toronto JoVE 63528 Biology Reconstitución funcional y la actividad del canal Las mediciones de Purificada Wildtype y mutante CFTR Proteína Paul D. W. Eckford1, Canhui Li1, Christine E. Bear1,2,3 1Programme in Molecular Structure and Function, Hospital for Sick Children, 2Department of Biochemistry, University of Toronto, 3Department of Physiology, University of Toronto JoVE 52427 Biology
Un ensayo basado en fluorescencia del potencial de membrana para el estudio funcional de alto rendimiento de dos canales iónicos endógenos en dos líneas celulares epiteliales Sunny Xia1,3, Michelle Di Paola3, Nicola L. Jones2,4,5, Christine E. Bear1,3,5 1Molecular Medicine, Hospital for Sick Children, 2Cell Biology, Hospital for Sick Children, 3Department of Physiology, University of Toronto, 4Department of Paediatrics, University of Toronto, 5Department of Biochemistry, University of Toronto JoVE 63528 Biology
Reconstitución funcional y la actividad del canal Las mediciones de Purificada Wildtype y mutante CFTR Proteína Paul D. W. Eckford1, Canhui Li1, Christine E. Bear1,2,3 1Programme in Molecular Structure and Function, Hospital for Sick Children, 2Department of Biochemistry, University of Toronto, 3Department of Physiology, University of Toronto JoVE 52427 Biology
