Christine E. Bear

A Bioengineered Three-Dimensional Cell Culture Platform Integrated with Microfluidics To Address Antimicrobial Resistance in Tuberculosis.

Dimeric cystic fibrosis transmembrane conductance regulator exists in the plasma membrane.

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.

Studies of the molecular basis for cystic fibrosis using purified reconstituted CFTR protein.

Epithelial cell chloride channel activity correlates with improved airway function in cystic fibrosis patients with the major mutant: Delta F508.

Evidence for a functional interaction between the ClC-2 chloride channel and the retrograde motor dynein complex.

CFTR directly mediates nucleotide-regulated glutathione flux.

The chloride channel ClC-4 contributes to endosomal acidification and trafficking.

Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore.

Phosphorylation-induced conformational changes of cystic fibrosis transmembrane conductance regulator monitored by attenuated total reflection-Fourier transform IR spectroscopy and fluorescence spectroscopy.

Molecular basis for the chloride channel activity of cystic fibrosis transmembrane conductance regulator and the consequences of disease-causing mutations.

A heteromeric complex of the two nucleotide binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) mediates ATPase activity.

Methods to study CFTR protein in vitro.

The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel.

Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods.

ATPase assay of purified, reconstituted CFTR protein.

Role of intramolecular and intermolecular interactions in ClC channel and transporter function.

Evaluation of the membrane-spanning domain of ClC-2.

Nucleotides bind to the C-terminus of ClC-5.

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.

ATP depletion inhibits the endocytosis of ClC-2.

The intact CFTR protein mediates ATPase rather than adenylate kinase activity.

Probing structure-function relationships and gating mechanisms in the CorA Mg2+ transport system.

Evidence for a superoxide permeability pathway in endosomal membranes.

Molecular basis for the ATPase activity of CFTR.

Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease.

A novel method for monitoring the cytosolic delivery of peptide cargo.

A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability.

An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice.

Functional rescue of DeltaF508-CFTR by peptides designed to mimic sorting motifs.

ClC transporters: discoveries and challenges in defining the mechanisms underlying function and regulation of ClC-5.

A chemical corrector modifies the channel function of F508del-CFTR.

Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise.

ATP induces conformational changes in the carboxyl-terminal region of ClC-5.

Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant.

Targeting the regulation of CFTR channels.

Structural basis for alginate secretion across the bacterial outer membrane.

Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy.

Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator.

Identification and validation of hits from high throughput screens for CFTR modulators.

Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein.

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.

Functional Rescue of F508del-CFTR Using Small Molecule Correctors.

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.

Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5.

Proton-dependent gating and proton uptake by Wzx support O-antigen-subunit antiport across the bacterial inner membrane.

Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention.

VX-809 and related corrector compounds exhibit secondary activity stabilizing active F508del-CFTR after its partial rescue to the cell surface.

Lung arginase expression and activity is increased in cystic fibrosis mouse models.

The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor.

The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation.

Efficient generation of functional CFTR-expressing airway epithelial cells from human pluripotent stem cells.

Acellular Lung Scaffolds Direct Differentiation of Endoderm to Functional Airway Epithelial Cells: Requirement of Matrix-Bound HS Proteoglycans.

Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity.

Directed differentiation of cholangiocytes from human pluripotent stem cells.

Facilitating Structure-Function Studies of CFTR Modulator Sites with Efficiencies in Mutagenesis and Functional Screening.

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.

Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.

Testing gene therapy vectors in human primary nasal epithelial cultures.

The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain.

Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants.

Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface.

Cystic fibrosis gene modifier SLC26A9 modulates airway response to CFTR-directed therapeutics.

Synergy of cAMP and calcium signaling pathways in CFTR regulation.

Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.

Orkambi® and amplifier co-therapy improves function from a rare mutation in gene-edited cells and patient tissue.

Editorial overview: Respiratory: Transformational therapies for cystic fibrosis.

Is a Genetic Modifier of Cystic Fibrosis That Regulates Attachment to Human Bronchial Epithelial Cells.

Structural effects of extracellular loop mutations in CFTR helical hairpins.

Transducing Airway Basal Cells with a Helper-Dependent Adenoviral Vector for Lung Gene Therapy.

Comprehensive mapping of cystic fibrosis mutations to CFTR protein identifies mutation clusters and molecular docking predicts corrector binding site.

Correctors of the Major Cystic Fibrosis Mutant Interact through Membrane-Spanning Domains.

The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF.

Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.

SLC6A14, an amino acid transporter, modifies the primary CF defect in fluid secretion.

Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators.

Activity of a novel antimicrobial peptide against Pseudomonas aeruginosa biofilms.

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.

Conversion of human and mouse fibroblasts into lung-like epithelial cells.

Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).

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.

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.

A Therapy for Most with Cystic Fibrosis.

Anti-Infectives Restore ORKAMBI Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of .

Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis-causing mutation.

The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor.

A helper-dependent adenoviral vector rescues CFTR to wild-type functional levels in cystic fibrosis epithelial cells harbouring class I mutations.

Allele-Specific Prevention of Nonsense-Mediated Decay in Cystic Fibrosis Using Homology-Independent Genome Editing.

Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants.

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.

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.

One-Step Formation of Protein-Based Tubular Structures for Functional Devices and Tissues.

Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTA.

Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator.

Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis.

A new platform for high-throughput therapy testing on iPSC-derived lung progenitor cells from cystic fibrosis patients.

Perspectives on the translation of in-vitro studies to precision medicine in Cystic Fibrosis.

Generation of functional ciliated cholangiocytes from human pluripotent stem cells.

High-Throughput Functional Analysis of CFTR and Other Apically Localized Proteins in iPSC-Derived Human Intestinal Organoids.

Antisense oligonucleotide splicing modulation as a novel Cystic Fibrosis therapeutic approach for the W1282X nonsense mutation.

Stage-Specific Generation of Human Pluripotent Stem Cell Derived Lung Models to Measure CFTR Function.

CFTR interactome mapping using the mammalian membrane two-hybrid high-throughput screening system.

A protocol for identifying the binding sites of small molecules on the cystic fibrosis transmembrane conductance regulator (CFTR) protein.