520 results on '"Bear, Christine E."'
Search Results
2. Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells
3. Clinical and functional efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis carrying the N1303K mutation
4. Antisense oligonucleotide splicing modulation as a novel Cystic Fibrosis therapeutic approach for the W1282X nonsense mutation
5. A new platform for high-throughput therapy testing on iPSC-derived lung progenitor cells from cystic fibrosis patients
6. Perspectives on the translation of in-vitro studies to precision medicine in Cystic Fibrosis
7. Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis
8. Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator
9. Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants
10. Collagen Tubular Airway‐on‐Chip for Extended Epithelial Culture and Investigation of Ventilation Dynamics.
11. 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
12. Allele-Specific Prevention of Nonsense-Mediated Decay in Cystic Fibrosis Using Homology-Independent Genome Editing
13. Benchmarking organ-specific responses to therapies in tissues differentiated from Cystic Fibrosis patient derived iPSCs
14. Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells
15. CFTR interactome mapping using the mammalian membrane two‐hybrid high‐throughput screening system
16. Generation of functional ciliated cholangiocytes from human pluripotent stem cells
17. The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF
18. Collagen Tubular Airway-on-Chip for Extended Epithelial Culture and Investigation of Ventilation Dynamics
19. Synthesis and characterization of a photoaffinity labelling probe based on the structure of the cystic fibrosis drug ivacaftor
20. Structural effects of extracellular loop mutations in CFTR helical hairpins
21. Synergy of cAMP and calcium signaling pathways in CFTR regulation
22. Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface
23. A Cell-Based Optimised Approach for Rapid and Efficient Gene Editing of Human Pluripotent Stem Cells
24. Correlation of Electrophysiological and Fluorescence-Based Measurements of Modulator Efficacy in Nasal Epithelial Cultures Derived from People with Cystic Fibrosis
25. Validating organoid-derived human intestinal monolayers for personalized therapy in cystic fibrosis
26. Validating organoid-derived human intestinal monolayers for personalized therapy in cystic fibrosis
27. The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain
28. Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop
29. Conversion of human and mouse fibroblasts into lung-like epithelial cells
30. Facilitating Structure-Function Studies of CFTR Modulator Sites with Efficiencies in Mutagenesis and Functional Screening
31. Acellular Lung Scaffolds Direct Differentiation of Endoderm to Functional Airway Epithelial Cells: Requirement of Matrix-Bound HS Proteoglycans
32. Testing gene therapy vectors in human primary nasal epithelial cultures
33. Orkambi® and amplifier co‐therapy improves function from a rare CFTR mutation in gene‐edited cells and patient tissue
34. Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants
35. Viscoelastic Notch Signaling Hydrogel Induces Liver Bile Duct Organoid Growth and Morphogenesis (Adv. Healthcare Mater. 23/2022)
36. Genetic, cell biological, and clinical interrogation of theCFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention
37. VX-809 and Related Corrector Compounds Exhibit Secondary Activity Stabilizing Active F508del-CFTR after Its Partial Rescue to the Cell Surface
38. Activity of a novel antimicrobial peptide against Pseudomonas aeruginosa biofilms
39. 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
40. The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor
41. A Fluorescence-based Assay of Membrane Potential for High-throughput Functional Study of Two Endogenous Ion Channels in Two Epithelial Cell Lines
42. Viscoelastic Notch Signaling Hydrogel Induces Liver Bile Duct Organoid Growth and Morphogenesis
43. A protocol for identifying the binding sites of small molecules on the cystic fibrosis transmembrane conductance regulator (CFTR) protein
44. Structural basis for alginate secretion across the bacterial outer membrane
45. Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia
46. Probing Conformational Rescue Induced by a Chemical Corrector of F508del-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutant
47. ATP Induces Conformational Changes in the Carboxyl-terminal Region of ClC-5
48. Stage‐Specific Generation of Human Pluripotent Stem Cell Derived Lung Models to Measure CFTR Function
49. Regulation of Ion Conductance in Human Skin Fibroblasts
50. High-Throughput Functional Analysis of CFTR and Other Apically Localized Proteins in iPSC-Derived Human Intestinal Organoids
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