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