about
Orkambi® and amplifier co-therapy improves function from a rare CFTR mutation in gene-edited cells and patient tissue.Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells.The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain.Correctors of the major Cystic Fibrosis mutant interact through membrane spanning domains.Comprehensive mapping of cystic fibrosis mutations to CFTR protein identifies mutation clusters and molecular docking predicts corrector binding site.Correctors of mutant CFTR enhance subcortical cAMP-PKA signaling through modulating ezrin phosphorylation and cytoskeleton organization.Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators.Anti-Infectives Restore ORKAMBI® Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of P. aeruginosaThe preclinical discovery and development of the combination of ivacaftor + tezacaftor used to treat cystic fibrosisFunctional 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 actionCholesterol 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
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description
researcher ORCID ID = 0000-0002-0237-4079
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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Onofrio Laselva
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P1153
56242776800
P31
P496
0000-0002-0237-4079