Pharmacological Inhibition of the Ubiquitin Ligase RNF5 Rescues F508del-CFTR in Cystic Fibrosis Airway Epithelia

Elvira Sondo, Federico Falchi, Emanuela Caci, +10

Cell chemical biology

FibrosisMolecular PhysiologyPathologyPulmonary PharmacologyPulmonary Fibrosis +4

Genetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation

Valeria Tomati, Elvira Sondo, Andrea Armirotti, +13

Scientific Reports

Discovery of a picomolar potency pharmacological corrector of the mutant CFTR chloride channel

Nicoletta Pedemonte, Fabio Bertozzi, Emanuela Caci, +12

Science Advances

The L467F-F508del Complex Allele Hampers Pharmacological Rescue of Mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients: The Value of the Ex Vivo Nasal Epithelial Model to Address Non-Responders to CFTR-Modulating Drugs

Elvira Sondo, Federico Cresta, Cristina Pastorino, +13

International Journal of Molecular Sciences

Relevance of Stereotyped B-Cell Receptors in the Context of the Molecular, Cytogenetic and Clinical Features of Chronic Lymphocytic Leukemia

Francesco Maura, Giovanna Cutrona, Sonia Fabris, +18

PLoS ONE

Chronic Lymphocytic LeukemiaImmunoglobulin Heavy-chain VariableMixed-phenotype Acute LeukemiaImmunologyPathology +16

Synthesis and structure–activity relationship of aminoarylthiazole derivatives as correctors of the chloride transport defect in cystic fibrosis

Emanuela Pesce, Marta Bellotti, Nara Liessi, +11

European Journal of Medicinal Chemistry

Aminoarylthiazole DerivativesMedicinal ChemistryChloride Transport DefectBiochemistryNatural Sciences +6

Synthesis and biological evaluation of novel thiazole- VX-809 hybrid derivatives as F508del correctors by QSAR-based filtering tools

Nara Liessi, Elena Cichero, Emanuela Pesce, +10

European Journal of Medicinal Chemistry

Combinatorial ChemistryMedicinal ChemistryDerivative (Chemistry)Natural SciencesF508del Correctors +9

High-throughput screening identifies FAU protein as a regulator of mutant cystic fibrosis transmembrane conductance regulator channel

Valeria Tomati, Emanuela Pesce, Emanuela Caci, +8

Journal of Biological Chemistry

Distinctive lipid signatures of bronchial epithelial cells associated with cystic fibrosis drugs, including Trikafta

Nara Liessi, Emanuela Pesce, Clarissa Braccia, +5

JCI Insight

Proteinlipid InteractionPathologyCell DeathCell LipidomeCellular Physiology +15

Rescue by elexacaftor-tezacaftor-ivacaftor of the G1244E cystic fibrosis mutation's stability and gating defects are dependent on cell background

Valeria Tomati, Stefano Costa, Valeria Capurro, +13

Journal of Cystic Fibrosis

Gating DefectsPathologyMolecular GeneticsGene ExpressionMedicine +2