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 O Laselva, C Bartlett, TNA Gunawardena, H Ouyang, PDW Eckford, ... European Respiratory Journal 57 (6), 2021 | 122 | 2021 |
Orkambi® and amplifier co‐therapy improves function from a rare CFTR mutation in gene‐edited cells and patient tissue SV Molinski, S Ahmadi, W Ip, H Ouyang, A Villella, JP Miller, PS Lee, ... EMBO molecular medicine 9 (9), 1224-1243, 2017 | 106 | 2017 |
Functional rescue of c. 3846G> A (W1282X) in patient-derived nasal cultures achieved by inhibition of nonsense mediated decay and protein modulators with complementary … O Laselva, PDW Eckford, C Bartlett, H Ouyang, TNA Gunawardena, ... Journal of Cystic Fibrosis 19 (5), 717-727, 2020 | 59 | 2020 |
Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells M Favia, MT Mancini, V Bezzerri, L Guerra, O Laselva, AC Abbattiscianni, ... American Journal of Physiology-Lung Cellular and Molecular Physiology 307 (1 …, 2014 | 59 | 2014 |
Comprehensive mapping of cystic fibrosis mutations to CFTR protein identifies mutation clusters and molecular docking predicts corrector binding site SV Molinski, VM Shahani, AS Subramanian, SS MacKinnon, G Woollard, ... Proteins: Structure, Function, and Bioinformatics 86 (8), 833-843, 2018 | 52 | 2018 |
Correctors of the major cystic fibrosis mutant interact through membrane-spanning domains O Laselva, S Molinski, V Casavola, CE Bear Molecular Pharmacology 93 (6), 612-618, 2018 | 48 | 2018 |
Thiodiglycolic acid is excreted by humans receiving ifosfamide and inhibits mitochondrial function in rats TM Visarius, H Bähler, A Küpfer, T Cerny, BH Lauterburg Drug Metabolism and Disposition 26 (3), 193-196, 1998 | 48 | 1998 |
ORKAMBI-mediated rescue of mucociliary clearance in cystic fibrosis primary respiratory cultures is enhanced by arginine uptake, arginase inhibition, and promotion of nitric … YS Wu, J Jiang, S Ahmadi, A Lew, O Laselva, S Xia, C Bartlett, W Ip, ... Molecular Pharmacology 96 (4), 515-525, 2019 | 46 | 2019 |
Correctors of mutant CFTR enhance subcortical cAMP–PKA signaling through modulating ezrin phosphorylation and cytoskeleton organization AC Abbattiscianni, M Favia, MT Mancini, RA Cardone, L Guerra, ... Journal of Cell Science 129 (6), 1128-1140, 2016 | 43 | 2016 |
Allele-specific prevention of nonsense-mediated decay in cystic fibrosis using homology-independent genome editing S Erwood, O Laselva, TMI Bily, RA Brewer, AH Rutherford, CE Bear, ... Molecular Therapy Methods & Clinical Development 17, 1118-1128, 2020 | 39 | 2020 |
Anti-Infectives Restore ORKAMBI® Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of P. aeruginosa O Laselva, TA Stone, CE Bear, CM Deber Biomolecules 10 (2), 334, 2020 | 39 | 2020 |
Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants O Laselva, C Bartlett, A Popa, H Ouyang, TNA Gunawardena, T Gonska, ... Journal of Cystic Fibrosis 20 (1), 106-119, 2021 | 38 | 2021 |
The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain O Laselva, S Molinski, V Casavola, CE Bear Biochemical Pharmacology 119, 85-92, 2016 | 38 | 2016 |
The era of CFTR modulators: improvements made and remaining challenges S Cuevas-Ocaña, O Laselva, J Avolio, R Nenna Breathe 16 (2), 2020 | 35 | 2020 |
The preclinical discovery and development of the combination of ivacaftor+ tezacaftor used to treat cystic fibrosis L Guerra, M Favia, S Di Gioia, O Laselva, A Bisogno, V Casavola, ... Expert Opinion on Drug Discovery 15 (8), 873-891, 2020 | 34 | 2020 |
Small-molecule drugs for cystic fibrosis: Where are we now? O Laselva, L Guerra, S Castellani, M Favia, S Di Gioia, M Conese Pulmonary Pharmacology & Therapeutics 72, 102098, 2022 | 31 | 2022 |
Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator O Laselva, Z Qureshi, ZW Zeng, EV Petrotchenko, M Ramjeesingh, ... Iscience 24 (6), 2021 | 30 | 2021 |
The CFTR mutation c. 3453G> C (D1152H) confers an anion selectivity defect in primary airway tissue that can be rescued by ivacaftor O Laselva, TJ Moraes, G He, C Bartlett, I Szàrics, H Ouyang, ... Journal of personalized medicine 10 (2), 40, 2020 | 26 | 2020 |
Molecular mechanism of action of trimethylangelicin derivatives as CFTR modulators O Laselva, G Marzaro, C Vaccarin, I Lampronti, A Tamanini, G Lippi, ... Frontiers in Pharmacology 9, 719, 2018 | 26 | 2018 |
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 … O Laselva, J McCormack, C Bartlett, W Ip, TNA Gunawardena, H Ouyang, ... Journal of Personalized Medicine 10 (4), 209, 2020 | 25 | 2020 |