Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTA^TM

by Onofrio Laselva, Maria C. Ardelean, Christine E. Bear
Published 2021-04-01

The era of CFTR modulators: improvements made and remaining challenges

by Sara Cuevas-Ocaña, Onofrio Laselva, Julie Avolio, Raffaella Nenna
Published 2020-06-01

Anti-Infectives Restore ORKAMBI^® Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of <i>P. aeruginosa</i>

by Onofrio Laselva, Tracy A. Stone, Christine E. Bear, Charles M. Deber
Published 2020-02-01

Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis‐causing mutation

by Onofrio Laselva, Steven Erwood, Kai Du, Zhenya Ivakine, Christine E. Bear
Published 2019-10-01

Anti-Inflammatory and Anti-Oxidant Effect of Dimethyl Fumarate in Cystic Fibrosis Bronchial Epithelial Cells

by Onofrio Laselva, Caterina Allegretta, Sante Di Gioia, Carlo Avolio, Massimo Conese
Published 2021-08-01

Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

by Stephanie Chin, Mohabir Ramjeesingh, Maurita Hung, June Ereño-Oreba, Hong Cui, Onofrio Laselva, Jean-Philippe Julien, Christine E. Bear
Published 2019-07-01

Allele-Specific Prevention of Nonsense-Mediated Decay in Cystic Fibrosis Using Homology-Independent Genome Editing

by Steven Erwood, Onofrio Laselva, Teija M.I. Bily, Reid A. Brewer, Alexandra H. Rutherford, Christine E. Bear, Evgueni A. Ivakine
Published 2020-06-01

The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor

by Onofrio Laselva, Theo J. Moraes, Gengming He, Claire Bartlett, Ida Szàrics, Hong Ouyang, Tarini N. A. Gunawardena, Lisa Strug, Christine E. Bear, Tanja Gonska
Published 2020-05-01

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 Modulato...

by Onofrio Laselva, Jacqueline McCormack, Claire Bartlett, Wan Ip, Tarini N. A. Gunawardena, Hong Ouyang, Paul D. W. Eckford, Tanja Gonska, Theo J. Moraes, Christine E. Bear
Published 2020-11-01

Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator

by Onofrio Laselva, Zafar Qureshi, Zhi-Wei Zeng, Evgeniy V. Petrotchenko, Mohabir Ramjeesingh, C. Michael Hamilton, Ling-Jun Huan, Christoph H. Borchers, Régis Pomès, Robert Young, Christine E. Bear
Published 2021-06-01

Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators

by Onofrio Laselva, Giovanni Marzaro, Christian Vaccarin, Ilaria Lampronti, Anna Tamanini, Giuseppe Lippi, Roberto Gambari, Giulio Cabrini, Christine E. Bear, Christine E. Bear, Christine E. Bear, Adriana Chilin, Maria C. Dechecchi
Published 2018-07-01