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

por Laselva, Onofrio, Ardelean, Maria C., Bear, Christine E.
Publicado 2021

Perspectives on the translation of in-vitro studies to precision medicine in Cystic Fibrosis

por Dumas, Marie-Pier, Xia, Sunny, Bear, Christine E., Ratjen, Felix
Publicado 2021

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

por Laselva, Onofrio, Erwood, Steven, Du, Kai, Ivakine, Zhenya, Bear, Christine E.
Publicado 2019

Anti-Infectives Restore ORKAMBI(®) Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of P. aeruginosa

por Laselva, Onofrio, Stone, Tracy A., Bear, Christine E., Deber, Charles M.
Publicado 2020

Functional Rescue of F508del-CFTR Using Small Molecule Correctors

por Molinski, Steven, Eckford, Paul D. W., Pasyk, Stan, Ahmadi, Saumel, Chin, Stephanie, Bear, Christine E.
Publicado 2012

The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor

por Broadbent, Steven D., Ramjeesingh, Mohabir, Bear, Christine E., Argent, Barry E., Linsdell, Paul, Gray, Michael A.
Publicado 2014

Proton-Dependent Gating and Proton Uptake by Wzx Support O-Antigen-Subunit Antiport Across the Bacterial Inner Membrane

por Islam, Salim T., Eckford, Paul D. W., Jones, Michelle L., Nugent, Timothy, Bear, Christine E., Vogel, Christian, Lam, Joseph S.
Publicado 2013

Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface

por Chin, Stephanie, Yang, Donghe, Miles, Andrew J., Eckford, Paul D. W., Molinski, Steven, Wallace, B. A., Bear, Christine E.
Publicado 2017

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

por Chin, Stephanie, Ramjeesingh, Mohabir, Hung, Maurita, Ereño-Oreba, June, Cui, Hong, Laselva, Onofrio, Julien, Jean-Philippe, Bear, Christine E.
Publicado 2019

A protocol for identifying the binding sites of small molecules on the cystic fibrosis transmembrane conductance regulator (CFTR) protein

por Laselva, Onofrio, Petrotchenko, Evgeniy V., Hamilton, C. Michael, Qureshi, Zafar, Borchers, Christoph H., Young, Robert N., Bear, Christine E.
Publicado 2022

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

por Erwood, Steven, Laselva, Onofrio, Bily, Teija M.I., Brewer, Reid A., Rutherford, Alexandra H., Bear, Christine E., Ivakine, Evgueni A.
Publicado 2020

Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein

por Wong, Amy P., Bear, Christine E., Chin, Stephanie, Pasceri, Peter, Thompson, Tadeo O., Huan, Ling-Jun, Ratjen, Felix, Ellis, James, Rossant, Janet
Publicado 2012

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

por Laselva, Onofrio, Bartlett, Claire, Gunawardena, Tarini N.A., Ouyang, Hong, Eckford, Paul D.W., Moraes, Theo J., Bear, Christine E., Gonska, Tanja
Publicado 2021

SLC6A14, an amino acid transporter, modifies the primary CF defect in fluid secretion

por Ahmadi, Saumel, Xia, Sunny, Wu, Yu-Sheng, Di Paola, Michelle, Kissoon, Randolph, Luk, Catherine, Lin, Fan, Du, Kai, Rommens, Johanna, Bear, Christine E
Publicado 2018

Activity of a novel antimicrobial peptide against Pseudomonas aeruginosa biofilms

por Beaudoin, Trevor, Stone, Tracy A., Glibowicka, Miroslawa, Adams, Christina, Yau, Yvonne, Ahmadi, Saumel, Bear, Christine E., Grasemann, Hartmut, Waters, Valerie, Deber, Charles M.
Publicado 2018

Correlation of Electrophysiological and Fluorescence-Based Measurements of Modulator Efficacy in Nasal Epithelial Cultures Derived from People with Cystic Fibrosis

por Gunawardena, Tarini N. A., Bozóky, Zoltán, Bartlett, Claire, Ouyang, Hong, Eckford, Paul D. W., Moraes, Theo J., Ratjen, Felix, Gonska, Tanja, Bear, Christine E.
Publicado 2023

Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity

por Malik, Firhan A., Meissner, Anja, Semenkov, Illya, Molinski, Steven, Pasyk, Stan, Ahmadi, Saumel, Bui, Hai H., Bear, Christine E., Lidington, Darcy, Bolz, Steffen-Sebastian
Publicado 2015

Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators

por Laselva, Onofrio, Marzaro, Giovanni, Vaccarin, Christian, Lampronti, Ilaria, Tamanini, Anna, Lippi, Giuseppe, Gambari, Roberto, Cabrini, Giulio, Bear, Christine E., Chilin, Adriana, Dechecchi, Maria C.
Publicado 2018

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

por Laselva, Onofrio, Moraes, Theo J., He, Gengming, Bartlett, Claire, Szàrics, Ida, Ouyang, Hong, Gunawardena, Tarini N. A., Strug, Lisa, Bear, Christine E., Gonska, Tanja
Publicado 2020

Synthesis and Properties of Molecular Probes for the Rescue Site on Mutant Cystic Fibrosis Transmembrane Conductance Regulator

por Alkhouri, Bashar, Denning, Robert A., Kim Chiaw, Patrick, Eckford, Paul D. W., Yu, Wilson, Li, Canhui, Bogojeski, Jovanka J., Bear, Christine E., Viirre, Russell D.
Publicado 2011