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Small-molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough

The Journal of clinical investigation, 2022-09, Vol.132 (18)

2022

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Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies

Frontiers in pharmacology, 2021-03, Vol.12, p.628722-628722

2021

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Established and novel human translational models to advance cystic fibrosis research, drug discovery, and optimize CFTR-targeting therapeutics

Current opinion in pharmacology, 2022-06, Vol.64, p.102210-102210, Article 102210

2022

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Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures

American journal of physiology. Lung cellular and molecular physiology, 2010-03, Vol.298 (3), p.L304-L314

2010

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The Legionella IcmS–IcmW protein complex is important for Dot/Icm‐mediated protein translocation

Molecular microbiology, 2005-02, Vol.55 (3), p.912-926

2005

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Recent progress in translational cystic fibrosis research using precision medicine strategies

Journal of cystic fibrosis, 2018-03, Vol.17 (2), p.S52-S60

2018

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A Novel Co-Culture Model Reveals Enhanced CFTR Rescue in Primary Cystic Fibrosis Airway Epithelial Cultures with Persistent Pseudomonas aeruginosa Infection

Cells (Basel, Switzerland), 2023-11, Vol.12 (22), p.2618

2023

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Cystic fibrosis airway inflammation enables elexacaftor/tezacaftor/ivacaftor-mediated rescue of N1303K CFTR mutation

ERJ open research, 2024-01, Vol.10 (1), p.746

2024

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Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis

Science translational medicine, 2014-07, Vol.6 (246), p.246ra96

2014

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DNAJB12 and Hsp70 triage arrested intermediates of N1303K-CFTR for endoplasmic reticulum-associated autophagy

Molecular biology of the cell, 2021-04, Vol.32 (7), p.538-553

2021

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Passive Immunization with a Polyclonal Antiserum to the Hemoglobin Receptor of Haemophilus ducreyi Confers Protection against a Homologous Challenge in the Experimental Swine Model of Chancroid

Infection and Immunity, 2011-08, Vol.79 (8), p.3168-3177

2011

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A small-molecule inhibitor and degrader of the RNF5 ubiquitin ligase

Molecular biology of the cell, 2022-11, Vol.33 (13), p.ar120-ar120

2022

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Immunization with the Haemophilus ducreyi Hemoglobin Receptor HgbA with Adjuvant Monophosphoryl Lipid A Protects Swine from a Homologous but Not a Heterologous Challenge

Infection and Immunity, 2010-09, Vol.78 (9), p.3763-3772

2010

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Personalised medicine for non-classic cystic fibrosis resulting from rare CFTR mutations

The European respiratory journal, 2020-07, Vol.56 (1), p.2000062

2020

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The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model

The European respiratory journal, 2018-12, Vol.52 (6), p.1801133

2018

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Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770

American journal of physiology. Lung cellular and molecular physiology, 2016-09, Vol.311 (3), p.L550-L559

2016

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Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment

Journal of cystic fibrosis, 2020-09, Vol.19 (5), p.746-751

2020

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Serial Isolates of Persistent Haemophilus influenzae in Patients with Chronic Obstructive Pulmonary Disease Express Diminishing Quantities of the HMW1 and HMW2 Adhesins

Infection and Immunity, 2008-10, Vol.76 (10), p.4463-4468

2008

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Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity

Scientific reports, 2019-07, Vol.9 (1), p.10310-13, Article 10310

2019

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Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation

Expert review of precision medicine and drug development, 2016-01, Vol.1 (3), p.235-243

2016

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Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia

JCI insight, 2018-02, Vol.3 (3)

2018

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Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia

JCI insight, 2019-04, Vol.4 (7)

2019

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Small-molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough

The Journal of clinical investigation, 2022-09, Vol.132 (18), p.1-15

2022

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A Novel Co-Culture Model Reveals Enhanced CFTR Rescue in Primary Cystic Fibrosis Airway Epithelial Cultures with Persistent IPseudomonas aeruginosa/I Infection

Cells (Basel, Switzerland), 2023-11, Vol.12 (22)

2023

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Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures

American journal of physiology. Lung cellular and molecular physiology, 2010, Vol.42 (3)

2010

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Small-molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough

Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies

Established and novel human translational models to advance cystic fibrosis research, drug discovery, and optimize CFTR-targeting therapeutics

Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures

The Legionella IcmS–IcmW protein complex is important for Dot/Icm‐mediated protein translocation

Recent progress in translational cystic fibrosis research using precision medicine strategies

A Novel Co-Culture Model Reveals Enhanced CFTR Rescue in Primary Cystic Fibrosis Airway Epithelial Cultures with Persistent Pseudomonas aeruginosa Infection

Cystic fibrosis airway inflammation enables elexacaftor/tezacaftor/ivacaftor-mediated rescue of N1303K CFTR mutation

Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis

DNAJB12 and Hsp70 triage arrested intermediates of N1303K-CFTR for endoplasmic reticulum-associated autophagy

Passive Immunization with a Polyclonal Antiserum to the Hemoglobin Receptor of Haemophilus ducreyi Confers Protection against a Homologous Challenge in the Experimental Swine Model of Chancroid

A small-molecule inhibitor and degrader of the RNF5 ubiquitin ligase

Immunization with the Haemophilus ducreyi Hemoglobin Receptor HgbA with Adjuvant Monophosphoryl Lipid A Protects Swine from a Homologous but Not a Heterologous Challenge

Personalised medicine for non-classic cystic fibrosis resulting from rare CFTR mutations

The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model

Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770

Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment

Serial Isolates of Persistent Haemophilus influenzae in Patients with Chronic Obstructive Pulmonary Disease Express Diminishing Quantities of the HMW1 and HMW2 Adhesins

Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity

Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation

Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia

Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia

Small-molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough

A Novel Co-Culture Model Reveals Enhanced CFTR Rescue in Primary Cystic Fibrosis Airway Epithelial Cultures with Persistent IPseudomonas aeruginosa/I Infection

Modulation of endocytic trafficking and apical stability of CFTR in primary human airway epithelial cultures

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