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Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators
The deletion of phenylalanine at position 508 (F508del) in cystic fibrosis transmembrane conductance regulator (CFTR) causes a severe defect in folding and trafficking of the chloride channel resulting in its absence at the plasma membrane of epithelial cells leading to cystic fibrosis. Progress in...
Autores principales: | , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6521598/ https://www.ncbi.nlm.nih.gov/pubmed/31143125 http://dx.doi.org/10.3389/fphar.2019.00514 |
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author | de Wilde, Gert Gees, Maarten Musch, Sara Verdonck, Katleen Jans, Mia Wesse, Anne-Sophie Singh, Ashvani K. Hwang, Tzyh-Chang Christophe, Thierry Pizzonero, Mathieu Van der Plas, Steven Desroy, Nicolas Cowart, Marlon Stouten, Pieter Nelles, Luc Conrath, Katja |
author_facet | de Wilde, Gert Gees, Maarten Musch, Sara Verdonck, Katleen Jans, Mia Wesse, Anne-Sophie Singh, Ashvani K. Hwang, Tzyh-Chang Christophe, Thierry Pizzonero, Mathieu Van der Plas, Steven Desroy, Nicolas Cowart, Marlon Stouten, Pieter Nelles, Luc Conrath, Katja |
author_sort | de Wilde, Gert |
collection | PubMed |
description | The deletion of phenylalanine at position 508 (F508del) in cystic fibrosis transmembrane conductance regulator (CFTR) causes a severe defect in folding and trafficking of the chloride channel resulting in its absence at the plasma membrane of epithelial cells leading to cystic fibrosis. Progress in the understanding of the disease increased over the past decades and led to the awareness that combinations of mechanistically different CFTR modulators are required to obtain meaningful clinical benefit. Today, there remains an unmet need for identification and development of more effective CFTR modulator combinations to improve existing therapies for patients carrying the F508del mutation. Here, we describe the identification of a novel F508del corrector using functional assays. We provide experimental evidence that the clinical candidate GLPG/ABBV-2737 represents a novel class of corrector exerting activity both on its own and in combination with VX809 or GLPG/ABBV-2222. |
format | Online Article Text |
id | pubmed-6521598 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-65215982019-05-29 Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators de Wilde, Gert Gees, Maarten Musch, Sara Verdonck, Katleen Jans, Mia Wesse, Anne-Sophie Singh, Ashvani K. Hwang, Tzyh-Chang Christophe, Thierry Pizzonero, Mathieu Van der Plas, Steven Desroy, Nicolas Cowart, Marlon Stouten, Pieter Nelles, Luc Conrath, Katja Front Pharmacol Pharmacology The deletion of phenylalanine at position 508 (F508del) in cystic fibrosis transmembrane conductance regulator (CFTR) causes a severe defect in folding and trafficking of the chloride channel resulting in its absence at the plasma membrane of epithelial cells leading to cystic fibrosis. Progress in the understanding of the disease increased over the past decades and led to the awareness that combinations of mechanistically different CFTR modulators are required to obtain meaningful clinical benefit. Today, there remains an unmet need for identification and development of more effective CFTR modulator combinations to improve existing therapies for patients carrying the F508del mutation. Here, we describe the identification of a novel F508del corrector using functional assays. We provide experimental evidence that the clinical candidate GLPG/ABBV-2737 represents a novel class of corrector exerting activity both on its own and in combination with VX809 or GLPG/ABBV-2222. Frontiers Media S.A. 2019-05-09 /pmc/articles/PMC6521598/ /pubmed/31143125 http://dx.doi.org/10.3389/fphar.2019.00514 Text en Copyright © 2019 de Wilde, Gees, Musch, Verdonck, Jans, Wesse, Singh, Hwang, Christophe, Pizzonero, Van der Plas, Desroy, Cowart, Stouten, Nelles and Conrath. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Pharmacology de Wilde, Gert Gees, Maarten Musch, Sara Verdonck, Katleen Jans, Mia Wesse, Anne-Sophie Singh, Ashvani K. Hwang, Tzyh-Chang Christophe, Thierry Pizzonero, Mathieu Van der Plas, Steven Desroy, Nicolas Cowart, Marlon Stouten, Pieter Nelles, Luc Conrath, Katja Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators |
title | Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators |
title_full | Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators |
title_fullStr | Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators |
title_full_unstemmed | Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators |
title_short | Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators |
title_sort | identification of glpg/abbv-2737, a novel class of corrector, which exerts functional synergy with other cftr modulators |
topic | Pharmacology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6521598/ https://www.ncbi.nlm.nih.gov/pubmed/31143125 http://dx.doi.org/10.3389/fphar.2019.00514 |
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