Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
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
_version_ 1783418995310854144
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
work_keys_str_mv AT dewildegert identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT geesmaarten identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT muschsara identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT verdonckkatleen identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT jansmia identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT wesseannesophie identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT singhashvanik identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT hwangtzyhchang identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT christophethierry identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT pizzoneromathieu identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT vanderplassteven identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT desroynicolas identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT cowartmarlon identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT stoutenpieter identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT nellesluc identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators
AT conrathkatja identificationofglpgabbv2737anovelclassofcorrectorwhichexertsfunctionalsynergywithothercftrmodulators