Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell

Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell

The F508 deletion (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) is the most common CF causing mutation. Here the authors show that cytosolic chaperones shift the F508del channel conformation to the native fold by kinetic and thermodynamic remodelling of the gating energ...

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Autores principales: Miklos Bagdany, Guido Veit, Ryosuke Fukuda, Radu G. Avramescu, Tsukasa Okiyoneda, Imad Baaklini, Jay Singh, Guy Sovak, Haijin Xu, Pirjo M. Apaja, Sara Sattin, Lenore K. Beitel, Ariel Roldan, Giorgio Colombo, William Balch, Jason C. Young, Gergely L. Lukacs
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2017
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Acceso en línea:https://doaj.org/article/e14a35b0276c400f88eedbb10dd40d9d
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spelling oai:doaj.org-article:e14a35b0276c400f88eedbb10dd40d9d2021-12-02T14:40:49ZChaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell10.1038/s41467-017-00444-42041-1723https://doaj.org/article/e14a35b0276c400f88eedbb10dd40d9d2017-08-01T00:00:00Zhttps://doi.org/10.1038/s41467-017-00444-4https://doaj.org/toc/2041-1723The F508 deletion (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) is the most common CF causing mutation. Here the authors show that cytosolic chaperones shift the F508del channel conformation to the native fold by kinetic and thermodynamic remodelling of the gating energetics towards that of wild-type CTFR.Miklos BagdanyGuido VeitRyosuke FukudaRadu G. AvramescuTsukasa OkiyonedaImad BaakliniJay SinghGuy SovakHaijin XuPirjo M. ApajaSara SattinLenore K. BeitelAriel RoldanGiorgio ColomboWilliam BalchJason C. YoungGergely L. LukacsNature PortfolioarticleScienceQENNature Communications, Vol 8, Iss 1, Pp 1-15 (2017)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
Miklos Bagdany
Guido Veit
Ryosuke Fukuda
Radu G. Avramescu
Tsukasa Okiyoneda
Imad Baaklini
Jay Singh
Guy Sovak
Haijin Xu
Pirjo M. Apaja
Sara Sattin
Lenore K. Beitel
Ariel Roldan
Giorgio Colombo
William Balch
Jason C. Young
Gergely L. Lukacs
Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell
description The F508 deletion (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) is the most common CF causing mutation. Here the authors show that cytosolic chaperones shift the F508del channel conformation to the native fold by kinetic and thermodynamic remodelling of the gating energetics towards that of wild-type CTFR.
format article
author Miklos Bagdany
Guido Veit
Ryosuke Fukuda
Radu G. Avramescu
Tsukasa Okiyoneda
Imad Baaklini
Jay Singh
Guy Sovak
Haijin Xu
Pirjo M. Apaja
Sara Sattin
Lenore K. Beitel
Ariel Roldan
Giorgio Colombo
William Balch
Jason C. Young
Gergely L. Lukacs
author_facet Miklos Bagdany
Guido Veit
Ryosuke Fukuda
Radu G. Avramescu
Tsukasa Okiyoneda
Imad Baaklini
Jay Singh
Guy Sovak
Haijin Xu
Pirjo M. Apaja
Sara Sattin
Lenore K. Beitel
Ariel Roldan
Giorgio Colombo
William Balch
Jason C. Young
Gergely L. Lukacs
author_sort Miklos Bagdany
title Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell
title_short Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell
title_full Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell
title_fullStr Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell
title_full_unstemmed Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell
title_sort chaperones rescue the energetic landscape of mutant cftr at single molecule and in cell
publisher Nature Portfolio
publishDate 2017
url https://doaj.org/article/e14a35b0276c400f88eedbb10dd40d9d
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