Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell
oleh: 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
| Format: | Article |
|---|---|
| Diterbitkan: | Nature Portfolio 2017-08-01 |
Deskripsi
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.