Can two wrongs make a right?:F508del-CFTR ion channel rescue by second-site mutations in its transmembrane domains

Autor: Stella Prins, Valentina Corradi, D. Peter Tieleman, David N. Sheppard, Paola Vergani

Publikováno v: Prins, S, Corradi, V, Sheppard, D N, Tieleman, D P & Vergani, P 2022, ' Can two wrongs make a right? F508del-CFTR ion channel rescue by second-site mutations in its transmembrane domains ', Journal of Biological Chemistry, vol. 298, no. 3, 101615, pp. 1-14 . https://doi.org/10.1016/j.jbc.2022.101615

Deletion of phenylalanine 508 (F508del), in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel, is the most common cause of cystic fibrosis (CF). F508 is located on nucleotide-binding domain 1 (NBD1) in contact with cytosoli

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Can two wrongs make a right? F508del-CFTR ion channel rescue by second-site mutations in its transmembrane domains.

Autor: Prins S; Department of Neuroscience, Physiology and Pharmacology, University College London, London, UK., Corradi V; Department of Biological Sciences, Centre for Molecular Simulation, University of Calgary, Calgary, Alberta, Canada., Sheppard DN; School of Physiology, Pharmacology and Neuroscience, University of Bristol, Bristol, UK., Tieleman DP; Department of Biological Sciences, Centre for Molecular Simulation, University of Calgary, Calgary, Alberta, Canada., Vergani P; Department of Neuroscience, Physiology and Pharmacology, University College London, London, UK. Electronic address: p.vergani@ucl.ac.uk.

Publikováno v: The Journal of biological chemistry [J Biol Chem] 2022 Mar; Vol. 298 (3), pp. 101615. Date of Electronic Publication: 2022 Jan 21.