Csanády L, Vergani P, Gadsby DC (2019) Structure, gating, and regulation of the CFTR anion channel. Physiol Rev 99:707–738. https://doi.org/10.1152/physrev.00007.2018

Article  CAS  PubMed  Google Scholar 

Farkas B, Tordai H, Padányi R, Tordai A, Gera J, Paragi G, Hegedüs T (2020) Discovering the chloride pathway in the CFTR channel. Cell Mol Life Sci 77:765–778. https://doi.org/10.1007/s00018-019-03211-4

Article  CAS  PubMed  Google Scholar 

Fatehi M, Linsdell P (2009) Novel residues lining the CFTR chloride channel pore identified by functional modification of introduced cysteines. J Membr Biol 228:151–164. https://doi.org/10.1007/s00232-009-9167-3

Article  CAS  PubMed  Google Scholar 

Gao X, Bai Y, Hwang T-C (2013) Cysteine scanning of CFTR’s first transmembrane segment reveals its plausible roles in gating and permeation. Biophys J 104:786–797. https://doi.org/10.1016/j.bpj.2012.12.048

Article  CAS  PubMed  PubMed Central  Google Scholar 

Ge N, Muise CN, Gong X, Linsdell P (2004) Direct comparison of the functional roles played by different membrane spanning regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Biol Chem 279:55283–55289. https://doi.org/10.1074/jbc.M411935200

Article  CAS  PubMed  Google Scholar 

Gong X, Linsdell P (2004) Maximization of the rate of chloride conduction in the CFTR channel pore by ion-ion interactions. Arch Biochem Biophys 426:78–82. https://doi.org/10.1016/j.abb.2004.03.033

Article  CAS  PubMed  Google Scholar 

Gong X, Burbridge SM, Cowley EA, Linsdell P (2002) Molecular determinants of au(CN)2– binding and permeability within the cystic fibrosis transmembrane conductance regulator Cl– channel pore. J Physiol 540:39–47. https://doi.org/10.1113/jphysiol.2001.013235

Article  CAS  PubMed  PubMed Central  Google Scholar 

Hoffmann B, Elbahnsi A, Lehn P, Décout J-L, Pietrucci F, Mornon J-P, Callebaut I (2018) Combining theoretical and experimental data to decipher CFTR 3D structures and functions. Cell Mol Life Sci 75:3829–3855. https://doi.org/10.1007/s00018-018-2835-7

Article  CAS  PubMed  Google Scholar 

Hwang T-C, Yeh J-T, Zhang J, Yu Y-C, Yeh H-I, Destefano S (2018) Structural mechanisms of CFTR function and dysfunction. J Gen Physiol 150:539–570. https://doi.org/10.1085/jgp.201711946

Article  CAS  PubMed  PubMed Central  Google Scholar 

Linsdell P (2001) Relationship between anion binding and anion permeability revealed by mutagenesis within the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Physiol 531:51–66. https://doi.org/10.1111/j.1469-7793.2001.0051j.x

Article  CAS  PubMed  PubMed Central  Google Scholar 

Linsdell P (2015) Interactions between permeant and blocking anions inside the CFTR chloride channel pore. Biochim Biophys Acta 1848:1573–1590. https://doi.org/10.1016/j.bbamem.2015.04.004

Article  CAS  PubMed  Google Scholar 

Linsdell P (2017) Architecture and functional properties of the CFTR channel pore. Cell Mol Life Sci 74:67–83. https://doi.org/10.1007/s00018-016-2389-5

Article  CAS  PubMed  Google Scholar 

Linsdell P (2018) Cystic fibrosis transmembrane conductance regulator (CFTR): making an ion channel out of an active transporter structure. Channels 12:284–290. https://doi.org/10.1080/19336950.2018.1502585

Article  PubMed  PubMed Central  Google Scholar 

Linsdell P, Hanrahan JW (1998) Adenosine triphosphate-dependent asymmetry of anion permeation in the cystic fibrosis transmembrane conductance regulator chloride channel. J Gen Physiol 111:601–614. https://doi.org/10.1085/jgp.111.4.601

Article  CAS  PubMed  PubMed Central  Google Scholar 

Linsdell P, Zheng S-X, Hanrahan JW (1998) Non-pore lining amino acid side chains influence anion selectivity of the human CFTR Cl– channel expressed in mammalian cell lines. J Physiol 512:1–16. https://doi.org/10.1111/j.1469-7793.1998.001bf.x

Article  CAS  PubMed  PubMed Central  Google Scholar 

Linsdell P, Evagelidis A, Hanrahan JW (2000) Molecular determinants of anion selectivity in the cystic fibrosis transmembrane conductance regulator chloride channel pore. Biophys J 78:2973–2982. https://doi.org/10.1016/S0006-3495(00)76836-6

Article  CAS  PubMed  PubMed Central  Google Scholar 

Linsdell P, Irving CL, Cowley EA, El Hiani Y (2021) Two positively charged amino acid side-chains in the inner vestibule of the CFTR channel pore play analogous roles in controlling anion binding and anion conductance. Cell Mol Life Sci 78:5213–5223. https://doi.org/10.1007/s00018-021-03859-x

Article  CAS  PubMed  Google Scholar 

McDonough S, Davidson N, Lester HA, McCarty NA (1994) Novel pore-lining residues in CFTR than govern permeation and open-channel block. Neuron 13:623–634. https://doi.org/10.1016/0896-6273(94)90030-2

Article  CAS  PubMed  Google Scholar 

Negoda A, El Hiani Y, Cowley EA, Linsdell P (2017) Contribution of a leucine residue in the first transmembrane segment to the selectivity filter region in the CFTR chloride channel. Biochim Biophys Acta 1859:1049–1058. https://doi.org/10.1016/j.bbamem.2017.02.014

Article  CAS  Google Scholar 

Negoda A, Hogan MS, Cowley EA, Linsdell P (2019) Contribution of the eighth transmembrane segment to the function of the CFTR chloride channel pore. Cell Mol Life Sci 76:2411–2423. https://doi.org/10.1007/s00018-019-03043-2

Article  CAS  PubMed  Google Scholar 

Richards FM (1974) The interpretation of protein structures: total volume, group volume distributions and packing density. J Mol Biol 82:1–14. https://doi.org/10.1016/0022-2836(74)90570-1

Article  CAS  PubMed  Google Scholar 

Sheppard DN, Travis SM, Ishihara H, Welsh MJ (1996) Contribution of proline residues in the membrane-spanning domains of cystic fibrosis transmembrane conductance regulator to chloride channel function. J Biol Chem 271:14995–15001. https://doi.org/10.1074/jbc.271.25.14995

Article  CAS  PubMed  Google Scholar 

Shteinberg M, Haq IJ, Polineni D, Davies JC (2021) Cystic fibrosis. Lancet 397:2195–2211. https://doi.org/10.1016/S0140-6736(20)32542-3

Article  CAS  PubMed  Google Scholar 

Smith SS, Steinle ED, Meyerhoff ME, Dawson DC (1999) Cystic fibrosis transmembrane conductance regulator. Physical basis for lyotropic anion selectivity patterns. J Gen Physiol 114:799–818. https://doi.org/10.1085/jgp.114.6.799

Article  CAS  PubMed  PubMed Central  Google Scholar 

Wei S, Roessler BC, Icyuz M, Chauvet S, Tao B, Hartman JL, Kirk KL (2016) Long-range coupling between the extracellular gates and the intracellular ATP binding domains of multidrug resistance protein pumps and cystic fibrosis transmembrane conductance regulator channels. FASEB J 30:1247–1262. https://doi.org/10.1096/fj.15-278382

Article  CAS  PubMed  Google Scholar 

Zeng ZW, Linsdell P, Pomès R (2023) Molecular dynamics study of Cl– permeation through cystic fibrosis transmembrane conductance regulator (CFTR). Cell Mol Life Sci 80:51. https://doi.org/10.1007/s00018-022-04621-7

Article  CAS  PubMed  PubMed Central  Google Scholar 

Zhang Z, Liu F, Chen J (2018) Molecular structure of the ATP-bound, phosphorylated human CFTR. Proc Natl Acad Sci USA 115:12757–12762. https://doi.org/10.1073/pnas.1815287115

Article  CAS  PubMed  PubMed Central  Google Scholar 

Zhou J-J, Fatehi M, Linsdell P (2007) Direct and indirect effects of mutations at the outer mouth of the CFTR chloride channel pore. J Membr Biol 216:129–142. https://doi.org/10.1007/s00232-007-9056-6

Article  CAS  PubMed  Google Scholar 

Zhou J-J, Li M-S, Qi J, Linsdell P (2010) Regulation of conductance by the number of fixed positive charges in the intracellular vestibule of the CFTR chloride channel pore. J Gen Physiol 135:229–245. https://doi.org/10.1085/jgp.200910327

Article  CAS  PubMed  PubMed Central  Google Scholar