The gating of the CFTR channel

O Moran - Cellular and Molecular Life Sciences, 2017 - Springer
Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel
expressed in the apical membrane of epithelia. Mutations in the CFTR gene are the cause of …

Molecular modelling and molecular dynamics of CFTR

I Callebaut, B Hoffmann, P Lehn, JP Mornon - Cellular and Molecular Life …, 2017 - Springer
The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a member of the
ATP-binding cassette (ABC) transporter superfamily that functions as an ATP-gated channel …

F508del disturbs the dynamics of the nucleotide binding domains of CFTR before and after ATP hydrolysis

B Abreu, EF Lopes, ASF Oliveira… - … Structure, Function, and …, 2020 - Wiley Online Library
The cystic fibrosis transmembrane conductance regulator (CFTR) channel is an ion channel
responsible for chloride transport in epithelia and it belongs to the class of ABC transporters …

Molecular dynamics simulation study on the structural instability of the most common cystic fibrosis-associated mutant ΔF508-CFTR

M Odera, T Furuta, Y Sohma… - Biophysics and …, 2018 - jstage.jst.go.jp
Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel that
belongs to the ATP binding cassette protein superfamily. Deletion of phenylalanine at …

Exploitation of a novel biosensor based on the full-length human F508del-CFTR with computational studies, biochemical and biological assays for the characterization …

P D'Ursi, M Uggeri, C Urbinati, E Millo, G Paiardi… - Sensors and Actuators B …, 2019 - Elsevier
Cystic fibrosis (CF) is mainly caused by the mutation F508del of the cystic fibrosis
transmembrane conductance regulator (CFTR) that is thus retained in the endoplasmic …

[HTML][HTML] Insights into channel dysfunction from modelling and molecular dynamics simulations

M Musgaard, T Paramo, L Domicevica, OJ Andersen… - …, 2018 - Elsevier
Developments in structural biology mean that the number of different ion channel structures
has increased significantly in recent years. Structures of ion channels enable us to …

Recent strategic advances in CFTR drug discovery: an overview

M Rusnati, P D'Ursi, N Pedemonte, C Urbinati… - International Journal of …, 2020 - mdpi.com
Cystic fibrosis transmembrane conductance regulator (CFTR)-rescuing drugs have already
transformed cystic fibrosis (CF) from a fatal disease to a treatable chronic condition …

Structure of wild type and mutant F508del CFTR: A small-angle X-ray scattering study of the protein–detergent complexes

NL Pollock, L Satriano, O Zegarra-Moran… - Journal of Structural …, 2016 - Elsevier
CFTR is an anionic channel expressed in epithelia whose mutations cause cystic fibrosis.
Wild (WT) and mutated (F508del) types were over-expressed in yeast, solubilised in the …

Drug Discovery Platforms for CFTR Modulators

N Pedemonte - Hodson and Geddes' Cystic Fibrosis, 2023 - taylorfrancis.com
Cystic fibrosis (CF), the most frequent lethal recessive disease, is caused by loss-of-function
mutations in the gene encoding the CF transmembrane conductance regulator (CFTR) …

[HTML][HTML] Structure-function relationships of CFTR in health and disease: the pancreas story

M Bouhamdan, X Youming, F Sun - Pancreapedia: The Exocrine …, 2018 - pancreapedia.org
In 1938, Dr. Dorothy Anderson published a paper describing the characteristic of Cystic
Fibrosis (CF) in the pancreas; the term of “cystic fibrosis” refers to the autopsy findings of …