Associated Papers
Functional stability of CFTR depends on tight binding of ATP at its degenerate ATP-binding site.
Yeh HI et al.
The Journal of Physiology, 2021
STRUCTURE, GATING, AND REGULATION OF THE CFTR ANION CHANNEL.
Csanády L et al.
Physiological Reviews, 2019
Structural mechanisms for defective CFTR gating caused by the Q1412X mutation, a severe Class VI pathogenic mutation in cystic fibrosis.
Yeh JT et al.
The Journal of Physiology, 2019
Ion channels as targets to treat cystic fibrosis lung disease.
Martin SL et al.
Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society, 2018
The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor.
Broadbent SD et al.
Pflugers Archiv : European Journal of Physiology, 2015
A single amino acid substitution in CFTR converts ATP to an inhibitory ligand.
Lin WY et al.
The Journal of General Physiology, 2014
Optimization of the degenerated interfacial ATP binding site improves the function of disease-related mutant cystic fibrosis transmembrane conductance regulator (CFTR) channels.
Tsai MF et al.
The Journal of Biological Chemistry, 2010
A stable ATP binding to the nucleotide binding domain is important for reliable gating cycle in an ABC transporter CFTR.
Shimizu H et al.
The Journal of Physiological Sciences : Jps, 2010
Potentiation of disease-associated cystic fibrosis transmembrane conductance regulator mutants by hydrolyzable ATP analogs.
Miki H et al.
The Journal of Biological Chemistry, 2010
Stable ATP binding mediated by a partial NBD dimer of the CFTR chloride channel.
Tsai MF et al.
The Journal of General Physiology, 2010
Gating of the CFTR Cl- channel by ATP-driven nucleotide-binding domain dimerisation.
Hwang TC et al.
The Journal of Physiology, 2009
State-dependent modulation of CFTR gating by pyrophosphate.
Tsai MF et al.
The Journal of General Physiology, 2009
Review. ATP hydrolysis-driven gating in cystic fibrosis transmembrane conductance regulator.
Muallem D et al.
Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences, 2009
Mechanism of G551D-CFTR (cystic fibrosis transmembrane conductance regulator) potentiation by a high affinity ATP analog.
Bompadre SG et al.
The Journal of Biological Chemistry, 2008
The two ATP binding sites of cystic fibrosis transmembrane conductance regulator (CFTR) play distinct roles in gating kinetics and energetics.
Zhou Z et al.
The Journal of General Physiology, 2006