Associated Papers
A cluster of inhibitory residues in the regulatory domain prevents activation of the cystic fibrosis transmembrane conductance regulator.
Wu M et al.
The Journal of Biological Chemistry, 2025
Global functional genomics reveals GRK5 as a cystic fibrosis therapeutic target synergistic with current modulators.
Botelho HM et al.
Iscience, 2025
PTI-801 (posenacaftor) shares a common mechanism with VX-445 (elexacaftor) to rescue p.Phe508del-CFTR.
Ferreira FC et al.
European Journal of Pharmacology, 2024
Identification of novel F508del-CFTR traffic correctors among triazole derivatives.
Bacalhau M et al.
European Journal of Pharmacology, 2023
Acting on the CFTR Membrane-Spanning Domains Interface Rescues Some Misfolded Mutants.
Baatallah N et al.
International Journal of Molecular Sciences, 2022
A small molecule CFTR potentiator restores ATP-dependent channel gating to the cystic fibrosis mutant G551D-CFTR.
Liu J et al.
British Journal of Pharmacology, 2022
Rescue of Mutant CFTR Trafficking Defect by the Investigational Compound MCG1516A.
Lopes-Pacheco M et al.
Cells, 2022
Rare Trafficking CFTR Mutations Involve Distinct Cellular Retention Machineries and Require Different Rescuing Strategies.
Ramalho SS et al.
International Journal of Molecular Sciences, 2021
Frequency of allele variations in the CFTR gene in a Mexican population.
Cantú-Reyna C et al.
Bmc Medical Genomics, 2021
Folding Status Is Determinant over Traffic-Competence in Defining CFTR Interactors in the Endoplasmic Reticulum.
Santos JD et al.
Cells, 2019
Substitution of Yor1p NBD1 residues improves the thermal stability of Human Cystic Fibrosis Transmembrane Conductance Regulator.
Xavier BM et al.
Protein Engineering, Design & Selection : Peds, 2017
CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR.
Wang Y et al.
British Journal of Pharmacology, 2014
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.
Xu Z et al.
The Journal of Physiology, 2014
Cystic fibrosis: CFTR correctors to the rescue.
Sheppard DN
Chemistry & Biology, 2011
Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms.
Roxo-Rosa M et al.
Proceedings of the National Academy of Sciences of the United States of America, 2006
Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis.
Chang XB et al.
Molecular Cell, 1999