Associated Papers
Trikafta restores thermodynamic coupling between two nucleotide binding domains for potentiating CFTR activity.
Wang G
Biomedicine & Pharmacotherapy = Biomedecine & Pharmacotherapie, 2026
CRISPR for cystic fibrosis: Advances and insights from a systematic review.
Nicosia L et al.
Molecular Therapy : the Journal of the American Society of Gene Therapy, 2025
Thermodynamic Coupling between Folding Correctors and the First of Dimerized Nucleotide Binding Domains in CFTR.
Wang G
Acs Bio & Med Chem Au, 2025
Thermodynamic coupling between folding correctors and the first of dimerized nucleotide binding domains in CFTR.
Wang G
Research Square, 2025
Effects of Point Mutations on the Thermal Stability of the NBD1 Domain of hCFTR.
Lublin L et al.
Journal of Chemical Information and Modeling, 2025
Proteostasis landscapes of cystic fibrosis variants reveal drug response vulnerability.
McDonald EF et al.
Proceedings of the National Academy of Sciences of the United States of America, 2025
Functional rescue of F508del-CFTR through revertant mutations introduced by CRISPR base editing.
Carrozzo I et al.
Molecular Therapy : the Journal of the American Society of Gene Therapy, 2025
Proteostasis Landscapes of Cystic Fibrosis Variants Reveals Drug Response Vulnerability.
McDonald EF et al.
Biorxiv : the Preprint Server for Biology, 2025
Allosteric inhibition of CFTR gating by CFTRinh-172 binding in the pore.
Gao X et al.
Nature Communications, 2024
The Folding Pathway of ABC Transporter CFTR: Effective and Robust.
van der Sluijs P et al.
Journal of Molecular Biology, 2024
Benchmarking AlphaMissense Pathogenicity Predictions Against Cystic Fibrosis Variants.
McDonald EF et al.
Biorxiv : the Preprint Server for Biology, 2024
Benchmarking AlphaMissense pathogenicity predictions against cystic fibrosis variants.
McDonald EF et al.
Plos One, 2024
Mutation accumulation in H. sapiens F508del CFTR countermands dN/dS type genomic analysis.
Hong JS et al.
Plos One, 2024
Folding correctors can restore CFTR posttranslational folding landscape by allosteric domain-domain coupling.
Soya N et al.
Nature Communications, 2023
CFTR Folding: From Structure and Proteostasis to Cystic Fibrosis Personalized Medicine.
McDonald EF et al.
Acs Chemical Biology, 2023
Rescue of Rare CFTR Trafficking Mutants Highlights a Structural Location-Dependent Pattern for Correction.
Zacarias S et al.
International Journal of Molecular Sciences, 2023
ABC-transporter CFTR folds with high fidelity through a modular, stepwise pathway.
Im J et al.
Cellular and Molecular Life Sciences : Cmls, 2023
Acting on the CFTR Membrane-Spanning Domains Interface Rescues Some Misfolded Mutants.
Baatallah N et al.
International Journal of Molecular Sciences, 2022
Systematic estimation of cystic fibrosis prevalence in Chinese and genetic spectrum comparison to Caucasians.
Ni Q et al.
Orphanet Journal of Rare Diseases, 2022
Structural Comparative Modeling of Multi-Domain F508del CFTR.
McDonald EF et al.
Biomolecules, 2022
Can two wrongs make a right? F508del-CFTR ion channel rescue by second-site mutations in its transmembrane domains.
Prins S et al.
The Journal of Biological Chemistry, 2022
A small molecule high throughput screening platform to profile conformational properties of nascent, ribosome-bound proteins.
Shishido H et al.
Scientific Reports, 2022
Nanomechanics combined with HDX reveals allosteric drug binding sites of CFTR NBD1.
Padányi R et al.
Computational and Structural Biotechnology Journal, 2022
A topological switch in CFTR modulates channel activity and sensitivity to unfolding.
Scholl D et al.
Nature Chemical Biology, 2021
Co-Translational Folding of the First Transmembrane Domain of ABC-Transporter CFTR is Supported by Assembly with the First Cytosolic Domain.
Kleizen B et al.
Journal of Molecular Biology, 2021
Stability Prediction for Mutations in the Cytosolic Domains of Cystic Fibrosis Transmembrane Conductance Regulator.
Bahia MS et al.
Journal of Chemical Information and Modeling, 2021
CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates.
Shishido H et al.
Nature Communications, 2020
Towards next generation therapies for cystic fibrosis: Folding, function and pharmacology of CFTR.
Bose SJ et al.
Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society, 2020
Disease-relevant mutations alter amino acid co-evolution networks in the second nucleotide binding domain of CFTR.
Ivey G et al.
Plos One, 2020
Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis-causing mutation.
Laselva O et al.
Faseb Bioadvances, 2019
Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR.
Bose SJ et al.
American Journal of Physiology. Lung Cellular and Molecular Physiology, 2019
Domain-interface dynamics of CFTR revealed by stabilizing nanobodies.
Sigoillot M et al.
Nature Communications, 2019
Identification of GLPG/ABBV-2737, a Novel Class of Corrector, Which Exerts Functional Synergy With Other CFTR Modulators.
de Wilde G et al.
Frontiers in Pharmacology, 2019
Cryo-EM Visualization of an Active High Open Probability CFTR Anion Channel.
Fay JF et al.
Biochemistry, 2018
Structural stability of purified human CFTR is systematically improved by mutations in nucleotide binding domain 1.
Yang Z et al.
Biochimica Et Biophysica Acta. Biomembranes, 2018
Mutations in SELENBP1, encoding a novel human methanethiol oxidase, cause extraoral halitosis.
Pol A et al.
Nature Genetics, 2018
Targeted Gene Next-Generation Sequencing in Chinese Children with Chronic Pancreatitis and Acute Recurrent Pancreatitis.
Xiao Y et al.
The Journal of Pediatrics, 2017
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
Stabilization of a nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator yields insight into disease-causing mutations.
Vernon RM et al.
The Journal of Biological Chemistry, 2017
Correcting CFTR folding defects by small-molecule correctors to cure cystic fibrosis.
Mijnders M et al.
Current Opinion in Pharmacology, 2017
Specific stabilization of CFTR by phosphatidylserine.
Hildebrandt E et al.
Biochimica Et Biophysica Acta. Biomembranes, 2017
Direct Measurement of Trafficking of the Cystic Fibrosis Transmembrane Conductance Regulator to the Cell Surface and Binding to a Chemical Chaperone.
Zhang Z et al.
Biochemistry, 2017