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Increased efficacy of VX-809 in different cellular systems results from an early stabilization effect of F508del- CFTR.
- Published in:
- Pharmacology Research & Perspectives, 2015, v. 3, n. 4, p. n/a, doi. 10.1002/prp2.152
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- Publication type:
- Article
CyFi-MAP: an interactive pathway-based resource for cystic fibrosis.
- Published in:
- Scientific Reports, 2021, v. 11, n. 1, p. 1, doi. 10.1038/s41598-021-01618-3
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- Publication type:
- Article
Additive Potentiation of R334W-CFTR Function by Novel Small Molecules.
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- Journal of Personalized Medicine, 2023, v. 13, n. 1, p. 102, doi. 10.3390/jpm13010102
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- Publication type:
- Article
Personalized Medicine Based on Nasal Epithelial Cells: Comparative Studies with Rectal Biopsies and Intestinal Organoids.
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- Journal of Personalized Medicine, 2021, v. 11, n. 5, p. 421, doi. 10.3390/jpm11050421
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- Publication type:
- Article
Assessment of Distinct Electrophysiological Parameters in Rectal Biopsies for the Choice of the Best Diagnosis/Prognosis Biomarkers for Cystic Fibrosis.
- Published in:
- Frontiers in Physiology, 2020, v. 11, p. N.PAG, doi. 10.3389/fphys.2020.604580
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- Publication type:
- Article
What Can RNA-Based Therapy Do for Monogenic Diseases?
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- Pharmaceutics, 2023, v. 15, n. 1, p. 260, doi. 10.3390/pharmaceutics15010260
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- Publication type:
- Article
Finding new medicines to fight CF: multiple steps of a success story.
- Published in:
- 2012
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- Publication type:
- Abstract
Drug Repurposing for Cystic Fibrosis: Identification of Drugs That Induce CFTR-Independent Fluid Secretion in Nasal Organoids.
- Published in:
- International Journal of Molecular Sciences, 2022, v. 23, n. 20, p. 12657, doi. 10.3390/ijms232012657
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- Publication type:
- Article
CFTR, Cell Junctions and the Cytoskeleton.
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- International Journal of Molecular Sciences, 2022, v. 23, n. 5, p. 2688, doi. 10.3390/ijms23052688
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- Publication type:
- Article
Rare Trafficking CFTR Mutations Involve Distinct Cellular Retention Machineries and Require Different Rescuing Strategies.
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- International Journal of Molecular Sciences, 2022, v. 23, n. 1, p. 24, doi. 10.3390/ijms23010024
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- Publication type:
- Article
Synergy in Cystic Fibrosis Therapies: Targeting SLC26A9.
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- International Journal of Molecular Sciences, 2021, v. 22, n. 23, p. 13064, doi. 10.3390/ijms222313064
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- Publication type:
- Article
Impact of KLF4 on Cell Proliferation and Epithelial Differentiation in the Context of Cystic Fibrosis.
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- International Journal of Molecular Sciences, 2020, v. 21, n. 18, p. 6717, doi. 10.3390/ijms21186717
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- Publication type:
- Article
Full Rescue of F508del-CFTR Processing and Function by CFTR Modulators Can Be Achieved by Removal of Two Regulatory Regions.
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- International Journal of Molecular Sciences, 2020, v. 21, n. 12, p. 4524, doi. 10.3390/ijms21124524
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- Publication type:
- Article
What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
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- International Journal of Molecular Sciences, 2020, v. 21, n. 9, p. 3133, doi. 10.3390/ijms21093133
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- Publication type:
- Article
BAG-1 Stabilizes Mutant F508del-CFTR in a Ubiquitin-Like-Domain-Dependent Manner.
- Published in:
- Cellular Physiology & Biochemistry (Karger AG), 2012, v. 30, n. 5, p. 1120, doi. 10.1159/000343303
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- Publication type:
- Article
Deletion of CFTR Translation Start Site Reveals Functional Isoforms of the Protein in CF Patients.
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- Cellular Physiology & Biochemistry (Karger AG), 2009, v. 24, n. 5/6, p. 335, doi. 10.1159/000257426
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- Publication type:
- Article
Establishment and Characterization of a Novel Polarized MDCK Epithelial Cellular Model for CFTR Studies.
- Published in:
- Cellular Physiology & Biochemistry (Karger AG), 2005, v. 16, n. 4-6, p. 281, doi. 10.1159/000089857
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- Publication type:
- Article
Cytoskeleton regulators CAPZA2 and INF2 associate with CFTR to control its plasma membrane levels under EPAC1 activation.
- Published in:
- Biochemical Journal, 2020, v. 477, n. 13, p. 2561, doi. 10.1042/BCJ20200287
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- Publication type:
- Article
CFTR mutations altering CFTR fragmentation.
- Published in:
- Biochemical Journal, 2013, v. 449, n. 1, p. 295, doi. 10.1042/BJ20121240
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- Publication type:
- Article
Mutation-class dependent signatures outweigh disease-associated processes in cystic fibrosis cells.
- Published in:
- Cell & Bioscience, 2023, v. 13, n. 1, p. 1, doi. 10.1186/s13578-023-00975-y
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- Publication type:
- Article
A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint.
- Published in:
- Science Signaling, 2015, v. 8, n. 377, p. 1, doi. 10.1126/scisignal.aaa1580
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- Publication type:
- Article
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene.
- Published in:
- Nature Genetics, 2013, v. 45, n. 10, p. 1160, doi. 10.1038/ng.2745
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- Publication type:
- Article
Processing of CFTR: Traversing the cellular mazeHow much CFTR needs to go through to avoid cystic fibrosis?
- Published in:
- Pediatric Pulmonology, 2005, v. 39, n. 6, p. 479, doi. 10.1002/ppul.20168
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- Publication type:
- Article
An open-source high-content analysis workflow for CFTR function measurements using the forskolin-induced swelling assay.
- Published in:
- Bioinformatics, 2020, v. 36, n. 24, p. 5686, doi. 10.1093/bioinformatics/btaa1073
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- Publication type:
- Article
Transcription-dependent spatial arrangements of CFTR and adjacent genes in human cell nuclei.
- Published in:
- Journal of Cell Biology, 2004, v. 166, n. 6, p. 815, doi. 10.1083/jcb.200404107
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- Publication type:
- Article
Enhanced Expression of Human Epididymis Protein 4 (HE4) Reflecting Pro-Inflammatory Status Is Regulated by CFTR in Cystic Fibrosis Bronchial Epithelial Cells.
- Published in:
- Frontiers in Pharmacology, 2021, v. 11, p. N.PAG, doi. 10.3389/fphar.2021.592184
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- Publication type:
- Article
2 Measurements of CFTR-Mediated Cl<sup>-</sup> Secretion in Human Rectal Biopsies Constitute a Robust Biomarker for Cystic Fibrosis Diagnosis and Prognosis.
- Published in:
- PLoS ONE, 2012, v. 7, n. 10, p. 1, doi. 10.1371/journal.pone.0047708
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- Publication type:
- Article
The K+ Channel Opener 1-EBIO Potentiates Residual Function of Mutant CFTR in Rectal Biopsies from Cystic Fibrosis Patients.
- Published in:
- PLoS ONE, 2011, v. 6, n. 8, p. 1, doi. 10.1371/journal.pone.0024445
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- Publication type:
- Article
Five Percent of Normal Cystic Fibrosis Transmembrane Conductance Regulator mRNA Ameliorates the Severity of Pulmonary Disease in Cystic Fibrosis.
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- American Journal of Respiratory Cell & Molecular Biology, 2002, v. 27, n. 5, p. 619, doi. 10.1165/rcmb.2001-0004OC
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- Publication type:
- Article
Changes in transcriptome of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies.
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- Respiratory Research, 2013, v. 14, n. 3, p. 1, doi. 10.1186/1465-9921-14-38
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- Publication type:
- Article
Changes in transcriptome of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies.
- Published in:
- 2013
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- Publication type:
- journal article
Assessing the residual CFTR gene expression in human nasal epithelium cells bearing CFTR splicing mutations causing cystic fibrosis.
- Published in:
- European Journal of Human Genetics, 2014, v. 22, n. 6, p. 784, doi. 10.1038/ejhg.2013.238
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- Publication type:
- Article
Human-Specific Cystic Fibrosis Transmembrane Conductance Regulator Antibodies Detect In Vivo Gene Transfer to Ovine Airways.
- Published in:
- American Journal of Respiratory Cell & Molecular Biology, 2006, v. 35, n. 1, p. 72, doi. 10.1165/rcmb.2005-0377OC
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- Publication type:
- Article
AMPK controls epithelial Na<sup>+</sup> channels through Nedd4-2 and causes an epithelial phenotype when mutated.
- Published in:
- Pflügers Archiv: European Journal of Physiology, 2009, v. 458, n. 4, p. 713, doi. 10.1007/s00424-009-0660-4
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- Publication type:
- Article
Exploring YAPI-centered networks linking dysfunctional CFTR to epithelial-mesenchymal transition.
- Published in:
- Life Science Alliance, 2022, v. 5, n. 9, p. 1, doi. 10.26508/lsa.202101326
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- Publication type:
- Article
A central role of the endoplasmic reticulum in the cell emerges from its functional contact sites with multiple organelles.
- Published in:
- Cellular & Molecular Life Sciences, 2020, v. 77, n. 23, p. 4729, doi. 10.1007/s00018-020-03523-w
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- Publication type:
- Article
Increases in cytosolic Ca<sup>2+</sup> induce dynamin- and calcineurin-dependent internalisation of CFTR.
- Published in:
- Cellular & Molecular Life Sciences, 2019, v. 76, n. 5, p. 977, doi. 10.1007/s00018-018-2989-3
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- Publication type:
- Article
Proteomic interaction profiling reveals KIFC1 as a factor involved in early targeting of F508del-CFTR to degradation.
- Published in:
- Cellular & Molecular Life Sciences, 2018, v. 75, n. 24, p. 4495, doi. 10.1007/s00018-018-2896-7
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- Publication type:
- Article
The SLC26A9 inhibitor S9‐A13 provides no evidence for a role of SLC26A9 in airway chloride secretion but suggests a contribution to regulation of ASL pH and gastric proton secretion.
- Published in:
- FASEB Journal, 2022, v. 36, n. 11, p. 1, doi. 10.1096/fj.202200313RR
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- Publication type:
- Article
Pediatric population with cystic fibrosis in the centre of Portugal: candidates for new therapies.
- Published in:
- Jornal de Pediatria, 2022, v. 98, n. 2, p. 212, doi. 10.1016/j.jped.2021.05.010
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- Publication type:
- Article
Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi.
- Published in:
- FEBS Journal, 2013, v. 280, n. 18, p. 4396, doi. 10.1111/febs.12392
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- Publication type:
- Article
Post-translational modifications of CFTR: insight into protein trafficking and cystic fibrosis disease.
- Published in:
- FEBS Journal, 2013, v. 280, n. 18, p. 4395, doi. 10.1111/febs.12345
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- Publication type:
- Article
Deletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperone.
- Published in:
- FEBS Journal, 2009, v. 276, n. 23, p. 7097, doi. 10.1111/j.1742-4658.2009.07421.x
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- Publication type:
- Article
Using the genome to correct the ion transport defect in cystic fibrosis.
- Published in:
- Journal of Physiology, 2023, v. 601, n. 9, p. 1573, doi. 10.1113/JP282308
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- Publication type:
- Article
Partial rescue of F508del-cystic fibrosis transmembrane conductance regulator channel gating with modest improvement of protein processing, but not stability, by a dual-acting small molecule.
- Published in:
- 2018
- By:
- Publication type:
- journal article
CFTR gene transfer to human cystic fibrosis pancreatic duct cells using a Sendai virus vector.
- Published in:
- Journal of Cellular Physiology, 2008, v. 214, n. 2, p. 442, doi. 10.1002/jcp.21220
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- Publication type:
- Article
Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.
- Published in:
- Journal of Physiology, 2014, v. 592, n. 9, p. 1931, doi. 10.1113/jphysiol.2014.271817
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- Publication type:
- Article
Protein Traffic Disorders: an Effective High-Throughput Fluorescence Microscopy Pipeline for Drug Discovery.
- Published in:
- Scientific Reports, 2015, p. 9038, doi. 10.1038/srep09038
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- Publication type:
- Article
Absence of EPAC1 Signaling to Stabilize CFTR in Intestinal Organoids.
- Published in:
- Cells (2073-4409), 2022, v. 11, n. 15, p. 2295, doi. 10.3390/cells11152295
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- Publication type:
- Article
Rescue of Mutant CFTR Trafficking Defect by the Investigational Compound MCG1516A.
- Published in:
- Cells (2073-4409), 2022, v. 11, n. 1, p. 136, doi. 10.3390/cells11010136
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- Publication type:
- Article