| 1 |
34666947 | Functional Restoration of CFTR Nonsense Mutations in Intestinal Organoids. | 2022 Mar |
1 |
| 2 |
34977268 | The sodium/glucose cotransporters as potential therapeutic targets for CF lung diseases revealed by human lung organoid swelling assay. | 2022 Mar 10 |
2 |
| 3 |
35086832 | Forskolin-induced Organoid Swelling is Associated with Long-term CF Disease Progression. | 2022 Jan 27 |
1 |
| 4 |
35110005 | Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype. | 2022 Mar |
2 |
| 5 |
35315358 | CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor. | 2022 Mar 22 |
1 |
| 6 |
35455747 | Theratyping of the Rare CFTR Variants E193K and R334W in Rectal Organoid-Derived Epithelial Monolayers. | 2022 Apr 14 |
1 |
| 7 |
35471184 | Molecular Dynamics and Theratyping in Airway and Gut Organoids Reveal R352Q-CFTR Conductance Defect. | 2022 Apr 26 |
1 |
| 8 |
32747394 | Correction of CFTR function in intestinal organoids to guide treatment of cystic fibrosis. | 2021 Jan |
1 |
| 9 |
33016182 | Identification of Compounds That Promote Readthrough of Premature Termination Codons in the CFTR. | 2021 Feb |
1 |
| 10 |
33236921 | Phospholipase C controls chloride-dependent short-circuit current in human bronchial epithelial cells. | 2021 Feb 1 |
1 |
| 11 |
33558100 | Targeting G542X CFTR nonsense alleles with ELX-02 restores CFTR function in human-derived intestinal organoids. | 2021 May |
2 |
| 12 |
33655768 | Impact of hypoxia and AMPK on CFTR-mediated bicarbonate secretion in human cholangiocyte organoids. | 2021 May 1 |
2 |
| 13 |
33971543 | LPS decreases CFTR open probability and mucociliary transport through generation of reactive oxygen species. | 2021 Jul |
1 |
| 14 |
34054511 | Enhanced Expression of Human Epididymis Protein 4 (HE4) Reflecting Pro-Inflammatory Status Is Regulated by CFTR in Cystic Fibrosis Bronchial Epithelial Cells. | 2021 |
2 |
| 15 |
34071719 | Health Characteristics of Patients with Cystic Fibrosis whose Genotype Includes a Variant of the Nucleotide Sequence c.3140-16T>A and Functional Analysis of this Variant. | 2021 May 28 |
1 |
| 16 |
34163370 | Alteration of Membrane Cholesterol Content Plays a Key Role in Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Channel Activity. | 2021 |
9 |
| 17 |
34330959 | Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis. | 2021 Jul 30 |
1 |
| 18 |
31126900 | Inactivation of CFTR by CRISPR/Cas9 alters transcriptional regulation of inflammatory pathways and other networks. | 2020 Jan |
2 |
| 19 |
31735562 | Forskolin-induced swelling of intestinal organoids correlates with disease severity in adults with cystic fibrosis and homozygous F508del mutations. | 2020 Jul |
1 |
| 20 |
32061518 | R117H-CFTR function and response to VX-770 correlate with mRNA and protein expression in intestinal organoids. | 2020 Sep |
1 |
| 21 |
32078860 | Targeting different binding sites in the CFTR structures allows to synergistically potentiate channel activity. | 2020 Mar 15 |
1 |
| 22 |
32159371 | The dual phosphodiesterase 3/4 inhibitor RPL554 stimulates rare class III and IV CFTR mutants. | 2020 May 1 |
2 |
| 23 |
32204475 | Phenotyping of Rare CFTR Mutations Reveals Distinct Trafficking and Functional Defects. | 2020 Mar 19 |
1 |
| 24 |
32377875 | An organoid model to assay the role of CFTR in the human epididymis epithelium. | 2020 Aug |
1 |
| 25 |
32485957 | Human Nasal Epithelial Organoids for Therapeutic Development in Cystic Fibrosis. | 2020 May 29 |
1 |
| 26 |
32801121 | Inhibition of Alk signaling promotes the induction of human salivary-gland-derived organoids. | 2020 Sep 28 |
2 |
| 27 |
33014932 | Functional Profiling of CFTR-Directed Therapeutics Using Pediatric Patient-Derived Nasal Epithelial Cell Models. | 2020 |
1 |
| 28 |
33111074 | Protocol for Application, Standardization and Validation of the Forskolin-Induced Swelling Assay in Cystic Fibrosis Human Colon Organoids. | 2020 Jun 19 |
3 |
| 29 |
33123317 | Upregulation of CFTR Protects against Palmitate-Induced Endothelial Dysfunction by Enhancing Autophagic Flux. | 2020 |
5 |
| 30 |
33367496 | An open-source high-content analysis workflow for CFTR function measurements using the forskolin-induced swelling assay. | 2020 Dec 26 |
2 |
| 31 |
30573361 | CFTR activation suppresses glioblastoma cell proliferation, migration and invasion. | 2019 Jan 22 |
1 |
| 32 |
28712885 | Detection of CFTR function and modulation in primary human nasal cell spheroids. | 2018 Jan |
1 |
| 33 |
29761302 | CFTR prevents neuronal apoptosis following cerebral ischemia reperfusion via regulating mitochondrial oxidative stress. | 2018 Jul |
2 |
| 34 |
30166324 | Stratifying infants with cystic fibrosis for disease severity using intestinal organoid swelling as a biomarker of CFTR function. | 2018 Sep |
3 |
| 35 |
28273890 | CFTR is involved in the regulation of glucagon secretion in human and rodent alpha cells. | 2017 Mar 7 |
1 |
| 36 |
28287550 | Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients. | 2017 Feb 11 |
4 |
| 37 |
28785019 | Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators. | 2017 Aug 7 |
1 |
| 38 |
26336913 | A synonymous codon change alters the drug sensitivity of ΔF508 cystic fibrosis transmembrane conductance regulator. | 2016 Jan |
1 |
| 39 |
26492939 | Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor). | 2016 Feb |
1 |
| 40 |
26509335 | rAAV-CFTRΔR Rescues the Cystic Fibrosis Phenotype in Human Intestinal Organoids and Cystic Fibrosis Mice. | 2016 Feb 1 |
2 |
| 41 |
26574187 | Hypercapnia modulates cAMP signalling and cystic fibrosis transmembrane conductance regulator-dependent anion and fluid secretion in airway epithelia. | 2016 Mar 15 |
2 |
| 42 |
26677983 | Human Enteroids as a Model of Upper Small Intestinal Ion Transport Physiology and Pathophysiology. | 2016 Mar |
1 |
| 43 |
27160424 | Potentiator synergy in rectal organoids carrying S1251N, G551D, or F508del CFTR mutations. | 2016 Sep |
5 |
| 44 |
27229121 | Identification of intestinal ion transport defects in microvillus inclusion disease. | 2016 Jul 1 |
1 |
| 45 |
27830759 | Marked increases in mucociliary clearance produced by synergistic secretory agonists or inhibition of the epithelial sodium channel. | 2016 Nov 10 |
1 |
| 46 |
25795456 | Influenza virus M2 targets cystic fibrosis transmembrane conductance regulator for lysosomal degradation during viral infection. | 2015 Jul |
1 |
| 47 |
26079370 | Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity. | 2015 |
2 |
| 48 |
26115671 | Junctional abnormalities in human airway epithelial cells expressing F508del CFTR. | 2015 Sep 1 |
1 |
| 49 |
26357939 | Mechanosensitive activation of CFTR by increased cell volume and hydrostatic pressure but not shear stress. | 2015 Nov |
2 |
| 50 |
23740188 | Evidence that two distinct crypt cell types secrete chloride and potassium in human colon. | 2014 Mar |
2 |