Research graph
References from Vanzacaftor/tezacaftor/deutivacaftor partially restores CFTR function of the L467F;F508del complex allele in primary nasal epithelial cells. Local targets link to admitted publications; unresolved targets remain external evidence.
Cystic fibrosis
10.1038/s41572-024-00538-6 · 2024 · External reference
The future of cystic fibrosis treatment: from disease mechanisms to novel therapeutic approaches
10.1016/s0140-6736(23)01608-2 · 2023 · External reference
Elexacaftor-Tezacaftor-Ivacaftor for Cystic fibrosis with a single Phe508del Allele
10.1056/nejmoa1908639 · 2019 · External reference
Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial
10.1016/s0140-6736(19)32597-8 · 2019 · External reference
Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis
10.1183/13993003.02153-2022 · 2023 · External reference
Effects of Elexacaftor/Tezacaftor/Ivacaftor therapy on CFTR function in patients with cystic fibrosis and one or two F508del alleles
10.1164/rccm.202110-2249oc · 2022 · External reference
Effects of Elexacaftor/Tezacaftor/Ivacaftor therapy on lung clearance index and Magnetic resonance imaging in patients with cystic fibrosis and one or two F508del alleles
10.1164/rccm.202201-0219oc · 2022 · External reference
Reduction of systemic inflammation by elexacaftor/tezacaftor/ivacaftor correlates with lung function improvement in cystic fibrosis
10.1183/13993003.00150-2025 · 2025 · External reference
L467F;F508del Complex allele in a heterozygous State with CFTRdele2,3: what to expect from CFTR modulators?
10.3390/ijms262311742 · 2025 · External reference
The L467F-F508del complex Allele hampers pharmacological rescue of mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in cystic fibrosis patients: the value of the ex vivo nasal epithelial model to address non-responders to CFTR-modulating drugs
10.3390/ijms23063175 · 2022 · External reference
Estimation of chloride channel residual function and assessment of targeted drugs efficiency in the presence of a complex allele [L467F;F508del] in the CFTR gene
10.3390/ijms251910424 · 2024 · External reference
Evaluation of the complex p. [Leu467Phe;Phe508del] CFTR allele in the intestinal organoids model: implications for therapy
10.3390/ijms231810377 · 2022 · External reference
Vanzacaftor-Tezacaftor as an alternative therapeutic resource for the ETI-Resistant L467F-F508del Allele: ex vivo prediction and exploratory clinical assessment
10.1016/j.jcf.2026.05.015 · 2026 · External reference
Vanzacaftor-tezacaftor-deutivacaftor versus elexacaftor-tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years and older (SKYLINE Trials VX20-121-102 and VX20-121-103): results from two randomised, active-controlled, phase 3 trials
10.1016/s2213-2600(24)00411-9 · 2025 · External reference
Personalized CFTR modulator therapy for G85E and N1303K homozygous patients with cystic fibrosis
10.3390/ijms241512365 · 2023 · External reference
The potentiator ivacaftor is essential for pharmacological restoration of F508del-CFTR function and mucociliary clearance in cystic fibrosis
10.1172/jci.insight.187951 · 2025 · External reference
Relationship between theratyping in nasal epithelial cells and clinical outcomes in people with cystic fibrosis
10.1183/13993003.01855-2024 · 2025 · External reference
CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis
10.1053/j.gastro.2004.07.006 · 2004 · External reference
Effects of Lumacaftor-Ivacaftor Therapy on cystic fibrosis transmembrane conductance regulator function in Phe508del homozygous patients with Cystic fibrosis
10.1164/rccm.201710-1983oc · 2018 · External reference
Lumacaftor-Ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR
10.1056/nejmoa1409547 · 2015 · External reference
Evaluation of elexacaftor-tezacaftor-ivacaftor treatment in individuals with cystic fibrosis and CFTR(N1303K) in the USA: a prospective, multicentre, open-label, single-arm trial
10.1016/s2213-2600(24)00205-4 · 2024 · External reference
French CFRNsg. Theratyping cystic fibrosis patients to guide elexacaftor/tezacaftor/ivacaftor out-of-label prescription
10.1183/13993003.00110-2023 · 2023 · External reference
High frequency of complex CFTR alleles associated with c.1521_1523delCTT (F508del) in Russian cystic fibrosis patients
10.1186/s12864-022-08466-z · 2022 · External reference
