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ProteomicsElexacaftor/VX-445-mediated CFTR interactome remodeling reveals differential correction driven by mutation-specific translational dynamics
Cystic fibrosis (CF) is one of the most prevalent lethal genetic diseases with over 2000 identified genetic variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Pharmacological chaperones such as Lumacaftor (VX-809), Tezacaftor (VX-661) and Elexacaftor (VX-445) treat muta...
ORGANISM(S): Homo sapiens (Human) 
2023-09-18 | PXD039773 | Pride
Ap-ms Elexacaftor Cf Cftr Trikafta Interactomics 
TranscriptomicsRescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers
In cystic fibrosis (CF), loss of CF transmembrane conductance regulator (CFTR)-dependent bicarbonate secretion precipitates the accumulation of viscous mucus in the lumen of respiratory and gastrointestinal epithelial tissues. We investigated whether the combination of elexacaftor (ELX), ivacaftor (...
ORGANISM(S): Homo sapiens 
2021-12-14 | GSE182347 | GEO
UnknownElexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Not available
S-EPMC7282384 | biostudies-literature
UnknownElexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment.
Not available
S-EPMC8494914 | biostudies-literature
UnknownRegulatory T cell enhancement in adults with cystic fibrosis receiving Elexacaftor/Tezacaftor/Ivacaftor therapy.
Not available
S-EPMC9978140 | biostudies-literature
UnknownRechallenge of Elexacaftor/Tezacaftor/Ivacaftor After Skin Rash in Two Pediatric Patients
Not available
S-EPMC9268115 | biostudies-literature
Genomics
Sputum microbiota in people with cystic fibrosis starting on Elexacaftor/Tezacaftor/Ivacaftor
PRJNA934375 | ENA
GenomicsRescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers
Rescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers
PRJNA755978 | ENA
UnknownPhysiologically-Based Pharmacokinetic-Led Guidance for Patients With Cystic Fibrosis Taking Elexacaftor-Tezacaftor-Ivacaftor With Nirmatrelvir-Ritonavir for the Treatment of COVID-19.
Not available
S-EPMC9087007 | biostudies-literature
UnknownOrganic Synthesis and Current Understanding of the Mechanisms of CFTR Modulator Drugs Ivacaftor, Tezacaftor, and Elexacaftor.
Not available
S-EPMC10891718 | biostudies-literature
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