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ProteomicsFolding correctors can retore CFTR post-translational folding landscape by allosteric domain-domain coupling
Numerous missense mutations cause misfolding and premature degradation of ATP-binding cassette (ABC)-transporters-transporters, accounting for several human conformational diseases with poorly under-stood molecular mechanisms. Recent breakthroughs in small molecule combination therapy led transforma...
ORGANISM(S): Homo sapiens (Human) 
2024-01-26 | PXD042481 | Pride
Biogenesis Post-translational protein folding Cystic fibrosis mutation Protein dynamics Coupled domain folding Hydrogen-deuterium exchange and mass spectrometry (hdx-ms) Abc-transporter Folding corrector drugs Allostery Cystic fibrosis transmembrane conductance regulator (cftr) 
UnknownFolding-function relationship of the most common cystic fibrosis-causing CFTR conductance mutants.
Not available
S-EPMC6339265 | biostudies-literature
ProteomicsProteostasis Landscapes of Selective versus Poorly Responsive CFTR Variants Reveals Structural Vulnerabilities to Correction
Cystic Fibrosis (CF) is a lethal genetic disorder caused by variants in CF transmembrane conductance regulator (CFTR). Many disease variants are treatable with corrector compounds, which enhance the folding and trafficking of CFTR. However, correctors fail to elicit a response for every CFTR variant...
ORGANISM(S): Homo sapiens (Human) 
2025-10-27 | PXD055156 | Pride
Proteomics Cf Cftr Interactomics Ap-ms Vx-445 
UnknownStructure-guided combination therapy to potently improve the function of mutant CFTRs.
Not available
S-EPMC6301090 | biostudies-literature
UnknownProlonged co-treatment with HGF sustains epithelial integrity and improves pharmacological rescue of Phe508del-CFTR.
Not available
S-EPMC6115363 | biostudies-literature
UnknownProteostasis Landscapes of Cystic Fibrosis Variants Reveals Drug Response Vulnerability.
Not available
S-EPMC11257600 | biostudies-literature
UnknownProteostasis landscapes of cystic fibrosis variants reveal drug response vulnerability.
Not available
S-EPMC12054793 | biostudies-literature
UnknownABC-transporter CFTR folds with high fidelity through a modular, stepwise pathway.
Not available
S-EPMC9825563 | biostudies-literature
UnknownPharmacological Responses of the G542X-CFTR to CFTR Modulators.
Not available
S-EPMC9263564 | biostudies-literature
UnknownLow free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor).
Not available
S-EPMC4728415 | biostudies-literature
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