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Article

Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein.

The Journal of biological chemistry - 16 Nov 2007

Wang Ying, Loo Tip W, Bartlett M Claire, Clarke David M

Abstract excerpt

The most common cause of cystic fibrosis (CF) is defective folding of a cystic fibrosis transmembrane conductance regulator (CFTR) mutant lacking Phe(508) (DeltaF508). The DeltaF508 protein appears to be trapped in a prefolded state with incomplete packing of the transmembrane (TM) segments, a defect that can be repaired by expression in the presence of correctors such as corr-4a, VRT-325, and VRT-532. To...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 17911111
DOI: 10.1074/jbc.C700175200

Genes

CFTR

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein.PMID 17911111Read ↗

Correctors promote folding of the CFTR in the endoplasmic reticulum.Semantic proximity Read ↗

Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants.Semantic proximity Read ↗

Repair of CFTR folding defects with correctors that function as pharmacological chaperones.Semantic proximity Read ↗

The chemical chaperone CFcor-325 repairs folding defects in the transmembrane domains of CFTR-processing mutants.Semantic proximity Read ↗

Rescuing cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by transcomplementation.Semantic proximity Read ↗

Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27.Semantic proximity Read ↗

Correctors of the Major Cystic Fibrosis Mutant Interact through Membrane-Spanning Domains.Semantic proximity Read ↗

Unravelling the Regions of Mutant F508del-CFTR More Susceptible to the Action of Four Cystic Fibrosis Correctors.Semantic proximity Read ↗

Corrector VX-809 stabilizes the first transmembrane domain of CFTR.Semantic proximity Read ↗

Correctors of the basic trafficking defect of the mutant F508del-CFTR that causes cystic fibrosis.Semantic proximity Read ↗

Introduction of the most common cystic fibrosis mutation (Delta F508) into human P-glycoprotein disrupts packing of the transmembrane segments.Semantic proximity Read ↗

A chemical corrector modifies the channel function of F508del-CFTR.Semantic proximity Read ↗

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