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Article

Correctors of the basic trafficking defect of the mutant F508del-CFTR that causes cystic fibrosis.

Current opinion in chemical biology - 1 Jun 2013

Birault Véronique, Solari Roberto, Hanrahan John, Thomas David Y

Abstract excerpt

Cystic fibrosis (CF) is the most frequent lethal genetic disease and the most frequent mutation is F508del-cystic fibrosis transmembrane regulator (CFTR). In common with some other protein trafficking diseases the mutant protein is functional but recognized by the cellular quality control system retained in the endoplasmic reticulum (ER) and degraded. There have been some recent impressive advances in developing...

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Source

Identifiers

PMID: 23711435
DOI: 10.1016/j.cbpa.2013.04.020

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 3 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Correctors of the basic trafficking defect of the mutant F508del-CFTR that causes cystic fibrosis.PMID 23711435Read ↗

Functional Rescue of F508del-CFTR Using Small Molecule CorrectorsSemantic proximity Read ↗

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

Corrector-mediated rescue of misprocessed CFTR mutants can be reduced by the P-glycoprotein drug pump.Semantic proximity Read ↗

Regulated recycling of mutant CFTR is partially restored by pharmacological treatment.Semantic proximity Read ↗

Identification of novel F508del-CFTR traffic correctors among triazole derivatives.Semantic proximity Read ↗

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

Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell linesSemantic proximity Read ↗

Pharmacological Correction of Cystic Fibrosis: Molecular Mechanisms at the Plasma Membrane to Augment Mutant CFTR Function.Semantic proximity Read ↗

Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors.Semantic proximity Read ↗

Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors.Semantic proximity Read ↗

Correctors modify the bicarbonate permeability of F508del-CFTR.Semantic proximity Read ↗

Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment.Semantic proximity Read ↗

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