Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Small-molecule correctors divert CFTR-F508del from ERAD by stabilizing sequential folding states.

Molecular biology of the cell - 1 Feb 2024

Riepe Celeste, Wąchalska Magda, Deol Kirandeep K, Amaya Anais K, Porteus Matthew H, Olzmann James A, Kopito Ron R

Abstract excerpt

Over 80% of people with cystic fibrosis (CF) carry the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR), a chloride ion channel at the apical plasma membrane (PM) of epithelial cells. F508del impairs CFTR folding causing it to be destroyed by endoplasmic reticulum associated degradation (ERAD). Small-molecule correctors, which act as pharmacological chaperones to divert...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Benzodioxoles
Share to Cystic Fibrosis

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Cystic Fibrosis Transmembrane Conductance Regulator

Share to Endoplasmic Reticulum-Associated Degradation

Share to Ligases

Share to Mitochondrial Proteins

Share to Protein Folding

Share to Ubiquitin-Protein Ligases

Source

Open in PubMed Open full text

Identifiers

PMID: 38019608
DOI: 10.1091/mbc.E23-08-0336
PMCID: PMC10881158

Genes

CFTR
RNF5
RNF185

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Small-molecule correctors divert CFTR-F508del from ERAD by stabilizing sequential folding states.PMID 38019608Read ↗

Small molecule correctors divert CFTR-F508del from ERAD by stabilizing sequential folding statesSemantic proximity Read ↗

CFTR Folding: From Structure and Proteostasis to Cystic Fibrosis Personalized Medicine.Semantic proximity Read ↗

Elexacaftor/VX-445-mediated CFTR interactome remodeling reveals differential correction driven by mutation-specific translational dynamics.Semantic proximity Read ↗

Chaperone-Independent Peripheral Quality Control of CFTR by RFFL E3 Ligase.Semantic proximity Read ↗

Elexacaftor/VX-445-mediated CFTR interactome remodeling reveals differential correction driven by mutation-specific translational dynamicsSemantic proximity Read ↗

Proteostasis Landscapes of Cystic Fibrosis Variants Reveals Drug Response VulnerabilitySemantic proximity Read ↗

Mechanism-based corrector combination restores ΔF508-CFTR folding and function.Semantic proximity Read ↗

Elucidation of Global Trends in the Effects of VX-661 and VX-445 on the Expression of Clinical CFTR VariantsSemantic proximity Read ↗

Regulation of CFTR Biogenesis by the Proteostatic Network and Pharmacological Modulators.Semantic proximity Read ↗

Mechanism of CFTR correction by type I folding correctors.Semantic proximity Read ↗

RNA Interference Screen to Identify Kinases That Suppress Rescue of ΔF508-CFTR*Semantic proximity Read ↗

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

⌁Select a neighboring publication to make it the new centre.