Discover Community Create You

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

Ribosomal frameshifting selectively modulates the assembly, function, and pharmacological rescue of a misfolded CFTR variant.

Proceedings of the National Academy of Sciences of the United States of America - 15 Oct 2024

Carmody Patrick J, Roushar Francis J, Tedman Austin, Wang Wei, Herwig Madeline, Kim Minsoo, McDonald Eli F, Noguera Karen, Wong-Roushar Jennifer, Poirier Jon-Luc, Zelt Nathan B, Pockrass Ben T, McKee Andrew G, Kuntz Charles P, Raju S Vamsee, Plate Lars, Penn Wesley D, Schlebach Jonathan P

Abstract excerpt

The cotranslational misfolding of the cystic fibrosis transmembrane conductance regulator chloride channel (CFTR) plays a central role in the molecular basis of CF. The misfolding of the most common CF variant (ΔF508) remodels both the translational regulation and quality control of CFTR. Nevertheless, it is unclear how the misassembly of the nascent polypeptide may directly influence the activity of the...

Read the complete abstract on PubMed

Topics

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

Share to Cystic Fibrosis
Share to Cystic Fibrosis Transmembrane Conductance Regulator

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Frameshifting, Ribosomal

Share to Mutation

Share to Nucleic Acid Conformation

Share to Protein Biosynthesis

Share to Protein Folding

Share to Ribosomes

Source

Open in PubMed Open full text

Identifiers

PMID: 39388263
DOI: 10.1073/pnas.2414768121
PMCID: PMC11494300

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Ribosomal frameshifting selectively modulates the assembly, function, and pharmacological rescue of a misfolded CFTR variant.PMID 39388263Read ↗

Slowing ribosome velocity restores folding and function of mutant CFTR.Semantic proximity Read ↗

Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression DefectSemantic proximity Read ↗

Correcting the F508del-CFTR variant by modulating eukaryotic translation initiation factor 3-mediated translation initiation.Semantic proximity Read ↗

CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates.Semantic proximity Read ↗

Feedback from the Nascent Chain Triggers Ribosomal Frameshifting and Transcript DecaySemantic proximity Read ↗

Removal of multiple arginine-framed trafficking signals overcomes misprocessing of delta F508 CFTR present in most patients with cystic fibrosis.Semantic proximity Read ↗

Quality control ensures fidelity in ribosome assembly and cellular healthSemantic proximity Read ↗

Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting.Semantic proximity Read ↗

Readthrough-induced misincorporated amino acid ratios guide mutant-specific therapeutic approaches for two CFTR nonsense mutationsSemantic proximity Read ↗

Strategies for correcting the delta F508 CFTR protein-folding defect.Semantic proximity Read ↗

A synonymous single nucleotide polymorphism in DeltaF508 CFTR alters the secondary structure of the mRNA and the expression of the mutant protein.Semantic proximity Read ↗

Ribosome. The complete structure of the 55S mammalian mitochondrial ribosome.Semantic proximity Read ↗

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