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Article

Endoplasmic reticulum-associated degradation of mutant CFTR requires a guanine nucleotide-sensitive step.

The international journal of biochemistry & cell biology - 1 Jan 2008

De Keukeleire Béatrice, Micoud Julien, Biard Julie, Benharouga Mohamed

Abstract excerpt

Proteasome degradation of endoplasmic reticulum (ER)-misfolded proteins requires retrograde transport from ER to the cytosol. To date, it is not clear whether this event constitutes the exclusive ER degradation process for non-native membrane proteins. Here we describe the role of GTP in the degradation of DeltaF508-CFTR and the alpha subunit of the T-cell receptor (TCRalpha), representative misfolded ER membrane...

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Source

Identifiers

PMID: 18280771
DOI: 10.1016/j.biocel.2007.12.015

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Endoplasmic reticulum-associated degradation of mutant CFTR requires a guanine nucleotide-sensitive step.PMID 18280771Read ↗

Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin.Semantic proximity Read ↗

Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP.Semantic proximity Read ↗

Degradation of CFTR by the ubiquitin-proteasome pathwaySemantic proximity Read ↗

Endocytic Trafficking Routes of Wild Type and ΔF508 Cystic Fibrosis Transmembrane Conductance RegulatorSemantic proximity Read ↗

Ricin cytotoxicity is sensitive to recycling between the endoplasmic reticulum and the Golgi complex.Semantic proximity Read ↗

The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells.Semantic proximity Read ↗

Enhanced cell-surface stability of rescued ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperonesSemantic proximity Read ↗

Small Heat-Shock Proteins Select ΔF508-CFTR for Endoplasmic Reticulum-associated DegradationSemantic proximity Read ↗

Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.Semantic proximity Read ↗

Protein translocation across the ER requires a functional GTP binding site in the alpha subunit of the signal recognition particle receptor.Semantic proximity Read ↗

Delta F508 CFTR pool in the endoplasmic reticulum is increased by calnexin overexpression.Semantic proximity Read ↗

Proteasome involvement in the degradation of the G(q) family of Galpha subunits.Semantic proximity Read ↗

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