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Article

Partial restoration of cAMP-stimulated CFTR chloride channel activity in DeltaF508 cells by deoxyspergualin.

The American journal of physiology - 1 Jul 1998

Jiang C, Fang S L, Xiao Y F, O'Connor S P, Nadler S G, Lee D W, Jefferson D M, Kaplan J M, Smith A E, Cheng S H

Abstract excerpt

Deletion of the codon encoding phenylalanine 508 (DeltaF508) is the most common mutation in cystic fibrosis (CF) and results in a trafficking defect. Mutant DeltaF508-CF transmembrane conductance regulator (CFTR) protein retains functional activity, but the nascent protein is recognized as abnormal and, in consequence, is retained in the endoplasmic reticulum (ER) and degraded. It has been proposed that this...

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Source

Identifiers

PMID: 9688848
DOI: 10.1152/ajpcell.1998.275.1.C171

Genes

CFTR

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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

Partial restoration of cAMP-stimulated CFTR chloride channel activity in DeltaF508 cells by deoxyspergualin.PMID 9688848Read ↗

Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epitheliaSemantic proximity Read ↗

The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70.Semantic proximity Read ↗

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

Activation of the unfolded protein response by deltaF508 CFTR.Semantic proximity Read ↗

Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein.Semantic proximity Read ↗

Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR.Semantic proximity Read ↗

The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.Semantic proximity Read ↗

Induction of HSP70 promotes DeltaF508 CFTR trafficking.Semantic proximity Read ↗

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

Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.Semantic proximity Read ↗

Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.Semantic proximity Read ↗

The Delta F508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells.Semantic proximity Read ↗

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