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

doi:10.1152/ajpcell.1998.275.1.C171

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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 abnorm...

Topics

1-Methyl-3-isobutylxanthine
Analysis of Variance
Calcium-Binding Proteins
Calnexin
Cell Line
Cyclic AMP
Cystic Fibrosis Transmembrane Conductance Regulator
Endoplasmic Reticulum
Epithelial Cells
Genetic Variation
Guanidines
HSP70 Heat-Shock Proteins
HSP90 Heat-Shock Proteins
Humans

Source

Open in PubMed

Identifiers

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

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 30 Aug 2026. Prepared record publication-9688848-2026-08-30.1. Manifest publication-graph-cohort-2026-08-30.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 30 Aug 2026.

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Immunosuppressive Agents

Membrane Potentials

Molecular Chaperones

Patch-Clamp Techniques