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Article

Alteration of the Cystic Fibrosis Transmembrane Conductance Regulator Folding Pathway

1 Mar 1996

Abstract excerpt

The cellular phenotype of the most common cystic fibrosis-causing mutation, deletion of phenylalanine 508 (deltaF508) in the amino-terminal nucleotide binding domain (NBD1) of the cystic fibrosis transmembrane conductance regulator (CFTR), is the inability of the mutant protein to fold and transit to the apical membrane of affected epithelial cells. Expressed NBD1s were purified and folded in vitro into soluble...

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Identifiers

PMID: 8631737
DOI: 10.1074/jbc.271.13.7261

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Related research

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Research trail→

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Alteration of the Cystic Fibrosis Transmembrane Conductance Regulator Folding PathwayPMID 8631737Read ↗

Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding.Semantic proximity Read ↗

Cystic fibrosis: a disease of altered protein folding.Semantic proximity Read ↗

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

Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1.Semantic proximity Read ↗

Impact of the ΔF508 Mutation in First Nucleotide-binding Domain of Human Cystic Fibrosis Transmembrane Conductance Regulator on Domain Folding and StructureSemantic proximity Read ↗

Diminished self-chaperoning activity of the DeltaF508 mutant of CFTR results in protein misfolding.Semantic proximity Read ↗

Structural stability of purified human CFTR is systematically improved by mutations in nucleotide binding domain 1.Semantic proximity Read ↗

Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function.Semantic proximity Read ↗

The primary folding defect and rescue of ΔF508 CFTR emerge during translation of the mutant domain.Semantic proximity Read ↗

The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis.Semantic proximity Read ↗

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

CFTR misfolds during native-centric simulations due to entropic penalties of native state formationSemantic proximity Read ↗

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