The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR. (Q40477013)

20 September 2017

title

The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR. (English)

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20 September 2017

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3

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20 September 2017

Kai Du

1

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20 September 2017

Manu Sharma

series ordinal

2

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20 September 2017

publication date

26 December 2004

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Nature Structural & Molecular Biology

20 September 2017

language of work or name

English

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published in

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20 September 2017

volume

12

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20 September 2017

issue

1

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20 September 2017

page(s)

17-25

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The multiplicity of domains in proteins

20 September 2017

cites work

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Defective protein folding as a basis of human disease

31 March 2020

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31 March 2020

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Identification of the cystic fibrosis gene: chromosome walking and jumping

31 March 2020

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Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

31 March 2020

1 reference

Structure and function of the CFTR chloride channel

31 March 2020

1 reference

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

31 March 2020

1 reference

Chaperoning the maturation of the cystic fibrosis transmembrane conductance regulator

31 March 2020

1 reference

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

31 March 2020

1 reference

Degradation of CFTR by the ubiquitin-proteasome pathway

31 March 2020

1 reference

Multiple proteolytic systems, including the proteasome, contribute to CFTR processing

31 March 2020

1 reference

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

31 March 2020

1 reference

The role of regulated CFTR trafficking in epithelial secretion

31 March 2020

1 reference

Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis

31 March 2020

1 reference

Cystic fibrosis transmembrane conductance regulator: solution structures of peptides based on the Phe508 region, the most common site of disease-causing DeltaF508 mutation

31 March 2020

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Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway

31 March 2020

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

31 March 2020

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Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes

31 March 2020

1 reference

Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR

31 March 2020

1 reference

Membrane topology and glycosylation of the human multidrug resistance-associated protein

31 March 2020

1 reference

Differential interactions of nucleotides at the two nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator

31 March 2020

1 reference

47 Characterization of polyclonal and monoclonal antibodies to cystic fibrosis transmembrane conductance regulator

31 March 2020

1 reference

Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.

31 March 2020

1 reference

COOH-terminal truncations promote proteasome-dependent degradation of mature cystic fibrosis transmembrane conductance regulator from post-Golgi compartments

31 March 2020

1 reference

A functional R domain from cystic fibrosis transmembrane conductance regulator is predominantly unstructured in solution

31 March 2020

1 reference

The Delta F508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells

31 March 2020

1 reference

Protein Folding Stability Can Determine the Efficiency of Escape from Endoplasmic Reticulum Quality Control

31 March 2020

1 reference

Destabilizing mutations promote membrane protein misfolding

31 March 2020

1 reference

Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation.

31 March 2020

1 reference

The First Nucleotide Binding Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is a Site of Stable Nucleotide Interaction, whereas the Second Is a Site of Rapid Turnover

31 March 2020

1 reference

Association of domains within the cystic fibrosis transmembrane conductance regulator

31 March 2020

1 reference

Principles of chaperone-assisted protein folding: differences between in vitro and in vivo mechanisms

31 March 2020

1 reference

Co-translational domain folding as the structural basis for the rapid de novo folding of firefly luciferase

31 March 2020

1 reference

Recombination of protein domains facilitated by co-translational folding in eukaryotes

31 March 2020

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Co-translational folding of an alphavirus capsid protein in the cytosol of living cells

31 March 2020

1 reference

Coordinated nonvectorial folding in a newly synthesized multidomain protein

31 March 2020

1 reference

Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator

31 March 2020

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Side chain and backbone contributions of Phe508 to CFTR folding

31 March 2020

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Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore

31 March 2020

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The amino-terminal portion of CFTR forms a regulated Cl- channel.

31 March 2020

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Severed molecules functionally define the boundaries of the cystic fibrosis transmembrane conductance regulator's NH(2)-terminal nucleotide binding domain

31 March 2020

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C-terminal truncations destabilize the cystic fibrosis transmembrane conductance regulator without impairing its biogenesis. A novel class of mutation

31 March 2020

1 reference

Structure of MsbA from Vibrio cholera: a multidrug resistance ABC transporter homolog in a closed conformation

31 March 2020

1 reference

The E. coli BtuCD structure: a framework for ABC transporter architecture and mechanism

31 March 2020

1 reference

Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure

31 March 2020

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The DeltaF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator

31 March 2020

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inferred from DOI database lookup

7 January 2021

based on heuristic

Identifiers

DOI

10.1038/NSMB882

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20 September 2017

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