(Q33973031)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

title

Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

main subject

cystic fibrosis

0 references

transmembrane protein

0 references

author

Patrick H Thibodeau

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

7

Sandra E Guggino

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

author name string

Xue-Mei Zhang

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

Xi-Tao Wang

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

Hongwen Yue

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

Steve W Leung

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

Philip J Thomas

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

language of work or name

English

0 references

publication date

7 October 2003

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

Journal of Biological Chemistry

19 December 2019

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

volume

278

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

issue

51

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

page(s)

51232-51242

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia

19 December 2019

cites work

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Competing solvent effects of polyols and guanidine hydrochloride on protein stability

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

A naturally occurring protective system in urea-rich cells: mechanism of osmolyte protection of proteins against urea denaturation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Mammalian osmolytes and S-nitrosoglutathione promote Delta F508 cystic fibrosis transmembrane conductance regulator (CFTR) protein maturation and function

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Expression and characterization of the cystic fibrosis transmembrane conductance regulator.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Membrane trafficking of the cystic fibrosis gene product, cystic fibrosis transmembrane conductance regulator, tagged with green fluorescent protein in madin-darby canine kidney cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Aggresomes: a cellular response to misfolded proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Ablation of internalization signals in the carboxyl-terminal tail of the cystic fibrosis transmembrane conductance regulator enhances cell surface expression

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Defective regulation of outwardly rectifying Cl- channels by protein kinase A corrected by insertion of CFTR

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

The two halves of CFTR form a dual-pore ion channel

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Molecular cloning and functional characterization of a GABA/betaine transporter from human kidney

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Taurine: an overview of its role in preventive medicine

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Betaine, ethanol, and the liver: a review

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Phagocyte oxidants and nitric oxide in cystic fibrosis: new therapeutic targets?

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Defective protein folding as a basis of human disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Protein misfolding, evolution and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Protein misfolding and disease; protein refolding and therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Defective aquaporin-2 trafficking in nephrogenic diabetes insipidus and correction by chemical chaperones

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Misfolding of mutant aquaporin-2 water channels in nephrogenic diabetes insipidus

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Pharmacological chaperones rescue cell-surface expression and function of misfolded V2 vasopressin receptor mutants

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Charge density-dependent strength of hydration and biological structure

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Towards a molecular level understanding of protein stabilization: the interaction between lysozyme and sorbitol

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Osmoregulatory changes in myo-inositol transport by renal cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Symptomatic and asymptomatic methylenetetrahydrofolate reductase deficiency in two adult brothers

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Betaine for treatment of homocystinuria caused by methylenetetrahydrofolate reductase deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Betaine, a promising new agent for patients with nonalcoholic steatohepatitis: results of a pilot study

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Effect of taurine supplements on fat absorption in cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M309076200

21 January 2018

Identifiers

DOI

10.1074/JBC.M309076200

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14532265%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

1 reference