Cystic fibrosis: a disease of altered protein folding (Q41725523)

8 October 2017

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title

Cystic fibrosis: a disease of altered protein folding (English)

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8 October 2017

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Qu BH

1

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8 October 2017

Strickland E

series ordinal

2

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8 October 2017

Thomas PJ

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3

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8 October 2017

1 October 1997

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Journal of Bioenergetics and Biomembranes

8 October 2017

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8 October 2017

29

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8 October 2017

5

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8 October 2017

483-490

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https://scigraph.springernature.com/pub.10.1023/a:1022439108101

8 October 2017

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cites work

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12 December 2020

Altered protein folding may be the molecular basis of most cases of cystic fibrosis

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12 December 2020

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

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12 December 2020

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

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Chloride impermeability in cystic fibrosis

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

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Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins

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12 December 2020

Identification of an ion channel-forming motif in the primary structure of CFTR, the cystic fibrosis chloride channel

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12 December 2020

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

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12 December 2020

The cystic fibrosis transmembrane conductance regulator. Effects of the most common cystic fibrosis-causing mutation on the secondary structure and stability of a synthetic peptide

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

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The spectrum of cystic fibrosis mutations

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Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.

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12 December 2020

Association of folding intermediates of glycoproteins with calnexin during protein maturation

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12 December 2020

Cystic Fibrosis Transmembrane Conductance Regulator: Nucleotide Binding to a Synthetic Peptide

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12 December 2020

Identification of revertants for the cystic fibrosis delta F508 mutation using STE6-CFTR chimeras in yeast

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12 December 2020

Calnexin: a membrane-bound chaperone of the endoplasmic reticulum

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12 December 2020

Defective protein folding as a basis of human disease

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12 December 2020

Disease-associated mutations in the fourth cytoplasmic loop of cystic fibrosis transmembrane conductance regulator compromise biosynthetic processing and chloride channel activity.

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12 December 2020

Effects of the delta F508 mutation on the structure, function, and folding of the first nucleotide-binding domain of CFTR.

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12 December 2020

Chaperones and protein folding

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12 December 2020

Mechanism of phage P22 tailspike protein folding mutations

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12 December 2020

Nucleoside triphosphates are required to open the CFTR chloride channel.

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12 December 2020

Mislocalization of ΔF508 CFTR in cystic fibrosis sweat gland

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12 December 2020

Global suppression of protein folding defects and inclusion body formation

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12 December 2020

Cystic fibrosis: a disease in electrolyte transport.

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12 December 2020

Class I histocompatibility molecule association with phosphorylated calnexin. Implications for rates of intracellular transport

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A dimerization motif for transmembrane α–helices

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Protein-catalysed protein folding.

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12 December 2020

Principles that govern the folding of protein chains

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12 December 2020

Principles of protein folding--a perspective from simple exact models.

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12 December 2020

Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway

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12 December 2020

Mechanism of protein stabilization by glycerol: preferential hydration in glycerol-water mixtures

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12 December 2020

Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections.

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12 December 2020

Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2

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12 December 2020

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

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12 December 2020

CFTR as a cAMP-dependent regulator of sodium channels

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12 December 2020

Thermolabile folding intermediates: inclusion body precursors and chaperonin substrates.

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12 December 2020

Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)

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12 December 2020

Correction of Defective Protein Kinesis of Human P-glycoprotein Mutants by Substrates and Modulators

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12 December 2020

Identification of the cystic fibrosis gene: genetic analysis

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Induction of fetal hemoglobin production in subjects with sickle cell anemia by oral sodium phenylbutyrate

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Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene

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12 December 2020

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

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12 December 2020

Degradation of CFTR by the ubiquitin-proteasome pathway

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12 December 2020

Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

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12 December 2020

Effect of cystic fibrosis-associated mutations in the fourth intracellular loop of cystic fibrosis transmembrane conductance regulator.

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12 December 2020

Why do some organisms use a urea-methylamine mixture as osmolyte? Thermodynamic compensation of urea and trimethylamine N-oxide interactions with protein

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Interaction of heat-shock protein 70 with p53 translated in vitro: evidence for interaction with dimeric p53 and for a role in the regulation of p53 conformation

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12 December 2020

Thermodynamic and kinetic examination of protein stabilization by glycerol

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12 December 2020

Inhibiting transthyretin amyloid fibril formation via protein stabilization

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Missense mutation (G480C) in the CFTR gene associated with protein mislocalization but normal chloride channel activity

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12 December 2020

Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator

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12 December 2020

Identifiers

DOI

10.1023/A:1022439108101

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Dimensions Publication ID

8 October 2017

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