(Q34502469)

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Cystic fibrosis: a brief look at some highlights of a decade of research focused on elucidating and correcting the molecular basis of the disease

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scholarly article

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

1 reference

Europe PubMed Central

Cystic fibrosis: a brief look at some highlights of a decade of research focused on elucidating and correcting the molecular basis of the disease (English)

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

cystic fibrosis

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author name string

Ko YH

1

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

Pedersen PL

2

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

publication date

1 December 2001

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

Journal of Bioenergetics and Biomembranes

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

33

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

6

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

513-521

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

Phosphorylation-regulated CI− channel in CHO cells stably expressing the cystic fibrosis gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cystic fibrosis transmembrane conductance regulator: the purified NBF1+R protein interacts with the purified NBF2 domain to form a stable NBF1+R/NBF2 complex while inducing a conformational change transmitted to the C-terminal region

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A recombinant polypeptide model of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator functions as an active ATPase, GTPase and adenylate kinase.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Modeling of nucleotide binding domains of ABC transporter proteins based on a F1-ATPase/recA topology: structural model of the nucleotide binding domains of the cystic fibrosis transmembrane conductance regulator (CFTR)

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The crystal structure of the MJ0796 ATP-binding cassette. Implications for the structural consequences of ATP hydrolysis in the active site of an ABC transporter

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Crystal structure of the ATP-binding subunit of an ABC transporter

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Regulation of the gating of cystic fibrosis transmembrane conductance regulator C1 channels by phosphorylation and ATP hydrolysis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Function of the R domain in the cystic fibrosis transmembrane conductance regulator chloride channel

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gene therapy for pulmonary diseases

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Coupling of CFTR Cl- channel gating to an ATP hydrolysis cycle

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Walker mutations reveal loose relationship between catalytic and channel-gating activities of purified CFTR (cystic fibrosis transmembrane conductance regulator)

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A novel Ca2+ pump expressed in brain, kidney, and stomach is encoded by an alternative transcript of the slow-twitch muscle sarcoplasmic reticulum Ca-ATPase gene. Identification of cDNAs encoding Ca2+ and other cation-transporting ATPases using an o

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mutant (delta F508) cystic fibrosis transmembrane conductance regulator Cl- channel is functional when retained in endoplasmic reticulum of mammalian cells.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structures of metal sites of oxidized bovine heart cytochrome c oxidase at 2.8 A.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Defective protein folding as a basis of human disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Regulation of CFTR channel gating

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structure of the alpha 1 subunit of horse Na,K-ATPase gene

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Expression of hybrid (Na+ + K+)-ATPase molecules after transfection of mouse Ltk-cells with DNA encoding the beta-subunit of an avian brain sodium pump

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR)

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Adenovirus-mediated gene transfer transiently corrects the chloride transport defect in nasal epithelia of patients with cystic fibrosis.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Research on cystic fibrosis: a journey from the Heart House

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structure of the recA protein–ADP complex

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Crystal structure of the cytochrome bc1 complex from bovine heart mitochondria

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

US gene therapy in crisis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structure at 2.8 A resolution of F1-ATPase from bovine heart mitochondria

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amino acid sequence of the amino-terminal 24 kDa fragment of the heavy chain of chicken gizzard myosin

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

CFTR: the nucleotide binding folds regulate the accessibility and stability of the activated state

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The nucleotide binding folds of the cystic fibrosis transmembrane conductance regulator are extracellularly accessible

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

ABC Transporters: From Microorganisms to Man

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Lack of conventional ATPase properties in CFTR chloride channel gating

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Coupling of ATP hydrolysis with channel gating by purified, reconstituted CFTR.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Gene therapy for the respiratory manifestations of cystic fibrosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

ATPase activity of the cystic fibrosis transmembrane conductance regulator

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Three-dimensional structure of adenyl kinase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The first nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator can function as an active ATPase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Probing the structural and functional domains of the CFTR chloride channel.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cystic fibrosis, lung infections, and a human tracheal antimicrobial peptide (hTAP)

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

ATP hydrolysis by a CFTR domain: pharmacology and effects of G551D mutation

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

ATP-dependent transport systems in bacteria and humans: relevance to cystic fibrosis and multidrug resistance

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cystic fibrosis. Vehicles for gene therapy.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Crystal structure of MalK, the ATPase subunit of the trehalose/maltose ABC transporter of the archaeon Thermococcus litoralis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Molecular cloning and immunological characterization of the gamma polypeptide, a small protein associated with the Na,K-ATPase

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

One-step affinity isolation of recombinant protein using the baculovirus/insect cell expression system

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Cystic fibrosis transmembrane conductance regulator: the first nucleotide binding fold targets the membrane with retention of its ATP binding function.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Intrinsic anion channel activity of the recombinant first nucleotide binding fold domain of the cystic fibrosis transmembrane regulator protein

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The cystic fibrosis transmembrane conductance regulator. Overexpression, purification, and characterization of wild type and delta F508 mutant forms of the first nucleotide binding fold in fusion with the maltose-binding protein.

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The 2.8-A structure of rat liver F1-ATPase: configuration of a critical intermediate in ATP synthesis/hydrolysis

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Crystal structures of the MJ1267 ATP binding cassette reveal an induced-fit effect at the ATPase active site of an ABC transporter

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Amino acid sequence of the plasma membrane ATPase of Neurospora crassa: deduction from genomic and cDNA sequences

1 reference

https://pubmed.ncbi.nlm.nih.gov/11804193

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1023/A:1012831322753

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

Dimensions Publication ID

0 references

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

2 August 2017

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