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Published in 2019 at "American journal of respiratory cell and molecular biology"
DOI: 10.1165/rcmb.2018-0314oc
Abstract: S-Nitrosothiols (SNOs) are endogenous signaling compounds with a diverse spectrum of beneficial airway effects that are both cGMP-dependent and -independent. SNOs are present in healthy human airways, but levels are low in the airways of…
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Keywords:
cftr maturation;
expression;
f508del cftr;
chip ... See more keywords
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1
Published in 2018 at "JCI insight"
DOI: 10.1172/jci.insight.98699
Abstract: In cystic fibrosis (CF), deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel causes misfolding and premature degradation. Considering the numerous effects of the F508del mutation on the assembly…
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Keywords:
cftr;
cystic fibrosis;
f508del cftr;
thymosin correct ... See more keywords
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Published in 2018 at "Frontiers in Pharmacology"
DOI: 10.3389/fphar.2018.01221
Abstract: There is still a high unmet need for the treatment of most patients with cystic fibrosis (CF). The identification and development of new Cystic Fibrosis Transmembrane conductance Regulator (CFTR) modulators is necessary to achieve higher…
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Keywords:
characterization novel;
cftr;
novel cftr;
cftr potentiators ... See more keywords
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2
Published in 2020 at "Biomolecules"
DOI: 10.3390/biom10020334
Abstract: Chronic infection and inflammation are the primary causes of declining lung function in Cystic Fibrosis (CF) patients. ORKAMBI® (Lumacaftor-Ivacaftor) is an approved combination therapy for Cystic Fibrosis (CF) patients bearing the most common mutation, F508del,…
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Keywords:
cftr function;
f508del cftr;
rescue;
human bronchial ... See more keywords
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Published in 2022 at "Biomolecules"
DOI: 10.3390/biom12030471
Abstract: Cystic fibrosis (CF) is a rare genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR), an epithelial anion channel expressed in several vital organs. Absence of functional CFTR results in imbalanced…
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Keywords:
multi domain;
cftr;
f508del cftr;
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Published in 2021 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms222313064
Abstract: SLC26A9, a constitutively active Cl− transporter, has gained interest over the past years as a relevant disease modifier in several respiratory disorders including Cystic Fibrosis (CF), asthma, and non-CF bronchiectasis. SLC26A9 contributes to epithelial Cl−…
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Keywords:
cystic fibrosis;
expression;
cftr;
f508del cftr ... See more keywords
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Published in 2022 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms23179612
Abstract: Cystic fibrosis (CF) is caused by mutations in the gene encoding of the cystic fibrosis transmembrane conductance regulator (CFTR), an anion-selective plasma membrane channel that mainly regulates chloride transport in a variety of epithelia. More…
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Keywords:
potential targets;
f508del cftr;
cftr;
kdm3b potential ... See more keywords
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1
Published in 2022 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms231810956
Abstract: Highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators have led to dramatic improvements in lung function in many people with cystic fibrosis (PwCF). However, the efficacy of CFTR modulators may be hindered by persistent…
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Keywords:
cftr amplifier;
function;
f508del cftr;
cftr ... See more keywords
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Published in 2022 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms232012274
Abstract: Cystic fibrosis is a hereditary disease mainly caused by the deletion of the Phe 508 (F508del) of the cystic fibrosis transmembrane conductance regulator (CFTR) protein that is thus withheld in the endoplasmic reticulum and rapidly…
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Keywords:
f508del cftr;
lumacaftor;
cystic fibrosis;
cftr ... See more keywords
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1
Published in 2022 at "Pharmaceuticals"
DOI: 10.3390/ph15030274
Abstract: Cystic fibrosis (CF) is a genetic disease affecting the lungs and pancreas and causing progressive damage. CF is caused by mutations abolishing the function of CFTR, a protein whose role is chloride’s mobilization in the…
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Keywords:
f508del cftr;
series;
cftr;
cftr correctors ... See more keywords