S-Nitrosylation of CHIP Enhances F508Del CFTR Maturation.
Sign Up to like & getrecommendations! 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… read more here.
Keywords: cftr maturation; expression; f508del cftr; chip ... See more keywords
Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.
Sign Up to like & getrecommendations! 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… read more here.
Keywords: cftr; cystic fibrosis; f508del cftr; thymosin correct ... See more keywords
Deleterious effect of Pseudomonas aeruginosa on F508del-CFTR rescued by elexacaftor/tezacaftor/ivacaftor is clinical strain-dependent in patient-derived nasal cells
Sign Up to like & getrecommendations! Published in 2024 at "ERJ Open Research"
DOI: 10.1183/23120541.00970-2024
Abstract: Graphical abstract Pseudomonas aeruginosa infection reduces ETI treatment efficacy. BAL: bronchoalveolar lavage; ETI: elexacaftor/tezacaftor/ivacaftor. Background The triple cystic fibrosis transmembrane conductance regulator (CFTR) modulators combination elexacaftor/tezacaftor/ivacaftor (ETI) has been approved for people with cystic fibrosis… read more here.
Keywords: f508del cftr; elexacaftor tezacaftor; pseudomonas aeruginosa; cftr ... See more keywords
Mutation accumulation in H. sapiens F508del CFTR countermands dN/dS type genomic analysis
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DOI: 10.1371/journal.pone.0305832
Abstract: Understanding the mechanisms that underlie de novo mutations (DNMs) can be essential for interpreting human evolution, including aspects such as rapidly diverging genes, conservation of non-coding regulatory elements, and somatic DNA adaptation, among others. DNM… read more here.
Keywords: f508del cftr; sapiens f508del; cftr; mutation accumulation ... See more keywords
Identification and Characterization of Novel CFTR Potentiators
Sign Up to like & getrecommendations! 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… read more here.
Keywords: characterization novel; cftr; novel cftr; cftr potentiators ... See more keywords
Anti-Infectives Restore ORKAMBI® Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of P. aeruginosa
Sign Up to like & getrecommendations! 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,… read more here.
Keywords: cftr function; f508del cftr; rescue; human bronchial ... See more keywords
Structural Comparative Modeling of Multi-Domain F508del CFTR
Sign Up to like & getrecommendations! 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… read more here.
Keywords: multi domain; cftr; f508del cftr;
Synergy in Cystic Fibrosis Therapies: Targeting SLC26A9
Sign Up to like & getrecommendations! 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−… read more here.
Keywords: cystic fibrosis; expression; cftr; f508del cftr ... See more keywords
KDM2A and KDM3B as Potential Targets for the Rescue of F508del-CFTR
Sign Up to like & getrecommendations! 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… read more here.
Keywords: potential targets; f508del cftr; cftr; kdm3b potential ... See more keywords
The CFTR Amplifier Nesolicaftor Rescues TGF-β1 Inhibition of Modulator-Corrected F508del CFTR Function
Sign Up to like & getrecommendations! 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… read more here.
Keywords: cftr amplifier; function; f508del cftr; cftr ... See more keywords
Virtual Drug Repositioning as a Tool to Identify Natural Small Molecules That Synergize with Lumacaftor in F508del-CFTR Binding and Rescuing
Sign Up to like & getrecommendations! 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… read more here.
Keywords: f508del cftr; lumacaftor; cystic fibrosis; cftr ... See more keywords