Src kinase inhibition reduces inflammatory and cytoskeletal changes in ΔF508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy
Sign Up to like & getrecommendations! Published in 2018 at "Hepatology"
DOI: 10.1002/hep.29400
Abstract: Cystic fibrosis transmembrane conductance regulator (CFTR), the channel mutated in cystic fibrosis (CF), is expressed by the biliary epithelium (i.e., cholangiocytes) of the liver. Progressive clinical liver disease (CF‐associated liver disease; CFLD) occurs in around… read more here.
Keywords: cystic fibrosis; kinase; src kinase; fibrosis transmembrane ... See more keywords
DNA Sequencing Analysis of Cystic Fibrosis Transmembrane Regulator Gene Identifies Cystic Fibrosis-Associated Variants in the Severe Asthma Research Program.
Sign Up to like & getrecommendations! Published in 2022 at "Pediatric pulmonology"
DOI: 10.1002/ppul.25939
Abstract: BACKGROUND Heterozygote carriers of potentially pathogenic variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene have increased asthma risk. However, the frequency and impact of CFTR variation among individuals with asthma is unknown. OBJECTIVE… read more here.
Keywords: fibrosis transmembrane; severe asthma; cystic fibrosis; potentially pathogenic ... See more keywords
Esc peptides as novel potentiators of defective cystic fibrosis transmembrane conductance regulator: an unprecedented property of antimicrobial peptides
Sign Up to like & getrecommendations! Published in 2021 at "Cellular and Molecular Life Sciences"
DOI: 10.1007/s00018-021-04030-2
Abstract: Mutations in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein lead to persistent lung bacterial infections, mainly due to Pseudomonas aeruginosa, causing loss of respiratory function and finally death of people affected by CF.… read more here.
Keywords: cystic fibrosis; property; cftr; fibrosis transmembrane ... See more keywords
Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator
Sign Up to like & getrecommendations! Published in 2021 at "iScience"
DOI: 10.1016/j.isci.2021.102542
Abstract: Summary Ivacaftor (VX-770) was the first cystic fibrosis transmembrane conductance regulator (CFTR) modulatory drug approved for the treatment of patients with cystic fibrosis. Electron cryomicroscopy (cryo-EM) studies of detergent-solubilized CFTR indicated that VX-770 bound to… read more here.
Keywords: transmembrane conductance; cystic fibrosis; conductance regulator; fibrosis ... See more keywords
Salivary Thiocyanate as a Biomarker of Cystic Fibrosis Transmembrane Regulator Function.
Sign Up to like & getrecommendations! Published in 2019 at "Analytical chemistry"
DOI: 10.1021/acs.analchem.9b01800
Abstract: Improved methods are needed to reliably assess Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) function in vivo in light of recent therapeutic developments targeting the CFTR protein. Oral fluid from patients with cystic fibrosis (CF) and… read more here.
Keywords: cystic fibrosis; colorimetry; fibrosis transmembrane; regulator ... See more keywords
Cystic Fibrosis Transmembrane Conductance Regulator Potentiation as a Therapeutic Strategy for Pulmonary Edema: A Proof-of-Concept Study in Pigs
Sign Up to like & getrecommendations! Published in 2017 at "Critical Care Medicine"
DOI: 10.1097/ccm.0000000000002720
Abstract: Objectives: To determine the feasibility of using a cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770/Kalydeco, Vertex Pharmaceuticals, Boston, MA), as a therapeutic strategy for treating pulmonary edema. Design: Prospective laboratory animal investigation. Setting: Animal… read more here.
Keywords: transmembrane conductance; cystic fibrosis; conductance regulator; pulmonary edema ... See more keywords
Nutritional and metabolic management for cystic fibrosis in a post-cystic fibrosis transmembrane conductance modulator era
Sign Up to like & getrecommendations! Published in 2022 at "Current Opinion in Pulmonary Medicine"
DOI: 10.1097/mcp.0000000000000917
Abstract: Purpose of review The introduction of highly effective cystic fibrosis transmembrane conductance regulator modulators has resulted in a paradigm shift towards treating underlying cause of cystic fibrosis (CF) rather than the ensuing complications. In this… read more here.
Keywords: cystic fibrosis; metabolic management; fibrosis transmembrane; fibrosis ... See more keywords
Cystic fibrosis: candidate selection and impact of the cystic fibrosis transmembrane conductance regulator therapy.
Sign Up to like & getrecommendations! Published in 2022 at "Current opinion in organ transplantation"
DOI: 10.1097/mot.0000000000000975
Abstract: PURPOSE OF REVIEW Over the past decade, the development of highly effective cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators has dramatically ameliorated the manifestations of CF for most patients. Perhaps most importantly, CFTR modulators… read more here.
Keywords: fibrosis transmembrane; therapy; lung; cystic fibrosis ... See more keywords
Prenatal cystic fibrosis transmembrane conductance regulator modulator therapy: A promising way to change the impact of cystic fibrosis.
Sign Up to like & getrecommendations! Published in 2023 at "Fetal diagnosis and therapy"
DOI: 10.1159/000530261
Abstract: INTRODUCTION Cystic fibrosis (CF) is a potentially severe disease. The development of new therapies with cystic fibrosis transmembrane conductance regulator (CFTR) modulators has been a great advance in the management of this condition because they… read more here.
Keywords: fibrosis transmembrane; modulator therapy; fibrosis; cystic fibrosis ... See more keywords
The Extrapulmonary Effects of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis
Sign Up to like & getrecommendations! Published in 2020 at "Annals of the American Thoracic Society"
DOI: 10.1513/annalsats.201909-671cme
Abstract: The effects of cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators on lung function, pulmonary exacerbations, and quality of life have been well documented. However, CF is a multiorgan disease, and therefore an evidence base… read more here.
Keywords: fibrosis transmembrane; cystic fibrosis; fibrosis; extrapulmonary effects ... See more keywords
ζ potential changing nanoparticles as cystic fibrosis transmembrane conductance regulator gene delivery system: an in vitro evaluation.
Sign Up to like & getrecommendations! Published in 2017 at "Nanomedicine"
DOI: 10.2217/nnm-2017-0115
Abstract: AIM Aim of the study was the development of ζ potential changing nanoparticles as gene delivery system for the cystic fibrosis transmembrane conductance regulator gene. METHODS Chitosan and carboxymethyl cellulose were modified with phosphotyrosine, a… read more here.
Keywords: transmembrane conductance; cystic fibrosis; delivery system; gene delivery ... See more keywords