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
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
Novel cystic fibrosis transmembrane conductance regulator variant in a cystic fibrosis patient
Sign Up to like & getrecommendations! Published in 2022 at "Internal Medicine Journal"
DOI: 10.1111/imj.15715
Abstract: A 17-year-old boy was assessed because of a 1-week history of thoracic pain without dyspnoea. The patient came from Pakistan 2 years earlier. He had a history of asthma and had delayed puberty. The family… read more here.
Keywords: history; conductance regulator; patient; transmembrane conductance ... See more keywords
Spectrum of Cystic Fibrosis Conductance Regulator Gene Mutations Reported in Pakistani Descent Cystic Fibrosis Patients.
Sign Up to like & getrecommendations! Published in 2022 at "Journal of the College of Physicians and Surgeons--Pakistan : JCPSP"
DOI: 10.29271/jcpsp.2022.08.1042
Abstract: Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This study aims to determine the genotypic and phenotypic spectrum of the CFTR gene mutations reported in the literature… read more here.
Keywords: gene mutations; cftr gene; conductance regulator; fibrosis ... See more keywords
Effectiveness and Safety of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Children With Cystic Fibrosis: A Meta-Analysis
Sign Up to like & getrecommendations! Published in 2022 at "Frontiers in Pediatrics"
DOI: 10.3389/fped.2022.937250
Abstract: Background and Aim Cystic fibrosis (CF) is a genetic disease that is difficult to treat and caused by dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Small molecules have been used to treat… read more here.
Keywords: meta analysis; conductance regulator; fibrosis transmembrane; fibrosis ... See more keywords
Genotype patterns for mutations of the cystic fibrosis transmembrane conductance regulator gene: a retrospective descriptive study from Saudi Arabia
Sign Up to like & getrecommendations! Published in 2020 at "Annals of Saudi Medicine"
DOI: 10.5144/0256-4947.2020.15
Abstract: ABSTRACT BACKGROUND: Cystic fibrosis (CF) occurs in populations in Saudi Arabia and the Gulf area. Approximately 2000 known variants have been identified for the CF transmembrane conductance regulator (CTFR) gene. Screening for ten of the… read more here.
Keywords: transmembrane conductance; cystic fibrosis; conductance regulator; saudi arabia ... See more keywords