Results 31 to 40 of about 10,277 (199)
Modulator Therapy for Cystic Fibrosis: An Exploration of Current Research [PDF]
, 2020 Developing a drug therapy that addresses the root cause of cystic fibrosis (CF) by increasing CFTR protein levels has long been a research challenge. After genetic therapy failed because a suitable delivery system could not be found, researchers began ...
Rombocos, Jessalyn
core +1 more source
Polymorphisms in the glutathione pathway modulate cystic fibrosis severity: a cross-sectional study [PDF]
, 2014 BACKGROUND: Cystic fibrosis (CF) clinically manifests with various levels of severity, which are thought to be modulated by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR), modifier genes, and the environment.
Antonio Fernando Ribeiro +3 more
core +1 more source
The Journal of Clinical Investigation, 2022 The vast majority of people with cystic fibrosis (CF) are now eligible for CF transmembrane regulator (CFTR) modulator therapy. The remaining individuals with CF harbor premature termination codons (PTCs) or rare CFTR variants with limited treatment ...
Rhianna E. Lee +15 more
doaj +1 more source
Interplay between CFTR Phosphorylation, CFTR-ATPase Activity, and Anion Flux [PDF]
, 2016 The cystic fibrosis (CF) transmembrane conductance regulator (CFTR, ABCC7), mutations of which cause CF, belongs to the ATP binding cassette (ABC) transporter family and works as a channel for small anions, such as chloride and bicarbonate. Anion channel
Esposito, Cinzia +3 more
core +1 more source
CFTR genotype and maximal exercise capacity in cystic fibrosis: a cross-sectional study [PDF]
, 2018 RATIONALE: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells. Variations of CFTR dysfunction among patients with CF may present an important determinant of aerobic exercise capacity in CF.
et al +4 more
core +1 more source
CFTR activity and mitochondrial function [PDF]
, 2013 Cystic Fibrosis (CF) is a frequent and lethal autosomal recessive disease, caused by mutations in the gene encoding the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
Santa Coloma, Tomás Antonio +1 more
core +1 more source
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation [PDF]
, 2011 BACKGROUND: Increasing the activity of defective cystic fibrosis transmembrane conductance regulator (CFTR) protein is a potential treatment for cystic fibrosis.
Bell, Scott C. +18 more
core +1 more source
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis
Scientific Reports, 2021 The availability of a simple, robust and non-invasive in vitro airway model would be useful to study the functionality of the cystic fibrosis transmembrane regulator (CFTR) protein and to personalize modulator therapy for cystic fibrosis (CF) patients ...
Maite Calucho +5 more
doaj +1 more source
‘”Why me, why now?” Using clinical immunology and epidemiology to explain who gets nontuberculous mycobacterial infection [PDF]
, 2016 BACKGROUND: The prevalence of nontuberculous mycobacterial (NTM) disease is rising. An understanding of known risk factors for disease sheds light on the immunological and physical barriers to infection, and how and why they may be overcome.
David M. Lowe +3 more
core +4 more sources
Physiological Reports, 2020 Introduction One method for assessing the in vitro response to CFTR‐modulating compounds is by analysis of epithelial monolayers in an Ussing chamber, where the apical and basolateral surfaces are isolated and the potential difference, short‐circuit ...
Preston E. Bratcher +4 more
doaj +1 more source