CF Fungal Disease in the Age of CFTR Modulators [PDF]
Mycopathologia, 2021 AbstractFungi are increasingly recognised to have a significant role in the progression of lung disease in Cystic fibrosis with Aspergillus fumigatus the most common fungus isolated during respiratory sampling. The emergence of novel CFTR modulators has, however, significantly changed the outlook of disease progression in CF.
Amelia Bercusson +2 more
openaire +4 more sources
Diagnosis of Cystic Fibrosis in Screened Populations [PDF]
, 2017 Objective Cystic fibrosis (CF) can be difficult to diagnose, even when newborn screening (NBS) tests yield positive results. This challenge is exacerbated by the multitude of NBS protocols, misunderstandings about screening vs diagnostic tests, and ...
Accurso, Frank J. +15 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
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
CFTR Modulator Therapy and Its Impact on Lung Transplantation in Cystic Fibrosis
Pulmonary Therapy, 2021 Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasian people and is caused by mutations in the gene encoding for the CF transmembrane conductance regulator (CFTR) protein. It is a multisystem disorder; however, CF lung disease
C. Benden, C. Schwarz
semanticscholar +1 more source
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
From CFTR biology toward combinatorial pharmacotherapy:expanded classification of cystic fibrosis mutations [PDF]
, 2016 More than 2000 mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) have been described that confer a range of molecular cell biological and functional phenotypes.
Avramescu, Radu G. +17 more
core +3 more sources
Lived experiences of individuals with cystic fibrosis on CFTR-modulators
BMC Pulmonary Medicine, 2022 Background CFTR-modulators are a category of drugs that facilitate trafficking and opening of the abnormal CFTR protein in individuals with cystic fibrosis (CF) who have certain genetic mutations.
Annelise Page +2 more
doaj +1 more source
Endogenous annexin A1 counter-regulates bleomycin-induced lung fibrosis [PDF]
, 2011 PMCID: PMC3212807This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided ...
Damazo, AS +5 more
core +3 more sources
Phagocytosis depends on TRPV2-mediated calcium influx and requires TRPV2 in lipids rafts: alteration in macrophages from patients with cystic fibrosis. [PDF]
, 2018 Whereas many phagocytosis steps involve ionic fluxes, the underlying ion channels remain poorly defined. As reported in mice, the calcium conducting TRPV2 channel impacts the phagocytic process.
A Abu-Arish +47 more
core +4 more sources